Ann Thorac Surg 2003;76:937-939
© 2003 The Society of Thoracic Surgeons
Case report
Desmoid tumor of the chest wall with pleural involvement
Thomas K. Varghese, Jr, MDa,
Rohit Gupta, MDb,
Anjana V. Yeldandi, MDb,
Sudhir R. Sundaresan, MD*a
a Division of Cardiothoracic Surgery, Department of Surgery, Chicago, Illinois, USA
b Department of Pathology, Northwestern University, Feinberg School of Medicine, Chicago, Illinois, USA
Accepted for publication February 15, 2003.
* Address reprint requests to Dr Sundaresan, Division of Thoracic Surgery, The Ottawa Hospital, General Campus, 6NW, Room 6356, 501 Smyth Rd, Ottawa, Ontario, Canada K1H 8L6
e-mail: ssundaresan{at}ottawahospital.on.ca
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Abstract
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Although there have been reports of desmoid tumors of the chest wall, pleural extension, as well as overall size greater than 20 cm, is rare. We present the case of a large desmoid tumor involving the left anterior chest wall, upper abdomen, and diaphragm, which impinged on the left lung and displaced the liver. Wide surgical excision, reconstruction, and differential diagnosis from fibrosarcoma are essential elements in the treatment of these rare tumors.
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Introduction
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Desmoid tumors (aggressive fibromatoses) are rare soft tissue tumors, accounting for 0.03% of all neoplasms [1]. Twenty percent of patients with desmoid tumors have involvement of the chest wall [2], usually presenting as a palpable mass. These tumors can be painful due to nerve involvement [3], or these tumors can be asymptomatic as in the majority of reported cases in the literature, which were approximately 5 to 10 cm in diameter [2]. We report a patient with a rare, significantly larger desmoid tumor of the chest wall with associated pleural involvement.
A 33-year-old woman with a past medical history of hypertension was referred for management of a chest wall mass. One year prior the patient had noted a small lump under the left breast, which had gradually increased in size. Over the course of 2 months, the patient had dyspnea on exertion and anterior chest pain develop, associated with a 20-pound weight loss. There was no antecedent history of trauma, exposure history, or significant surgical history. Initially the patient was admitted to the intensive care unit at a community hospital after presenting with acute onset of shortness of breath. On physical examination there was a palpable 10 x 10-cm left anterior chest wall mass. Computed tomographic scan revealed a massive soft tissue tumor extending through the chest wall into the left pleural space, causing compression of the left lung. There was associated extension to the upper abdomen with rightward displacement of the liver (Fig 1).
Open biopsy suggested a desmoid tumor. During her hospital stay, she had an acute deep venous thrombosis develop and subsequently bilateral pulmonary emboli. She was anticoagulated, had Greenfield filter placement, and was discharged home to permit some degree of recovery before surgery. At the time of admission to our institution she had clinically improved, although she was still using supplemental oxygen. Through a modified Mercedes-Benz incision, the tumor of the left thoraco-abdominal region and portion of the diaphragm were resected. The specimen was removed with margins of at least 4 cm in all directions, including removal of portions of rectus and intercostal muscles with attached ribs and sternum. An oval piece of 2-mm Gore-Tex patch (W.L. Gore & Assoc, Flagstaff, AZ) was used to reconstruct the chest wall and periphery of the diaphragm, and a large piece of bidirectional 1-mm Gore-Tex (W.L. Gore & Assoc) was used to reconstruct the abdominal wall. Chest tube and drains were placed, followed by skin closure.
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Fig 1. Computed tomographic scan demonstrating large tumor of chest wall with intraabdominal extension displacing the liver. (T = tumor; L = liver.)
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Postoperative recovery was unremarkable, and the patient was discharged on postoperative day 12. The patient was readmitted a week later with complaints of serous drainage from the abdominal wound. On physical examination there was separation of a 2-inch area in the upper portion of the abdominal incision. Reexploration revealed a dehisced abdominal wall Gore-Tex (W.L. Gore & Assoc) without evidence of infection, which was removed. Because of the low evisceration risk, after thorough irrigation the skin incision was closed over drains. Postoperative recovery was unremarkable, and the patient continues to do well 7 months later.
The specimen was a bilobed mass weighing 4.35 kg, measuring 20 x 15 x 9 cm intrathoracic, and 20 x 12 x 12 cm extrathoracic (Fig 2).
Sectioning revealed an infiltrative, firm pale-white mass with a trabeculated cut surface (Fig 3).
Microscopy demonstrated a variably cellular spindle cell lesion with fascicular architecture and dense collagenous bands. The cells had tapered nuclei and indistinct cytoplasm (Fig 4).
Throughout the majority of the specimen, mitotic activity was less than one tenth per high-power field; however, a single area displayed 2 mitoses per 10 high-power field. Atypical mitotic figures and cytologic atypia were absent. The tumor cells were negative for p53, estrogen, and progesterone receptors.
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Fig 2. Surgical specimen. (A = abdominal extension; CW = chest wall; D = diaphragm; E = extrathoracic portion.)
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Comment
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Desmoid tumors arise from fascia and connective tissue of the muscular layers and invade surrounding structures. Desmoid tumors may occur at the site of any fascia with approximately 50% arising in the abdominal region [4]. Of the extra-abdominal sites, the chest wall is the most common [5], whereas the other sites are the shoulder girdle and inguinal regions.
Although the cause is not well defined, there are some recognized predisposing factors. A history of trauma can be found in 25% of patients [4]. There is also a close association with familial adenomatous polyposis and Gardner’s syndrome, suggesting the role of an intrinsic genetic defect in the development of desmoid tumors. Estrogen has also been implicated in the multifactorial development of desmoids because they tend to occur in women of reproductive age, some tumors express estrogen and progesterone receptors, and reports illustrate regression with tamoxifen therapy [6].
Treatment of these tumors involves surgical resection, stressing wide resection of the involved soft tissues and bony structures. Radiation therapy is usually used when a wide local excision cannot be accomplished. Definitive diagnosis requires histologic assessment when the main differential diagnosis is fibrosarcoma. Focal increased cellularity, bland cytology, rare mitotic activity, and absence of herringbone pattern favor fibromatosis over fibrosarcoma [7]. Extra-abdominal desmoid tumors have a high local recurrence rate after resection. In a recent series of chest wall desmoid tumors [8], although the overall 5-year survival rate was 93%, the 5-year local recurrence rate despite aggressive surgical intervention was 29%. This lends credence to the fact that continued vigilance in our patient for recurrence is warranted even after attaining optimal wide tumor-free surgical resection margins. However, these tumors do not metastasize, and previously reported metastases probably represents fibrosarcomas rather than desmoid tumors.
Our patient had a typical age of presentation, but the size and location of her lesion was unusual. Although chest wall fibromatosis is well described, few cases involve the pleura. In the literature, most tumors range from 5 to 10 cm and rarely exceed 20 cm; our patient had intra- and extra-thoracic components greater than 20 cm. Although this tumor had an isolated area with increased mitotic activity, histologic appearance is the best characteristic of aggressive fibromatosis. This case illustrates the complexities involved in the treatment of these rare tumors.
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References
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- Weiss S.W., Goldblum J.R. Fibromatoses. In: Weiss S.W., Goldblum J.R., eds. Enzinger and Weiss’s soft tissue tumors, 4th edit St. Louis, MO: Mosby, Inc, 2001:320-329.
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Abstract
Introduction
