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Showing papers in "The American Journal of Surgical Pathology in 1996"


Journal ArticleDOI
TL;DR: The Sydney System for the classification of gastritis emphasized the importance of combining topographical, morphological, and etiological information into a schema that would help to generate reproducible and clinically useful diagnoses as discussed by the authors.
Abstract: The Sydney System for the classification of gastritis emphasized the importance of combining topographical, morphological, and etiological information into a schema that would help to generate reproducible and clinically useful diagnoses. To reappraise the Sydney System 4 years after its introduction, a group of gastrointestinal pathologists from various parts of the world met in Houston, Texas, in September 1994. The aims of the workshop were (a) to establish an agreed terminology of gastritis; (b) to identify, define, and attempt to resolve some of the problems associated with the Sydney System. This article introduces the Sydney System as it was revised at the Houston Gastritis Workshop and represents the consensus of the participants. Overall, the principles and grading of the Sydney System were only slightly modified, the grading being aided by the provision of a visual analogue scale. The terminology of the final classification has been improved to emphasize the distinction between the atrophic and nonatrophic stomach; the names used for each entity were selected because they are generally acceptable to both pathologists and gastroenterologists. In addition to the main categories and atrophic and nonatrophic gastritis, the special or distinctive forms are described and their respective diagnostic criteria are provided. The article includes practical guidelines for optimal biopsy sampling of the stomach, for the use of the visual analogue scales for grading the histopathologic features, and for the formulation of a comprehensive standardized diagnosis. A glossary of gastritis-related terms as used in this article is provided.

4,511 citations


Journal ArticleDOI
TL;DR: The participants concluded that nasal T/natural killer (NK) cell lymphoma is a distinct clinicopathologic entity highly associated with Epstein-Barr virus (EBV) and in situ hybridization for EBV an be very valuable in early diagnosis, especially if tissue is sparse.
Abstract: A workshop jointly sponsored by the University of Hong Kong and the Society for Hematopathology explored the definition, differential diagnosis, and epidemiology of angiocentric lymphomas presenting in the nose and other extranodal sites. The participants concluded that nasal T/natural killer (NK) cell lymphoma is a distinct clinicopathologic entity highly associated with Epstein-Barr virus (EBV). In situ hybridization for EBV an be very valuable in early diagnosis, especially if tissue is sparse. The cytologic spectrum is broad, ranging from small or medium-sized cells to large transformed cells. Histologic progression often occurs with time. Necrosis is nearly always present, and angioinvasion by tumor cells is seen in most cases. Nasal T/NK cell lymphoma has a characteristic immunophenotype: CD2-positive, CD56-positive, but usually negative for surface CD3. Cytoplasmic CD3 can be detected in paraffin sections. Clonal T-cell receptor gene rearrangement is not found. Tumors with an identical phenotype and genotype occur in other extranodal sites, most commonly in the skin, subcutis, and gastrointestinal tract, and should be referred to as nasal-type T/NK cell lymphomas. The differential diagnosis includes lymphomatoid granulomatosis, blastic or monomorphic NK cell lymphoma/leukemia, CD56-positive peripheral T-cell lymphoma, and enteropathy-associated T-cell lymphoma.

724 citations


Journal ArticleDOI
TL;DR: Using the actuarial curves within this study, physicians will be able to more accurately determine a patient's risk of progression following radical prostatectomy based on a combination of the radical prostateCTomy Gleason score, extent of capsular penetration, and status of surgical margins of resection.
Abstract: We studied 721 men with clinically confined disease who underwent radical prostatectomy. No patient received preoperative or postoperative radiotherapy or hormone therapy until progression occurred. For those men without progression, the mean follow-up was 6.5 years with a median of 6 years (range 1

558 citations


Journal ArticleDOI
TL;DR: This dahlins bone tumors general aspects and data on 11 087 cases book will probably make you feel curious and help you to have willing to reach all benefits.
Abstract: When there are many people who don't need to expect something more than the benefits to take, we will suggest you to have willing to reach all benefits. Be sure and surely do to take this dahlins bone tumors general aspects and data on 11 087 cases that gives the best reasons to read. When you really need to get the reason why, this dahlins bone tumors general aspects and data on 11 087 cases book will probably make you feel curious.

518 citations


Journal ArticleDOI
TL;DR: The poorly recognized low-grade myxofibrosarcoma is emphasized, as proper diagnosis and treatment and scrupulous follow-up are mandatory to avoid local recurrence and gradual tumor progression to a higher-grade neoplasm that may then metastasize.
Abstract: Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients. We analysed the clinicopathologic features in a series of 75 patients. All patients were adults (range, 22-91 years; median, 66 years) with an approximately equal incidence in men and women. Thirty-five tumors arose in the lower and 25 in the upper extremities, nine on the trunk, two each in the retroperitoneum and the head and neck region, and one each in the pelvis and penis. Forty-eight cases (69.5%) were located in dermal or subcutaneous tissues. Distinctive histologic features included the following: a commonly nodular growth pattern; a myxoid matrix containing elongated, curvilinear capillaries; and fusiform, round or stellate tumor cells with indistinct cell margins, slightly eosinophilic cytoplasm, and hyperchromatic atypical nuclei. These lesions varied from a hypocellular, mainly myxoid, and purely spindle-cell appearance (low-grade neoplasms) to high-grade, pleomorphic (malignant fibrous histiocytoma-like) lesions with multinucleated giant cells, high mitotic activity, and areas of necrosis. Immunohistochemistry in 44 cases revealed only vimentin and occasional actin positivity. Ultrastructurally, tumor cells had a fibroblastic phenotype. DNA flow cytometry and proliferation analysis showed an association between aneuploidy and histologic grade. An average follow-up of 45 months (range, 5-300 months) in 60 cases has revealed local recurrence in 33 cases (54%). Thirteen patients developed metastases, and 13 tumor-related deaths occurred. A short interval to first local recurrence was associated with poor clinical outcome. The rate of local recurrence was independent of histologic grade, but only intermediate and high-grade neoplasms metastasized. The depth of the primary lesion did not influence the incidence of local recurrence. However, in deep-seated neoplasms, the incidence of metastases was higher and the percentage of tumor-related deaths was twice as high as in superficially located lesions, reflecting the fact that deep-seated lesions tended to be higher-grade, larger tumors. Myxofibrosarcoma tends to become progressively higher grade in recurrences, as demonstrated in five cases in our series. The poorly recognized low-grade myxofibrosarcoma is emphasized, as proper diagnosis and treatment and scrupulous follow-up are mandatory to avoid local recurrence and gradual tumor progression to a higher-grade neoplasm that may then metastasize.

