Germ cell tumor associated primitive neuroectodermal tumors

Purpose: This retrospective review was done to assess the prognosis and res ponse in patients presenting with primitive neuroectodermal tumor admired w ith germ cell tumor. Materials and Methods: Of the 40 patients treated at our institution from 1 984 to 1999, 15 had initial stage I and 25 had initial metastatic disease, Median followup after the diagnosis was 25 months (range 4 to 142). Results, Of the 40 patients 15 presented with clinical stage I disease, inc luding 9 treated with retroperitoneal lymph node dissection and 6 who elect ed surveillance. Seven of the 9 patients had normal lymph nodes and all con tinuously had no evidence of disease, Two of the 9 patients had lymph nodes involved with teratoma with or without primitive neuroectodermal tumor. Re troperitoneal relapse in 5 of the 6 patients on surveillance was treated wi th cisplatin based chemotherapy followed by post-chemotherapy retroperitone al lymph node dissection, Residual primitive neuroectodermal tumor was note d in 4 of the 5 patients and only 3 of 6 are currently without disease at a median followup Of 17 months (range 15 to 69). A total of 25 patients pres ented with metastatic disease, of whom 23 underwent cisplatin based chemoth erapy. Only 3 patients achieved complete remission with chemotherapy alone and 2 of the 3 subsequently relapsed. Of the remaining 20 patients 16 under went post-chemotherapy retroperitoneal lymph node dissection including 11 w ith primitive neuroectodermal tumor in the resected specimen. Two of these 11 patients have continuously had no evidence of disease, while an addition al 3 currently have no evidence of disease after further therapy, Teratoma was present in the resected specimen in 5 of 16 patients, of whom 2 have co ntinuously had no evidence of disease, while an additional 2 currently have no evidence of disease after further surgical resection. Therefore, 11 of 25 patients who presented with metastatic disease currently have no evidenc e of disease at a median followup of 19 months (range 2 to 111). Conclusions: Primitive neuroectodermal tumor in the orchiectomy specimen ha s adverse prognostic significance, This condition in the retroperitoneum is potentially curable by retroperitoneal lymph node dissection but rarely er adicated by chemotherapy, Therefore, we recommend retroperitoneal lymph nod e dissection for all clinical stage I cases with primitive neuroectodermal tumor in the orchiectomy specimen. Patients who present with metastatic pri mitive neuroectodermal tumor should be treated aggressively with surgical r esection as an integral part of the therapeutic strategy.