Chemotherapy for desmoid tumors in association with familial adenomatous polyposis.
OBJECTIVE
This study was designed to assess the effect of chemotherapy on complex desmoid tumors associated with familial adenomatous polyposis.


METHODS
Five patients (3 males, 2 females; age range, 29-45 years) had symptomatic, unresectable intra-abdominal desmoid tumors in association with familial adenomatous polyposis that were unresponsive to conventional medical therapy. Each patient was treated with a cytotoxic chemotherapeutic regimen consisting of doxorubicin and dacarbazine followed by carboplatin and dacarbazine. Response to treatment was assessed by measurement of tumor size using computerized tomography. Follow-up has been for a mean of 22 (range, 10-30) months.


RESULTS
One patient has had a complete response, and three patients have had a partial response, with a reduction in tumor volume of at least 50 percent. One patient had a minimum response to treatment and developed a rapid increase in tumor size on cessation of therapy. Complications of treatment included febrile neutropenia, severe epistaxis, and subclavian vein thrombosis.


CONCLUSIONS
The cytotoxic chemotherapeutic regimen described is effective in the treatment of selected unresectable desmoid tumors associated with familial adenomatous polyposis and should be considered in symptomatic patients who do not respond to conventional medical therapy.
Schnitzler
M
M
Steve Atanas Familial Gastrointestinal Cancer Registry, Mount Sinai Hospital and University of Toronto, Ontario, Canada.
Cohen
Z
Z
Blackstein
M
M
Berk
T
T
Gallinger
S
S
Madlensky
L
L
McLeod
R
R
D011125:Adenomatous Polyposis Coli / Q000150:complications*; D000328:Adult; D000903:Antibiotics, Antineoplastic / Q000008:administration & dosage / Q000009:adverse effects; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000009:adverse effects; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D016190:Carboplatin / Q000008:administration & dosage / Q000009:adverse effects; D003606:Dacarbazine / Q000008:administration & dosage / Q000009:adverse effects; D004317:Doxorubicin / Q000008:administration & dosage / Q000009:adverse effects; D004844:Epistaxis / Q000139:chemically induced; D005260:Female; D005334:Fever / Q000139:chemically induced; D018221:Fibromatosis, Abdominal / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009503:Neutropenia / Q000139:chemically induced; D012074:Remission Induction; D013350:Subclavian Vein; D013927:Thrombosis / Q000139:chemically induced; D014057:Tomography, X-Ray Computed
D000903:Antibiotics, Antineoplastic; D000970:Antineoplastic Agents; D003606:Dacarbazine; D004317:Doxorubicin; D016190:Carboplatin