474 citations


Journal ArticleDOI
TL;DR: Calretinin is a most useful marker for the positive identification of malignant mesotheliomas in routinely fixed and embedded tissue sections and in cytological preparations of serous effusions.
Abstract: Immunohistochemistry is a powerful diagnostic adjunct in the differential diagnosis between malignant mesothelioma (especially of the epithelial type) and adenocarcinoma metastatic to the serous membranes. Most of the immunological probes commonly used, however, recognize antigens expressed by the epithelial malignancies and absent from mesothelial cells and mesotheliomas. Probes suitable for the positive identification of mesotheliomas are comparatively scarce and much less commonly used because of their reduced sensitivity and specificity, their unsuitability for staining routinely fixed and embedded tissues, or their lack of commercial availability. We now document that two different polyclonal antisera to calretinin consistently immunostain mesothelial cells and malignant mesotheliomas both in routinely fixed and embedded tissue sections and in cytological preparations of serous effusions. The diagnostic sensitivity of this novel immunocytochemical approach reached 100%, allowing immunostaining of all 44 mesotheliomas investigated, which included five biphasic and three sarcomatoid types. The specificity of calretinin immunoreactivity was checked against 294 adenocarcinomas of different origin (19 serosal metastases and 275 primary tumors potentially able to metastatize to serosal membranes) relevant for the discussion of the differential diagnosis with malignant mesothelioma: only 28 cases showed focal immunoreactivity for calretinin. We conclude that calretinin is a most useful marker for the positive identification of malignant mesotheliomas.

415 citations


Journal ArticleDOI
TL;DR: A unique, previously unreported pancreatic tumor occurring in a 60-year-old woman who was preoperative diagnosed on cytoaspiration as having clear cell carcinoma is reported, proposing this new entity be named clear cell "sugar" tumor of the pancreas.
Abstract: We report at unique, previously unreported pancreatic tumor occurring in a 60-year-old woman who was preoperative diagnosed on cytoaspiration as having clear cell carcinoma. The resected tumor consisted of a population of large epithelioid cells with clear or eosinophilic, granular cytoplasm, rich in glycogen, with nuclear pleomorphism and no mitotic activity. In spite of the epithelioid appearance, the tumor cells were negative for epithelial (CAM 5.2, KL1, AE1-AE3), endocrine (neuron-specific enolase [NSE], chromogranin A), and acinar (lipase, amylase) markers and positive for actin and melanogenesis-related marker HMB 45. Ultrastructurally, the neoplastic cells showed membrane-bound granules; no evidence of either epithelial or melanocytic differentiation was present. These morphophenotypic features have never been reported in a pancreatic tumor and overlap those of clear cell "sugar" tumor of the lung. The same morphophenotypic features are observed in a family of lesions characterized by the presence of the perivascular epithelioid cell that also includes lymphangiomyomatosis and angiomyolipoma. The present case may be considered a novel member of this family of lesions. We propose this new entity be named clear cell "sugar" tumor of the pancreas.

351 citations


Journal ArticleDOI
TL;DR: The comigrations of the single homoduplexes generated from both the carcinoma cells and sarcoma cells in six different malignant mixed tumors obtained from four different organs indicated clonal identity and monoclonality in all cases.
Abstract: Malignant mixed tumors (carcinosarcomas) are examples of unusual neoplasms whose occurrences have been observed in increasingly diverse sites but whose pathogenesis remains a complete mystery. Two antithetical hypotheses that have been advanced to explain the histogenesis of these tumors include the convergence hypothesis, which proposes an origin from two or more stem cells (multiclonal hypothesis), and the divergence hypothesis, which proposes an origin from a single totipotential stem cell that differentiates into separate epithelial and mesenchymal directions (monoclonal hypothesis). To test these hypotheses, a novel strategy for the determination of clonality from as few as 100 tumor cells obtained by enzymatic digestion of either fresh or formalin-fixed, paraffin-embedded tissues and cell sorting was used that exhibited the polymerase chain reaction (PCR) in amplifying a 511-bp region located within the first intron of the human hypoxanthine phosphoribosyl transferase gene, a site that contains inactive X chromosomal obligately methylated HpaII/MspI sites and single-base allelic polymorphisms in 5% females. Carcinoma cells gated on the basis of fluorescein isothiocyanate (FITC)-anti-cytokeratin and sarcoma cells gated on the basis of FITC-antivimentin or FITC-anti-desmin were sorted to homogeneity on FACSTAR and then subjected to genomic DNA extraction and Hpa II digestion before PCR amplification and subsequent analysis of the product on denaturing gradient gel electrophoresis. The comigrations of the single homoduplexes generated from both the carcinoma cells and sarcoma cells in six different malignant mixed tumors obtained from four different organs indicated clonal identity and monoclonality in all cases. These findings of monoclonality were confirmed independently by two other methods of clonality determination. The findings of a monoclonal origin of carcinosarcomas support the single totipotential stem-cell-divergence hypothesis.

345 citations


Journal ArticleDOI
TL;DR: A group of serous neoplasms that have distinctive morphologic features and that are often associated with progressive, invasive disease are described, characterized by a filigree pattern of highly complex micropapillae arising directly from large, bulbous papillary structures.
Abstract: According to the International Federation of Gynecology and Obstetrics (FIGO) and the World Health Organization (WHO), stromal invasion, defined as destructive infiltrative growth, is the sole criterion used to distinguish serous borderline tumors from invasive serous carcinomas of the ovary. Although this criterion effectively identifies most malignant tumors, it does not permit the identification of a small subset of well-differentiated ovarian carcinomas that do not display destructive infiltrative growth but that may be associated with malignant behavior. In this study, we describe a group of such serous neoplasms that have distinctive morphologic features and that are often associated with progressive, invasive disease. We have designated these tumors micropapillary serous carcinomas (MPSC). They are characterized by a filigree pattern of highly complex micropapillae arising directly from large, bulbous papillary structures. The micropapillae are covered by round to cuboidal cells with a high nuclear-to-cytoplasmic ratio. Typical serous borderline tumors tend to display a hierarchical pattern of branching terminating in small papillae or tufts, and the cells covering the papillae tend to be more columnar and often ciliated compared with cells of MPSC. We reviewed more than 400 cases of serous ovarian borderline tumors and well-differentiated serous carcinomas and identified 26 cases of MPSC. Seventeen tumors lacked destructive infiltrative growth (noninvasive), and nine contained areas of invasion ranging from minimal to extensive. Eight of the 26 tumors were stage I, and none of the patients developed recurrence whether or not their tumors had demonstrable invasion. In contrast, of the 16 women presenting with stage II disease or higher and who had more than 1 year of follow-up, eight (50%) have either died of intra-abdominal carcinomatosis or are alive with carcinoma. Twenty-four (92%) of MPSCs contained areas of serous borderline tumor. The frequent association of MPSCs with serous borderline tumors suggests that MPSCs arise from the latter and may account for the few cases of serous borderline tumors that have been associated with progression to invasive carcinoma.

