Nasopharyngeal mucoepidermoid carcinoma with mastermind-like transcriptional coactivator 2 translocation: A case report and comprehensive literature review of a rare entity.

Chan-Fong Chio, Wan-Pang Sam, Sio-In Wong, Cheong-Un Sio, Lai-Fong Kok, Ki-Man Pai, Thazin Hlaing
Author Information
  1. Chan-Fong Chio: Department of Anatomical Pathology, Conde de São Januário Hospital, Macao SAR, China. ORCID
  2. Wan-Pang Sam: Department of Otorhinolaryngology, Conde de São Januário Hospital, Macao SAR, China.
  3. Sio-In Wong: Department of Anatomical Pathology, Conde de São Januário Hospital, Macao SAR, China.
  4. Cheong-Un Sio: Department of Otorhinolaryngology, Conde de São Januário Hospital, Macao SAR, China.
  5. Lai-Fong Kok: Department of Anatomical Pathology, Conde de São Januário Hospital, Macao SAR, China.
  6. Ki-Man Pai: Department of Otorhinolaryngology, Conde de São Januário Hospital, Macao SAR, China.
  7. Thazin Hlaing: Department of Anatomical Pathology, Conde de São Januário Hospital, Macao SAR, China.

Abstract

Mucoepidermoid carcinoma is one of the most common malignant tumors in salivary glands and is usually associated with mastermind-like transcriptional coactivator 2 (MAML2) rearrangement. Primary nasopharyngeal mucoepidermoid carcinoma is extremely rare, and MAML2 status was reported in only two studies. Herein, we present a 70-year-old male patient with incidentally found nasopharyngeal mucoepidermoid carcinoma. MAML2 translocation was detected by fluorescence in situ hybridization test. Additionally, we conducted a comprehensive literature review and summarized the clinicopathological features of this rare condition. Nasopharyngeal mucoepidermoid carcinoma shows a similar mean age at diagnosis and gender ratio to those of mucoepidermoid carcinoma in salivary glands. More than half of the patients exhibit high histologic grade at the time of diagnosis. As MAML2 status is unreported in almost all published cases, further studies are needed to explore the incidence and prognostic value of MAML2 rearrangement in nasopharyngeal mucoepidermoid carcinoma.

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