Showing papers in "Československá patologie in 2000"

[Intestinal T-cell lymphoma].

Abstract: In our study, 7 bioptical specimens of intestinal T-cell lymphoma are described. This tumour occurs in the small intestine of adults who may have a history of malabsorption. The patients present clinically with intestinal perforation, enterorrhagia, or ileus. The gross appearance varies: the tumour may take the form of multiple ulcers or of a huge exulcerated lesion. The cytomorphological features are variable. Bizarre multinucleated cells may be sometimes present. The reactive cellular background is usually dominated by histiocytes or eosinophilic granulocytes. Immunohistochemically, the tumour cells stain in reactions with antibodies to pan T-cell markers, occasionally to CD8 and CD30. The positivity of tumour cells for markers of intraepithelial T-cells and for cytotoxic molecules has been demonstrated previously.

Kaposiform hemangioendothelioma in adult. Report of a case with amianthoid-like fibrosis and angiectases.

Abstract: A rare case of kaposiform hemangioendothelioma in adult is reported. The 11 x 7 x 5 cm tumor was excised from deep subcutis of the abdominal region in 37-year-old man. No signs of Kasabach-Merritt syndrome or lymphangiomatosis were present. Besides typical pattern of kaposiform hemangioendothelioma, following unusual features were found: dilated vessels producing a gross impression of spindle cell hemangioma, areas of amianthoid-like fibrosis, and diffuse immunoreactivity for CD99. The differential diagnosis included mainly spindle cell hemangioma (hemangioendothelioma), hemangiopericytoma-like solitary fibrous tumor, and Kaposi's sarcoma.

Plexiform fibrohistiocytic tumor of soft tissues and bone

Abstract: Plexiform fibrohistiocytic tumor is a rare lesion which has been reported only in the dermis and subcutis so far. We present two cases in this location and an additional one localized in the proximal epiphysis of the tibia. A case with crural location showed predominance of plexiform tumorous nodes with fibrohistiocytic arrangement. The nodes were composed of spindle-shaped myofibroblasts with admixture of histiocytes and giant multinucleated osteoclast-like cells. In another case the tumor of axillary soft tissues featured mostly plexiform bundles of spindle-shaped myofibroblast-like cells reacting positively with actin and CD 68 antibodies; ultrastructurally, they contained numerous dense lysosomal inclusions with myelin figures. The bone tumor was composed of hyalinized fibroblastic component with disperse fibrohistiocytic nodes. Despite a semimalignant character of the lesion there was no recurrence in our cases during the 2-6 year postoperative period.

Composite multicystic mesothelioma and adenomatoid tumor of the ovary: additional observation suggesting common histogenesis of both lesions.

Abstract: An unusual case of composite multicystic mesothelioma and adenomatoid tumor of the ovary is reported. The gradual transitions between both tumor components were well apparent. This observation indicates a histogenetic relationship between adenomatoid tumor and benign multicystic mesothelioma.

Diagnosis of malacoplakia in a transplanted kidney by needle biopsy

Abstract: Malakoplakia is an uncommon inflammatory condition rarely involving parenchyma of transplanted kidney We report a 44-year-old female recipient of a cadaveric renal allograft treated with cyclosporin A and prednisone After transplantation, E coli and Citrobacter bacteruria occurred and three years later decreased graft function developed Percutaneous needle biopsy was performed and diagnosis of malakoplakia was established Histologically, interstitial sheets of plasmocytes and histiocytes with periodic acid-Shiff positive cytoplasm containing Michaelis-Gutmann bodies were present Ultrastructurally, phagolysosomes containing membrane fragments and various developmental stages of inclusions to fully developed Michaelis-Gutmann bodies were found The patient was treated with co-piperacillin and subsequently pefloxacin and renal functions improved after six months follow-up Our case suggests that malakoplakia represents an abnormal defective histiocytic response to the infection in the setting of immunosuppressive therapy

Fetal rhabdomyoma of the mucosa of the lip (case report)

Abstract: In a 15-year-old male the foetal type of rhabdomyoma was diagnosed in the mucosa of the lower lip. Diagnosis of this relatively rare tumour prevents possible mistaking for a malignant process and an inadequate therapeutic procedure.

Atypical oxyphilic metaplasia of the uterine cervix. Report of a case.

Abstract: A new example of atypical oxyphilic metaplasia (AOM) of the uterine endocervix occurring in a 37-year-old female is presented. This rare lesion is worrisome in appearance but entirely indolent and needs to be distinguished from glandular atypia or adenocarcinoma in situ of the cervix. In the present case, there were seen transitions between AOM and tubal metaplasia in some cervical glands. Additionally, similar eosinophilic and ciliated changes were found in the endometrium. These findings suggest that AOM, tubal metaplasia of the cervix and eosinophilic metaplasia of the endometrium may be related from the pathogenetic viewpoint.

Benign glomus tumor of the superior posterior mediastinum.