314 citations


Journal ArticleDOI
TL;DR: The recognition that SBTs can be divided into benign and malignant subtypes provides the basis for replacing the borderline category and tumors displaying a micropapillary growth pattern (MPSC) and SBTs with invasive implants should be classified as carcinomas and treated accordingly.
Abstract: Poor outcome in serous borderline tumors (SBT) of the ovary is limited to patients with advanced stage disease. This study was designed to determine whether there are histologic features among advanced-stage SBTs (International Federation of Gynecology and Obstetrics [FIGO] stages II and III) that predict behavior. The 65 cases in the study were divided into three groups: typical SBTs, with noninvasive implants (51 cases), SBTs with invasive implants (three cases), and a recently described tumor, designated micropapillary serous carcinoma (MPSC) (11 cases), a proliferative serous ovarian neoplasm that often lacks destructive infiltrative growth but appears to behave as a low-grade invasive carcinoma. When the tumor lacks infiltrative growth, as it did in the 11 cases in this series, it qualifies as a borderline tumor. After censoring nontumor deaths, the 5- and 10-year actuarial survival rates were 98% for SBTs with noninvasive implants, 33% for SBTs with invasive implants, and 81% at 5 years and 71% at 10 years for MPSCs. The mean follow-up was 100 months. Two (4%) of 51 patients with SBTs with noninvasive implants subsequently developed invasive carcinoma, and one (2%) died of carcinoma. In contrast, two (67%) of three women with SBTs accompanied by invasive implants developed invasive carcinoma, and both died of disease. Finally, of the 11 patients with MPSC, seven (64%), all of whom had invasive implants, developed recurrences of invasive carcinoma and/or died of tumor. MPSCs had significantly higher rates of mortality (p < 0.001) and recurrence as invasive carcinoma (p < .002) than SBTs with noninvasive implants. The recognition that SBTs can be divided into benign and malignant subtypes provides the basis for replacing the borderline category. The benign subgroup is composed of typical SBTs, including those with noninvasive implants for which the term atypical proliferative serous tumor is appropriate. In contrast, tumors displaying a micropapillary growth pattern (MPSC) and SBTs with invasive implants should be classified as carcinomas and treated accordingly.

308 citations


Journal ArticleDOI
TL;DR: Patients with CVID and XLAG manifest a spectrum of abnormalities in the gastrointestinal tract, with patterns superficially resembling graft-versus-host disease, inflammatory bowel disease, and Whipple's disease, but often lacking some of the diagnostic features of the diseases.
Abstract: Review of the medical records of 43 patients with common variable immunodeficiency (CVID) and 23 patients with X-linked agammaglobulinemia (XLAG) revealed a high incidence of chronic gastrointestinal complaints, most commonly diarrhea. Thirty-eight biopsies, four small-bowel resection specimens, and one autopsy from 10 patients with CVID and one patient with XLAG showed a wide range of abnormalities. A pattern resembling acute graft-versus-host disease, with apoptotic bodies and lymphocytes in crypts, was seen in the stomach (four patients), small bowel (three patients), and colon (three patients). Small-bowel specimens from three CVID patients with malabsorption showed mild to severe villous atrophy. Three CVID patients had Giardia in biopsies. Two cases of small bowel lymphoma associated with nodular lymphoid hyperplasia were identified in CVID patients. One patient's small bowel contained foamy histiocytes in the lamina propria, resembling Whipple's disease or chronic granulomatous disease, with numerous apoptotic bodies in crypts. Ultrastructurally, the histiocytes contained cellular debris. The patient with XLAG had recurrent fissuring necrosis of small bowel resembling Crohn's disease; a patient with CVID had colitis with features similar to ulcerative colitis. Poorly formed granulomas were seen in the stomach (one CVID patient) and the colon (two CVID patients). Lymphocyte populations were dominated by T cells; B cells were scarce except in lymphoid follicles in CVID patients with nodular lymphoid hyperplasia. Patients with CVID and XLAG manifest a spectrum of abnormalities in the gastrointestinal tract, with patterns superficially resembling graft-versus-host disease, inflammatory bowel disease, and Whipple's disease, but often lacking some of the diagnostic features of the diseases. Many of the CVID patients with chronic gastrointestinal complaints (62%) also had evidence of autoimmune phenomena, suggesting that in some patients the inflammatory process in the gastrointestinal tract has an autoimmune component.

Journal ArticleDOI
TL;DR: The term LCG is preferred to avoid confusion with the term histiocytosis X because there is evidence that the LC is not a member of the mononuclear phagocyte system and hence not a tissue macrophage, and because the use of the term "histiocyte" has become a convenience in much of the literature when reporting incompletely understood diseases.
Abstract: We summarize our experience with 238 cases of Langerhans cell granulomatosis (LCG), 198 of whom were followed for a median period of 10.5 years. Our patients did well unless overtreated, and no deaths were attributed to the disorder itself. The disease may appear in unifocal or multifocal form, and treatment is based on this fact. Virtually all patients recovered completely except for occasional residual orthopedic problems or residual diabetes insipidus. Several of the patients underwent subsequent pregnancies without difficulty. The granulomas primarily occur in bone, but lung, skin, and lymph nodal involvement is not uncommon. Involvement of thyroid, thymus, and other sites is rare. The hallmark of the disease is the accumulation of Langerhans cells (LCs). We review the pathology of LCG by histology, electron microscopy, and immunolabeling. LCs originally were identified in squamous epithelium, but these cells are part of the widespread system of dendritic cells. The latter cells, which arise from CD34+ progenitors, are specialized and efficient antigen-presenting cells for T-cell-mediated immunity. In LCG, however, the major associated cells are not T cells, but mature eosinophils: hence the original name eosinophilic granuloma. Confusion about terminology has been based upon the scanty and rather crude pathology reports in the original literature. The term histiocytosis X was meant to cover a spectrum of three diseases--eosinophilic granuloma, Hand-Schuller-Christian disease (HSC), and Letterer-Siwe disease (LS)--but HSC and LS have no basis in pathology and hence the terms are meaningless. The term HSC has become a synonym for multifocal eosinophilic granuloma (LCG). The term LS has been used in reporting a number of benign, malignant, or unknown conditions. We prefer the term LCG to avoid confusion with the term histiocytosis X because there is evidence that the LC is not a member of the mononuclear phagocyte system and hence not a tissue macrophage, and because the use of the term "histiocyte" has become a convenience in much of the literature when reporting incompletely understood diseases.

