Malignant peripheral nerve sheath tumor of adrenal gland with heterologus osseous differentiation in a case of Von Recklinghausen's disease.

Malignant peripheral nerve sheath tumor (MPNST) of the adrenal gland is extremely rare. Most of them occur in association with neurofi bromatosis, ganglioneuroma or as part of a composite tumor such as pheochromocytoma. Only seven cases of MPNST of the adrenal gland have been reported in the literature till date. Discriminating this entity from other soft tissue sarcomas and gastrointestinal stromal tumor of the adrenal gland has important diagnostic and therapeutic implications. Moreover, the tumor size and pattern of expression for certain immunohistochemical markers may serve as independent predictors of aggressiveness. Herein we present a 24-years-old male with features of Von Recklinghausen’s disease who presented with large left adrenal gland malignant peripheral nerve sheath tumor.

Embryonal sarcoma of liver: an undifferentiated rare primary solid and mucoid to cystic tumour.

Undifferentiated (embryonal) sarcoma of the liver is a rare primary malignant tumor of the liver occurring almost exclusively in childhood with characterisitic findings on gross and microscopy. Careful sampling of the tumor shows various immature undifferentiated elements and infiltration of the tumor into the surrounding capsule. CT findings could be suggestive of a hamartoma, as in our case. We report a case of hepatic embryonal sarcoma in a ten-year old male child presenting with an abdominal mass.

Aggressive angiomyxoma presenting as a vulval polyp.

Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor, occurring predominantly in the pelvic-perineal region of adults and carries a high risk for local relapse and hence the need to differentiate it from the other mesenchymal tumors occurring in this region. Presentation as a pedunculated polyp, like in our case, is unusual for this rare tumor. Except for positive surgical margins, there are no clinical or histological means for predicting the tumor recurrence. A diligent long-term follow-up is mandatory. Though rare, this tumor needs to be considered in the differential diagnosis of vulval polyps.