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Showing papers in "Československá patologie in 2004"


Journal Article
TL;DR: The confirmation of the intrathecal IgG synthesis by oligoclonal bands in CSF and the immunohistochemical detection of IgG deposits on membranes of Purkinje cells, neurons of Cerebellar granular layer and in cerebellar nuclei in this case support the presumed autoimmune nature of the disease.
Abstract: Paraneoplastic syndromes related to soft tissue tumors are very uncommon and an association of paraneoplastic diseases with a malignant fibrous histiocytoma (MFH) has not been reported so far. Opsoclonus-myoclonus is a rare paraneoplastic nervous system syndrome, that was well documented in adult patients with neoplasms particularly of the lung and breast. A 77-year-old woman developed typical opsoclonus-myoclonus syndrome and rapidly progressed to coma. The opsoclonus and generalized myoclonus continued until the patient's death seven months later. An autopsy examination identified a pleomorphic MFH in the retroperitoneum without a metastatic spread. Microscopic examination of the cerebellum revealed an atrophy of the granular layer along with a marked patchy loss of Purkinje cells, and a loss of neurons in the cerebellar nuclei accompanied by distinct peridental demyelination and astrogliosis. A moderate loss of neurons and neuronal chromatolysis were observed also in the inferior olivary nuclei. However, the omnipause neurons of the nucleus raphe interpositus, that are supposed to be responsible for opsoclonus generation under pathological conditions, as well as ocular brain stem nuclei were all intact. We failed to prove the presence of known antineuronal anti-Yo, anti-Hu or anti-Ri autoantibodies in both serum and cerebrospinal fluid (CSF) of the patient. However, the confirmation of the intrathecal IgG synthesis by oligoclonal bands in CSF and the immunohistochemical detection of IgG deposits on membranes of Purkinje cells, neurons of cerebellar granular layer and in cerebellar nuclei in our case support the presumed autoimmune nature of the disease.

10 citations


Journal Article
TL;DR: A rare case of solitary metastasis of follicular carcinoma of the thyroid gland into the petroclival region in a 58-year-old woman and the primary focus of carcinoma within the thyroid is demonstrated.
Abstract: We describe a rare case of solitary metastasis of follicular carcinoma of the thyroid gland into the petroclival region in a 58-year-old woman. The metastasis was the first and only manifestation of the disease. The histology of the tumor, differential diagnosis and clinical course are discussed. In a few similar cases described so far the tumor was always a well or moderately differentiated follicular carcinoma located in the petroclival region. As in this presentation, these cases also clinically mimic a meningioma. The differential diagnosis includes adenoma of the thyroid gland and thyroid gland dystopia. We demonstrated the primary focus of carcinoma within the thyroid after its detailed examination initiated by our finding. The clinical outcome of such rare cases is usually excellent, much better than in metastases of papillary carcinoma into the brain.

10 citations


Journal Article
TL;DR: Testing a series of commercially accessible antibodies against melanoma-associated antigens revealed it to be a favourable supplement for the bioptical or cytological diagnosis of malignant melanoma in case the traditional/conventional combination of S-100 protein and HMB-45 antibody fails.
Abstract: In certain primary and metastatic malignant melanomas diagnostic problems may arise due to their cytologic features and/or absence of synthesis of melanin. As the "classic" combination of S-100 protein and HMB-45 may occasionally fail to stain cells of malignant melanoma, we have tested a series of commercially accessible antibodies which were so far not compared by other authors in the three most frequent subtypes of this tumor. In surgical specimens from 104 cutaneous malignant melanomas (40 nodular melanomas, 46 superficially spreading malignant melanomas and 18 lentigo maligna melanomas) the staining intensity and the proportion of neoplastic cells stained with antibodies to S-100 protein, HMB-45, NKI/C3, NKI/beteb, MART 1 (Melan A), KBA 62 and Mitf was semiquantitatively analysed. The use of this group of antibodies against melanoma-associated antigens revealed it to be a favourable supplement for the bioptical or cytological diagnosis of malignant melanoma in case the traditional/conventional combination of S-100 protein and HMB-45 antibody fails. According to the authors' experience the antibody against KBA 62 has shown to be the most effective antibody followed by the antibodies against MART-1 (Melan A) and NKI/C3.

