The isolated retroperitoneal malignant schwannoma unassociated with Vo
n Recklinghausen's disease is an unusual neoplasm, representing 0.01%
of all retroperitoneal malignant neoplasms, with a poor prognosis, and
an average survival at 5 years of 50% in patients treated by radical
exeresis. At present, it is impossible, without histologic and immunoh
istochemical examinations, to differentiate it from other isolated ret
roperitoneal sarcomatous neoplasms. The authors report a case of retro
peritoneal malignant schwannoma 20 cm in diameter in a 62-year-old wom
an surgically treated by radical exeresis. Postoperative complications
were absent, and the patient, discharged from the hospital on the 12t
h postoperative day, died 8 months later of diffuse metastases, withou
th local relapse. Despite the patient's short survival, the authors be
lieve radical surgery to be the best therapeutic choice. Only surgery
can establish a final diagnosis and can offer the best chance of survi
val and a significant and sometimes prolonged relief of symptomatology
.