Primary primitive neuroectodermal tumor of the kidney.

Renal primitive neuroectodermal tumor (PNET) is a rare entity. It should be differentiated from other primary renal malignancies. The differentiation of the malignant small round cell tumors may be challenging; however, it is essential for better management of the patient. We report a 45-year-old male having complaints of pain in the abdomen, hematuria, and mass in the abdomen for 6 months. Abdominal and pelvic sonography finding showed cystic-solid, right renal mass suggestive of malignancy. On histopathology diagnosed as malignant small round blue cell tumor suggestive of primitive neuroectodermal tumor. On immunohistochemistry, CD99 positivity confirmed the diagnosis of primary PNET of the kidney. We are presenting this case for its rarity, clinical presentation, and pathological findings.

High-grade myxofibrosarcoma-presented as a large mass of right upper arm.

Myxofibrosarcoma is one of the rare soft tissue sarcomas. We present a case of a 65-year-old male having large soft tissue mass over right upper arm associated with surface ulceration. On histopathological study tumor was diagnosed as myxofibrosarcoma - high grade according to modified FNCLCC grading system. Like many other tumors of connective tissue, soft tissue sarcoma exhibits high recurrence. In our case, tumor showed features of high grade with local recurrence, large size; however, no evidence of metastasis was noted. For this unpredictable clinical behavior, we are presenting this case.

Complete Pentalogy of Cantrell (POC) with Phocomelia and Other Associated Rare Anomalies.

We are reporting a rare case of Complete Pentalogy of Cantrell (CPOC) with phocomelia and other associated anomalies such as encephalocoele, craniofacial defects, limb defects and a flexion deformity, with club foot in right lower limb. Antenatal ultrasonography done in a 20 year old primigravida revealed multiple thoraco-abdominal and CNS anomalies in a foetus with an average gestational age of 18.2 weeks. Foetal autopsy done following termination of the pregnancy revealed a combination of defects, based on which the diagnosis of Complete Pentalogy of Cantrell with associated anomalies was given. To the best of our knowledge, this is the first case of Complete Pentalogy of Cantrell with phocomelia which has been seen in the world.

Adrenocortical carcinoma presenting as a rupture and extensive retroperitoneal haemorrhage.

Adrenocortical carcinoma (ACC) is an extremely rare tumour. We are reporting a 45-year-male patient who had a history of sudden severe worsening epigastric pain and fullness in abdomen, with giddiness. The radiological investigations showed a large right suprarenal mass with extensive destruction and retroperitoneal haemorrhage, with extra capsular, periportal and liver metastases. Exploratory laparotomy was done for excisions of mass and surrounding tissue. On histopathological examination, diagnosis was given as Adreno Cortical Carcinoma with capsular, vascular, and soft tissue nodular involvement.

Huge dedifferentiated liposarcoma of the left thigh with a high grade fibrosarcomatous differentiation and a local recurrence.

Dedifferentiated liposarcoma is one of the variants of liposarcoma which has a more aggressive course. It constitutes less than 10% of all the liposarcomas and is often found in the retroperitoneum and the mediastinum. We are reporting a rare case of a 60 years old female who presented with a huge, soft tissue mass in the left thigh with a past history of an operation which was done15 years back. The histopathological examination showed a well differentiated liposarcoma with a high grade fibrosarcomatous differentiation. Immunohistochemical studies confirmed the dedifferentiated liposarcoma with the high grade fibrosarcomatous differentiation. To the best of our knowledge, this is the 11th case in the literature with rare findings.