myofibromatosis
Pronunciation: my-oh-fy-broh-mah-TOH-sis
A benign fibroblastic/myofibroblastic proliferative disorder of infancy and childhood, presenting as solitary or multiple nodules in soft tissue, bone, and viscera.
Full Definition
Infantile myofibromatosis is the most common fibrous tumour of infancy, characterised by nodular proliferations of plump spindle cells with a myofibroblastic immunophenotype. Lesions may be solitary (myofibroma) or multicentric (myofibromatosis). Visceral involvement carries a worse prognosis due to cardiopulmonary or gastrointestinal complications. Histologically, the tumour displays a biphasic pattern of pale myoid zones and haemangiopericytoma-like areas. Editors should distinguish the solitary form ('myofibroma') from the multicentric designation ('myofibromatosis') to ensure diagnostic precision in reports.
Usage
Usage note: Do not abbreviate as 'MF' in pathology documents, as this overlaps with 'mycosis fungoides'.
In Context
- "The pathology report confirmed multicentric myofibromatosis with involvement of the skull, mandible, and soft tissue of the neck." — Surgical pathology report
- "Editors should note that 'myofibromatosis' implies multiple lesions, whereas 'myofibroma' denotes a solitary tumour." — Editorial style guide