Malignant peripheral nerve sheath tumor of the lower labial mucosa: case report and literature review

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon soft tissue sarcoma with a predilection for the extremities and trunk of adults, rarely occurring in the oral cavity. To date, 10 cases of MPNSTs affecting the lower labial mucosa have been reported in the English language literature. We...

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Published in:Oral surgery, oral medicine, oral pathology and oral radiology oral medicine, oral pathology and oral radiology, 2016-08, Vol.122 (2), p.e64-e68
Main Authors: do Amaral, Thiago Lucena, DDS, Valiati, Renato, DDS, PhD, de Andrade, Bruno Augusto Benevenuto, DDS, PhD, Rumayor Piña, Alicia, DDS, MSc, Torres, Sandra Regina, DDS, PhD, Romañach, Mário José, DDS, PhD, Agostini, Michelle, DDS, PhD
Format: Article
Language:English
Subjects:
Aged
Biomarkers, Tumor - analysis
Dentistry
Diagnosis, Differential
Humans
Immunohistochemistry
Lip - innervation
Lip Neoplasms - diagnosis
Lip Neoplasms - pathology
Lip Neoplasms - surgery
Male
Nerve Sheath Neoplasms - diagnosis
Nerve Sheath Neoplasms - pathology
Nerve Sheath Neoplasms - surgery
Surgery
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Summary:Malignant peripheral nerve sheath tumor (MPNST) is an uncommon soft tissue sarcoma with a predilection for the extremities and trunk of adults, rarely occurring in the oral cavity. To date, 10 cases of MPNSTs affecting the lower labial mucosa have been reported in the English language literature. We describe an additional case of MPNST in the lower labial mucosa of a 67-year-old male patient, who exhibited a painful swelling with a history of 4 months of evolution. Microscopic examination showed dense plexiform fascicles of spindle cells with wavy atypical nuclei. Mitotic figures and nuclear pleomorphism were evident. Immunohistochemical analysis of tumor cells revealed positivity for S-100 protein, CD56, CD34, and neuron-specific enolase but was negative for neurofilament protein, glut-1, claudin-1, desmin, and smooth muscle actin. Ki-67 labeling was 20%. The final diagnosis was MPNST. The lesion was surgically removed with wide margins, with no signs of recurrence after 4 years of follow-up.
ISSN:2212-4403
2212-4411
DOI:10.1016/j.oooo.2015.11.009