Asian Cardiovasc Thorac Ann 2006;14:359-362
© 2006 Asia Publishing EXchange Ltd
Desmoid Tumors of the Chest Wall
Charalambos Zisis, PhD,
Apostolos Dountsis, MD,
Athanasios Nikolaides, MD1,
Jubrail Dahabreh, FETCS
Department of Thoracic Surgery
1 Department of Anesthesiology, Athens Medical Center, Athens, Greece
For reprint information contact: Charalambos Zisis, MD Tel: 30 210 965 1639 Fax: 30 210 722 4449 Email: chzisis{at}otenet.gr, 17A, Patriarchou Grigoriou str, Glyfada 166 74, Greece.
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ABSTRACT
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Chest wall desmoid tumors (DT) are rare pathologic entities with microscopic features similar to, or undistinguishable from, fibromas or fibrosarcomas. From 1996 to 2001, four patients with DT were surgically managed in our department. Their ages ranged from 27 years to 43 years (mean 32.25 years, median 29.5 years). A resection of the lesion was performed with negative margins of 4 cm around the tumor (wide resection). A reconstruction of the chest wall was also performed with polytetrafluoroethylene (PTFE) in 2 patients and methylmethacrolate with Marlex mesh in 1 patient. One patient had a recurrence 15 months later, and was admitted for complementary resection, and remains disease-free for 5 years. The rest 3 patients are disease-free for 6 months to 5 years. Resection must include all adjacent, overlying and underlying musculature as well as soft tissues and any spare skin from the procedure should be used. Prognosis after a wide resection is good.
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INTRODUCTION
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Chest wall tumors are rare and present a diversity of histological features. The management of these tumors depends on the histology, size, and depth of invasion. An accurate knowledge of pathology is essential to ensure successful treatment. Desmoid tumors (DT) belong to the group of soft tissue sarcomas and are characteristically associated with familial syndromes, such as familial adenomatous polyposis. They are very uncommon tumors, with multiple potential primary anatomical locations, without metastatic burden, but with a tendency towards local invasiveness and recurrence. The management of these tumors requires specific consideration. They classically appear between the 3rd and 4th decades of life with a female to male ratio of 1.5 : 1.1 Since their pathological features are similar to a low-grade fibrosarcoma, differential diagnosis is difficult.
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PATIENTS AND METHODS
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Four patients with DT of the chest wall were surgically managed between 1996 and 2001 (Table I
). There were three females and one male. Their ages ranged from 27 to 43 years (mean age 32.25 years, median age 29.5 years). Initial symptoms included a painful mass in 2 patients and a simple palpable mass without pain in the other 2 patients. Preoperative evaluations included cerebral, thoracic and abdominal computed tomography (CT) scanning, bone scanning and pulmonary functional tests. A preoperative incisional biopsy without adequate tissue sampling was performed in one patient at another hospital. Postoperatively, diagnostic investigation with gastroscopy and coloscopy was performed in all patients in order to exclude Gardner’s syndrome. None of them were diagnosed to have this syndrome.
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RESULTS
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Two patients (case 1 and case 2) had a wide chest wall resection and reconstruction with polytetrafluoroethylene (PTFE). Both of them have no signs of recurrence after 5 years, and 6 months, respectively (Figure 1A, 1B).
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Figure 1A. Preoperative thoracic CT-scan of case 2 in Table 1 revealing tumor located in the right anterior chest wall.
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Figure 1B. Postoperative thoracic CT-scan of the same patient after resection and reconstruction of the chest wall with PTFE.
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Two female patients (case 3 and case 4) had recurrent tumors. Case 3 had been initially managed conservatively in order to avoid amputational manipulations; hence, a margin less than 3 cm was chosen. Consequently, the disease recurred in 15 months. The patient is now free of symptoms 5 years after complementary management and reconstruction with Marlex mesh and methylmethacrolate (Figure 2A, 2B). Case 4 had resection of a primary tumor located in the posterior chest wall. She had recurrences at 2 years and 4 years, which were managed surgically. Because of the recurrent nature of the disease, a frozen section was taken to differentiate between scar fibrosis and lesion. Complementary resection of the clavicle and of the tip of the scapula was performed in order to achieve safe wide margins.
