Mammary analogue secretory carcinoma of the parotid gland as a secondary malignancy in a childhood survivor of atypical teratoid rhabdoid tumor.
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.
School of Medicine, Wake Forest University, Winston-Salem, NC, USA, jennifer.susan.woo@gmail.com.
Jennifer
J
Woo
Raja R
RR
Seethala
S Joseph
SJ
Sirintrapun
D000293:Adolescent; D002277:Carcinoma / Q000235:genetics / Q000473:pathology*; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D009381:Neoplasms, Radiation-Induced / Q000235:genetics / Q000473:pathology*; D016609:Neoplasms, Second Primary / Q000235:genetics / Q000473:pathology*; D010307:Parotid Neoplasms / Q000235:genetics / Q000473:pathology*; D018335:Rhabdoid Tumor / Q000188:drug therapy / Q000532:radiotherapy; D017741:Survivors; D013724:Teratoma / Q000188:drug therapy / Q000532:radiotherapy