Annals of Thoracic Medicine
: 2009  |  Volume : 4  |  Issue : 3  |  Page : 146--148

Huge intrathoracic desmoid tumor

Majdi Ibrahim, Hasan Sandogji, Abdullah Allam 
 Department of Thoracic Surgery, King Fahad Hospital, Almadinah Almunawarah, Saudi Arabia

Correspondence Address:
Hasan Sandogji
King Fahad Hospital, Almadinah Almunawarah
Saudi Arabia


Desmoid tumors are soft-tissue neoplasms arising from fascial or musculo-aponeurotic structures. Most reported thoracic desmoid tumors originate from the chest wall. However, intrathoracic desmoid tumors are rare. We present a case of a 35-year-old male patient complaining of mild shortness of breath. The patient was diagnosed to have a huge intrathoracic desmoid tumor, which was successfully resected.

How to cite this article:
Ibrahim M, Sandogji H, Allam A. Huge intrathoracic desmoid tumor.Ann Thorac Med 2009;4:146-148

How to cite this URL:
Ibrahim M, Sandogji H, Allam A. Huge intrathoracic desmoid tumor. Ann Thorac Med [serial online] 2009 [cited 2019 Dec 6 ];4:146-148
Available from:

Full Text

Desmoid tumors are soft-tissue neoplasms arising from fascial or musculo-aponeurotic structures. [1],[2] Most reported thoracic desmoid tumors originate from the chest wall. However, intrathoracic desmoid tumors are rare. [3] They are classified as benign as they do not metastasize. [4] Desmoid tumors can, however, exhibit rapid local growth, and clinically they can mimic sarcomas. Their histological appearance can also resemble some malignant neoplasm such as low-grade sarcomas, rendering the differential diagnosis difficult. [5] Desmoid tumors account for approximately 3.5% of fibrous tumors, 0.3% of all solid tumors [6] and only 0.03% of all the neoplasms. [7] Chest wall desmoids account for approximately 20% of all desmoid tumors. Patients with these lesions are often asymptomatic and thus commonly present with lesions greater than 10 cm in size. [8]

 Case Report

A 35-year-old male patient was admitted to the emergency room complaining of mild shortness of breath of 12 weeks' duration. He had history of road traffic accident about 1 year back, with unilateral lower limb fracture. Otherwise, the history was unremarkable.

Clinical examination of the patient showed stable vital signs, one small left supraclavicular lymph node, trachea shifted to the right and decreased air entry on the left side with dull percussion note; otherwise, no abnormality was detected. Blood work-up did not show significant changes. Chest x-ray was done, and it showed homogenous opacity on left hemi-thorax and shifting of the mediastinum to the right. So, CT scan of the chest was done, and it revealed huge left-side intrathoracic mass pushing the mediastinum to the right side, with complete collapse of the left lung [Figure 1]. Fine-needle aspiration of the lymph node was inconclusive. So, incisional biopsy of the intrathoracic mass was done, which showed desmoid tumor.

The patient was prepared for surgery, posterolateral thoracotomy was done and about 20 × 15 × 20 cm lobulated intrathoracic tumor was found. The tumor was attached to the apicolateral portion of the chest wall near the brachial plexus; so we excised the tumor completely except a very small part which was adherent to the area of brachial plexus, and decortication of the left lung was done [Figure 2]. The histopathological study of the mass confirmed the diagnosis. The postoperative recovery period went smoothly and the left lung completely expanded [Figure 3]. The patient was referred to our oncologist, who referred the patient to the oncology center in Jeddah for completion of treatment. The patient has not come for follow-up in our hospital till now.


