Malignant melanotic nerve sheath tumour of the neck:report of one case and review of literature
LV Xu
LIU Xia
Abstract：Objective To investigate the clinicopathological and prognostic features of malignant melanotic nerve sheath tumour(MMNST).Methods Clinicopathological data of one case of MMNST was collected and analyzed,PRKAR1A and BRAFV600E gene mutations were tested,with review of the literature.Results Our patient was a 25-years-old male presented with a mass in the right neck for the past ten days.Microscopically,the lesion was composed of spindle and epithelioid cells arranged in interlacing fascicles or sheets with pleomorphic,hyperchromatic nuclei.The nucleoli were prominent.A large amount of melanin and scattered psammoma body-like calcification could be seen in the background.Immunohistochemical staining for HMB45,MART-1,S-100 and MMR produced positive result.Ki-67 index was 5％.PRKAR1A frameshift mutation was identified while BRAFV600E mutation was not detected.Conclusion MMNST is a rare soft tissue tumor.Complete resection is the main treatment.The prognosis is uncertain and long-term follow-up is required.
Keywords：Malignant melanotic nerve sheath tumourPsammoma bodyCarney complex
Publication Date：2023-09-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 852-855 )