Primary intracranial leiomyosarcoma in an immunocompetent patient: case report
We report a case of intracranial leiomyosarcoma (LMS) arising after resection of neurofibroma at the cerebellopontine angle. A 45-year-old immunocompetent woman presented with recurrence of a tumor 9 years after resection performed in another hospital. Magnetic resonance imaging demonstrated a heterogeneously enhancing, dura-based mass at the left cerebellopontine angle. The tumor was subtotally removed via lateral suboccipital craniotomy. LMS was diagnosed based on histological and immunohistochemical findings. Postoperatively, although the patient was treated using local radiotherapy, she died due to rapid regrowth of the tumor. Reevaluation of the specimen obtained in the first operation led to a diagnosis of neurofibroma. Both LMS and neurofibroma rarely occur intracranially. LMS is generally thought to arise from smooth muscle cells of the blood vessels or pluripotent mesenchymal cells. In this case, LMS might also have originated from smooth muscle cells of the vessels in the neurofibroma, possibly associated with mechanical and/or heat stimulation during the previous surgery.
KeywordsCerebellopontine angle Leiomyosarcoma Neurofibroma Smooth muscle cell
The authors wish to thank Dr. Takanori Hirose of Tokushima Prefectural Central Hospital for offering the immunohistochemical photomicrograph of h-caldesmon.
The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article.
- 6.Scheithauer BW, Louis DN, Hunter S, Woodruff JM, Antonescu CR (2007) Neurofibroma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO Classification of Tumours of the Central Nervous System, 4th edn. WHO, Lyon, France, pp 156–157Google Scholar