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DESMOID TUMOUR IN FAMILIAL POLYPOSIS OF THE COLON
Author(s) -
Hughes E. S. R.,
Hueston J. T.
Publication year - 1960
Publication title -
australian and new zealand journal of surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.111
H-Index - 51
eISSN - 1445-2197
pISSN - 0004-8682
DOI - 10.1111/j.1445-2197.1960.tb03098.x
Subject(s) - medicine , fibromatosis , familial adenomatous polyposis , abnormality , pathology , gardner syndrome , soft tissue , dermatology , colorectal cancer , cancer , psychiatry
Summary Familial polyposis is associated sometimes with an abnormal behaviour in certain other tissues. Osteomata, soft tissue tumours, sebaceous cysts and intra‐abdominal fibrous tissue masses may co‐exist with familial polyposis. A case is recorded in which a desmoid tumour appeared in the wound after surgical treatment of familial polyposis. This is the 17th case to be reported in the literature. Pathologically, the tumour appears to be histologically distinct. from palmar and plantar fibromatosis nor does it seem to be inflammatory in origin. Desmoid tumours in surgical incisions are rare but the number that have been reportedin familial polyposis seems to indicate that in this disease there is a special tendency. The desmoid tumour may or may not be associated with fibrous tissue masses and osteomas elsewhere. In the case reported here these were present. It is known that this latter abnormality is transmitted as a Mendelian dominant. The curious association with polyposis has suggested two closely linked genes, or a pleotropic gene; a more acceptable theory is that the mutant gene has but a single action many steps removed from any clinically obvious manifestations. Recurrence after removal is so common that it seems excision should be very wide, to avoid the involved fascia1 sheets. The prognosis of these desmoids has not been established and it is possible that removal is not essential.