Glandular peripheral nerve sheath tumors

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Abstract
Peripheral nerve tumors (PNT) containing glands are uncommon. Which types of PNT contain glands is a matter of controversy, a factor bearing on the prognosis of these tumors. The authors reviewed the files of 11 patients with glandular PNT seen in their laboratory and 27 patients reported in the literature. The 11 instances of glandular PNT seen in their laboratory affected male and female patients equally; patients ages ranged from 8 to 68 years (mean, 28 years). Six patients had neurofibromatosis-1 (NF-1). Eleven of the tumors were histologically malignant PNT, and one was a benign neurofibroma. There were no schwannomas (neurilemomas). The glands were discrete, usually localized to a few areas and in every patient were lined by a keratin-positive epithelium, which in two patients was malignant. One tumor also contained areas of rhabdomyosarcoma, chondrosarcoma, and osteosarcoma (a pluridirectional malignant PNT). Treatment in all patients was some form of surgical resection, followed by radiation in three and chemotherapy in two. Follow-up data were available for nine patients; six of eight patients died with disease. Review of the literature revealed two purported glandular schwannomas (neurilemomas). The authors think these patients had schwannomas containing trapped skin adnexa. Overall, 74% of the patients had NF-1. Ninety-two percent of the tumors were histologically malignant, and 23% of the malignant tumors were pluridirectional malignant PNT. Of the 21 patients for whom follow-up was available, 71% died with tumor. Most glandular PNT are histologically malignant and often are fatal.