Head and Neck Pathology

, Volume 8, Issue 2, pp 194–197

Mammary Analogue Secretory Carcinoma of the Parotid Gland as a Secondary Malignancy in a Childhood Survivor of Atypical Teratoid Rhabdoid Tumor

  • Jennifer Woo
  • Raja R. Seethala
  • S. Joseph Sirintrapun
Case Report

DOI: 10.1007/s12105-013-0481-9

Cite this article as:
Woo, J., Seethala, R.R. & Joseph Sirintrapun, S. Head and Neck Pathol (2014) 8: 194. doi:10.1007/s12105-013-0481-9

Abstract

We report the first case of mammary analogue secretory carcinoma (MASC) arising as a secondary malignancy in a 14 years old child with a history of atypical teratoid rhabdoid tumor (ATRT). Although MASC and ATRT are both rare malignancies, they do not share the same genetic and molecular profiles. MASC is a salivary malignancy characterized by a t(12;15)(p13;q25) translocation, resulting in an ETV6-NTRK3 fusion product encoding for a tyrosine kinase. ATRT is a highly malignant pediatric tumor characterized by a chromosome 22 mutation in the hSNF5/INI1 gene, encoding for a chromatin remodeling protein. Additionally, although mucoepidermoid carcinoma has been described as a secondary malignancy post-therapy for head and neck tumors, MASC has only been reported as a primary malignancy. Our patient was treated with a complete resection of his left sided ATRT at age 3 followed postoperatively with chemoradiotherapy. At age 14 he underwent a parotidectomy for his 1 year history of a left sided preauricular mass and was subsequently diagnosed with MASC. We not only report a case of two rare malignancies in one patient, but also the first case of MASC arising as a secondary malignancy.

Keywords

Mammary analogue secretory carcinoma Parotid gland Atypical teratoid rhabdoid tumor 

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Jennifer Woo
    • 1
  • Raja R. Seethala
    • 1
  • S. Joseph Sirintrapun
    • 1
  1. 1.School of MedicineWake Forest UniversityWinston-SalemUSA

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