Malignant Peripheral Nerve Sheath Tumor With Divergent Glandular Differentiation
| Autor: | Yurina Miki, Khin Thway |
|---|---|
| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty Soft Tissue Neoplasm Malignant peripheral nerve sheath tumor Soft Tissue Neoplasms Biology medicine.disease Glandular Differentiation Pathology and Forensic Medicine 03 medical and health sciences 030104 developmental biology 0302 clinical medicine 030220 oncology & carcinogenesis medicine Immunohistochemistry Humans Surgery Sarcoma Anatomy Neurofibromatosis Differential diagnosis Neoplasm Recurrence Local Nerve sheath neoplasm Neurilemmoma |
| Zdroj: | International journal of surgical pathology. 25(4) |
| ISSN: | 1940-2465 |
| Popis: | Malignant peripheral nerve sheath tumors (MPNST) are soft tissue neoplasms with evidence of nerve sheath differentiation. They usually arise from peripheral nerves or from preexisting benign nerve sheath neoplasms, often in patients with neurofibromatosis type 1 (NF1). The histologic diagnosis of MPNST is challenging as their morphology is highly variable, and there has been a lack of routine diagnostic immunohistochemical markers and specific genetic aberrations. Although divergent differentiation is well documented in MPNST, it is most frequently toward mesenchymal elements. Differentiation toward epithelial elements is very rare, and we illustrate a case of MPNST with glandular differentiation, comprising prominent well-formed glands, with a brief discussion of biphasic (spindle and glandular) neoplasms in the differential diagnosis. An index of suspicion for MPNST is necessary, due to the differing management from tumors in its differential diagnosis, and because of the potential for therapies toward molecular targets in future. |
| Databáze: | OpenAIRE |
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