Preferred Label : Dermatofibrosarcoma protuberans;
ICD-11 definition : Dermatofibrosarcoma protuberans is a locally invasive tumour arising in the dermis
and showing fibroblastic differentiation. It typically presents as a very slowly enlarging
firm nodular dermal mass on the trunk or proximal limbs. In its early stages it may
be mistaken both clinically and histologically for a benign fibrous histiocytoma.
Local recurrence after resection is common.;
ICD-11 synonym : Bednar tumour of unspecified site; DFSP - [dermatofibrosarcoma protuberans]; pigmented dermatofibrosarcoma;
ICD-11 acronym : DFSP;
Origin ID : 1579898301;
Automatic exact mappings (from CISMeF team)
Dermatofibrosarcoma protuberans is a locally invasive tumour arising in the dermis
and showing fibroblastic differentiation. It typically presents as a very slowly enlarging
firm nodular dermal mass on the trunk or proximal limbs. In its early stages it may
be mistaken both clinically and histologically for a benign fibrous histiocytoma.
Local recurrence after resection is common.
