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CONGENITAL SOLITARY FIBROMATOSIS OF SOFT TISSUES, A VARIANT OF CONGENITAL GENERALIZED FIBROMATOSIS
Author(s) -
Kindblom LarsGunnar,
Termén Gunnar,
SäeSÖderbergh Johan,
Angervall Lennart
Publication year - 1977
Publication title -
acta pathologica microbiologica scandinavica section a pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.909
H-Index - 88
eISSN - 1600-0463
pISSN - 0365-4184
DOI - 10.1111/j.1699-0463.1977.tb00454.x
Subject(s) - fibromatosis , hemangiopericytoma , solitary fibrous tumor , fibroma , differential diagnosis , pathology , medicine , fibrosarcoma , anatomy , aggressive fibromatosis , biology , stem cell , cd34 , genetics
A report of 2 cases of solitary fibromatosis in a 10‐day‐old boy and a girl 3 years and 10 months old is presented. Both lesions were deep‐seated and showed a nodular and infiltrating growth, predominantly built‐up by immature fibroblast‐like cells and including hemangiopericytoma‐like areas. One of the lesions also showed leiomyoma‐like areas. An ultrastructural study, however, revealed no intra‐cytoplasmatic myofilaments. At follow‐up examinations after 21 years and 1 year, respectively, there were no signs of recurrences or metastases. These 2 cases are considered to represent a solitary form of congenital generalized fibromatosis. The differential diagnosis from infantile hemangiopericytoma and fibrous lesions seen in infancy and early childhood, such as infantile fibrosarcoma, diffuse infantile fibromatosis, extra‐abdominal desmoid, fibrous hamartoma of infancy and juvenile aponeurotic fibroma, is discussed.