9 citations


Journal Article
TL;DR: Results show that GIS has some features that are more typical for neurofibroma than for conventional schwannoma, and in surgical pathology practice, a finding of intratumoral axons and positivity for claudin-1 and especially for EMA should not preclude diagnosis of GIS.
Abstract: Gastrointestinal schwannoma (GIS) is a distinctive and extremely rare lesion showing some differences from conventional soft tissue schwannoma and some similarities with common soft tissue neurofibroma. Soft tissue neurofibromas and schwannomas differ by contents of specific types of nerve sheath cells, such as Schwann cells, perineurial cells and CD34+ cells. To compare GIS with these soft tissue lesions, eight cases of typical GIS were studied immunohistochemically to evaluate their nerve sheath cell types. Epithelial membrane antigen (EMA) and claudin-1 as perineurial cell markers, and neurofilament protein as a marker for intratumoral axons were used. In addition, the tumors were stained for cytokeratin, CD117 (C-KIT), alpha-muscle specific actin, S100 protein and CD34. EMA- and claudin-positive cells were seen in 2 (25%) and one lesion, respectively, thus resembling cellular composition of neurofibroma. Intratumoral neurofilament protein positive axons otherwise typical of neurofibroma were found inside 4 tumors (50%). CD34 positivity was found in 6 tumors (75%) and often revealed a diffuse pattern as seen in neurofibroma and not a zonal pattern as described in schwannomas. These results show that GIS has some features that are more typical for neurofibroma than for conventional schwannoma. In surgical pathology practice, a finding of intratumoral axons and positivity for claudin-1 and especially for EMA should not preclude diagnosis of GIS.

8 citations


Journal Article
TL;DR: Platinum based chemotherapy is effective in the management of children and adolescents with mixed germ cell tumors of the ovary and Chemosensitivity of these tumours allows most girls to have conservative surgery with possible preservation of reproductive function.
Abstract: Mixed germ cell tumours of the ovary are rare malignant neoplasms containing combinations of two or more types of germ cell elements. The aim of the study was to review biopsy examinations, medical records, treatment strategy, follow-up and outcome of all girls treated for mixed germ cell tumour of the ovary at the Department of Pediatric Oncology, University Hospital Motol during the period 1979-2002. Archival slides of all tumours were reviewed and tumours were classified according to the WHO system. The clinical data on surgical treatment, chemotherapy and radiotherapy used and follow-up information were obtained in all girls. The staging was reviewed retrospectively on the basis of surgical and pathological findings and results of imaging investigations, and it was outlined according to the most recent FIGO criteria and TNM classification. Sixteen girls with mixed germ cell tumour of the ovary, age range 3 years 11 months to 17 years 8 months (median 12 years) were treated. All girls presented with unilateral tumour of the ovary and all underwent surgery as an initial treatment. The most common presenting symptom was abdominal pain, occurring in ten patients. The original diagnosis of mixed histology was confirmed in all cases; in five cases the tumour contained three histologic components, in eleven cases the tumour consisted of two germ cell types. All tumours contained elements of yolk sac tumour, followed by immature teratoma, embryonal carcinoma, dysgerminoma and mature teratoma. At the time of diagnosis three patients had stage I disease, four patients stage II, seven stage III and two stage IV disease. All patients were treated with chemotherapy after surgery, predominantly with platinum-based regimens (PVB, BEP). Three patients treated initially with MAC (metothrexate, dactinomycin, cyclophosphamide) were diagnosed in the early eighties. In seven girls with advanced disease treated in the early years, radiotherapy was administered to the pelvis or whole abdomen. Overall survival and event-free survival were 80% and 81.3% respectively (median follow-up time 7.6 years). Three patients have died from the disease, two progressed on treatment (MAC), one girl relapsed three months after finishing therapy, no further therapy was administered. One girl underwent resection of tumour of her remaining ovary 24 months after original diagnosis. Histology showed mixed serous and mucinous cystadenoma. The latest examinations revealed that all other patients were in good health. Microscopic examination should be extensive and careful to find out all types of malignant germ cell elements. Platinum based chemotherapy is effective in the management of children and adolescents with mixed germ cell tumors of the ovary. Chemosensitivity of these tumours allows most girls to have conservative surgery with possible preservation of reproductive function.

