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.
Rosai–Dorfman disease (RDD) is a rare, nonmalignant disorder of histiocyte proliferation which usually involves the cervical lymph nodes. Isolated extranodal RDD is rare which mimics other neoplastic processes thus causing diagnostic difficulties to the clinicians and radiologists. Here we report a case of 32 year old lady who presented with swelling in right thigh, clinically diagnosed as soft tissue neoplasm. Histopathology revealed pathognomonic findings of RDD showing sheets of large pale histiocytes showing emperipolesis along with dense lymphplasmacytic infiltrate. In extranodal RDD the typical histological findings of RDD are less common and there is pronounced fibrosis compared to nodal disease. The pathological diagnosis may be challenging in such cases. The surgical pathologist should have a high index of suspicion in such cases so that aggressive therapy can be avoided. The course of extranodal disease is generally less indolent and can be aggressive if vital organs are involved.
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