Congenital Myoepithelial Carcinoma of Soft Tissue Associated With Cystic Myoepithelioma. (February 2018)

• Record Type: Journal Article
• Authors: Baldovini, Chiara; Sorrentino, Stefania; Alves, Cesar Augusto; Piatelli, Gianluca; Garaventa, Alberto; Morana, Giovanni; Nozza, Paolo
• Is Part Of: International journal of surgical pathology. Volume 26:Number 1(2018)
• Page Start: 78
• Page End: 83
• Publication Date: 2018-02
• Subjects: myoepithelial carcinoma -- myoepithelioma -- soft tissue -- congenital -- cystic; Pathology, Surgical -- Periodicals; 617.07
• DOI: 10.1177/1066896917718936
• Languages: English
• ISSNs: 1066-8969

Abstract

Myoepithelial neoplasms of soft tissue represent a heterogeneous group of lesions, encompassing both benign entities as myoepithelioma (ME) and highly aggressive tumors as myoepithelial carcinoma (MEC). We describe a case of pediatric soft tissue MEC with peculiar features that may lead to misdiagnosis: congenital onset and presence of a benign component with predominant cystic structure. Few cases of congenital MEC have been reported, but the coexistence of MEC with ME is even more rare, accounting for less than 1% of myoepithelial tumors. Moreover, an extensive cystic appearance had never been described in either ME or MEC of soft tissue. Despite several predictors of poor prognosis, the patient has been showing a favorable clinical course since the administration of ICpE (ifosfamide, cisplatin, and etoposide) chemotherapy. This report provides valuable information in the differential diagnosis of cystic congenital tumors and supports a possible efficacy of adjuvant combined treatment for patients with localized disease after surgery.