Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1261473 (sarcoma)
 25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Five cases of alveolar soft part sarcoma, 5 cases of granular cell tumor, and 6 cases of paraganglioma were investigated immunohistochemically to examine the expression of tissue-specific intermediate filaments (cytokeratin, vimentin, desmin, and glial fibrillary acidic protein (GFAP], actin, myoglobin, and nervous tissue markers (S-100 protein, neuron-specific enolase, and Leu-7). In alveolar soft part sarcomas, some of the tumor cells were positive for desmin, but negative for nervous tissue markers. The tumor cells of granular cell tumors were stained with anti-S-100 protein antibody, but not with anti-neuron-specific enolase antibody. In contrast, the tumor cells of paragangliomas were positive for neuron-specific enolase, but not for S-100 protein except for stellate cells surrounding the tumor cell nests. This immunohistochemical approach was valuable for the differential diagnosis of these three tumors. Furthermore, the complete absence of cytokeratin in all of the tumor cells may be helpful in distinguishing these three tumors from metastatic carcinoma in soft tissue. The histogenesis of alveolar soft part sarcoma is a matter of controversy. The result that besides desmin actin was also demonstrated in some of the tumor cells may support the myogenic origin of this tumor.
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PMID:Alveolar soft part sarcoma, granular cell tumor, and paraganglioma. An immunohistochemical comparative study. 353 89

We report an unusual case of renal sarcoma in a young adult. Histological examination demonstrated many similarities to the histopathological features of clear cell sarcoma of the kidney. Immunohistochemically, none of the intrinsic tumor cells showed positive staining with the antibodies against the intermediate filament proteins, epithelial membrane antigen, S100 protein, neuron-specific enolase, Leu-7 or myoglobin. The clinical course of this tumor was that of high grade malignancy, resulting in death with generalized metastases 13 months after tumor resection.
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PMID:Unusual renal sarcoma in a young adult: its similarities to clear cell sarcoma of the kidney. 356 Mar 36

Rhabdoid sarcoma is a tumor of unknown etiology that usually occurs in the kidneys of infants and small children. We report an adolescent with a rhabdoid sarcoma of the chest wall. In addition to the patient's age and the site of the tumor, other unusual features of this case were as follows: positive staining of tumor cells with neuron-specific enolase, the presence of chronic, active, hepatitis that apparently developed coincident with the sarcoma, and the presence of widespread hemosiderosis. Two of the patient's siblings died in infancy with degenerative neurologic disease, hepatomegaly, and multiple congenital anomalies. The histochemical findings and family history lend support to previous suggestions that some rhabdoid sarcomas may be of neural crest origin and may be heritable lesions.
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PMID:Extrarenal rhabdoid sarcoma. 374 41

To date, the histogenesis of alveolar soft part sarcoma has been considered to be of paraganglioma origin, striated muscle cell origin, or as a malignant granular cell myoblastoma, neural neoplasm, or renin-producing tumor. Further studies for these existing theories were performed based on various methods. The negative formaldehyde-induced fluorescence and the immunohistochemical absence of neuron-specific enolase were against the paraganglioma theory. The immunohistochemical absence of myelin proteins (P2 protein and PO protein) and S-100 protein were against the malignant granular cell myoblastoma and neural neoplasm theory. Furthermore, there was a totally negative immunohistochemical finding for renin in the tumor cells, and the biochemical relationship of the tumor and renin was completely negated. The contradiction in a well-known report that the components of crystals were considered to be Z-band materials such as tropomyosin was referred to based on recent myological data. Concurrently, the absence of tropomyosin was immunohistochemically demonstrated. Hence, the issues on the histogenesis of alveolar soft part sarcoma and the identity of the characteristic crystalloids remain open for discussion.
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PMID:Alveolar soft-part sarcoma. A review on its histogenesis and further studies based on electron microscopy, immunohistochemistry, and biochemistry. 619 31

