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Special Feature |

Image of the Month—Diagnosis FREE

[+] Author Affiliations

Section Editor: S. Rozycki, Md Grace, MD

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Arch Surg. 2004;139(2):224. doi:10.1001/archsurg.139.2.224.
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Published online

Figure 1. Computed tomographic scan of the abdomen and pelvis demonstrating a large, homogeneous mass of approximately 23 × 15 cm.

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Figure 1.
Graphic Jump Location

Figure 2. Biopsy specimen taken from the intra-abdominal tumor showing the same appearance as the incisional biopsy specimens taken from the mesentery: a paucicellular to cellular proliferation of bland-looking fibroblasts, infiltrating muscle and engulfing muscle fibers (black arrowheads). No mitoses are seen. Immunohistochemical analysis was negative for CD117 (poly C-kit) and estrogen receptors but weakly positive for progesterone receptors.

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Figure 2.
Graphic Jump Location

Desmoid tumors are benign in origin and do not metastasize. However, they can be aggressive locally and have a tendency for local recurrence after excision.1,2Their ability for local infiltration can result in significant deformity, morbidity, and mortality. Desmoid tumors in the general population are rare, accounting for 0.03% of all neoplasms.3,4There is an association between the occurrence of desmoid tumors and familial adenomatous polyposis coli, especially in patients with Gardner syndrome.3,4Both intra- and extra-abdominal desmoid tumors occur more frequently in patients with familial adenomatous polyposis coli, with an incidence of 3.5% to 32%.4,5

The treatment of choice for desmoid tumors is radical excision, both for primary tumors and recurrent lesions. However, a radical resection of mesenteric desmoid tumors is often not feasible owing to the inevitable preservation of vital anatomic structures, as was the case in our patient. In such cases, treatment with sulindac, tamoxifen, or toremifene citrate may alleviate symptoms.57Radiotherapy has only a minor role, with some reports of complete and partial remission following adjuvant radiotherapy.2,8Some attempts have been made to use chemotherapy, but, with a partial response in 50% to 60% of cases, the results have been inconclusive.9,10

In the patient described, diagnoses other than desmoid tumor were also considered, including gastrointestinal stromal tumor, fibrosarcoma, and ovarian carcinoma. We considered gastrointestinal stromal tumor because of the rectal blood loss. These tumors occur in the entire gastrointestinal tract and may also arise from the omentum, mesenteries, and retroperitoneum. Such tumors may become large and occur more often in men than women, with the highest incidence around 50 to 60 years of age. Unlike the specimen described, they are positive for CD117 on histologic examination.11Fibrosarcoma seemed plausible because of the tumor's rapid growth and the fact that it is usually difficult to distinguish between low-grade fibrosarcoma and desmoid tumor. Ovarian carcinoma was considered because of the tumor's enormous size; however, ovarian carcinoma is often cystic. In accordance with the patient's history of Gardner syndrome, a desmoid tumor was thought to be the most likely option and turned out to be the case.

Corresponding author: Joost M. Klaase, MD, PhD, Department of Surgery, Medisch Spectrum Twente, PO Box 50.000, 7500 KA Enschede, the Netherlands (e-mail: j.klaase@ziekenhuis-mst.nl).

Accepted for publication March 15, 2003.

