Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Extraskeletal osteosarcoma is a rare, highly malignant, osteoid formation mesenchymal neoplasm in the absence of bone involvement, associated with exceptionally poor prognosis. It frequently arises in the soft tissues of the extremities or in the retroperitoneum, but rarely in visceral organ. We describe a primary osteosarcoma of the liver in a 70-year-old man who presented with an episode of fever, accompanied by abdominal discomfort, after an accident abdominal strike. Ultrasonography and computed tomography revealed a large heterogeneous mass with areas of dense calcification involving most of the right lobe of liver. Radiography did not show evidence of primary tumor or primary bone lesion at any other site. Histologically, the tumor showed an essentially similar appearance as osteosarcoma originating in the skeleton, comprised of polygonal or spindle shaped cells, along with abundant eosinophilic lace-like osteoids, or irregularly arranged bone trabeculae. Immunohistochemistry showed that the tumor cells were positive for vimentin, CD10, and focally for SMA and CD56, but negative for other lineage-specific markers. Thus, the findings favored a primary hepatic osteosarcoma. This patient received palliative chemotherapy to ease the signs of his sickness due to the large size of the tumor and he died 4 moths later.
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PMID:Primary Osteosarcoma of the Liver: Case Report and Literature Review. 3035 50

Rarely, salivary gland tumors such as mucoepidermoid carcinoma, mammary analogue secretory carcinoma and mucinous carcinoma arise as primary tumors from ectopic or metaplastic salivary gland tissue adjacent to or within the thyroid gland. We report for the first time a case of primary salivary acinic cell carcinoma (AcCC) adjacent to the thyroid gland in a 71-year-old female patient with Crohns disease and a previous history of malignant melanoma. Following the development of a nodule adjacent to the left thyroid lobe, a fine-needle aspiration biopsy was reported as consistent with a follicular lesion of undetermined significance (Bethesda III). A left-sided hemithyroidectomy was performed. A circumscribed lesion measuring 33 mm was noted adjacent to the thyroid and trapping parathyroid, it was composed of solid nests and glands with microcystic and follicular patterns. The tumor was negative for thyroid, parathyroid and paraganglioma markers, but positive for pan-cytokeratins, CK7, CD10, CD117, androgen receptor and HNF-beta. A metastasis of a thyroid-like renal cell carcinoma was suspected but ruled out, and the patient had no evident lesions on extensive radiology of the urogenital, pulmonary and GI tracts. Based on the morphology, a diagnosis of AcCC was suggested, and confirmed with DOG1 and PAS-diastase staining. Molecular analyses pinpointed a constitutional ASXL1 variant of uncertain significance, but no fusion events. The patient had no radiological or clinical evidence of parotid, submandibular or sublingual tumors postoperatively, and the excised lesion was therefore assumed to be a primary tumor. We here detail the morphological and immunophenotypic profile of this previously undescribed perithyroidal tumor.
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PMID:Perithyroidal Salivary Gland Acinic Cell Carcinoma: Morphological and Molecular Attributes of a Unique Lesion. 3251 64


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