Presacral Schwannoma in a Patient with von Recklinghausens Disease: Case Report

• Published in: The Japanese Journal of Gastroenterological Surgery Vol. 35; no. 4; pp. 436 - 439
• Main Authors: Ishikawa, Nami, Iwata, Takako, Matoba, Naoyuki, Kameoka, Nobuhisa, Watanabe, Masato, Satoh, Kazuhiro, Masuda, Hiroshi, Fujii, Terumasa
• Format: Journal Article
• Language: Japanese
• Published: The Japanese Society of Gastroenterological Surgery 2002
• Subjects: neurofibromatosis 1; presacral tumor; schwannoma
• ISSN: 0386-9768; 1348-9372
• DOI: 10.5833/jjgs.35.436

A 53-year-old woman admitted with presacral tumor confused initially with ovarian cystoma was diagnosed with von Recklinghausen's disease based on physical findings, and an elastic hard mass was palpable on the posterior wall of the rectum. CT scan and MRI showed a solid cystic tumor 7 cm in diameter in the presacral space. Angiography showed a hypervascular mass fed by the middle and left lateral sacral arteries. We suspected the tumor was a malignant peripheral nerve sheath tumor, and extirpated it. The specimen was a 8×6×5 cm yellowish solid cystic tumor weighing 130g. The histopathological diagnosis was schwannoma. Presacral schwannoma in a patient with von Recklinghausen's disease is quite rare, and has not, to our knowledge, been reported in the literature except for 1 case.