Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The production and detailed immunostaining properties of a new rat monoclonal antibody (ICR.2) to epithelial membrane antigen are reported. The antibody was selected for its ability to compete with the polyclonal antiserum (M7), used in the original immunohistological studies, in order that it might serve as a direct replacement in diagnosing epithelial tumours. Most of the staining reactions on normal tissues were identical to those previously reported with M7 but there were some important differences. They included: positivity of renal and adrenal capsular fibroblasts, perineurium, some myoepithelial and smooth muscle cells, occasional osteoblasts and squamous and thyroid follicular epithelium in the normal state. The intercellular canaliculi of sweat glands and secretory canaliculi of gastric oxyntic cells were clearly demonstrated. These staining reactions could be obtained with M7 when a sensitive detection system was used although the results were usually weak and inconsistent. Nearly all adenosquamous and transitional carcinomas were positive. The remaining tumours fell into three major groups: (1) those which were consistently or nearly consistently negative--melanoma, seminoma, rhabdomyosarcoma, alveolar soft part sarcoma, adrenal cortical carcinoma, granulocytic sarcoma, paraganglioma, non-Hodgkin's lymphoma. Hodgkin's disease and embryonal carcinoma: (2) those which were either negative or positive with distinctive patterns of staining--basal cell carcinoma, embryonal tumours: and (3) non-epithelial tumours that were consistently positive--epithelioid sarcoma, synovial sarcoma, osteosarcoma, chordoma and myeloma--or positive in a significant minority of cases--leiomyosarcoma, malignant fibrous histiocytoma, clear cell sarcoma of tendon sheath, various neuroectodermal tumours.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detailed investigation of the diagnostic value in tumour histopathology of ICR.2, a new monoclonal antibody to epithelial membrane antigen. 169 88

Identification of growth factors and receptors in mesenchymal tumors may be crucial to understanding of growth regulation in sarcomas. During an immunohistochemical study of the expression of growth factors and receptors in human soft tissue tumors (STT), only 1 antisera capable of working in paraffin-embedded tissue was noted. A detailed study of 141 STT was undertaken to determine the frequency of expression of nerve growth factor receptor (NGF-R), its specificity and sensitivity for neural tumors, and the effect of fixation on detection. In normal mesenchymal tissue, only nerve sheath and perivascular staining was seen. No immunoreactivity was seen in many tumors including rhabdomyosarcoma, angiosarcoma, liposarcoma, Ewing's sarcoma, and alveolar soft part sarcoma. Less than 15% of tumors of smooth muscle, fibrous, or fibrohistiocytic origin showed immunoreactivity, usually focal. In contrast, a high frequency of immunoreactivity was noted in tumors of neural origin (74%). This included granular cell tumors (100%), Schwannoma/neurofibroma (91%), malignant Schwannoma (78%), neuroblastoma/neuroepithelioma (60%), and paraganglioma (57%). A high rate of reactivity was also seen in synovial sarcomas (80%), undifferentiated sarcomas (60%), and hemangiopericytomas (43%), suggesting a potential relationship to the neural phenotype. Among the neural tumors, Bouin's fixation was superior to formalin, suggesting that immunoreactivity for NGF-R is affected by fixation. This antibody may be a useful adjunct marker diagnostically.
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PMID:Expression of nerve growth factor receptor in paraffin-embedded soft tissue tumors. 245 20

Nonchromaffin paraganglioma (NCP), also called glomus body tumor or chemodectoma, is rarely found in the orbit. The behavior of orbital nonchromaffin paraganglioma may potentially be more aggressive than in other head and neck locations. Diagnosis depends on electron microscopic demonstration of membrane-bound neurosecretory granules. Results of histopathologic study show a well-circumscribed lesion without a true capsule with alveolar or organoid arrangements of epithelioid cells within a reticulin framework with thin-walled blood vessels. Cells are polygonal with round or oval nuclei containing rare mitotic figures and pale-staining cytoplasm. Differential diagnosis includes alveolar soft-part sarcoma, alveolar rhabdomyosarcoma, neuroblastoma, carcinoid, and granular cell tumor. Of 29 previously reported cases of orbital NCP, 16 have been reclassified as alveolar soft-part sarcoma. The authors report a patient with an electron microscopically established orbital NCP, with the history of a contralateral glomus jugulare tumor irradiated 14 years previously.
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PMID:Orbital nonchromaffin paraganglioma. A case report and review of the literature. 255 86

