Malignant peripheral nerve sheath tumor with glandular elements arising in bone: a potential diagnostic pitfall

Abstract

We report a unique case of a malignant peripheral nerve sheath tumor with glandular differentiation arising in the tibial epiphysis of a 30 year old woman with no other significant medical history. Extensive histochemical and immunohistochemical analysis showed the epithelium lining the glands to exhibit gastrointestinal and neuroendocrine differentiation. The patient’s clinical course had been one of multiple recurrences and distant metastases, almost exclusively of the spindle cell component of this biphasic neoplasm, culminating in the patient’s demise. The differential diagnosis is discussed along with ancillary studies helpful in elucidating the appropriate diagnosis when confronted with a biphasic neoplasm exhibiting unequivocal glandular differentiation.