title: Aggressive fibromatosis (desmoid tumor) is a monoclonal disorder.
abstract: Aggressive fibromatosis (also called deep fibromatosis or desmoid tumor) is a proliferation of cytologically benign-appearing fibrocytes, often resulting in significant functional loss. The nature of the lesion is controversial: some evidence suggests that it is a reactive process, whereas other evidence supports a neoplastic etiology. The pattern of X chromosome inactivation, using a technique based on polymerase chain reaction (PCR) amplification of a hypervariable CAG repeat region flanking Hhal restriction sites of the human androgen receptor gene, was determined in four cases in which cryopreserved tumor and adjacent normal tissue were available. All four tumors demonstrated a monoclonal pattern, while the adjacent normal tissues demonstrated a polyclonal pattern. This demonstrates that aggressive fibromatosis is proliferation of cells derived from a single clone with a growth advantage, and thus is likely a neoplastic process.
authors.affiliation: Division of Orthopaedic Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: B A
authors.initials: BA
authors.lastname: Alman
authors.forename: M E
authors.initials: ME
authors.lastname: Pajerski
authors.forename: S
authors.initials: S
authors.lastname: Diaz-Cano
authors.forename: K
authors.initials: K
authors.lastname: Corboy
authors.forename: H J
authors.initials: HJ
authors.lastname: Wolfe
mesh_terms: D001132:Arm; D002999:Clone Cells; D003001:Cloning, Molecular; D005260:Female; D018222:Desmoid Tumors / Q000235:genetics* / Q000473:pathology*; D006801:Humans; D007866:Leg; D016133:Polymerase Chain Reaction; D011944:Receptors, Androgen / Q000235:genetics; D012729:Sex Chromosome Aberrations / Q000235:genetics / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000235:genetics* / Q000473:pathology*; D014960:X Chromosome / Q000473:pathology
chemical_list: D011944:Receptors, Androgen