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Ann Thorac Surg 2004;78:1219-1223
© 2004 The Society of Thoracic Surgeons

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Original article: general thoracic

Chest Wall Desmoid Tumors: Results of Surgical Intervention

^a Division of General Thoracic Surgery, Mayo Clinic College of Medicine, Rochester, Minnesota, USA
^b Section of Biostatistics, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

Accepted for publication March 2, 2004.

^* Address reprint requests to Dr Deschamps, Division of General Thoracic Surgery, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905, USA
deschamps.claude{at}mayo.edu

Presented at the Fiftieth Annual Meeting of the Southern Thoracic Surgical Association, Bonita Springs, FL, Nov 13–15, 2003.

	Abstract

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
BACKGROUND: We are analyzing our experience with treatment of desmoid tumors of the chest wall and adjacent structures.

METHODS: A retrospective review was undertaken of the records of all patients who underwent surgical management for a desmoid tumor of the chest between January 1980 and December 2001 at one institution. Fifty-three patients (24 men and 29 women) were identified, whose median age was 39 years (range 10 to 78 years).

RESULTS: The desmoid tumor involved the chest wall exclusively in 25 patients (47%) and both the chest wall and adjacent structures in 28 (53%). Twenty-eight patients (53%) had previous resections for a desmoid tumor. Three patients also had previous radiation therapy. A wide radical resection was attempted in all 53 patients; resection was complete in 44. Seven patients had positive microscopic margins and 2 had gross residual disease. Complications were noted in 6 patients (11%); no operative deaths occurred. Median hospitalization was 6 days (range 1 to 124 days). Nineteen patients (36%) had postoperative radiation therapy (12 had complete resection and 7 had positive margins). Follow-up was complete in 51 patients (96%) and ranged from 2 weeks to 21 years (median 53 months). At the end of the review 46 patients were alive with no recurrence; 3 were alive with local recurrence and 2 died (1 from metastatic breast cancer and 1 from unknown cause). Five-year overall probability of developing a local recurrence was 37.5% (95% confidence interval, 20.2% to 53.3%). Recurrence occurred in 8 of 9 patients with positive margins (89%) and 8 of 44 with negative margins (18%). Factors adversely affecting the rate of postoperative recurrence were reoperation (p = 0.0199), positive margins (p < 0.0001), and postoperative radiation therapy (p = 0.0027). Eleven patients (22%) required reoperation at a median of 24.6 months postoperatively (range 11 to 78 months).

CONCLUSIONS: Desmoid tumors involving the chest and adjacent structures are locally aggressive tumors with a high recurrence rate. Wide radical resection should be attempted whenever possible. Positive margins at resection, reoperation and postoperative radiation are associated with a high risk of local recurrence.

	Introduction

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
Desmoid tumors (DT) are rare soft tissue tumors whose etiology is not completely understood [1, 2]. Extraabdominal DT involve mainly the extremities or the chest wall [3–5]. Irrespective of whether DT is considered an aggressive variant of fibromatosis or a true neoplasm, these tumors are usually managed by wide radical resection [4, 6]. Resection, however, may be a challenge, particularly for recurrent tumors or those involving vital structures of the neck or shoulder [7].

For this report we reviewed our experience in patients who underwent resection of DT of the chest wall and adjacent structures.

	Patients and Methods

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
We reviewed all patients who underwent resection of the chest and adjacent structures for DT at the Mayo Clinic in Rochester, Minnesota, from January 1980 though December 2001. One patient denied use of his data for research purposes and was excluded. The records of the other patients were analyzed for age, sex, symptoms, previous treatment, diagnostic studies, procedure, operative morbidity and mortality, and adjuvant treatment. Tumors were classified as simple if only the chest wall was involved and complex if the tumor involved both the chest wall and adjacent structures. Follow-up data were acquired from review of the medical record. Operative deaths included patients who died within the first 30 days after surgery or during the same hospitalization. Recurrence rates were analyzed.

The cumulative probability of tumor recurrence was estimated using the method of Kaplan and Meier [8]. Rates are reported with 95% confidence intervals (CI). The effects of potential risk factors on local recurrence were evaluated using log-rank tests [9, 10]. All statistical tests were two-sided with the threshold of significance set at p < 0.05. Our Institutional Review Board granted approval for this study.

The study population included 53 patients (24 men and 29 women). Median age at the time of resection was 39 years (range 10 to 78 years). Twenty-eight patients (53%) had had a previous resection for DT, 3 of whom also had previous radiation therapy. Past medical history included local trauma in 4 patients; 7 patients had 10 cancers (3 breast, 2 colon, 2 melanoma, and renal, neurosarcoma, and esophageal 1 each). Another patient was known to have familial adenomatous polyposis (Gardner's syndrome).

The DT was limited to the chest wall in 25 patients. In addition to the chest wall, the scapula was involved in 11 patients, the neck in 10, the clavicle in 9, the axilla in 5, the spine in 4, the brachial plexus in 2, and the thoracic outlet in 2. The tumors were classified as simple in the 25 patients with chest wall involvement only (47%) and complex in the 28 with involvement of both the chest wall and adjacent structures (53%).

Pain waspresent in 22 patients (42%) and limitation of shoulder motion in 5 (9%). A palpable mass was noted in 35 patients (66%). Five patients (10%) had no signs or symptoms. A computed tomography of the chest was performed in 34 patients (64%) and magnetic resonance images were obtained in 17 (32%). A preoperative biopsy (incisional in 12 and fine needle aspiration in 9) was done in 21 patients (40%) and was diagnostic in 13 (25%).

