Eur J Cardiothorac Surg 2001;19:580-583
© 2001 Elsevier Science NL
Desmoid tumors of the chest wall
El Hassane Kabiria,
Said Al Aziza,
Abderrahman El Maslouta,
Abdelatif Benosmanb
a Department of Thoracic Surgery, Ibn Sina University Hospital, Rabat, Morocco
b Service de Chirurgie Thoracique, CHU Ibn Sina, Rabat, Morocco
Received 2 May 2000;
received in revised form 31 January 2001;
accepted 21 February 2001.
Corresponding author. 12 C 15 Rue Alloysia, Hay Riad 10100, Rabat, Morocco. Tel.: +212-37-71-17-37; fax: +212-61-81-85-45
e-mail: hassankabiri{at}yahoo.com
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Abstract
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Objective: Desmoid tumors of the chest wall are uncommon fibromatous tumors characterized by their local invasion and frequent recurrences. They are considered low-grade malignant sarcomas. We analyze our results and evaluate our experience in their surgical treatment. Patients and methods: From 1980 and 1999, eight patients were operated in our service for desmoid tumors of the chest wall. There were five males and three females with a mean age of 32 years old (17–52 years). The diagnosis was established on the basis of clinical signs and imaging studies (Chest X-ray and computed tomography). Surgery was done by direct approach of the tumors. Results: None of the patients had Gardner's syndrome. Complete resection was done in seven of the eight cases. Histology confirmed all cases. The follow-up ranged from 24 and 180 months, for a mean of 82 months. There was one death from cardiac and renal failure 3 months after a fifth resection associated with adjuvant radiation therapy. No other post-operative complications were observed. Recurrences occurred in 50% of cases, all treated by surgery, associated to radiotherapy in one case. Conclusion: Surgical treatment of primary desmoid tumors of the chest wall requires wide local resection. In cases of recurrences, multimodality therapies should be considered.
Key Words: Desmoid tumor • Chest wall • Surgery • Recurrences
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1. Introduction
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Desmoid tumors are rare connective tumors. They account for approximately 3.5% of fibrous tumors, 0.3% of all solid tumors [1], and only 0.03% of all the neoplasms [2]. Local invasion and frequent recurrences [3–7] characterize this tumor, considered a low-grade sarcoma even if it does not metastasize [5,8].
In this report we describe the surgical treatment, analyze our results and review the literature.
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2. Patients and methods
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In a retrospective study of the last 20 years, from January 1980 to December 1999, eight patients were treated for desmoid tumor of the chest wall.
None of the patients had a Gardner's syndrome, meaning that none presented cutaneous cysts, bone signs or intestinal polyposis (checked by colonoscopy).
CT needle biopsy was done in four cases and the suggested diagnosis at that time was a non-specific inflammatory process. Surgical biopsy was done in two cases with a positive diagnosis for both. In two cases (patient 5 and 8) local recurrences of a previously treated cancer was suspected and the diagnosis of desmoid tumor was made only post-operatively.
Surgical treatment of these tumors consisted of a direct approach and complete local resection of each tumor in seven cases and one incomplete excision with microscopic positive margins. The pleura was entered in four cases (cases with rib resection) after precise the upper limit of the tumor, the intercostal space was opened, the lower limit was fixed and all the structures involved by the tumor between this limits was excised. In all cases we have excised skin, subcutaneous tissue, muscle, ribs, intercostal muscles and parietal pleura, there was no pulmonary resection. The number of ribs removed varied, two or three with margins of 5 cm, so that chest wall reconstruction was necessary in only three cases. In one case, a myocutaneous flap was solely done for chest reconstruction, the tumor being larger than 7 cm but the chest wall was having been modified by a previous procedure.
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3. Results
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There were five males and three females with a mean age of 32 years old, ranging from 17 to 52 years. Two patients had previous operations for other types of cancer: one had lobectomy for left lung carcinoma 13 years before, and another had a mastectomy for right breast carcinoma 6 years previously.
Clinical presentation consisted essentially of chest pain in 62.5% of cases. All other patients (37.5%) were asymptomatic. All underwent chest X-ray and computed tomography (CT). There were three right and five left-sided lesions on the chest. Sagitally, seven of them were lateral and one anterior. Their size ranged from 2 to 13 cm for a mean of 6 cm. Chest wall infiltration was deep in five cases and superficial in three cases.
The thoracic cavity was drained in four cases (patients with costal resection) for a period of 1 to 3 days. There were no post-operative complications and no mortality. The mean hospital stay was of 4.1 days, ranging 3–7 days.
The mean follow-up is 82 months, from 24 to 180. Fifty percent of patients presented one or multiple recurrences (1–5, mean 2.75) and were treated each time by local resection. In one case, adjuvant radiation therapy (50 Gy) was done after the 5th recurrence on a female patient who had right mastectomy 6 years before. She died 3 months later from cardiac and renal failure.