Journal ArticleDOI
TL;DR: It was concluded that intraductal prostatic adenocarcinoma is a common morphologic entity with precisely defined histologic criteria and a unique biologic significance, as reflected by an enhanced capacity for extensive spread within ducts and perineural spaces.
Abstract: Malignant epithelial masses within prostatic duct lumens have been equated with several conflicting entities, including Gleason cribriform grade 3 carcinoma and cribriforming dysplasia. We identified 51 radical prostatectomy cancers containing intraductal lesions among 130 cases, with total cancer volumes between 4 and 10 cc. Such lesions with duct lumen-spanning septa or masses were rare in areas away from invasive cancer (22 foci), while dysplasia (prostatic intraepithelial neoplasia) was common (1,490 foci). Consequently, these lesions were interpreted as being part of the evolution of invasive carcinoma rather than precursors; they were designated "intraductal carcinoma" as distinct from dysplasia. Intraductal cancer areas within invasive carcinoma usually represented cancer extension within the branches of a single segment of the duct-acinar system from near the urethra to the gland capsule. In 51% of cases with intraductal spread, the invasive component produced large ( > 0.5 mm) tumor masses in perineural spaces, which in turn correlated strongly with extensive capsule penetration and frequent positive surgical margins selectively at the superior nerve pedicle. The amount of grade 4/5 cancer, the amount of intraductal carcinoma, and the large perineural tumor mass appeared to be related to postprostatectomy progression of cancer, as measured by elevation of ultrasensitive serum prostate-specific antigen. It was concluded that intraductal prostatic adenocarcinoma is a common morphologic entity with precisely defined histologic criteria and a unique biologic significance, as reflected by an enhanced capacity for extensive spread within ducts and perineural spaces. It was proposed that the diversity of diagnoses attached to most cribriform malignant lesions can be unified by the concept of this single entity.

Journal ArticleDOI
TL;DR: The behavior of these tumors is more akin to that of a low-grade soft tissue sarcoma than a malignant lymphoma and is characterized by local recurrences and occasional metastases.
Abstract: Follicular dendritic cell (FDC) tumor is an extremely rare malignant neoplasm with approximately 17 well-documented cases in the literature. We report 13 additional cases of this distinctive neoplasm. There were seven men and six women, with a mean age of 46.5 years (range, 27-62 years). There was i

Journal ArticleDOI
TL;DR: It is concluded that intraductal oncocytic papillary neoplasm is a distinctive pancreatic tumor that is usually intradUCTal but may develop invasive carcinoma and should be treated with complete resection.
Abstract: We describe the clinical and pathologic features of 11 intraductal oncocytic papillary neoplasms of the pancreas, a hitherto unrecognized tumor. The patients were six men and five women, and most of the tumors were in the head (head: body/tail = 8:3). The mean patient age was 62 (range, 39-78), and the average tumor size was 6 cm. Grossly the tumors exhibited mucin-filled cysts containing nodular papillary projections. Dilated ducts communicating with the main tumor were sometimes noted. Microscopically the cystic structures appeared to represent dilated ducts containing intraductal tumor. The tumors were characterized by variably complex, arborizing papillary structures. The papillae had thin, delicate fibrovascular cores with focal myxoid changes and were lined by stratified oncocytic cells. Goblet cells and intra-epithelial mucin-containing lumina were present, the latter resulting in a characteristic cribriform pattern. The exuberance of the epithelial proliferation varied from case to case and between different regions within individual tumors; solid sheets of cells were often identified. Although the degree of cytologic atypia was not generally severe, the complexity of the architecture justified a designation of intraductal oncocytic papillary carcinoma in 10 of the 11 cases. In nine cases the tumor was entirely intraductal; one case exhibited focal microinvasion and another showed widespread invasive carcinoma, the invasive elements appearing cytologically similar to the intraductal papillary components. The oncocytic cells stained positively with phosphotungstic acid hematoxylin and Novelli stains. Immunohistochemically, all cases stained positively for B72.3, and five cases showed focal, weak luminal membrane staining for carcinoembryonic antigen. Ultrastructurally many of the cells were packed with mitochondria, and mucin was also identified. Seven patients were alive and free of tumor from 1 month to 3 years (average, 1 year) after resection. Two patients died postoperatively. The remaining two patients died with no evidence of disease at 2.5 and 5 years, the latter following a recurrence at 2.5 years. We conclude that intraductal oncocytic papillary neoplasm is a distinctive pancreatic tumor that is usually intraductal but may develop invasive carcinoma and should be treated with complete resection.

Journal ArticleDOI
TL;DR: That nodal nevi are selectively present in melanoma patients raises the possibility of their origin from nodal melanocytes influenced by tumor products, and may indicate that the nevocytes of cutaneous nevi can be disrupted and displaced by the growth of an adjacent melanoma.
Abstract: Nevocytes in melanoma-draining lymph nodes can be mistaken for melanoma metastases and may possibly transform to melanoma. During the development of a new technique for managing high-risk primary melanomas, selective lymph node dissection, we examined 4,821 nodes from 208 melanoma patients by light microscopy and immunohistochemistry. Nodal nevi were identified in 49 of 226 lymphadenectomy specimens (22%), a frequency considerably higher than previously recorded (5-6%). Nevi occurred in 57 of 4,821 nodes (1.2%), in 84% of patients in one node, in 13% of patients in two nodes, and in 3% of patients in three nodes. Nevocytes were detected in hematoxylin and eosin-stained sections in 38 of 49 cases (78%) and exclusively by immunocytochemistry with an antibody to S-100 protein in 11 of 49 (22%). Nevi were in the peripheral capsule in 93% of cases and in internal trabecula in the remaining 7%. Nevocytes surrounded a small vessel in 33% of cases. Nevi were more frequent in axillary (37 of 140, 26%) and cervical nodes (seven of 40, 18%) than in inguinal nodes (five of 46, 11%). Nevi were more frequent in sentinel nodes, the first nodes on the lymphatics draining a primary melanoma (11 of 284, 3.9%), than in nonsentinel nodes (46 of 4,537, 1.01%; p < 0.0008). One of 1,071 nodes from 50 patients with breast cancer (0.1%) and none of 521 nodes from 50 patients with pelvic cancer contained nevocytes. That nodal nevi are selectively present in melanoma patients raises the possibility of their origin from nodal melanocytes influenced by tumor products. Alternatively, the association may indicate that the nevocytes of cutaneous nevi can be disrupted and displaced by the growth of an adjacent melanoma.