Abstract: An unusual location of a benign glomus tumour, outside of the constantly located regions, e.g. in the subungual location or deeply sited in extremities, was diagnosed in a 56-year-old white female in her posterior upper mediastinum. The single similar case report was published before the era of electron microscopy and immunohistochemistry and single cases of atypical and malignant forms in this unusual location were published only recently. The tumour measuring 5 x 4 x 2 centimeters has caused cough and was associated with occasional righ-sided chest pain. Its rich vascular supply has caused intensive intraoperative bleeding. The postoperative course was uneventful and the patient is free of neoplastic disease or symptoms six years after surgery. Numerous mast cells present within the tumour's interstices must be considered in relation to the possible pathogenesis of the up to now unexplained pain in glomus tumours.

Morphologic and clinical sequelae of focal ischemic lesions

Abstract: The diseases of vessels, mainly of those in brain are one of the most serious problems of the medical practice. The encephalomalacia or cerebral infarctions are usually caused by transient or permanent obstruction of the brain arteries lumen. Beside local dysfunction of vessels the obstructions could be based on embolic events originating in the heart. Such an obstructions are resulting in global and focal cerebral ischaemias. Arterial occlusion results in cerebral ischaemia and the lack of oxygen (anoxia) which leads to reversible or irreversible injury of the nervous cells in the ischaemic region. The local cell injury or cell death causes attraction of macrophages invading into the devitalized tissue within 72-96 hours after the beginning of the ischaemia. The aim of this study was to find out the correlation between asymptomatic or symptomatic course regarding localisation of the ischaemic lesions in the cerebral tissue. Our anatomical findings were collected from 318 autopsies, and reports on postmortem examinations during the period between September-December 1998. The grossing of the brain was carried out by using of Virchow's method. Atherosclerosis, hypertension, and diabetes mellitus were found to be the main risk factors for the production of focal cerebral ischaemia. Of those patients with focal cerebral ischaemia atherosclerosis had 87.5%, 44.3% were suffering from hypertension, and 25% from diabetes mellitus. The focal ischaemia analysed in this study originated from arterial stenosis or thromboembolic obstructions. We divided the lesions into 3 groups according to their size. The most frequently apparent lesions (72%) were the small cysts (0-10 mm in diameter)-lacunae. The majority of them (90%) was found in the basal ganglia. The second group consisted of postmalatic pseudocysts (10-30 mm in diameter), and the third group was represented by encephalomalatic lesions which were larger than 30 mm. Cerebral ischaemic lesions were present in 27.8% of the studied cases. Nevertheless, more than the half (56.8%) of the affected brains (postmalatic pseudocysts, lacunae and malaciae) belongs to the group of patients who were clinically asymptomatic. The asymptomatic lesions, having negative results in the patient's history, and the clinical course were localised mainly in the basal ganglia of both sides and in the frontal part of the right (nondominant) hemisphere.

Biological characteristics of multiple myeloma

Abstract: On a series of thirty trephine bone marrow biopsies from patients with multiple myeloma, the authors evaluated expression of markers of cell proliferation or of its blockade (Ki-67, PCNA, topoisomerase IIa, cyclin D-1, AgNOR, and p27kip1) and markers indicating multidrug resistance (P-170 and Bcl-2). Expression of Ki-67 and of topoisomerase IIa was unfrequent. Marked positivity of PCNA was expressed in about one third of cases, negative staining was exceptional. No expression of cyclin D-1 was noted. Positivity of p27kip1 was frequent. P-170 was demonstrated in a small number of cases, Bcl-2 was strongly positive in most cases. The results characterise multiple myeloma as a tumour with low proliferation rate and, simultaneously, with high resistance to apoptosis.

[Angiogenesis in tumors. Part I. Its role in the determination of biological characteristics of tumors; factors controlling the onset and development of angiogenesis].

Abstract: The tumor stroma gains increasing attention in recent years. Angiogenesis (AG) is defined as formation of new vessels from the endothelium of the preexisting vasculature. The vascular bed does not only provide supply of nutrition factors and oxygen as well as elimination of metabolic waste, but the neo-formed vessels represent also a gate for lymphogenous and hematogenous metastatic spread of the tumor. For tumor growth beyond the size of several mm, AG must be started to form vascular supply of tumor cells. After the switch to angiogenic phenotype, the tumor starts to exhibit rapid, almost exponential growth. AG is a complex process, involving degradation of the basement membrane of preexisting vessel, proliferation of endothelial buds or solid strands towards the angiogenic stimulus, maturation of endothelial cells with formation of a luminized capillary, connection of such capillaries with creation of vascular loops, and finally formation of a functional vessel, surrounded by basement membrane and pericytes. The most intensive AG is observed at the periphery of the tumor. AG is regulated by numerous angiogenic and anti-angiogenic factors, which can be synthesized and released by tumor cells themselves, stromal elements, as well as inflammatory cells, namely macrophages. The issues of regulation of AG and importance of AG for tumor growth, progression and metastasis are discussed.

