Inflammatory Myofibroblastic Tumor of the Orbit in an 8-Month Old.
Ophthalmic Plast Reconstr Surg
; 36(3): e65-e68, 2020.
Article
en En
| MEDLINE
| ID: mdl-31880682
ABSTRACT
Inflammatory myofibroblastic tumor is a mesenchymal neoplasm, commonly seen in the lung and abdominopelvic region of children. The authors present an 8-month-old female with a 2-month history of left-sided proptosis. Examination was significant for left-sided proptosis, a left exotropia and hypotropia, left supraduction and adduction deficits, and left optic disc elevation. MRI imaging revealed an extraconal left superomedial orbital mass with globe displacement and proptosis. Left anterior orbitotomy with excisional biopsy showed a solid mass composed of an infiltrative proliferation of bland spindle cells in a variably myxoid background with associated perivascular lymphoplasmacytic infiltration. Immunohistochemistry was positive for ALK-1 and CD34 and demonstrated focal positivity for S100. Fluorescence in-situ hybridization showed an additional copy of the 3'ALK gene (46%) in interphase cells examined. Next generation targeted sequencing found a DCTN1/ALK fusion. Findings were consistent with inflammatory myofibroblastic tumor. To the authors' knowledge, this is one of the largest primary orbital inflammatory myofibroblastic tumors in the youngest reported patient.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Órbita
/
Neoplasias de Tejido Muscular
Límite:
Child
/
Female
/
Humans
/
Infant
Idioma:
En
Revista:
Ophthalmic Plast Reconstr Surg
Asunto de la revista:
OFTALMOLOGIA
Año:
2020
Tipo del documento:
Article
País de afiliación:
Estados Unidos