Journal ArticleDOI
TL;DR: SCH is a primary benign vascular neoplasm or malformation similar to angiomatosis in which alterations in blood flow might explain some of the secondary features, and areas of diminished blood flow result in vascular collapse with formation of the "cellular" zones, and Areas of vascular engorgement with stasis promote thrombosis and organization.
Abstract: Seventy-eight cases of spindle cell hemangioendothelioma (SCH) were studied to reevaluate its pathogenesis and determine its long-term behavior. Most of the original findings were confirmed by this study. The tumor occurred at all ages (8-78 years; median, 32 years; mean, 34 years). Males and female

Journal ArticleDOI
TL;DR: Endoscopic criteria for GERD have a morphologic counterpart in capillary congestion and hemorrhage into the papillae, which have largely been ignored by pathologists as secondary to biopsy trauma, and a biopsy protocol that maximizes the chances of detecting changes of GERD is suggested.
Abstract: Histologic changes indicative of gastroesophageal reflux disease (GERD) are found on both sides of the squamocolumnar junction (Z-line). In the gastric cardia, inflammation is found as part of GERD in the absence of Helicobacter pylori or other causes of gastritis (carditis). The squamous mucosa is the location most likely to show inflammatory changes, such as neutrophils or eosinophils, close to the Z-line, whereas traditional reactive changes in the squamous mucosa are found only in biopsies taken at least 3 cm above the Z-line. Endoscopic criteria for GERD have a morphologic counterpart in capillary congestion and hemorrhage into the papillae, which have largely been ignored by pathologists as secondary to biopsy trauma. A biopsy protocol that maximizes the chances of detecting changes of GERD is suggested. The traditional definition of Barrett's esophagus as requiring 3 cm of glandular mucosa extending into the esophagus is no longer tenable. However, even the concept of short-segment Barrett's esophagus, in which less than 3 cm of intestinalized mucosa is present, often as tongues, is being challenged because random biopsies immediately distal to the Z-line may also show intestinal metaplasia when Barrett's esophagus is unsuspected endoscopically. Moreover, it is difficult or impossible to determine whether these changes indicate the earliest lesion of Barrett's esophagus or intestinal metaplasia in native cardiac mucosa. It is suggested that Barrett's esophagus be redefined as intestinal metaplasia in the lower esophagus. It is presently unclear whether patients with such minimal Barrett's epithelium are at increased risk for adenocarcinoma or require surveillance. Successful therapy for GERD results in healing of disease in squamous mucosa and may result in regression of Barrett's epithelium. In the stomach it may be associated with temporary regression of H. pylori and associated inflammation, migration of H. pylori into the oxyntic mucosa, hypertrophy and hyperplasia of parietal cells, and a variant of fundic gland polyps. Some patients may be at risk for accelerated atrophic gastritis if inflammation is present before therapy.

Journal ArticleDOI
TL;DR: PMLCL can be combined with other diffuse large B-cell lymphomas on morphologic grounds; it is not associated with EBV and should be managed like other high-grade lymphomas of equivalent histology.
Abstract: Among non-Hodgkin's lymphomas, primary mediastinal large B-cell lymphoma (PMLCL) has been considered a separate entity that has specific clinical and histological aspects and a poor prognosis. In this study, we reexamined the clinicopathologic features and the response to current treatment of 141 PMLCL and compare them with 916 nonmediastinal large B-cell lymphomas (NMLCL) recorded in the same period and treated with similar combined chemotherapy. The clinical features of PMLCL at diagnosis were largely homogeneous and distinct from NMLCL, with a predilection for young women (59% with a mean age of 37 years versus 42% with a mean age of 54 years), bulky tumor (77% versus 7%, p < 10(4)), high serum lactic dehydrogenase (LDH) level 76% versus 51%, p < 10(4)), and frequent intrathoracic extension to adjacent organs such as pleura, pericardium, and lung. By contrast, extrathoracic or hematologic dissemination was uncommon (2% of bone marrow involvement versus 17%). All patients had diffuse large B-cell nonimmunoblastic, nonanaplastic lymphomas. Histological analysis of the 141 PMLCL evaluated two common patterns: the presence of large cells with clear cytoplasm (found in 38% of cases) and the presence of fibrosis (marked in 25% of cases). The presence of clear cells or intense fibrosis did not constitute prognostic indicators. Immunologic and molecular analysis assessed the profile of bcl-2 expression and the presence of Epstein-Barr virus (EBV) in PMLCL: 30% expressed a high level of bcl-2 protein; EBER RNAs were detected by in situ hybridization in only two of the 41 cases tested. Monotypic light chain restriction could be demonstrated in seven of the 41 PMLCL tested on fixed-section. Treated with polychemotherapy regimens without radiotherapy, 79% of PMLCL patients achieved a complete remission compared with 68% in the NMLCL patient group (p = 0.01). Overall, 3-year survival rates were estimated at 66 and 61%, respectively (p = 0.05), and disease-free survival rates were not significantly different (61 versus 64%). Stratified analysis on the International Prognostic Index (based on age, tumor stage, serum LDH level, and performance status) showed no difference in the overall and disease-free survivals between the two lymphoma groups. In conclusion, PMLCL can be combined with other diffuse large B-cell lymphomas on morphologic grounds; it is not associated with EBV. It responds favorably to treatment and should be managed like other high-grade lymphomas of equivalent histology. However, the uncommon clinical presentation makes it a distinct entity.

