Retroperitoneal Malignant Schwannoma. A Case Report

Abstract

The isolated retroperitoneal malignant schwannoma unassociated with Von 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 immunohistochemical examinations, to differentiate it from other isolated retroperitoneal sarcomatous neoplasms. The authors report a case of retroperitoneal malignant schwannoma 20 cm in diameter in a 62-year-old woman surgically treated by radical exeresis. Postoperative complications were absent, and the patient, discharged from the hospital on the 12th postoperative day, died 8 months later of diffuse metastases, without local relapse. Despite the patient's short survival, the authors believe radical surgery to be the best therapeutic choice. Only surgery can establish a final diagnosis and can offer the best chance of survival and a significant and sometimes prolonged relief of symptomatology.

Hemorrhagic, calcified, and ossified benign retroperitoneal schwannoma

“…[8] Malignant schwannomas are very rare and usually associated with von Recklinghausen disease. [9] Most schwannomas are benign and show either monosomy 22 or loss of 22q material. [10] They usually affect adult patients aged 20 to 50 years [11] and arise from almost anywhere in the body, while head, neck, and extremities are the most common sites.…”

Analysis of 82 Cases of Retroperitoneal Schwannoma

“…Hayasaka et al reported that MRI of benign schwannomas typically shows hypointensity on T 1 ‐weighted images and hyperintensity on T 2 ‐weighted images, although this was noted in only 57% of their cases 2 . A definitive diagnosis is based on pathological and histological results and the tumours were strongly positive for S‐100 protein 11 …”

A Case of an Incidentally Detected Retroperitoneal Schwannoma: Current Approaches to Diagnosis and Treatment of Schwannoma

“…While USG showed a hypoechoic mass with uniformly limited and minimal heterogeneous internal echoes in imaging, because of the low vascularity of the schwannomas, hypodense, well-defined mass is seen with contrast-enhanced CT (6). The definitive diagnosis can only be made if a strong S-100 positivity is detected after histopathological evaluation (7). In addition, the differentiation of benign or malignant schwannoma is important and necessary to determine the follow-up and treatment strategy.…”