John Macfarlane first described desmoid tumors in 1832. [9] The overall incidence of desmoid tumors is approximately 2-4 cases per million of the population per year. [10] They are rare tumors, which were given different names: Desmoid fibroma, aggressive fibromatosis, desmomas, and desmoplastic fibroma. Currently the name sarcoma of low-grade malignancy [12] or aggressive fibromatosis [6],[11] is preferred due to its vulnerability to local invasion and frequent recurrences even after complete surgical resection. [9]

They most often arise from the musculo -aponeurosis of the abdominal wall. [13] Common sites of extra-abdominal desmoid tumors include the extremities, head and neck, and chest wall. The incidence of chest wall desmoid tumor has been reported to be 10% to 28%. [1] Although desmoid tumors of the chest wall account for approximately 20% of all desmoid tumors, only 26 case reports (including our case) of intrathoracic desmoid tumors have appeared in the English literature. [10],[14],[15]

Several pathophysiological hypotheses are suggested. [16] Abnormal scarring secondary to previous surgery [6],[17],[18],[19],[20],[21] or chest trauma [22],[23] ; hormonal factor, particularly estrogen; genetic (familial) predisposition, in relation to clonal abnormalities carried on Y chromosome, or the long arm of fifth, which is related to chromosome playing Gardner's syndrome. As many as 33% to 38% of patients with Gardner's syndrome develop desmoid tumors, but only 2% of patients with desmoid tumors have Gardner's syndrome or other pathology (familial adenomatous polyposis, osteomas and other soft-tissue neoplasms) [16],[24],[25],[26],[27],[28] or abnormalities in connective tissue synthesis. Symptoms are rare and result mainly from the local mass effect of tumor encroachment on vital structures or erosion of adjacent bone or joint tissue. [13]

Complete resection of the tumor with a clear surgical margin is currently the mainstay of curative treatment for desmoid tumors. The recurrence rate is high and varies from 29% to 54% in some reported series. [29] Regular follow-up imaging is mandatory even when surgical margins are free of tumors. Re-excision is recommended for local recurrent disease. Other treatment methods, including radiation, chemotherapy, c-AMP modulation, estrogen, and prostaglandin inhibition, have been tried with varying success. [30]