Journal ArticleDOI
TL;DR: It is indicated that cutaneous specific manifestations of B-CLL present with characteristic histologic, immunophenotypic, and molecular patterns and is likely that prognosis in these patients is probably not affected by skin involvement.
Abstract: The clinical and histopathologic features of specific skin infiltrates in patients with B-cell chronic lymphocytic leukemia (B-CLL) have rarely been reported in detail. In this study we analyzed the clinical, histopathologic, immunophenotypic, and molecular features of 84 skin lesions from 42 patients (M:F = 1.3:1; mean age, 66.0 years; range, 42-83 years) with specific cutaneous manifestations of B-CLL. The duration of B-CLL before skin manifestations varied from 0 to 142 months (mean, 39 months). In seven patients (16.7%), skin lesions represented the first sign of disease. Clinical presentations included localized or generalized erythematous papules, plaques, nodules, and large tumors. Ulceration was uncommon. In six patients lesions were confined at the sites of scars from previous herpes zoster (four patients) or herpes simplex (two patients) eruptions. Histologically, three main patterns were recognized: (a) patchy perivascular and periadnexal, (b) nodular-diffuse, and (c) band-like. Cytomorphologically, small monomorphous lymphocytes predominated. Proliferation centers were observed in only four specimens. In two patients presenting with tumors, a high content of large cells with feature of centroblasts and immunoblasts was found (Richter's syndrome). Immunohistologic analyses were performed on paraffin-embedded specimens in 40 biopsies from 20 patients and on cryostat sections in 17 biopsies from 11 patients. Neoplastic B lymphocytes in all cases showed an aberrant phenotype (paraffin sections: CD20+/CD5+/CD43+; cryostat sections: CD19+/CD5+; immunoglobulin light-chain restriction). Proliferation markers (Ki67, PCNA, MIB1) stained 5 to 80% of cells (mean, 25%; median, 20%). Polymerase chain reaction performed in nine cases on paraffin-embedded tissues using consensus primers for immunoglobulin heavy-chain genes showed a monoclonal population of B lymphocytes in all cases. Several discrete bands in addition to the prominent ones were noted in five cases, indicating the additional presence of B lymphocytes whose immunoglobulin genes were not monoclonally but oligoclonally rearranged. Follow-up data could be obtained from 31 patients. The two patients with Richter's syndrome died after 5 and 8 months, respectively. The 5-year survival of patients with small-cell cutaneous B-CLL was 66.6%. Our study indicates that cutaneous specific manifestations of B-CLL present with characteristic histologic, immunophenotypic, and molecular patterns. Prognosis in these patients is probably not affected by skin involvement.

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TL;DR: The results support the existence of ALT as a distinct tumor subtype that is different from ordinary lipoma and from spindle or pleomorphic lipoma, albeit histogenetically closely related to them, and supports the proposed pathogenetic link between ALT and dedifferentiated liposarcoma.
Abstract: Fifty-nine cases of atypical lipomatous tumors (ALT) of soft tissue (atypical lipomas, well-differentiated liposarcomas) were studied morphologically and cytogenetically as part of an international collaborative study. Forty-nine cases were deeply seated (including retroperitoneum), and 10 were superficial. Clonal chromosomal abnormalities were found in 55 cases (93%). Supernumerary ring or giant marker chromosomes (RGCs), the sole consistent alteration, were found in 37 ALTs (63%). They were more common in tumors that were large (p < 0.001), deeply seated (p < 0.005), that contained lipoblasts (p < 0.05), and that had marked cytologic atypia (p < 0.05). In a relatively short follow-up period (average, 3 years), only three of 59 cases recurred, one resulting in the patient's death. All three cases had RGCs. Also, five of the six cases that underwent dedifferentiation had RGCs, indicating that RGCs are associated not only with low-grade malignant behavior (in the form of local recurrence) but also with the potential for tumor progression. When the karyotypic profile of ALT was compared with that of 233 other types of adipose tissue tumors similarly analyzed by the authors, a statistically highly significant correlation (p < 0.0001) was found between ALT and RGCs. These results support the existence of ALT as a distinct tumor subtype that is different from ordinary lipoma and from spindle or pleomorphic lipoma, albeit histogenetically closely related to them. It also supports the proposed pathogenetic link between ALT and dedifferentiated liposarcoma. The association between chromosomal and morphologic findings indicates the potential role of karyotypic analysis in the differential diagnosis of ALT with ordinary lipoma, spindle or pleomorphic lipoma, hibernoma, and myxoid liposarcoma.

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TL;DR: In this series, patients with multifocal tumors did not have a better prognosis than those with unicentric disease, and too few patients have received adjuvant chemotherapy to evaluate its usefulness in this setting.
Abstract: Epithelioid hemangioendothelioma of bone is a rare tumor of vascular origin. A series of 40 cases from the Mayo Clinic files was studied to define the pathologic features of this disease and to evaluate any histologic or clinical factor influencing outcome. More than 50% of the tumors were multicentric, with a predilection for bones of a particular anatomical area. In this series, patients with multifocal tumors did not have a better prognosis than those with unicentric disease. We could not predict the outcome of cases on the basis of the histologic features. Visceral involvement was the most important criterion in predicting prognosis. Resection is still the primary treatment for these tumors. Radiotherapy may be useful for surgically inaccessible tumors. Too few patients have received adjuvant chemotherapy to evaluate its usefulness in this setting.

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TL;DR: A new definition was developed based on the distribution of anaplastic changes within the tumor: FA applies only to tumors with anaplasia confined to one or a few discrete loci within the primary tumor, with no anaplia or marked nuclear atypia elsewhere.
Abstract: Anaplasia, defined by the presence of extreme nuclear and mitotic atypia, is a potent marker of adverse prognosis in Wilms tumor (WT). Anaplastic WT cells apparently have increased resistance to therapy rather than increased aggressiveness. The distribution of anaplasia should therefore have critical prognostic relevance. The original definitions for focal anaplasia (FA) and diffuse anaplasia (DA) were based on quantitative rather than topographical criteria and lacked prognostic significance. A new definition was developed based on the distribution of anaplastic changes within the tumor: FA applies only to tumors with anaplasia confined to one or a few discrete loci within the primary tumor, with no anaplasia or marked nuclear atypia elsewhere. This revised definition was evaluated in 165 cases with anaplastic WT entered on the third and fourth National Wilms Tumor Study. Only three relapses and one death occurred among 39 cases with FA, regardless of tumor stage, a result comparable to that for nonanaplastic WT. Eight children with metastases at diagnosis and FA in the primary tumor were alive and free of relapse; 22 of 23 children with stage IV DA WT died of tumor. This new definition reinforces the importance of carefully documenting the exact site from which each tumor section is obtained.

