RESUMEN
Ovarian Sertoli Leydig cell tumors (SLCT) accounts for less than 0.5% of all ovarian malignancies. The incidence of primary extra-ovarian SLCT is extremely rare with reported cases occurring in young adult women till now. We report case of primary retroperitoneal extra-ovarian SLCT in a seven-year girl child without any hormonal manifestation. She presented with complaint of left side abdominal swelling associated with intermittent pain for a duration of six months. CT scan revealed a huge retroperitoneal space-occupying lesion abutting the dorsal vertebrae and present posterior to pancreas, spleen and left kidney. The tumor was diagnosed as extraovarian Sertoli Leydig cell tumor with intermediate differentiation on histopathology and immunohistochemistry.
RESUMEN
Retroperitoneal liposarcoma is an uncommon malignant mesenchymal tumour of adipocytic differentiation. Giant retroperitoneal liposarcoma is a rare entity which achieves such a large tumour bulk without any significant clinical symptoms due to deep seated location of the tumour. Though incidence of distant metastasis is less but local recurrence is fairly common. Surgical management is the key of management in retroperitoneal liposarcoma in spite of its large size and local invasion to vital organs. We are reporting a case of giant retroperitoneal liposarcoma of 27.5 Kg in a 45 year old female patient.
RESUMEN
Extrarenal Wilms' tumor is a very exceptional tumor which is defined as Wilms' tumor found anywhere other than in the kidneys. Until now less than 60 cases have been reported in the English literature. The tumor can be located in the retroperitoneum, inguinal canal, uterus, cervix, testes, skin and even in the thorax. The diagnosis is almost always made after surgical intervention. Distant metastasis of this tumor has only been reported in the case of a 6-year-old girl who developed Wilms' tumor in the inguinal canal with lung involvement. In this paper we report a case of extrarenal Wilms' tumor in retroperitoneum with distant metastasis to lungs, ribs and pelvic bone in a 6- year old male. The tumor was successfully removed without tumor cell spillage. The patient was diagnosed as favorable histologic group, in which the tumor consists of epithelial, blastemal and mesenchymal components without teratomatous elements. He received combination chemotherapy according to NWTS-IV guidelines and radiation to involved lungs, and has been alive and well for the last 6 months without severe complication or relapse.