Secretory Carcinoma in Children and Young Adults: A Case Series

Secretory carcinoma (SC), previously known as mammary analogue secretory carcinoma, is a rare salivary gland neoplasm that typically presents as a slow-growing painless lesion in the head and neck. SC occurs mainly in adults but has been described in children with the youngest reported patient diagn...

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Bibliographic Details
Published in:Pediatric and developmental pathology 2022-03, Vol.25 (2), p.155-161
Main Authors: Simon, Caroline T, McHugh, Jonathan B, Rabah, Raja, Heider, Amer
Format: Article
Language:English
Subjects:
Adolescent
Biomarkers, Tumor - genetics
Breast Neoplasms
Carcinoma - pathology
Child
Female
Humans
Male
Mammary Analogue Secretory Carcinoma - pathology
Salivary Gland Neoplasms - diagnosis
Salivary Gland Neoplasms - genetics
Salivary Gland Neoplasms - surgery
Young Adult
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Summary:Secretory carcinoma (SC), previously known as mammary analogue secretory carcinoma, is a rare salivary gland neoplasm that typically presents as a slow-growing painless lesion in the head and neck. SC occurs mainly in adults but has been described in children with the youngest reported patient diagnosed at five years of age. In children the gender distribution has been reported as female to male ratio of 1:1.2. SC is generally considered a low-grade malignancy with characteristic morphological features and immunological profile. SC also harbors ETV6-NTRK3 fusion (t(12;15)(p13:q25)). Surgical resection with or without lymph node dissection is the standard treatment, with generally favorable clinical outcomes. Here we present a single institution case series of six patients (ages 9-21) with SC and a review of the previously described pediatric cases. Our small series showed male predominance in pediatric patients with predominantly low-grade and stage tumors. All cases underwent complete surgical resections and when follow up is available there was no evidence of recurrences or metastases. To the best of our knowledge, this is the only SC case series comprised exclusively of pediatric and youth patients.
ISSN:1093-5266
1615-5742
DOI:10.1177/10935266211046996