22 undifferentiated tumours of the Salivary Gland Register (University of Hamburg) were studied by conventional light microscopical and immunocytochemical methods to elucidate the heterogeneity of this tumour group. The following observations were made in this collective: 18 tumours displayed one or more markers for the epithelial character and were classified as carcinomas. 10 carcinomas were considered as primary ones and 8 were considered as secondary ones (metastatic or invasive "per continuitatem"). Primary carcinomas were subclassified as poorly differentiated variants of a distinctive type of salivary gland tumours, as follows: 6 cases of carcinoma in pleomorphic adenoma, and one case each of mucoepidermoid tumour, adenocarcinoma, salivary duct carcinoma and epidermoid carcinoma. Secondary carcinomas were subclassified as follows: 3 epidermoid carcinomas, 3 nasopharyngeal carcinomas and 2 bronchial carcinomas. One tumour positive for S-100 protein and NSE (Neuron-specific enolase) was classified as a metastatic melanoma. Another tumour positive for vimentin and actin was classified as a rhabdomyosarcoma of the periglandular tissue. Two tumours lacked any markers studied here and were regarded as a malignant paraganglioma and an undifferentiated lymphoma, respectively. The differential diagnosis of the undifferentiated tumours of salivary glands and the special problems of this tumour group are discussed.
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PMID:Undifferentiated tumours of salivary glands. Immunocytochemical investigations and differential diagnosis of 22 cases. 303 6

A case is described of a child presenting with an extremely large orbital tumour, which had an alveolar pattern on histological examination. The differential diagnosis, which included alveolar rhabdomyosarcoma, alveolar soft-part sarcoma and non-chromaffin paraganglioma, could only be made on electron microscopic examination. Exact diagnosis was important because of prognostic and therapeutic implications. The presence of neurosecretory granules in tumour cells was demonstrated on electron microscopy, confirming the diagnosis on non-chromaffin paraganglioma, a benign tumour which is extremely rare in this site.
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PMID:Paraganglioma of the orbit - an exercise in diagnosis. A case report. 626 55

This is a clinicopathologic study of 17 cases of alveolar soft-part sarcoma of the orbit. The mean age of patients was 23 and the median was 18 (range, 11 months to 69 years); 13 patients (76.5 per cent) were women and four were men (ratio, 3.25:1). The right and left orbits were equally involved (eight patients each), and in one the laterality was not specified. Histologically, the tumors had a distinctive organoid pattern outlined by thin-walled capillaries and were composed of nests of large polyhedral cells with abundant, finely granular, acidophilic cytoplasm. Approximately two thirds of the tumors had diagnostic PAS-positive diastase-resistant crystalline structures. Histologically, the differential diagnosis included nonchromaffin paraganglioma, granular cell tumor, metastatic renal cell carcinoma, vascular tumor, alveolar rhabdomyosarcoma, and amelanotic malignant melanoma. Electron microscopic studies of one tumor disclosed mitochondria with abnormal cristae, increased amounts of glycogen, and cytoplasmic crystalline structures with a periodicity of 8 to 10 nm. Smaller membrane-bound electron-dense granules appeared to be precursors of the crystals. Follow-up studies showed that eight patients were alive and well (median follow-up period, 11.4 years). Six of the eight patients at the time of diagnosis were 20 years of age or younger. A ninth patient was alive and well 13 years after excision of the orbital mass and four years after bilateral thoracotomy with resection of nine pulmonary nodules. Two patients died as a result of metastatic disease, one 14 years and the other 21 years after initial orbital surgery. Two patients died of other causes, one of whom had pulmonary metastases at autopsy. The follow-up period on two recent cases was less than three years, and two patients were lost to follow-up. The disease pursued an indolent clinical course. Surgery offers the best chance to control the disease.
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PMID:Alveolar soft-part sarcoma of the orbit: a clinicopathologic analysis of seventeen cases and a review of the literature. 707 38

