Congenital Myoepithelial Carcinoma of Soft Tissue Associated With Cystic Myoepithelioma.
Int J Surg Pathol
; 26(1): 78-83, 2018 Feb.
Article
in En
| MEDLINE
| ID: mdl-28675958
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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Soft Tissue Neoplasms
/
Carcinoma
/
Myoepithelioma
/
Head and Neck Neoplasms
Type of study:
Prognostic_studies
/
Risk_factors_studies
Limits:
Humans
/
Male
/
Newborn
Language:
En
Journal:
Int J Surg Pathol
Journal subject:
PATOLOGIA
Year:
2018
Document type:
Article
Affiliation country:
Italia