Mycosis fungoides: cutaneous T-cell lymphoma.

Mycosis fungoides (MF) is the most common group of cutaneous T-cell lymphomas. It is a rare non-Hodgkin’s lymphoma of mature, skin-homing, clonal, malignant T lymphocytes, usually observed in mid to late adulthood, that initially presents in the skin as patches, plaques, tumors, or generalized erythema (erythroderma) and can involve the lymph nodes and peripheral blood. In this review, we survey the MF literature of the last decade and highlight the major trends.

Stewart-Treves syndrome: a rare entity.

Stewart-Treves syndrome is a rare, deadly cutaneous angiosarcoma that develops in long - standing chronic lymphedema. Most commonly, this tumor is a result of lymphedema induced by radical mastectomy to treat breast cancer. Stewart-Treves syndrome occurs in 0.5% of patients, who survive mastectomy for more than 5 years. The mean age at appearance of the angiosarcoma is 62 years, and the mean interval between mastectomy and the appearance of the tumor is 10.5 years. The exact pathogenesis is not known so far. On histopathology, vascular channels infiltrate the normal structures in a disorganized fashion, as if trying to line every available tissue space with a layer of endothelial cells. The prognosis is poor as it is very aggressive. Only early diagnosis and prompt radical excision with or without adjuvant radiotherapy, can promote survival.

Hydroa vacciniforme: a very rare photodermatosis.

Hydroa Vacciniforme (HV) is a rare, acquired and chronic paediatric disorder that is characterized by photosensitivity and recurrent crops of skin lesions on sun-exposed skin, such as the face, ears and hands that heal with vacciniforme scarring. The pathogenesis of HV is unknown. No chromosome abnormality has been identified so far. HV patients have no abnormal laboratory results. The histopathologic features are distinctive and demonstrate intraepidermal multilocular vesicles and cellular necrosis. Most cases remit spontaneously by late adolescence.

Reactive perforating collagenosis.

Reactive Perforating Collagenosis (RPC) is a rare disease of elimination of altered collagen through the epidermis. The disease exists in childhood form with autosomal recessive mode of inheritance and an adult form acquired in association with diseases such as Diabetes Mellitus (DM), Chronic Renal Failure (CRF), hypothyroidism, lymphoma, hyperparathyroidism, neurodermatitis, AIDS, pulmonary fibrosis, scabies and herpes zoster infection.

Giant primary mesenteric liposarcoma: a rare case report.

Primary mesenteric liposarcoma is a rare malignant tumor of mesenchymal origin. These tumors are often found to be of substantial size upon first clinical presentation. We report a case of primary mesenteric liposarcoma in a 55 year old man who underwent laparotomy for the clinical/radiological impression of a mesenteric cyst. FNAC was inconclusive due to suboptimal cellularity. On laparotomy there were 2 masses (diameter- 19 and 14 cms) weighing approximately 8.5 kilograms together. Histopathology showed features of atypical lipomatous tumor / well differentiated liposarcoma (ALT/WDLS) with mixed histological pattern (e.g. lipoma like, sclerosing and myxoid areas) varying from area to area. Prognosis of ALT/WD liposarcoma depends upon its anatomic location and most aggressive histological subtype. This case is being presented because of its huge dimensions, rarity of the site and mixed histological pattern.