Macroscopic stomach tumors induced in Sprague-Dawley rats during two chronic bioassays with the acetanilide herbicide butachlor at a dietary concentration of 3000 ppm, were evaluated histologically and immunohistochemically in order to determine their identity and pathogenesis. The tumors, which occurred primarily in female rats, were a heterogeneous series, including a few consisting wholly or partly of classic solid or anaplastic epithelium, but with the majority containing diffusely distributed primitive neoplastic cells. The latter had either the general appearance of undifferentiated epithelium or presented a more "mesenchyme-like" pattern where the cells were epithelioid, blastema-like, neuroendocrine-like or sarcoma-like with fascicular disposition. Gastric glandular profiles were also present, usually located near the periphery of the tumors, but in some cases extending into the diffuse tumor tissue. Most of the tumors displayed variable immunohistochemical reactivity for cytokeratin, vimentin and neuron-specific enolase but were negative for muscle-specific actin or desmin except in the stromal tracts. Detailed examination of all available gastric tissue revealed the presence of additional microscopic neoplasms and precursor hyperplastic lesions. All of these were typical gastric neuroendocrine cell lesions (gastric carcinoids) originating in the fundic mucosa but occasionally invading submucosally, and consisting of epithelial cells in organized clusters, rosettes or primitive tubules. The enterochromaffin-like (ECL) nature of these microscopic neoplasms and precursor lesions was substantiated by strong immunohistochemical reactivity for cytokeratin, neuron-specific enolase and chromogranin A, and a negative reaction for vimentin. One microscopic tumor showed a transition from differentiated neuroendocrine type in the fundic mucosa to a dispersed "mesenchyme-like" pattern in the submucosal extension. An additional finding in the butachlor-treated male and female rats was atrophy of the fundic mucosa involving, in particular, reduction in the numbers of parietal cells. This effect was dose-related, being most severe in the high-dose (3000 ppm) females. On the basis of their morphological characteristics, coupled with the continuity evident in the microscopic lesions, it is concluded that the macroscopic stomach tumors associated with the dietary administration of butachlor are poorly differentiated gastric carcinoids, in some cases admixed with a non-neuroendocrine epithelial element. Fundic ECL and stem cells are known to be under the trophic influence of gastrin, which is apparently responsible for the induction of the tumors associated with butachlor administration. Gastric tumor development involving gastrin is recognized as a secondary, hormonal mechanism of carcinogenesis, demonstrating a dose-threshold phenomenon.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Identity and pathogenesis of stomach tumors in Sprague-Dawley rats associated with the dietary administration of butachlor. 758 Jan 13

Four cases of chondroid syringoma containing large numbers of hyaline or plasmacytoid cells are described. Three cases occurred in the hand and one in the foot. Hyaline cells are commonly seen in mixed tumours and myoepitheliomas of salivary glands and rarely in chondroid syringomas. The hyaline-cell change in three of the cases initially caused diagnostic difficulties and the possibility of sarcoma was raised in two cases. In addition to the characteristic hyaline cells, the presence of tubulo-glandulo-ductal structures, benign squamous epithelium and myxochondroid stroma aided diagnosis. Immunohistochemically, the hyaline cells exhibited positivity for vimentin, cytokeratin, S-100 protein, carcino-embryonic antigen, focal glial fibrillary acidic protein (3 cases), neuron-specific enolase (3 cases) and focal alpha-smooth muscle actin (2 cases). Occasional cells were Ber EP4 positive (2 cases). In some cells, a striking peripheral ring-like positivity for cytokeratin and S-100 protein was noted. Ultrastructurally, desmosomes, varying numbers of tonofibrils and non-bundling intermediate filaments were seen. Scanty fine filaments with vague focal densities were detected in some cells. Our studies suggest that the hyaline cells represent modified epithelial as well as myoepithelial cells. One of our cases also exhibited collagenous spherulosis.
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PMID:Chondroid syringoma with hyaline cell change. 768 53

