Fibromatosis (Desmoid Tumor) Involving the Orbit and Cheek

Desmoid tumor (aggressive fibromatosis) is an aggressive fibroblastic proliferation of well circumscribed, locally invasive, differentiated fibrous tissue. For patients with desmoid tumors that are not amenable to surgery or radiation therapy, the use of hormonal agents and nonsteroidal antiinflammatory drugs (NSAIDs) have been attempted, with some success. The use of chemotherapy also has been reported to have activity. Seven patients (5 males and 2 females) with a median age of 40 years (range, 17-66 years) who received cytotoxic chemotherapy (combinations of cyclophosphamide and doxorubicin; mitomycin, doxorubicin, and cisplatin; and ifosfamide and etoposide) for desmoid tumor were reviewed retrospectively. Five patients were found to have recurrent tumors. Four patients had familial adenomatous polyposis. Four patients had failed tamoxifen and six had failed NSAIDs prior to receiving cytotoxic chemotherapy. In six patients the desmoid tumor was intraabdominal and one tumor had occurred on the buttock. Patients received a median number of six cycles of chemotherapy (range, two to eight cycles). Objective disease regression occurred in 3 patients. There was an apparent clinical benefit in six patients with the duration of benefit ranging from 3 months to 15 years. The chemotherapy was well tolerated and no treatment-related mortality was reported. The results of the current study indicate that the use of combination chemotherapy for desmoid tumors may provide long-term clinical benefits. Copyright 2003 American Cancer Society

The primary goal of treatment for desmoid tumors is complete surgical resection to achieve negative margins. In adults with unresectable or recurrent lesions, treatment options include noncytotoxic and cytotoxic drugs, but little is known about nonsurgical treatment in children. Between 1992 and 2003 six children (four girls, two boys) with a median age of 2.5 years (range 11 months to 9 years) received multimodal adjuvant therapy for unresectable or recurrent desmoid tumors. Primary treatment consisted of noncytotoxic treatment with tamoxifen (1 mg/kg orally, twice daily) and diclofenac (2 mg/kg rectally, twice daily), whereas two children with life-threatening tumor progression in addition received treatment intensification with weekly vinblastine (6 mg/m intravenously) and methotrexate (30 mg/m intravenously). Of the four children with unresectable tumors, two achieved remarkable tumor shrinkage and two had stable disease, whereas two patients were disease-free for 3.7 and 2.6 years after nonradical resection. Median observation time was 3.1 years (range 1-11 years). Treatment was generally well tolerated; only one patient developed pubertal acceleration after a duration of tamoxifen treatment of 9.3 years. Because of the potential life-threatening situation, the management of children with unresectable or recurrent desmoid tumors requires a multidisciplinary approach. Nonaggressive therapy with tamoxifen and diclofenac may be the first treatment choice in these patients, but in patients with progressive disease, cytotoxic chemotherapy is indicated. Weekly administration of vinblastine and methotrexate seems to be safe and effective in these children.

Desmoid tumors are are, nonmalignant neoplasms that show locally aggressive growth but lack the potential to metastasize. Common anatomic sites include the extremity, abdominal wall, and mesentery. Little is reported about clinical features and outcome of desmoid tumors of the head and neck. Twenty-one patients with desmoid tumors of the head and neck treated at MSKCC between July 1982 and June 1999 were identified from our inpatient tumor database. All patients underwent surgical resection and were prospectively followed. Clinicopathologic features and treatment modalities were evaluated. Patient age at diagnosis was 22 to 76 years, with a female-male ratio of 16:5. Most tumors were located in the supraclavicular fossa or the muscles of the neck (n = 19). Patients most commonly had a painless mass (n = 13) or neurologic symptoms including pain or neurologic deficit (n = 8). Extent of resection was limited to the tumor in nine patients. In the other 12, structures including the accessory or phrenic nerve, parts of the brachial plexus, or bony structures were resected. Persistent neurologic or functional deficits after surgery were noted in 12 patients. Five of 21 patients had recurrences (24%). Eleven patients received radiation therapy. In this small population, no obvious benefit for patients receiving radiation vs patients who were not irradiated, regardless of their surgical margin status, was seen. Treatment for recurrence was re-resection or re-resection plus radiation. Eighteen patients remained free of disease after treatment, two patient have stable disease and one died of other causes. No patients died of their disease. Desmoid tumors of the head and neck are rare, fibrous neoplasms with a good prognosis but significant morbidity. Function-preserving surgery should be a primary goal to minimize morbidity. In this small group of patients, the benefits of radiation therapy in patients with positive margins could not be clearly demonstrated and should be balanced against radiation-related morbidity.