Skip Navigation LinksHome > May 2013 - Volume 35 - Issue 4 > Mast Cell Sarcoma in an Infant: A Case Report and Review of...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e318279e392
Clinical and Laboratory Observations

Mast Cell Sarcoma in an Infant: A Case Report and Review of the Literature

Bautista-Quach, Marnelli A. MD*; Booth, Cassie L. MD*; Kheradpour, Albert MD; Zuppan, Craig W. MD*; Rowsell, Edward H. MD, PhD*; Weiss, Lawrence MD; Wang, Jun MD*

Collapse Box

Abstract

Mast cell diseases comprise a spectrum of disorders including cutaneous mastocytosis, indolent or aggressive systemic variants including leukemia, and unifocal tumor formations such as benign extracutaneous mastocytoma or aggressive mast cell sarcoma (MCS). Many mast cell diseases are associated with aberrancy of c-KIT proto-oncogene resulting in tyrosine kinase activity, typically exhibiting point mutation in codon 816. MCS is an exceedingly rare clinicopathologic entity characterized by a unifocal accumulation of neoplastic mast cells that grow in a locally destructive manner. We report a case in a 2-year-old boy who was initially diagnosed at 8 months of age with atypical cutaneous mastocytoma of the right ear with subsequent aggressive, destructive growth pattern; features that were most consistent with MCS. So far, MCS has been documented in the literature in at least 6 human cases. To the best of our knowledge, our case represents the first MCS in an infant. Thorough multimodal approach with strict follow-up is relevant in appropriately diagnosing this rare entity, particularly in differentiating this lesion from other neoplasms that are more likely to occur in infancy.

© 2013 Lippincott Williams & Wilkins, Inc.

Login

Article Tools

Share

Search for Similar Articles
You may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search.

Connect With Us

Twitter
twitter.com/JPHOonline

For additional oncology content, visit LWW Oncology Journals on Facebook.