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TL;DR: The data indicate that diverticular disease-associated chronic sigmoid colitis expresses morphological features traditionally reserved for idiopathic inflammatory bowel disease, which permits distinction from Crohn's disease and ulcerative colitis.
Abstract: A clinical syndrome of chronic colitis unique to the sigmoid colon harboring diverticular was recently reported; its histopathological appearance has not been fully elucidated. In this study, the authors analyzed the clinical and pathological features of 23 patients (age range, 38-87 years; median age, 72 years) with diverticular disease-associated chronic colitis. Nineteen presented with hematochezia; four had abdominal pain. Colonoscopic visualization of the mucosa showed patchy or confluent granularity and friability affecting the sigmoid colon encompassing diverticular ostia. Colonic mucosae proximal and distal to the sigmoid were endoscopically normal. Mucosal biopsy specimens showed features of idiopathic inflammatory bowel disease that included plasmacellular and eosinophilic expansion of the lamina propria (100%), neutrophilic cryptitis (100%) with crypt abscesses (61%), basal lymphoid aggregates (100%), distorted crypt architecture (87%), basal plasmacytosis (61%), surface epithelial sloughing (61%), focal Paneth cell metaplasia (48%), and granulomatous cryptitis (26%). Concomitant rectal biopsies obtained in five patients demonstrated histologically normal mucosa. Fourteen patients treated with high-fiber diet or antibiotics or both improved clinically, as did nine patients administered sulfasalazine or 5-aminosalicylic acid. Five patients underwent sigmoid colonic resection, three for stricture with obstruction and two for chronic blood loss anemia. Among a control population of 23 age- and gender-matched patients with diverticular disease without luminal surface mucosal abnormality, none required resection during the same follow-up period. By Fisher's exact test, a statistically significant difference in outcome for patients with and without colitis was detected (p = 0.049). In addition, three patients developed ulcerative proctosigmoiditis 6, 9, and 17 months after the onset of diverticular disease-associted colitis. The data indicate that diverticular disease-associated chronic sigmoid colitis expresses morphological features traditionally reserved for idiopathic inflammatory bowel disease. Its clinical and endoscopic profiles permit distinction from Crohn's disease and ulcerative colitis. Patients with chronic colitis in conjunction with diverticula are at increased risk for sigmoid colonic resection. Diverticular disease-associated chronic colitis may also precede the onset of conventional ulcerative proctosigmoiditis in some cases.

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TL;DR: It is concluded that two main pathways operate in gastric carcinogenesis, both starting from H. pylori gastritis and both leading to phenotypically variable, often mixed gastric/intestinal tumor growth.
Abstract: Investigation of extensively sampled nontumor gastric mucosa from 205 early gastric cancers showed Helicobacter pylori colonization in 85% of cases, including 100% of diffuse and 78% (83% in 97 cases with Swiss rolls) of glandular or mixed cancers. Intestinal metaplasia, including its type III variant, was prominent in the mucosa associated with glandular and mixed (but not diffuse) early cancers. Both glandular (usually called intestinal) and diffuse-type cancers showed admixtures of intestinal and gastric tumor cell phenotypes. Both p53 gene mutations and p53 protein immunostaining were essentially restricted to glandular or mixed cancers and associated dysplastic lesions. Their appearance in the advanced stage of diffuse cancer was partly due to a change of the histologic pattern from glandular to diffuse during progression of some tumors. Loss of laminin, β 1 integrin, or zonula adherens junctions was a common finding in both early and advanced diffuse cancer. It is concluded that two main pathways operate in gastric carcinogenesis, both starting from H. pylori gastritis and both leading to phenotypically variable, often mixed gastric/intestinal tumor growth. However, only one of the two pathways involves intestinal metaplasia, its type III variant, p53 gene alteration, and dysplasia to end in glandular cancer. In the other pathway, diffuse cancer apparently arises directly from hyperplastic, sometimes atypical necks of mostly nonmetaplastic gastric glands, through primary involvement of genes affecting cell-cell and cell-matrix junctional proteins.

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Carney Ja1, J A Ferreiro
TL;DR: The epithelioid blue nevus is important because of its strong association with the Carney complex, and affected patients (and their relatives) should be considered at risk for other elements of the syndrome, especially cardiac myxoma.
Abstract: We report on 21 pigmented nevi that occurred in 11 patients (6 male and 5 female patients) age 3 to 39 years. All patients had the Carney complex (myxomas, spotty skin pigmentation, endocrine overactivity, and schwannomas); six patients were members of three families. The nevi occurred on the extremities and trunk, less frequently on the head and neck, and were multiple in five patients. Clinically, they were darkly pigmented, domed, and small (less than 1 cm) and commonly interpreted as "blue nevi." Microscopically, they featured heavily pigmented, poorly circumscribed, dermal lesions that displayed two types of melanocytes: one intensely pigmented, globular, and fusiform; the other lightly pigmented, polygonal, and spindle. The melanocytes were situated among the dermal collagen bundles singly, in short rows and small groups, and occasionally in fascicles. Nuclei of the lightly pigmented, polygonal and spindle cells were vesicular with very pale chromatin and a single prominent nucleolus. Seven tumors were each part of a combined nevus. After excision, none of the tumors recurred or metastasized. The epithelioid blue nevus is important because of its strong association with the Carney complex. Therefore, affected patients (and their relatives) should be considered at risk for other elements of the syndrome, especially cardiac myxoma.

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TL;DR: An expanded criteria to distinguish betweenLeiomyomas and leiomyosarcomas of the vulva is proposed and the sarcomas should be excised conservatively, with long-term, careful follow-up.
Abstract: The clinical and pathological features of 25 smooth-muscle tumors of the vulva were analyzed. The patients ranged in age from 17 to 67 (mean, 37.6) years; two were pregnant. Twenty-three tumors were 1.5 to 16 (mean, 5.2) cm in greatest dimension; the size of two tumors was unknown. Microscopic examination showed that 16 tumors were circumscribed, six had focally infiltrative margins, and the margins could not be evaluated in three tumors. Fourteen tumors were composed mainly of spindle cells; two of these tumors had prominent myxoid stroma. Seven tumors were predominantly epithelioid and had a prominent hyalinized or myxoid stroma; often the cells had a plexiform pattern. Four tumors contained an approximately equal number of epithelioid and spindle cells. Ten tumors had mild, nine moderate, and six severe cytologic atypia. Mitotic figures ranged from 0 to 10 (average, 1.8) per 10 high-power fields (hpf). Immunohistochemically, all the tumors stained for one or more muscle markers. Thirteen of 17 tumors were positive for estrogen receptors, and 16 of 18 were positive for progesterone receptors. Follow-up information ranging from 1 month to 19 years (average, 5 years) was available in 19 cases. Four tumors recurred locally, and one patient with recurrent tumor died of metastases 7 months after the initial operation. We propose an expanded criteria to distinguish between leiomyomas and leiomyosarcomas of the vulva. Tumors that manifest three or all of the four following features should be considered sarcomas: > or = 5 cm in greatest dimension, infiltrative margins, > or = 5 mitotic figures per 10 hpf, and moderate to severe cytologic atypia. Those that have only one of these characteristics should be diagnosed as leiomyoma, and those that exhibit only two of these features should be considered benign but atypical leiomyomas. The sarcomas should be excised with widely negative margins; the leiomyomas and the atypical leiomyomas should be excised conservatively, with long-term, careful follow-up.