6 citations


Journal Article
TL;DR: The clinicopathologic and immunohistochemical findings of three oncocytic tumors of the adrenal cortex are discussed, which demonstrated focal positivity with antibodies to neuron-specific enolase and synaptophysin and mostly focally weak positivity for cytokeratins.
Abstract: Oncocytic neoplasms of the adrenal gland are rare, as described in literature. Only 27 cases have been reported up to now in world literature. Here we describe our experience. In this report we discuss the clinicopathologic and immunohistochemical findings of three oncocytic tumors of the adrenal cortex. Two tumors were found during examination of the patients for other reasons. These tumors were hormonally inactive. One tumor manifested by the virilization of the patient. Immunohistochemical examination demonstrated in all tumors focal positivity with antibodies to neuron-specific enolase and synaptophysin and mostly focally weak positivity for cytokeratins. Very low mitotic activity was found in two tumors. Criteria for evaluation of biological character of this type of tumors are not established.

6 citations


Journal Article
TL;DR: Human thyroid peroxidase (hTPO) is a membrane protein with a key role in the thyroid hormones synthesis and is of limited value in the diagnosis of thyroid malignancy.
Abstract: Human thyroid peroxidase (hTPO) is a membrane protein with a key role in the thyroid hormones synthesis. Loss of hTPO was described in malignant tumours of the thyroid gland. hTPO was tested as a marker of malignancy. Immunohistochemical study of hTPO in 321 thyroid lesions (45 malignant tumours, 72 benign tumours, 199 benign non-tumours lesions, and 5 normal thyroid glands) is presented. The sensitivity of hTPO in predicting malignancy in thyroid is 64%, and the specificity is 87%. Thus, hTPO is of limited value in the diagnosis of thyroid malignancy. The authors discuss the role of hTPO as a marker of differentiation.

4 citations


Journal Article
TL;DR: Two cases of solitary fibrous tumor of the pleura with features of malignancy are described, one macroscopically circumscribed and the other characteristically "patternless" on low power examination.
Abstract: Two cases of solitary fibrous tumor of the pleura with features of malignancy are described. In the first case, the tumor macroscopically showed noncircumscribed growth. Microscopically, on low power examination, the tumor was characteristically "patternless", with alternation of cellular areas and hypocellular, prominently collagenized areas. There was an infiltrative growth present at the margins. Cytological atypias were not present. In the second case, the tumor was macroscopically circumscribed. Microscopically, on low power examination, the tumor had characteristical "patternless" appearance again. Pleomorphic cells with high mitotic activity dominated in cellular areas on high power examination. The infiltrative pattern of growth was not present at the margins. Both tumors were classified as malignant solitary fibrous tumors of the pleura, or fibrosarcomas of the pleura. The criteria of malignancy for solitary fibrous tumor are discussed.

2 citations


Journal Article
TL;DR: Reactive angioendotheliomatosis is presented in a 46-year-old male patient with a history of alcoholism and pulmonary tuberculosis and the suggested clinical diagnosis was KS, which was confirmed by surgical pathologists.
Abstract: Reactive angioendotheliomatosis (RAE) is a rare disorder with clinical presentation of multiple cutaneous lesions. RAE mimics mainly Kaposi sarcoma (KS) and, rarely angiosarcoma, and it usually arises in association with other systemic disease. The associated disease is mostly chronic infection. RAE presents diagnostic problems for both the clinician and pathologist. We present RAE in a 46-year-old male patient with a history of alcoholism and pulmonary tuberculosis. Multiple, rapidly progressive, itching and burning, and focally confluent cutaneous erythematous and purple macules, plaques and nodules of various size and wide distribution appeared during a course of antituberculotic therapy. The suggested clinical diagnosis was KS. The lesions were repeatedly evaluated by biopsy. Histologically, there were poorly marginated proliferations of capillaries in the dermis which extended focally into the subcutaneous tissue. Associated erythrocyte extravasations, stromal hemosiderin depositions and mild chronic inflammatory infiltrates were also present in the lesion. Adnexal and neuronal extension of the lesional vessels was an intriguing finding, not described in the literature until now. The microscopical findings were interpreted by surgical pathologists as capillary hemangioma and as "angioblastoma of Nakagawa" (one and two times, respectively).

