- See:
Soft Tumor Menu:
- Discussion:
- also called malignant schwannoma or
fibrosarcoma of nerve sheath;
- extracompartmental high grade tumor that develops from sheaths (Schwann cells) of
peripheral nerves;
- usually is a primary lesion occuring along proximal major nerve trunks,
- tumor is usually asymptomatic until it causes neuropraxia;
- gross inspection reveals a large fusiform mass;
- rather than being displaced by tumor, associated nerve enters proximal pole of the tumor and exits the distal pole;
-
associated conditions:
-
neurofibromatosis:
- neurosarcoma occassionally develops as secondary transformation of
neurofibromatosis;
- hence, look for the physical exam and radiographic characteristics of this disorder;
- prognosis for patients with primary neurosarcoma is more favorable than for those with malignant transformation of neurofibromatosis;
- often the transition to malignancy is heralded by new onset neurologic deficit;
- Histology:
- fibrillar pattern is loose & have with increased cellularity;
- multiple mitotic figures, & wavy eosinophilic pattern of neural tissue with comma shaped nuclei;
- in an undifferentiated lesion, electron microscopic examination is needed to distinguish it from
fibrosarcoma.
- Treatment:
- wide excision w/ wide margin, if possible;
- otherwise radical ampuatation is considered;
Malignant peripheral nerve sheath tumors: A clinicopathologic study of 120 cases.
BS Ducatman et al.
Cancer. Vol 57. 1987. p 2006-2021.
Survival Data for Patients with Malignant Schwannoma.