Only about 2% of the urinary tract are not of urothelial origin. Our knowledge of their morphology and biology is mainly based on single case reports, and therefore apart from a few exceptions very poor. Generally, the most often affected site is the urinary bladder (79.2%), followed by the urethra (12.7%), pelvis (4.9%) and ureter (3.2%). The urinary bladder also is the only organ in which all different histological types of these tumors were described. According to their histogenesis non-urothelial tumors (NUT) can be classified by the following main groups: soft tissue tumors, mixed epithelial and non epithelial tumors (carcinosarcomas), neuroendocrine carcinomas, carcinoids, malignant lymphomas, malignant melanomas and extragonadal germ cell tumors. Moreover some very interesting tumor-like lesions, like malakoplakia and inflammatory pseudosarcoma, mainly occur in this region. About 75% of all NUT of the urinary tract belong to the soft tissue tumors. Rhabdomyosarcomas in children and leiomyomas and -myosarcomas in adults are the more frequent histological types. Leiomyosarcomas can easily be confused with other tumor types or even with inflammatory pseudotumors. The use of immunohistochemistry to achieve a correct diagnosis is mandatory but not always successful. A relatively frequent tumor occurring in the bladder of young adults is the paraganglioma (pheochromocytoma), which usually produces typical symptoms of catecholamine excess. Carcinosarcomas of the urinary bladder contain both epithelial and mesenchymal components. They have to be distinguished from collision tumors (coexistent but separate carcinoma and sarcoma), spindle cell transitional carcinomas as well as from carcinomas with osseous or cartilaginous metaplasia. Carcinoids and neuroendocrine carcinomas developed from the neuroendocrine cells scattered all over the transitional epithelium of the bladder. Neuroendocrine carcinomas of the bladder are also called "oat cell carcinomas" since they show the same histological features and immunoreactivity as the oat cell carcinomas of the lung. They share also the same poor prognosis. The affection of the urinary tract in generalized malignant lymphomas and leukemias occur in more than 30% of cases. Lymphomas, primarily localised in the urinary bladder are, however, extremely rare. The most frequent ones are low grade non Hodgkin lymphomas, although 3 cases of Hodgkin disease and a few cases of primary extramedullary plasmacytoma of the bladder have been reported, too.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Non-urothelial tumors of the urinary tract]. 751 Dec 78

Paraganglioma (extra-adrenal pheochromocytoma) is a rare tumor, particularly in childhood. Those in the female genital tract are exceptionally rare, with only 9 cases reported in detail since 1926. All were seen in adults and only two arose in the vagina. This study examined the incidence of this tumor in the childhood population of the West Midlands region of the United Kingdom since 1957 and found 4 cases: 2 abdominal, 1 para-aortic, and 1 carotid body. A recent additional case is reported in the vagina of a child aged 11 years, who presented with heavy vaginal bleeding in the absence of hypertension. Initial diagnosis suggested rhabdomyosarcoma and near complete excision was carried out. Since this is the first such case to be described in a child, the outcome can only be assumed. Although histopathological examination suggested the benign nature of the tumor, implying that surgical excision was sufficient treatment, close follow-up is recommended.
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PMID:Paraganglioma of the vagina: the first report of a rare tumor in a child. 984 49

We report two cases of digestive/intra-abdominal PEComa. The first lesion developed in the caecum of a 36-year-old woman, the second in the pararectal region of a 35-year-old woman. The first tumor was formed from spindle cells arranged in fascicles, the second contained predominantly epithelioid cells with prominent nucleoli. Immunohistochemically, tumor cells expressed smooth muscle actin and melanocyte markers (HMB45), S-100 protein and CD117 were negative. Based on the morphologic aspect and, above all, on the immunohistochemical study the diagnosis of PEComa was retained for both lesions. In the gastrointestinal tract, the principal differential diagnoses of PEComas are gastrointestinal stromal tumors, particularly the round cell/epithelioid subtype, and metastases of carcinoma and melanoma. Other differential diagnoses include rhabdomyosarcoma, paraganglioma, leiomyosarcoma, and clear cell sarcoma.
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PMID:Digestive PEComas: a solution when the diagnosis fails to "fit". 1561 43

Modern imaging techniques have great importance in the diagnosis and therapy of skull-base pathologies. Many of these lesions, especially in relation to their specific location, can be evaluated using CT and MR imaging. Tumors commonly found in the anterior skull base include carcinoma, rhabdomyosarcoma, esthesioneuroblastoma and meningioma. In the central cranial fossa, nasopharyngeal carcinoma, metastases, meningioma, pituitary adenoma and neurinoma have to be considered. The most common neoplasms of the posterior skull base, including the CP angle, are neurinoma, meningioma, nasopharyngeal carcinoma, chordoma and paraganglioma. One major task of imaging is the evaluation of the exact tumor extent as well as its relationship to the neighboring neurovascular structures. The purpose of this review is to recapitulate the most important anatomical landmarks of the skull base. The typical imaging findings of the most common tumors involving the skull base are also presented.
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PMID:[Skull base tumors]. 1609 41


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