2 citations


Journal Article
TL;DR: This very rare case demonstrates the importance of careful examination of all spindle cell lesions of the kidney and the most important step within differential diagnosis is distinguishing of sarcomatoid differentiation in renal cell carcinoma.
Abstract: Authors report clinicopathological features of an unusual case of composite renal lesion occuring in 32-year-old Caucasian male. The patient was followed for cystic lesion of retroperitoneal-renal region for 5 years. He was indicated for resection of the cystic lesion because of changes of the retroperitoneal mass on CT scan. A cyst was located on upper renal pole. A huge cystic mass filled mainly by necrotic material was resected and submitted for histological examination. The wall of the cyst was composed of fibrous tissue, indistinguishable from inflammatory pseudotumor on histological level. The vital intracystic tissue was formed by well-differentiated papillary renal cell carcinoma. The most important step within differential diagnosis is distinguishing of sarcomatoid differentiation in renal cell carcinoma. This very rare case demonstrates the importance of careful examination of all spindle cell lesions of the kidney.

2 citations


Journal Article
TL;DR: The view that the gastric cardiac mucosa is not a physiological structure but that it results from glandular metaplasia of the distal esophageal mucosa due to gastroesophageaal reflux is supported.
Abstract: The development of the esophageal and gastric mucosa in the gastroesophageal junction was studied in 61 fetuses of 13-41 weeks of the gestational age. During the 13th-15th week, the esophageal multilayered epithelium was covered by a continuous layer of columnar mucous ciliated cells which were present only focally till the 25th week and disappeared later. Before the 15th week, the gastric mucosa was formed by pits only. The glands started as proliferating tubules in the basal parts of the pits in the 15th week. Further, they differentiated into oxyntic glands. The mucosa of the corpus was fully developed in the 27th week. The cardiac mucosa was absent in all the 10 fetuses examined between the 27th and 41st week of gestation. This supports the view that the gastric cardiac mucosa is not a physiological structure but that it results from glandular metaplasia of the distal esophageal mucosa due to gastroesophageal reflux.

Journal Article
TL;DR: The authors described immunohistological and molecular genetic findings in series of 21 tumours with spindle and epithelioid cells histology of the stomach, finding that the tumour could be classified as a transitional form between leiomyoma and GIST.
Abstract: The authors described immunohistological and molecular genetic findings in series of 21 tumours with spindle and epithelioid cells histology of the stomach. In 18 cases the tumours were KIT (CD117) positive and the diagnosis of gastrointestinal stromal tumour (GIST) was confirmed. Three cases were KIT (CD117) negative. According to additional immunohistological markers (desmin and smooth muscle actin positivity) two of them were categorized as leiomyomas. The immunohistological profile of the third case showed that the tumour could be classified as a transitional form between leiomyoma and GIST. All but one KIT (CD117) positive tumours were also CD34 positive. In other three KIT (CD117) positive cases up to 10% of CD34 positive cells were found. Desmin was negative in KIT (CD117) positive cases. S100 protein was positive in three KIT (CD117) positive cases ranging from single cells to 10% of cells. Nine tumours were NSE positive. In our study the connection between proliferation factors (Ki67 and PCNA) and the mitotic index was not established. Risk factors were identified based on the size of the tumours and the mitotic index. Very low and low risk of aggressive behaviour included 12 cases, intermediate risk category 5 cases, high risk category 4 cases. For molecular genetic examination, DNA was extracted from formalin-fixed, paraffin-embedded tissues. Exon 11 was analyzed by SSCP (single-strand conformational polymorphism analysis) with following sequencing. Deletion was found in 7 cases, point mutation in one case, silent point mutation in one case and in two cases the examination could not be detected. In 10 cases (47%) a "wild type" was found. We suggest that other exons, e.g. 9, 13, 17, (which were not examined) and genes than KIT gene could also trigger tyrosine-kinase activity.


Journal Article
TL;DR: A unique case of congenital muscular dystrophy simulating a juvenile polymyositis in a muscle biopsy is presented, and a profound reduction of alpha-dystroglycan and less pronounced secondary deficiency of alpha 2-laminin were found.
Abstract: The congenital muscular dystrophies (CMD, MDC) represent a heterogeneous group of autosomal recessive disorders manifesting in infancy by muscle weakness and hypotonia. Approximately 40% of patients with CMD have a primary deficiency of the laminin alpha 3. chain of merosin (laminin-2) due to mutations in LAMA2 gene. Laminin-2 bound to alpha-dystroglycan forms a link between actin--associated cytoskeletal proteins and the components of extracellular matrix. Disruption of this axis is responsible for several forms of muscular dystrophy. A unique case of congenital muscular dystrophy simulating a juvenile polymyositis in a muscle biopsy is presented. A profound reduction of alpha-dystroglycan and less pronounced secondary deficiency of alpha 2-laminin were found. All known forms of CMD were excluded, and the disorder was diagnosed as so far undescribed form of CMD. The mutation in a gene encoding the protein, that seems to play a role in a glycosylation of alpha-dystroglycan, is presumed.