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Figure 2A. Postoperative chest X-Ray of case 3 in Table 1 with methylmethacrolate and Marlex mesh used for the reconstruction of the chest wall (sandwich technique) in the right upper pulmonary field.
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None of the patients received postoperative therapy, as the resection of the tumor was radical in all cases. Postoperative mechanical ventilator support was not required and the postoperative course was uneventful without cosmetic malformation. Paradoxical respiration did not occur.
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DISCUSSION
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Desmoid tumors are sporadically diagnosed and are correlated, in many cases, especially in pediatric populations, with Gardner’s syndrome. The most common location is in the extremities; first in the lower, and second, in the upper extremities.
According to clinical experience, desmoid tumors, also called deep fibromatoses, should be considered as soft tissue benign tumors (equivalent to low-grade fibrosarcoma but without metastatic potential). Pathologically, they are characterized by bland-appearing fibroblasts, indistinct margins, and ability to spread by local invasion and recurrence2. The precise distinction between the subtly different pathologic entities appears problematic and the current terminology expresses this ambiguity.3 Their size at presentation can be large, because of the asymptomatic nature of the lesion in a large number of patients.4 Their usual clinical appearance is swelling, resulting in a palpable mass or pain due to nerve involvement, which can also give rise to sensory and motor symptoms.5
Chest X-Rays show a soft-tissue mass associated with the ribs, scapula or paravertebral tissues, possibly with periosteal reactive changes or destruction, whereas on computed tomography and on T1-weighted magnetic resonance, DT show attenuation similar to that of muscles.6,7 Incisional biopsy before a definite surgical resection has been advocated, but tumor spillage and contamination may result.8 Skilled cytopathologists can also make a diagnosis through the use of fine-needle aspiration or core needle biopsy in nearly 90% of patients.9
Data concerning treatment and results are limited because of the small number of cases. Recurrence is usual because of the local aggressive nature of the lesion and, when margins are microscopically invaded in the case of incomplete resections. The main problems performing a safe resection with negative margins are:
- Difficulties in manipulation due to limited width in adjacent tissues. Sometimes the surgeon chooses not to extend the resection to avoid affecting vital organs. The effects on the functional, physiologic, cosmetic and psychological field of the patient are also valid aspects.
- Difficulties in diagnosis by pathologists. In many cases, the initial diagnosis of negative margins in frozen section may be transformed in permanent section because of microscopical invasion.
It is crucial to have a safe extended resection in the initial operation, because recurrence creates additional problems due to adhesions, difficulties in manipulation, the need for more amputational operations and, difficulties in differential diagnosis (between simple postoperative fibrosis on a scar and pathological process from the tumor). Recurrence rates for chest wall desmoids have been reported between 25% and 75%. Five-year probability of developing local recurrence has been estimated as 37.5% with factors adversely affecting the rate of postoperative recurrence including positive margins.10 A wide resection with negative margins of at least 4 cm is proposed to minimize any chance of recurrence. The extension of the resection should include all adjacent overlying and underlying soft tissue and muscle layers. The skin should be intact and should be preserved to cover the defects. Although they do not metastisize, DT can cause significant morbidity and occasionally death through locoregional invasion of critical structures.2 Prompt follow-up evaluation in all their life, and aggressive management of the recurrences, without compromising the principles of oncological surgery can secure favorable prognosis.
With the application of reconstructive techniques, morbidity is minimal and cosmetic results satisfactory. Prosthetic material (PTFE and methylmethacrolate) show good performance without complications. Postoperative radiotherapy has been proposed for positive margins or when there are concerns about the adequacy of the margin. The results seem encouraging.11 Neoadjuvant chemoradiotherapy treatment protocols have also been applied12 and adjuvant chemotherapy, as well as other types of systematic biological treatments have been proposed in order to achieve local control of the disease13. However, the ideal treatment has not been established yet, and for this moment, surgery remains the cornerstone of an efficacious management.
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ABSTRACT
INTRODUCTION
