RESUMEN

We herein report the case of a 2-year-old girl with neurofibromatosis type 1 (NF1), who presented with a 12-cm mass in the right retroperitoneum and underwent tumor resection. Histologically, the tumor was composed of two distinct components: one was teratoma, showing mature morphology; and the other was embryonal rhabdomyosarcoma. An interphase fluorescence in situ hybridization (FISH) analysis of the rhabdomyosarcoma component revealed the absence of isochromosome 12p. Although it is well known that rhabdomyosarcoma occurs in infantile NF1, and that rhabdomyosarcoma can arise from teratoma as a somatic-type malignancy, to the best of our knowledge, this is the first case of an infantile NF1 patient, who developed rhabdomyosarcoma within a retroperitoneal teratoma. The absence of chromosome 12p alteration suggests a possibility that the rhabdomyosarcoma occurred due to the NF1 background, not as a somatic-type malignancy of germ cell tumor.

Asunto(s)

Neoplasias Primarias Múltiples/patología , Neurofibromatosis 1/patología , Neoplasias Retroperitoneales/patología , Rabdomiosarcoma Embrionario/patología , Teratoma/patología , Preescolar , Femenino , Humanos , Neoplasias Primarias Múltiples/diagnóstico , Neurofibromatosis 1/diagnóstico , Neoplasias Retroperitoneales/diagnóstico , Rabdomiosarcoma Embrionario/diagnóstico , Teratoma/diagnóstico

RESUMEN

Retmperitoneal ganglioneuroma is a rare neurogenic benign tumor.The prognosis of patients was good when the tumor was completely resected,while the surgical procedure is complicated.In March of 2013,a male patient with complex retroperitoneal ganglioneuroma was treated at the First Affiliated Hospital of Chongqing Medical University.A hypoechoic solid lesion (size,6.5 cm ×4.5 cm) adjacent to the head of the pancreas was detected by color Doppler ultra-sonography 9 months ago,and no any other clinical symptoms were detected.Perioperative abdominal computed tomography and the surgery confirmed that the tumor (size,8.5 cm × 7.5 cm × 4.5 cm) was located beneath the pancreas,encompassing thc ccliac artery,hepatic artery,splenic artery and superior mesenteric artery,surrounding the head and uncinate process of the pancreas,making it impossible to be separated.The tumor was closely connected with the portal vein,superior mesenteric vein,splenic vein and left renal vein.The tumor was separated from the major blood vessels,the body and tail of the pancreas,while the tumor could not be resected from the pancreatic head,and thus tumor resection and pancreaticoduodenectomy were performed.The surgery was extremely diffcult,but the complete removal of tumor was successfully achieved without excision of the major blood vessels and the patient recovered well.