Melanotic schwannoma is a rare form of pigmented neural tumor commonly arising from the posterior spinal nerves and ganglia. Two variants have been described, psammomatous and nonpsammomatous. 50% of psammomatous tumors are associated with Carney complex. The biologic behavior of the tumor is difficult to predict and slightly over 10% of the tumors follow malignant course. We present a case of psammomatous melanotic schwannoma as part of Carney complex in a 67-year-old male. Clinical examination revealed oral mucosal and abdominal skin pigmentation. Magnetic resonance imaging showed an intradural extramedullary lesion at D8-D12 level. Intraoperative squash smear study showed sheets of spindle cells with abundant intracytoplasmic melanin pigmentation and few psammoma bodies. Based on clinical, radiologic, and histopathological findings with immunohistochemistry correlation a final diagnosis of psammomatous melanotic schwannoma was rendered.
Gliosarcoma (GS) is a variant of IDH wild type of glioblastoma (WHO grade IV). It is characterized by biphasic tissue pattern showing glial and mesenchymal components. The glial component is usually similar to glioblastoma and the sarcomatous component is usually spindle cell type. Rarely the sarcomatous component can show additional lines of mesenchymal differentiation like cartilage, bone, smooth muscle, skeletal muscles etc. These tumours are more firm and discrete because of the high content of reticulin and collagen in the sarcomatous component. Originally these tumours were thought to arise from sarcomatous transformation of proliferating blood vessels or dedifferentiation of an advanced glioma. The recent studies show that both glial and sarcomatous component show identical mutations in p53, PTEN and TERT genes and suggest a monoclonal origin from a common stem cell. There is no significant difference in prognosis between glioblastoma and gliosarcoma. Here we report a case of gliosarcoma with chondrosarcomatous differentiation in a 52 year old gentleman. The case is presented here for its rarity.
Angiomyolipomas are benign mesenchymal neoplasms consisting of variable proportion of mature adipose tissue, thick walled blood vessels and smooth muscle bundles. Although initially thought to be hamartomas, now they are considered to be tumours derived from perivascular epithelioid cells(PEC) hence grouped under a family of tumours known as PEComas. The tumour mainly involves kidneys and are more common in patients with tuberous sclerosis. However extrerenal angiomyolipomas are being increasingly described with liver and retroperitoneum being the most common extrarenal sites. Other sites described include nasal cavity, oral cavity, colon, lung, skin, adrenal glands and bladder. Here we report two cases of extrarenal angiomyolipomas of paratesticular and subcutaneous region, the rarely described sites in the literature. There were no clinical signs of tuberous sclerosis in both cases. Although literature suggests that these tumours show immunohistochemical coexpression of myogenic and melanocytic markers with consistent expression of HMB-45, our both cases were negative for HMB-45. However both cases showed classical triphasic morphology. Although rare, it is important to screen these patients for tuberous sclerosis associated lesions for early diagnosis.
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