Journal Article
TL;DR: A case ofColumnar cell carcinoma of the thyroid gland is described in 69-year-old man and clinical and histological features of this tumour in fine-needle aspirates are presented.
Abstract: Columnar cell carcinoma of the thyroid gland is a very rare malignant tumour with characteristic histological features. The tumour is characterized by typical aggressive behaviour and unfavourable prognosis. We describe a case of this carcinoma in 69-year-old man. We present clinical and histological features of this tumour. In addition, we present features of this tumour in fine-needle aspirates, which were described only in a few cases in literature so far.

Journal Article
TL;DR: A new method for assessment of proliferative activity of plasma cells, which can be used on archived formalin-fixed, paraffin embedded material is presented.
Abstract: The assessment of proliferative activity of plasma cells in multiple myeloma has prognostic significance. The percentage of the proliferating plasma cells (plasma cell labelling index--PCLI) at the time of the diagnosis correlates with survival, and is increased during transition from the plateau phase to the relapse. Flow cytometry and immunofluorescence examination of bone marrow aspirates are used for the assessment of the PCLI. This study describes a method for evaluation of PCLI in formalin-fixed, paraffin embedded material. Bone marrow biopsies from 31 patients with relapsing (n = 10) and newly diagnosed (n = 21) multiple myeloma were examined by double immunohistochemical staining: anti-CD 138 (Syndecan 1) and anti--Ki-67. The percentage of plasma cells positive for Ki-67 were counted by an image analysis system. New cases of multiple myeloma showed 0.7 to 12.7% positivity of Ki-67 labelled plasma cells (median 4.75), the relapsing cases showed 4.4 to 22.4% positivity (median 7.75). Statistic analysis revealed prognostic significance (p = 0.046). This study presents a new method for assessment of proliferative activity of plasma cells, which can be used on archived formalin-fixed, paraffin embedded material.

Journal Article
TL;DR: The method of composing large images from individual parts was used for digitizing the skin lymphoma collection of the Institute of Dermatology, University Hospital, Zürich, and gave good results even in images with background or holes in the tissue.
Abstract: Composing microscopic images of very high resolution from several parts posed some problems. One of them was the necessity to adjust the focusing level when moving from one part to another. Re-focusing lead to problems with joining the image parts, which did not correspond exactly, and the area of image fusion was noticeable. A computer program was developed to overcome these problems. Our program worked with all the image parts together to find their optimal order for image fusion. Individual image parts were joined using a steep gradient running along a randomly generated curve. This method gave good results even in images with background or holes in the tissue. The method of composing large images from individual parts was used for digitizing the skin lymphoma collection of the Institute of Dermatology, University Hospital, Zurich. This collection of digital images is a part of the 6th version of Hypertext atlas of Dermatopathology at www.muni.cz/atlases.

Journal Article
TL;DR: In the opinion, apoplexia uteri is caused by the state of permanent hypoperfusion leading to passive hyperaemia, and it is related to the degree of the arterial stenosis.
Abstract: This is a detailed histological autopsy study of 47 cases of macroscopically recognisable flat intraendometrial haemorrhage into the atrophic lining of the uterine cavity. The average age of the studied women was 71 years. The majority of the deceased patients (61%) had suffered from various cardiovascular diseases or acute abdomen; the rest had disseminated carcinoma, chronic lung, kidney or liver diseases. The most common cause of death was cardiovascular failure (68%), followed by respiratory failure, cerebrovascular accident and renal or liver failure. We have observed serious vascular changes in other organs in 22 cases (46%), many of these affected the gastrointestinal tract. The histological examination has always showed congestion of the endometrium and myometrium. In 38 cases there was also marked haemorrhage into the endometrial stroma which occasionally extended into the myometrium. The intensity of the bleeding resembled a haemorrhagic infarction in several instances. The myometrial arteries exhibited a variable degree of atherosclerosis with narrowing of their lumen. In our opinion, apoplexia uteri is caused by the state of permanent hypoperfusion leading to passive hyperaemia, and it is related to the degree of the arterial stenosis.