A case of low-grade fibromyxoid sarcoma in the thigh of a 21 year old female is described. The patient had a fist-sized well-defined mass in her left thigh that enlarged over a 6 month period. Histologically, the neoplasm showed contrasting fibrous and myxoid areas with a swirling growth pattern. Cellularity was low to moderate, and the stromal cells were benign looking without mitoses or nuclear pleomorphism. The tissue was not noticeably vascular. Some stromal cells were aggregated around the blood vessels. The stromal cells were immunoreactive to vimentin, but were negative to keratin, desmin, alpha-smooth muscle actin, actin HHF35, S-100 protein, neuron-specific enolase, and epithelial membrane antigen. Ultrastructural examinations of the stromal cells revealed well-developed rough endoplasmic reticulum, mitochondria, pinocytotic vesicles, and numerous intermediate-sized filaments in the cytoplasm. These findings seem to indicate that the stromal cells were fibroblastic in origin. The occurrence of the tumor in a young adult, its location and its large, well defined borders together with the characteristics revealed through histological investigation, indicated that it was in fact what has been termed by Evans as a low-grade fibromyxoid sarcoma.
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PMID:A case of low-grade fibromyxoid sarcoma of the thigh. 783 81

Gastrointestinal autonomic nerve (GAN) tumors, also known as plexosarcomas, are a rare distinct subtype of the gastrointestinal stromal tumors. These tumors are usually histologically low-grade, epithelioid or spindle-cell neoplasms that can be distinguished from the other gastrointestinal stromal tumors on the basis of their unique ultrastructural features. A 66-year-old female presented with a histologically high-grade sarcoma of the small bowel. Ultrastructural studies showed features of a GAN tumor. The light microscopic and ultrastructural features are described. The tumor cells gave strong, diffuse staining for vimentin and synaptophysin, and weak focal staining for neuron-specific enolase and S100. While usually presenting as low-grade neoplasms on histologic examination, this case demonstrates that GAN tumors should be considered in the differential diagnosis of a histologically high-grade sarcoma of the gastrointestinal tract, especially when evidence of smooth muscle, peripheral nerve sheath, or neuroblastic origin is not forthcoming.
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PMID:Gastrointestinal autonomic nerve tumor presenting as high-grade sarcoma. Case report and review of the literature. 808 18

We report a case of Askin's tumor, primitive neuroectodermal tumor (PNET) in the chest wall, with review of reported cases of PNET. Our case was the ninth of Askin's tumor and the 36th of PNET in Japan. A 12-year-old girl was hospitalized because of a large right intrathoracic mass. The serum neuron-specific enolase (NSE) was 11.4 ng/ml, slightly increased, and the urinary excretion of catecholamine metabolites was normal. The needle biopsy specimens were diagnosed as small round cell sarcoma. At surgery we found an encapsulated multilobular mass in the region. The chest wall was resected partially to extirpate it completely. Its macroscopic and microscopic features were almost similar to the description of Askin et al. The immunohistochemical staining of the cells was positive for NSE, strongly suggesting the neurogenic origin. After surgery, intensive chemotherapy with autologous bone marrow transplant was performed, and she has been living without sign of recurrence for two years.
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PMID:[A case of primitive neuroectodermal tumor in the chest wall ("Askin's tumor")]. 817 3

A 64-year-old black man had a primary alveolar soft part sarcoma of the tongue. It is unusual for this rare tumor to develop in this site in the elderly age group. The patient survived nearly 3 years with disease. When the patient expired, a complete autopsy was performed. The biopsy material of the primary tumor showed characteristic light microscopic appearance, intracellular periodic acid-Schiff positive inclusions after diastase reaction, and electron microscopic presence of crystals. Immunohistochemistry for neuron-specific enolase was positive. The autopsy confirmed widespread visceral and lymphatic metastases. The atypical features of this case, including the site of the primary lesion, age of the patient at diagnosis, immunohistochemistry of the tumor, and natural history of this neoplasm, suggest that alveolar soft part sarcoma may behave differently when arising in an extraskeletal site, and could possibly have a different histogenesis from the typical alveolar soft part sarcoma found in a younger population.
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PMID:Primary alveolar soft part sarcoma of the tongue of an elderly man. A case report and review of the literature. 835 Nov 25


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