Pathology of paraneoplastic syndromes. Review

Abstract: A review of current opinion on paraneoplastic syndrome pathology is presented. It is supplemented by a reference to the author's own cases (paraneoplastic cerebellar degeneration with Purkinje cell lesion and vacuolar spongiform degeneration of the granuloneuronal layer of the cerebellar cortex).

Neuronal ceroid lipofuscinosis. Closing chapter of a long story

Abstract: Neuronal ceroid lipofuscinoses represent a group of diseases which has until quite recently resisted definite elucidation of the underlying defect(s) on the molecular level. The common feature of all the NCLs is a serious and progressive neurological disorder, accompanied, with only few exceptions, by retinal degeneration. Visceral symptoms, despite the presence of the storage process, are absent, or minimal. There are many clinical variants of the disease process, among which a set of standard, historical phenotypes exists found to be linked to specific genotypes. The disorder is inherited and transmitted as an autosomal recessive trait. At the cellular level, it is featured by lyzosomal storage of autofluorescent hydrophobic material, the substantial part of which consists of hydrophobic proteins and esterified dolichol. The dominant protein is the subunit c of mitochondria ATP synthase. In one NCL type (NCL1) the dominant proteins are saposins A and D. Ultrastructural appearance is membranous with several relatively specific patterns with some tendency to condensation or, namely in NCL3 to vacuolar distension. Amorphous appearance is associated with NCL1. The impact of the disease process on the cell biology differs substantially depending on the cell type. The brain neurons are most seriously affected and degenerate, whereas other cell types mostly survive without detectable deterioration. Pathogenesis at the molecular level is now being elucidated using the modern molecular biology techniques, which have already enabled unravelling of a set of genes controlling majority of the standard historical phenotypes. The original infantile form of NCL (NCL1) is now defined as palmitoyl protein thioesterase deficiency (gen at the 1p32 locus), the late infantile form (NCL2) as pepstatin resistant proteinase deficiency (gen at the 11p15.5 locus) and the original juvenile form (NCL3) as a defect of the specific gene (locus 16p11.2-12.3), the product of which, the NCL3 protein, still lacks functional characterization. Two gene loci have been identified in the so-called early juvenile, or variant late infantile NCL. One of them is in the 13q21 locus (NCL5 or Finnish variant late infantile form), the second is in the 15q21-23 one (NCL6). Kufs form remains the least defined form of NCL. Recently two novel NCL variants were described with specific loci. Thanks to introduction of molecular genetic based diagnosis it was possible to recognize, besides the standard phenotype, existence of further phenotypic variants. The phenotype based scheme of NCL has thus been definitely substituted by classification based on genotype and biochemistry.

A localized form of metastatic pulmonary calcification in a patient after renal transplantation

Abstract: A 51-year-old woman with a 20-year history of renal transplantation during the end-stage of chronic renal failure caused by tubulointerstitial nephritis. She died of chronic graft rejection. An interstitial calcification limited to 1. and 2. left segments was encountered as an incidental autoptic finding. There were two unusual microscopic findings, i.e., no involvement of arterioles and larger vessel inclusive veins, and a foreign-body response to some of the calcified tissue.

Proliferative activity in pulmonary carcinoids

Abstract: AIM On the basis of known Ki-67 dependence on tumor malignancy in some lesions, we compared this marker expression quantitatively in pulmonary typical and atypical carcinoid tumors and attempted to predict their biological behavior especially in cases associated with tumorous lymphadenopathy, satellites, and carcinoid tumorlets. METHOD Using material from surgically treated patients, we examined 54 cases of pulmonary carcinoids divided into five groups. 1. Forty-two typical carcinoids (TC), 2. Twelve atypical carcinoids (AC) diagnosed according to modified Arrigoni's criteria (Travis et al., 1998), 3. Thirty-two TC without metastases, satellites, and tumorlets (M, S, T), 4. Eight AC without M, S, T, and 5. Fourteen TC and AC associated with M, S, T. Groups 3, 4, and 5 were formed of cases selected from group 1 and 2. The proliferate activity was evaluated by Ki-67 (MIB-1, Immunotech France, 1:25). Its nuclear labeling was counted in more than 50 HPF and calculated as a number of positive nuclei in 10 HPF. The Fisher exact test was used for statistical analysis. RESULTS The Ki-67 nuclear expression was found in 19 (45%) out of 42 TC and in 9 (75%) out of 12 AC. In the set of TC without metastases (M, S, T), the Ki-67 positive labeling was found in 14 (44%) out of 32 cases (group III) and in six (75%) out of eight AC (group IV). In all TC and AC tumors with M, S, T (group V), the Ki-67 expression was encountered in 8 (57%) out of 14 cases. The Fisher exact test showed no significant difference between all examined groups. CONCLUSION No statistically significant difference was found in Ki-67 expression in pulmonary typical and atypical carcinoids. It appears to be a factor which can not be used for tumor prognosis prediction or adjuvant therapy indication in surgically treated patients.