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Keywords:

  • desmoid tumors;
  • cytotoxic chemotherapy;
  • nonsteroidal anti-inflammatory drugs (NSAIDs);
  • clinical benefit

Abstract

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. REFERENCES

BACKGROUND

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

The results of the current study indicate that the use of combination chemotherapy for desmoid tumors may provide long-term clinical benefits. Cancer 2003;97:1134–35. © 2003 American Cancer Society.

DOI 10.1002/cncr.11189

Desmoid tumor (aggressive fibromatosis) is a rare tumor of fibroblastic origin.1–3 Because of their propensity to recur locally, treatment usually is comprised of wide local excision with or without radiation therapy.4, 5 Patients with recurrent, unresectable desmoid tumors often die because of the locally aggressive nature of the tumor. Treatment options have included the use of hormonal agents such as tamoxifen as well as nonsteroidal antiinflammatory drugs (NSAIDs).6–8 The use of combination chemotherapy with vinblastine sulfate and methotrexate has been reported to be effective in adults as well as in children with desmoid tumors.9–12 Herein, we report our experience using cytotoxic chemotherapy for patients with desmoid tumors.

MATERIALS AND METHODS

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. REFERENCES

Between 1976 and July of 1998, seven patients treated at the Mayo Clinic with desmoid tumors who received cytotoxic chemotherapy were identified. Seven patients (5 males and 2 females) with a median age of 40 years (range, 17–66 years) were identified. Five patients had recurrent desmoid tumors and four patients had a history of 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 in the abdomen and pelvis and in one case the tumor developed on the buttock. The combination chemotherapy regimens were comprised of cyclophosphamide and doxorubicin (600 mg/m2 and 60 mg/m2, respectively, repeated every 3 weeks); ifosfamide and etoposide (2500 mg/m2 and 100 mg/m2, respectively, daily 3 times repeated every 3–4 weeks); and mitomycin, doxorubicin, and cisplatin (8 mg/m2, 40 mg/m2, and 60 mg/m2, respectively, repeated every 4 weeks). Patient charts were reviewed for objective disease regression, symptom control, and other clinical benefits. In addition, the duration of clinical benefit was assessed as well as the toxicity to chemotherapy.

RESULTS

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. REFERENCES

Patients received a median of six cycles of chemotherapy (range, two–eight cycles). An objective response was noted in three patients. The duration of chemotherapy was 3–12 months. There was a clinical benefit reported in 6 patients with the duration of the clinical benefit ranging from 3 months to 15 years. (Table 1). Patients appeared to tolerate chemotherapy well with no treatment-related mortality reported.

Table 1. Patient Characteristics
CaseAge (yrs)GenderGeneticTAMNSAIDLocationRecurrentChemoBenefit
  1. TAM: tamoxifen; NSAID: nonsteroidal antiinflammatory drugs; Chemo: chemotherapy; F: female; intraabdo: intraabdominal; CTX: cyclophosphamide; ADR: doxorubicin; M: male; FAP: familial adenomatous polyposis; IFOS: ifosfamide; VP-16: etoposide; MAP: mitomycin, doxorubicin, and cisplatin; abd wall: abdominal wall.

166FNoNoNoIntraabdoYesCTX/ADRYes
240MFAPYesYesIntraabdoYesIFOS/VP-16Yes
348MFAPYesYesIntraabdoYesIFOS/VP-16Yes
435MNoNoYesButtockNoIFOS/VP-16 MAPYes
527FFAPYesYesAbd wallYesIFOS/VP-16 MAPYes
650MFAPYesYesIntraabdoYesIFOS/VP-16Stable
717MNoNoYesIntraabdoNoIFOS/VP-16Yes

DISCUSSION

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. REFERENCES

The treatment of patients with recurrent, unresectable desmoid tumors usually is palliative. Once patients have failed hormonal therapy or the use of NSAIDs, treatment options are limited. The use of a combination chemotherapy regimen comprised of doxorubicin and dacarbazine as well as a low-dose regimen with methotrexate and vinblastine has demonstrated activity.9–12 In the current study, the chemotherapy used was the antisarcoma regimens ifosfamide and etoposide and mitomycin, doxorubicin, and cisplatin chemotherapy and the findings confirmed the benefit of chemotherapy. Although their histologic appearance might suggest otherwise, desmoid tumors do respond to cytotoxic chemotherapy and in some patients there is marked symptomatic and durable disease regression that occasionally persists after the discontinuation of chemotherapy. Despite minimal objective tumor shrinkage, some other patients in the current study experienced symptomatic benefit from the chemotherapy.

The current study does not provide a direct comparison with other chemotherapy regimens but does provide evidence that chemotherapy should be regarded as an option for patients with unresectable desmoid tumors.

REFERENCES

  1. Top of page
  2. Abstract
  3. MATERIALS AND METHODS
  4. RESULTS
  5. DISCUSSION
  6. REFERENCES