At the last follow-up visit, seven patients were free of disease on physical examination and radiological investigations (Table 1).
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4. Discussion
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John Macfarlane first described desmoid tumors in 1832. 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 [9] or aggressive fibromatosis [1,10] is preferred due to its tendency to local invasion and frequent recurrences even after complete surgical resection.
Desmoid tumors are most frequently located in the abdomen, which represents 50% of cases. Extra-abdominal sites are mainly the scapular girdle, the pelvic girdle and distal ends of the lower limbs [10]. The chest wall represents 8–10% of cases [3], and the tumor is exceptionally intrathoracic [11].
These tumors are usually more frequent in females than in males, in their twenties (18 and 30 years old) [10]. Our series shows a reverse ratio but other series present variable sex ratio too.
- Several physiopathological hypothesis are suggested [12]:
- Abnormal scarring secondary to previous surgery (two cases in our series) [1,13,14], or chest trauma [15].
- Hormonal factor: particularly oestrogen.
- Genetic (familial) predisposition, in relation with clonal abnormalities carried on Y chromosome or the long arm of fifth, which is related to chromosome playing Gardner's syndrome. Thirty-three percent to 38% of patients with Gardner's syndrome develop desmoid tumors but only 2% of patients with desmoid tumor have Gardner's syndrome or other pathology (familial adenomatous polyposis, osteomas, and other soft tissue neoplasms) [12,16].
- Abnormalities in connective tissue synthesis.
Desmoid tumors were usually non-inflammatory asymptomatic masses; they become symptomatic with compresses of surrounding structures, especially nerves. Usually, they will do so when already relatively large imposing important surgical mutilations.
Imaging investigations (Chest X-ray, CT, MRI) [5] will precise the characteristics of the tumor. Needle biopsy has minimal interest, being frequently negative like in four cases of our series. Surgical biopsy and histology will confirm the diagnosis.
Desmoid tumors [4,5,11] start from connective tissue of fascias, aponeurosis or muscle striae. They do not show any significant mitotic activity or cytological aspect of malignancy. Electronic microscopy shows fibroblastic or myofibroblastic proliferation with intracellular collagen fibrillae [17]. This fibrillae are normally synthesized in the extra-cellular space from precursors secreted by the cells. The presence of these mature fibrillae outside cytoplasm is characteristic of desmoid tumors and this aspect is exceptionally observed in fibroblastic cancers, mesenchymatous tumors or inflammatory process [17].
Treatment of desmoid tumors is still not standardized. It should combine several modalities to prevent the important risk of recurrences.
- Surgery is the best primary treatment, but sometimes the tumor is non-resectable because of extension to vital structures. Surgical margins must be negative in order to minimize the risk of recurrence [18]. In cases of major chest resections, different reconstructive procedures are possible: prosthetic material, autologous tissue [4,19,20], pasteurized autogenous bone grafts [6]. We did none of these in our series.
- Radiotherapy is used either as adjuvant to surgery or for cases of incomplete resections [8,14].
- Others treatments not used in our series are proposed for unoperable, non-resectable or recurrent desmoid tumors:
- Chemotherapy: it is not formally suggested but can be given on the basis of two arguments [15,16,21]: similar origin as sarcomas (connective tissue) and the difficulty to distinct desmoid tumor from low-grade fibrosarcomas. It is used as adjuvant therapy to the surgical treatment. Different drugs can be used (Doxorubicin, Vinblastine, Methotrexate, Association Doxorubicine- Dactinomycin- Vincristine...).
- Colchicine, this therapy has been reported to reduce the size of the tumor 40 [17].
- Hormonal treatment (analogues of oestrogen) is indicated when there are oestrogen receptors in the resected tumors.
- Non-steroidal anti-inflammatory drugs associated to ascorbic acid [11,17,22].
- Posner and coworkers [23] suggest different factors of severe prognosis:
- 18–30 age group,
- Multiple recurrences,
- Incomplete excision,
- Positive margins of resection,
- Absence of adjuvant radiation therapy.
Our experience did not show that age influenced the prognosis. The worst factors of severe prognosis in our cases were deep location and the positive margins of resection. In our series the patients except one (Patient 5) did not have adjuvant radiotherapy, we think that the absence of radiotherapy not influenced the prognosis but perhaps was responsible of the elevated recurrence rate.
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5. Conclusion
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Desmoid tumors are rare, benign but locally aggressive tumors by their reported important risk of local recurrences of 40% [11], 75% [4] to 100% [24]. Surgery is stilling the best treatment though sometimes mutilations in some cases of large tumors.
The follow-up of desmoid tumors must be on a long [5]. Late recurrences can occur even completely excised lesions, may be in relation with some permeation node.
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Top
Abstract
1. Introduction