1Posner MC, Shiu MH, Newsome JL, Hajdu SI, Gaynor JJ, Brennan MF. The desmoid tumor. Not a benign disease. Arch Surg 1989;124:191-6.
2Borzellino G, Minicozzi AM, Giovinazzo F, Faggian G, Iuzzolino P, Cordiano C. Intra-thoracic desmoid tumor in a patient with a previous aortocoronary bypass. World J Surg Oncol 2006;10:4:43.
3Iqbal M, Rossoff LJ, Kahn L, Lackner RP. Intrathoracic desmoid tumor mimicking primary lung neoplasm. Ann Thorac Surg 2001;71:1698-700.
4Easter DW, Halasz NA. Recent trends in the management of desmoid tumors. Summary of 19 cases and review of the literature. Ann Surg 1989;210:765-9.
5Oddsson SJ, Kristvinsson H, Jónsson JG, Torfason B, Guethbjartsson T. Desmoid tumor of chest wall-an important differential diagnosis to malignancies. Laeknabladid 2006;92:777-80.
6Aaron AD, O'Mara JW, Legendre KE, Evans SR, Attinger A, Montgomery EA. Chest wall fibromatosis associated with silicone breast implants. Surg Oncol 1996;5:93-9.
7Kinzbrunner B, Ritter S, Domingo J, Rosenthal CJ. Remission of rapidly growing desmoid tumors after tamoxifen therapy. Cancer 1983;54:2011-4.
8Allen PJ, Shriver CD. Desmoid tumors of the chest wall. Semin Thorac Cardiovasc Surg 1999;11:264-9.
9Kabiri EH, Al Aziz S, El Maslout A, Benosman A. Desmoid tumors of the chest wall. Eur J Cardiothorac Surg 2001;19:580-3.
10Tajima S, Hironaka M, Oshikawa K, Bando M, Ohno S, Saito K, et al. Intrathoracic sporadic desmoid tumor with the beta-catenin gene mutation in exon 3 and activated cyclin D1. Respiration 2006;73:558-61. Epub 2005 Aug 30.
11Wilcken N, Tattersall MN. Endocrine therapy of desmoid tumors. Cancer 1991;68:1384-8.
12Mendez-Fernandez M, Gard DA. The desmoid tumors: Benign neoplasm, not a benign disease. Plastic Reconstr Surg 1991;87:956-60.
13Saw E, Yu GS, Mell M. Desmoid tumor of the sternum presenting as an anterior mediastinal mass. Eur J Cardiothorac Surg 1997;11:384-6.
14Takeshima Y, Nakayori F, Nakano T, Shimizu K, Yamashita Y, Toge T, et al. Extra-abdominal desmoid tumor presenting as an intrathoracic tumor: Case report and literature review. Pathol Int 2001;51:824-8.
15Winer-Muram HT, Bowman LC, Parham D. Intrathoracic desmoid tumor: CT and MRI appearance. South Med J 1994;87:1007-9.
16Bridge JA, Sreekantaiah C, Mouron B, Neff J, Sandberg AA, Wolman SR. Clonal chromosomal abnormalities in desmoid tumors: Implications of histopathogenesis. Cancer 1992;69:430-6.
17Shimizu J, Kawaura Y, Tatsuzawa Y, Maeda K, Oda M, Kawashima A. Desmoid tumor of the chest wall following chest surgery: Report of a case. Surg Today 1999;29:945-7.
18Dale PS, Wardlaw JC, Wootton DG, Resnick JI, Giuliano AE. Desmoid tumor occurring after reconstruction mammaplasty for breast carcinoma. Ann Plast Surg 1995;35:515-8.
19Schiller VL, Arndt RD, Brenner RJ. Aggressive fibromatosis of the chest associated with a silicone breast implant. Chest 1995;108:1466-8.
20Khanfir K, Guinebretiere JM, Vanel D, Barreau-Pouhaer L, Bonvalot S, Le Cesne A. Unusual problems in breast cancer and a rare lung cancer case. Case 2. Aggressive fibromatosis of the chest wall arising near breast prosthesis. J Clin Oncol 2003;21:2216-8.
21Lynch JJ, Parvizi J, Scheithauer BW, Krauss WE. Development of postoperative fibromatosis after resection of an intraspinal meningioma. Case report. J Neurosurg 1999;90:121-4.
22Icard P, Le Rochais JP, Galateau F, Evrard C. Desmoid fibromatosis of the shoulder and of the upper chest wall following a clavicular fracture. Eur J Cardio-thorac Surg 1999;15:723-5.
23Wiel Marin A, Romagnoli A, Carlucci I, Veneziani A, Mercuri M, Destito C. Thoracic desmoid tumors: A rare evolution of rib fracture. Etiopathogenesis and therapeutic considerations. G Chir 1995;16:341-3.
24Seiter K, Kemeny N. Successful treatment of a desmoid tumor with doxorubicin. Cancer 1993;71:2242-4.
25Mole MT, Goldstraw P, Sheppard MN. Desmoid tumor in thoracotomy scar 5 years after excision of a left giant atrial appendage aneurysm in female with a family history of Gardner's syndrome. Thorac Cardiovasc Surg 1992;40:300-2.
26Mariani L, Martinoli S. Desmoid tumor and Gardner syndrome: A little known surgical dilemma. Presentation of 2 cases. Helv Chir Acta 1990;57:67-71.
27Eden CG, Breach NM, Goldstraw P. Treatment of desmoid tumors in Gardner's syndrome. Thorax. 1992;47:662-3.
28Loccufier A, Vanhulle A, Moreels R, Deruyter L, Legley W. Gardner syndrome and desmoid tumors. Acta Chir Belg 1993;93:230-2.
29Brodsky JT, Gordon MS, Hajdu SI, Burt M. Desmoid tumors of the chest wall: A locally recurrent problem. J Thorac Cardiovasc Surg 1992;104:900-3.
30Thompson DH, Khan A, Gonzalez C, Auclair P. Juvenile aggressive fibromatosis: Report of three cases and review of the literature. Ear Nose Throat J 1991;70:462-8.