Brueckl  WMPreuss  JMWein  A  et al.  Ki-67 expression and residual tumour (R) classification are associated with disease-free survival in desmoid tumour patients. Anticancer Res. 2001;213615- 3620
PubMed
Baliski  CRTemple  WJArthur  KSchachar  NS Desmoid tumors: a novel approach for local control. J Surg Oncol. 2002;8096- 99
PubMed Link to Article
Ponz de Leon  MVaresco  LBenatti  P  et al.  Phenotype-genotype correlations in an extended family with adenomatosis coli and an unusual APC gene mutation. Dis Colon Rectum. 2001;441597- 1604
PubMed Link to Article
Naylor  EWGardner  EJRichards  RC Desmoid tumors and mesenteric fibromatosis in Gardner's syndrome: report of kindred 109. Arch Surg. 1979;1141181- 1185
PubMed Link to Article
Clark  SKNeale  KFLandgrebe  JCPhillips  RK Desmoid tumours complicating familial adenomatous polyposis. Br J Surg. 1999;861185- 1189
PubMed Link to Article
Picariello  LBrandi  MLFormigli  L  et al.  Apoptosis induced by sulindac sulfide in epithelial and mesenchymal cells from human abdominal neoplasms. Eur J Pharmacol. 1998;360105- 112
PubMed Link to Article
Soravia  CBerk  TMcLeod  RSCohen  Z Desmoid disease in patients with familial adenomatous polyposis. Dis Colon Rectum. 2000;43363- 369
PubMed Link to Article
Zelefsky  MJHarrison  LBShiu  MHArmstrong  JGHajdu  SIBrennan  MF Combined surgical resection and iridium 192 implantation for locally advanced and recurrent desmoid tumors. Cancer. 1991;67380- 384
PubMed Link to Article
Poritz  LSBlackstein  MBerk  TGallinger  SMcLeod  RSCohen  Z Extended follow-up of patients treated with cytotoxic chemotherapy for intra-abdominal desmoid tumors. Dis Colon Rectum. 2001;441268- 1273
PubMed Link to Article
Patel  SREvans  HLBenjamin  RS Combination chemotherapy in adult desmoid tumors. Cancer. 1993;723244- 3247
PubMed Link to Article
Miettinen  MEl-Rifai  WSobin  LHLasota  J Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a review. Hum Pathol. 2002;33478- 483
PubMed Link to Article

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Figures

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Figure 1.
Graphic Jump Location
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Figure 2.
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Tables

References

Brueckl  WMPreuss  JMWein  A  et al.  Ki-67 expression and residual tumour (R) classification are associated with disease-free survival in desmoid tumour patients. Anticancer Res. 2001;213615- 3620
PubMed
Baliski  CRTemple  WJArthur  KSchachar  NS Desmoid tumors: a novel approach for local control. J Surg Oncol. 2002;8096- 99
PubMed Link to Article
Ponz de Leon  MVaresco  LBenatti  P  et al.  Phenotype-genotype correlations in an extended family with adenomatosis coli and an unusual APC gene mutation. Dis Colon Rectum. 2001;441597- 1604
PubMed Link to Article
Naylor  EWGardner  EJRichards  RC Desmoid tumors and mesenteric fibromatosis in Gardner's syndrome: report of kindred 109. Arch Surg. 1979;1141181- 1185
PubMed Link to Article
Clark  SKNeale  KFLandgrebe  JCPhillips  RK Desmoid tumours complicating familial adenomatous polyposis. Br J Surg. 1999;861185- 1189
PubMed Link to Article
Picariello  LBrandi  MLFormigli  L  et al.  Apoptosis induced by sulindac sulfide in epithelial and mesenchymal cells from human abdominal neoplasms. Eur J Pharmacol. 1998;360105- 112
PubMed Link to Article
Soravia  CBerk  TMcLeod  RSCohen  Z Desmoid disease in patients with familial adenomatous polyposis. Dis Colon Rectum. 2000;43363- 369
PubMed Link to Article
Zelefsky  MJHarrison  LBShiu  MHArmstrong  JGHajdu  SIBrennan  MF Combined surgical resection and iridium 192 implantation for locally advanced and recurrent desmoid tumors. Cancer. 1991;67380- 384
PubMed Link to Article
Poritz  LSBlackstein  MBerk  TGallinger  SMcLeod  RSCohen  Z Extended follow-up of patients treated with cytotoxic chemotherapy for intra-abdominal desmoid tumors. Dis Colon Rectum. 2001;441268- 1273
PubMed Link to Article
Patel  SREvans  HLBenjamin  RS Combination chemotherapy in adult desmoid tumors. Cancer. 1993;723244- 3247
PubMed Link to Article
Miettinen  MEl-Rifai  WSobin  LHLasota  J Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a review. Hum Pathol. 2002;33478- 483
PubMed Link to Article

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