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TL;DR: Second-opinion pathological evaluation of prostate biopsy before radical prostatectomy is cost effective and has a major impact on clinical treatment for a subset of patients.
Abstract: Despite numerous studies evaluating second-opinion surgical programs, we are unaware of work evaluating the cost effectiveness of a second opinion for pathology prior to surgery. One of six pathologists reviewed the pathology of the outside needle biopsies of 535 consecutive men referred to Johns Hopkins Hospital for radical prostatectomy over a 12-month period (from October 1993 until October 1994) before the men underwent surgery. Of the 535 needle biopsies initially diagnosed on the outside as adenocarcinoma of the prostate, seven (1.3%) were reclassified as benign upon pathology review at Johns Hopkins Hospital. The most common lesion misinterpreted as adenocarcinoma was adenosis or less pronounced examples of adenosis consisting of foci of crowded glands (five cases). Foci of atrophy in the remaining two cases were misdiagnosed as adenocarcinoma of the prostate. Upon subsequent clinical work up, six of seven men were considered not to have adenocarcinoma, and their surgery was cancelled. The cost for reviewing all 535 preoperative needle biopsies was $44,883, which included the cost of immunohistochemical studies for high-molecular-weight cytokeratin and repeat biopsies and ultrasounds in men whose diagnoses were reversed. The total cost of the radical prostatectomies had the six men undergone surgery was estimated at $85,686, including hospitalization, anesthesia, radical prostatectomy pathology, and surgery. This cost savings did not include other costs resulting from lost wages, morbidity, or potential litigation. Second-opinion pathological evaluation of prostate biopsy before radical prostatectomy is cost effective and has a major impact on clinical treatment for a subset of patients.

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TL;DR: The strong expression of latent membrane protein restricted to the tumor cells and the clonality of the EBV suggest that the virus may be involved in the pathogenesis of this tumor and not present merely as a "bystander."
Abstract: Tumors of the follicular dendritic cell are uncommon, and most occur as primary lymph node tumors. We report a case of primary follicular dendritic cell tumor of the liver that was initially reported as an inflammatory pseudotumor. The neoplasm recurred as two separate tumor masses 30 months after complete resection of the "hepatic inflammatory pseudotumor." It showed a wide spectrum of morphologic features ranging from areas with fascicles of very bland spindle cells amidst a background population of lymphocytes, reminiscent of inflammatory pseudotumor, to areas of dispersed sheets of highly pleomorphic tumor cells with a relative paucity of reactive inflammatory cells. The diagnosis was confirmed by positive immunohistochemical staining with CD21, CD35, R4/23, and Ki-M4 and by ultrastructural demonstration of convoluted interdigitating cell processes joined by desmosomes. The background lymphocytes were oligoclonal, CD8-positive T cells. In situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was positive in the tumor cells in the original and recurrent tumors. More importantly, the cells showed identical episomal clonal EBV on Southern blot analysis, implying that the initial and recurrent tumors are due to clonal proliferation of EBV-positive neoplastic follicular dendritic cells. The tumor cells expressed latent membrane protein but not EBV-encoded nuclear antigen 2 (EBNA2) or ZEBRA. Such gene expression is very similar to that of Hodgkin's disease and nasopharyngeal carcinoma. The strong expression of latent membrane protein restricted to the tumor cells and the clonality of the EBV suggest that the virus may be involved in the pathogenesis of this tumor and not present merely as a "bystander."

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TL;DR: It is found that a round cell component of > 5% portends a higher risk of metastasis or death from disease, and only those areas that are unequivocally round cell liposarcoma should be designated as high grade.
Abstract: Round cell liposarcoma, a high-grade sarcoma, is a poorly differentiated form of myxoid liposarcoma, which is low grade. It is not known, however, how much of a round cell component within an otherwise typical myxoid liposarcoma results in a neoplasm that behaves as a high-grade sarcoma. Twenty-nine cases of myxoid liposarcoma of the extremities with or without a component of round cell liposarcoma were studied to semiquantitate the amount of round cell component needed to adversely affect prognosis. An estimate of the percent of necrosis, round cell liposarcoma, myxoid liposarcoma, and transitional areas was obtained for each slide on all cases. Transitional areas were defined as those that displayed an increased cellularity compared with typical myxoid liposarcoma, but in which the cells remained spindled, did not have overlapping nuclear borders, and retained an easily discernible plexiform vascular pattern. The amount of necrosis was subtracted from the total material available for evaluation, and a composite estimate of the percent of round cell, myxoid, and transitional areas was obtained. Two tumors were located on the upper extremity, 27 on the lower extremity; tumor size ranged from 3 to 30 cm (median, 14 cm). All 29 tumors had a myxoid component, with a range from 12 to 100% (median, 73%). The range of transitional component for all 29 tumors was 0 to 88% (median, 11%). Twenty-one tumors had transitional areas (range, 4-88%). The range of round cell component for all 29 tumors was 0 to 58% (median, 0%). Twelve tumors had round cell areas (range, 1-58%). Seventeen patients are either alive without disease, or died from unrelated causes at 24-202 months (median, 96 months). Twelve patients are either alive with metastases or died of disease at 10 to 180 months (median, 53 months). Patients with >5% round cell component in their initial tumor had a statistically significant higher rate of metastasis or death due to disease than those with ≤5% round cell liposarcoma (p = 0.05). In addition, patients with myxoid liposarcoma with transitional areas did not fare worse than those with myxoid liposarcoma alone. In conclusion, we found that a round cell component of >5% portends a higher risk of metastasis or death from disease. Furthermore, transitional areas alone do not appear to alter the prognosis of myxoid liposarcoma. Thus, only those areas that are unequivocally round cell liposarcoma should be designated as high grade.