Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literaturePerivascular epithelioid cell tumors (PEComas) include different morphological entities originating from perivascular epitheliod cells. Their clinical behavior is not predictable, and there are no strict histologic criteria for malignancy, although larger tumors with infiltrative growth, hypercellularity, cellular atypia, atypical mitoses, and necrosis generally have a malignant course. Pediatric PEComas are rare, with less than 40 cases reported, mostly in children older than 5 years. We describe a case of malignant PEComa of the ligamentum teres in a 2-year-old girl, characterized by the occurrence of local relapse after primary treatment with chemotherapy and surgery and poor response to imatinib mesilate and temsirolimus used after further analyses confirmed p70S6K expression involved in the mTOR pathway.
Management of unresectable solid papillary cystic tumor of the pancreas. A case report and literature reviewPancreatic solid papillary cystic tumor is a rare neoplasm with an excellent prognosis if surgical excision is complete. We report on a case and review 47 more cases extracted from the published literature to assess the treatment options when solid papillary cystic tumor is considered unresectable. Chemotherapy and radiotherapy were beneficial in a limited number of patients, but therapeutic decisions must be made bearing in mind that patients may be long-term survivors without any treatment because of the tumor's slow growth.
An unusual case of female hypospadias associated with a pelvic massFemale hypospadias is a rare malformation. We report on an unusual case that presented with a urethral dimple in the perineum in association with a pelvic soft tissue sarcoma compressing and displacing the urethra. The diagnosis was made by cystoscopy, and, as the patients remained asymptomatic, no treatment was eventually required for her urologic malformation. This is, to our knowledge, the first reported case of female hypospadias associated with a perineal urethral dimple. The possible pathogenesis of the malformation is discussed also in relation to the simultaneous presence of a pelvic mass.