Successful management strategy for giant primary hepatic rhabdomyosarcoma in 15-year-old male: a case report.

Primary hepatic rhabdomyosarcoma is an exceedingly rare type of sarcomas. A 15-year-old boy was admitted to the hospital with abdominal pain. Serial investigation showed a giant heterogeneous mass, with a diameter of 15 cm, located in the right lobe of the liver (segment 4a, 4b, 5, 6, 7, 8), with clear margins, non-homogeneous density in computerized tomography and the Positron Emission Tomography Scan Multi Slice revealed a peripherally slightly metabolically active hepatic mass (205 × 134 × 208 mm). Histopathological examination and the Immunohistochemical showed embryonal rhabdomyosarcoma. The management strategy involved multiple cycles of ifosfamide and doxorubicin and monitoring the tumor size, until the tumor size was suitable for the surgery. The management of hepatic rhabdomyosarcoma is a surgical challenge due to the late onset of symptoms, a limited number of reported cases, and poor prognosis. Surgical excision is the treatment of choice for primary hepatic rhabdomyosarcoma and combination between chemotherapy, surgery showed good results.

Liver Transplant for Primary Hyperoxaluria Type 1: Results of Sequential, Combined Liver and Kidney, and Preemptive Liver Transplant.

OBJECTIVES: Primary hyperoxaluria type 1 is an autosomal recessive disorder that causes overproduction and urinary excretion of oxalate. Liver transplant has been suggested as a treatment for primary hyperoxaluria type 1 since the defective enzyme is expressed in the liver. This study aimed to investigate results of combined liver and kidney, sequential, and preemptive livertransplantin patients with primary hyperoxaluria type 1. MATERIALS AND METHODS: In this cohort study, we followed patients with primary hyperoxaluria type 1 who underwent liver transplant at our centerin Shiraz, Iran. Clinical and laboratory data of patients were gathered, and major outcomes, including renal failure after liver transplant, rejection, and mortality were recorded. Survival of patients was analyzed by the Kaplan-Meier method. RESULTS: Our study included 24 patients. There were 16 male (66.6%) and 8 female (33.33%) patients. Thirteen patients were in the pediatric age group (age < 18 y), and 11 patients were adults (age ≥ 18 y). Thirteen patients underwent sequential transplant, 8 patients underwent combined liver and kidney transplant, and 3 patients underwent preemptive transplant. All patients received organs from deceased donors. There were no statistically significant differences in mortality, rejection, and hemodialysis after transplant between those with sequential transplant and those with combined liver and kidney transplant (P > .05). CONCLUSIONS: Liver transplant can be considered a treatment for patients with primary hyperoxaluria type 1. Combined liver and kidney transplant and preemptive liver transplant could be proper options for these patients.