Extraabdominal fibromatosis in retroperitoneal space
© Kikkawa et al. 2004
Received: 25 May 2004
Accepted: 03 October 2004
Published: 03 October 2004
Fibromatosis or desmoid tumor covers a broad spectrum of benign fibrous tissue proliferations. It is characterized by infiltrative growth and a tendency towards recurrence; however, unlike sarcoma, it never metastasizes.
We report on a case of extraabdominal fibromatosis originating from the retroperitoneal space in a 43-year-old woman. Seven years earlier she had undergone ureterolysis and ureteroureterostomy for ureteral obstruction. Computed tomography revealed a tumor between the iliocostalis and the psoas muscle. Histopathological evaluation revealed uniform proliferation of spindle cells, with a moderate amount of collagen fibers, suggesting extraabdominal fibromatosis (desmoid tumor). The tumor was surgically resected, and since then, the patient has remained asymptomatic without any restrictions of daily living activities and without any signs of tumor recurrence during the two-year follow-up.
Complete resection is the treatment of choice. Adjuvant therapy using non steroidal anti-inflammatory agents, tamoxifen, interferon, anti-neoplastic agents, and radiotherapy, either alone or in combination finds application for unresectable or recurrent cases.
The term "fibromatosis" covers a broad spectrum of benign fibrous tissue proliferations, the biological behavior of which is similar to both benign fibrous lesions and fibrosarcoma. Like fibrosarcoma, fibromatosis is characterized by infiltrative growth and a tendency towards recurrence; however, unlike sarcoma, it never develops metastasis . Therefore, the most important strategy is to prevent direct invasion into adjacent tissues. Extraabdominal fibromatosis principally originates from the connective tissue of muscles and the overlying fascia or aponeurosis. It may occur in a variety of anatomical locations, including the muscles of the shoulder, the chest wall and back, thigh, and head and neck. However, solitary occurrence is rare in retroperitoneal space [1, 2]. Here, we report on a case of extraabdominal fibromatosis in the retroperitoneum. Resection was successfully performed, and the patient has been tumor-free for two years after surgery.
Extraabdominal fibromatosis may occur in a variety of anatomic locations; the principle sites of the involvement are the shoulder, chest wall and back, thigh and head and neck. Origin of extraabdominal fibromatosis from any mesenchymal tissue is now well recognized [1, 2]. Several authors have reported retroperitoneal fibromatosis in patients with familial adenomatous polyposis (Gardner syndrome) [2, 4–6], however, solitary occurrence of fibromatosis is very rarely reported [1, 2, 7–9].
Our patient did not have a family history and upper gastrointestinal endoscopy, colonoscopy, or opthalmoscopy were normal suggesting that our patient may be negative for the syndrome. The exact histological origin of the tumor remains to be verified. The findings of CT suggested an origin from paravertebral muscles. Interestingly, this assumption was corroborated by a computed tomography performed in April 2000, which revealed that the previous tumor was located intramuscularly
Principally, complete resection is the therapy of choice for this type of tumors . Adjuvant therapy using non steroidal anti-inflammatory drugs (NSAIDs), tamoxifen, interferon, anti-neoplastic agents, radiation, and a combinations of these, have been reported for cases that are difficult to resect , the exact benefit offered by them is not known due to thin literature. Radiation therapy is accepted as an effective treatment after incomplete resection [11, 12]. Recently, preoperative radiotherapy was reported to be useful for the local control .
In our case the tumor detection was delayed because the psychiatric status of our patient which has been unstable for several years. As wide resection of the tumor reduces the risk of recurrence, an early diagnosis is required for this type of tumor, which is difficult as most of these patients are asymptomatic. While the silent area contains several vital organs, extraabdominal fibromatosis should be considered for the differential diagnosis for such a lesion.
Written consent was obtained from the patient for publication. We thank Mr. Bernd Wuesthoff for editing the manuscript.
- Weiss SW, Goldblum JR: Fibromatosis. Soft tissue tumors (Edited by: Weiss SW, Goldblum JR). St. Louis, Mosby 2001, 309–346.
- Campanacci M, Enneking WF: Aggressive fibromatosis. Bone and soft tissue tumors (Edited by: Campanacci M, Enneking WF). Padova (Italy): Springer Verlag 1999, 925–936.
- Cleeve LK: Retroperitoneal Fibrosis, Tumors, and Cysts. Urologic Surgery (Edited by: Glenn JF). Philadelphia, Lippincott 1991, 404–413.
- Gurbuz AK, Giardiello FM, Petersen GM, Krush AJ, Offerhaus GJ, Booker SV, Kerr MC, Hamilton SR: Desmoid tumors in familial adenomatous polyposis. Gut 1994, 35:377–381.View ArticlePubMed
- Heiskanen I, Jarvinen HJ: Occurrence of desmoid tumors in familial adenomatous polyposis and results of treatment. Int J Colorectal Dis 1996, 11:157–162.View ArticlePubMed
- Rodriguez-Bigas MA, Mahoney MC, Karakousis CP, Petrelli NJ: Desmoid tumors in patients with familial adenomatous polyposis. Cancer 1994, 74:1270–1274.View ArticlePubMed
- Goldblum J, Fletcher JA: Desmoid-type fibromatosis. World Health Organization classification, pathology, and genetics of tumors of soft tissue and bone (Edited by: Fletcher CDM, Unni KK, Mertens F). Lyon, IARC press 2002, 83–84.
- Pignatti G, Barbanti-Brodano G, Ferrari D, Gherlinzoni F, Bertoni F, Bacchini P, Barbieri E, Giunti A, Campanacci M: Extraabdominal desmoid tumor. A study of 83 cases. Clin Orthop 2000, 375:207–213.View ArticlePubMed
- Douglas PJ, Antonio NG, Ivy PA: Local control of extra-abdominal desmoid tumors. J Bone Joint Surg Am 1996, 78:848–854.
- Overhaus M, Decker P, Fischer HP, Textor HJ, Hirner A: Desmoid tumors of the abdominal wall:A case report. [http://www.wjso.com/content/1/1/11] World J Surg Oncol 2003, 1:11.View ArticlePubMed
- Karakousis CP, Mayordomo J, Zografos GC, Driscoll DL: Desmoid tumors of the trunk and extremity. Cancer 1993, 72:1637–1641.View ArticlePubMed
- Schulz-Ertner D, Zierhut D, Mende U, Harms W, Branitzki P, Wannenmacher M: The role of radiation therapy in the management of desmoid tumors. Strahlenther Onkol 2002, 178:78–83.View ArticlePubMed
- O'Dea FJ, Wunder j, Bell RS, Griffin AM, Catton C, O'sullivan B: Preoperative radiation therapy is effective in the treatment of fibromatosis. Clin Orthop 2003, 415:19–24.View ArticlePubMed
This article is published under license to BioMed Central Ltd. This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.