A wide radical resection was attempted in all 53 patients. Full-thickness chest wall resection was performed in 30 patients (57%) and partial-thickness resection in 23 (43%). The median number of ribs resected was three (range one to six ribs). Associated procedures performed included removal of a portion of scapula in 11 patients, sternum in 10, clavicle in 9, wedge excision of the lung in 5, vertebrae in 4, a portion of brachial plexus in 1, and a forequarter amputation in 1. Resection was complete in 44 patients. Seven patients had resection margins that demonstrated microscopic DT; 2 patients had gross residual disease. Twenty-one patients (40%) were reconstructed with Gore-Tex soft tissue patch (W.L. Gore and Assoc, Flagstaff, AZ), 3 (6%) with Prolene mesh (Ethicon, Somerville, NJ), and 1 (2%) with Vicryl mesh (Ethicon). Soft tissue coverage was achieved with transposed muscle in 13 patients (25%) and omentum in 2 (4%). The muscles used for soft tissue coverage included pectoralis major in 6 patients, latissimus dorsi in 4, external oblique in 2, and rectus abdominis in 1.

	Results

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
Complications occurred in 6 patients (11%): pneumonia in 2 patients and wound infection, bleeding, chyle leak, and axillary vein thrombosis in 1 patient each. No operative deaths occurred. Median hospitalization was 6 days (range 1 to 124 days). Nineteen patients (36%) had postoperative external beam radiation therapy, including 7 patients who had positive margins.

Follow-up was complete in 51 patients (96%) and ranged from 4 weeks to 21 years (median 53 months). Forty-six patients were alive at the end of this report without evidence of recurrent DT and 3 were alive with local recurrence. Two patients died—1 from metastatic breast cancer and 1 from unknown cause.

Overall, local recurrences occurred in 16 patients (30%). The rate of recurrence, however, varied according to whether the DT had been previously resected, the margins contained residual disease, or radiation was administered postoperatively. Recurrence developed in 8 of the 9 patients (89%) with positive margins compared with only 8 of the 44 (18%) with negative margins. Recurrences developed in 14 of the 28 patients (50%) who had a prior resection for DT compared with only 2 of the 25 (8%) who did not have a previous resection. In patients with negative margins, recurrence occurred in 8 of the 30 patients (27%) who did not have postoperative radiation therapy compared with none of the 12 (0%) who had radiation therapy. The only recurrences in the postoperative radiation group occurred in patients with positive margins. Recurrences occurred in 4 of the 21 patients (19%) who had a preoperative biopsy compared with 12 of the 32 (38%) who did not. Recurrence occurred in 5 of the 25 patients (20%) who were classified as simple and 11 of the 28 (39%) who were classified as complex.

Two-, 3-, and 5-year probability of developing a local recurrence was 8.9% (95% CI, 0.2% to 16.91%), 23.4% (95% CI, 9.5% to 35.2%), and 37.5% (95% CI, 20.2% to 53.3%), respectively (Fig 1). Factors affecting recurrence were analyzed by univariate analysis (Table 1). Factors adversely affecting the rate of postoperative recurrence were reoperation (p = 0.0199) (Fig 2), ss positive margins (p < 0.0001) (Fig 3), ss and postoperative radiation therapy (p = 0.0027) (Fig 4). Factors not affecting the rate of postoperative recurrence included age, sex, preoperative biopsy, partial- versus full-thickness resection, and simple versus complex tumor.

View larger version (10K): [in this window] [in a new window]   Fig 1. Overall probability of developing a local recurrence in 51 patients who underwent desmoid tumor (DT) resection. Zero time on the abscissa represents the date of DT resection. Vertical bars represent 95% confidence intervals.

View larger version (12K): [in this window] [in a new window]   Fig 2. Probability of developing a local recurrence in the 25 patients who underwent resection for an initial desmoid tumor (DT) versus the 28 patients who underwent resection for recurrent tumor. Zero time on the abscissa represents the date of DT resection. - - - - = reoperation; —— = primary operation. p = 0.0199.

View larger version (13K): [in this window] [in a new window]   Fig 3. Probability of developing a local recurrence in the 8 patients who had positive surgical margins after resection for desmoid tumor (DT) versus the 44 patients who had negative margins. Zero time on the abscissa represents the date of DT resection. - - - - = positive margins; —— = negative margins. p < 0.0001.

View larger version (13K): [in this window] [in a new window]   Fig 4. Probability of developing a local recurrence in the 19 patients who received postoperative radiation after resection for desmoid tumor (DT) versus the 33 patients who received no radiation. Zero time on the abscissa represents the date of DT resection. - - - - = postoperative radiation; —— = no radiation. p = 0.0027.

	Comment

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 
Grossly, DT are poorly circumscribed and located in the muscular aponeurotic structures. These tumors are firm, rubbery, and have a remarkable tendency to infiltrate into surrounding structures [2]. Although distant spread has not been documented in long-term follow-up studies, these tumors have a strong propensity to recur locally after resection [2, 5, 6]. For this reason, wide radical resection with 2- to 4-cm negative margins whenever possible has been considered essential to successful management [7]. However, because of the propensity of the tumor to invade vital structures, obtaining negative margins for DTs involving the chest region often represents a therapeutic challenge for the thoracic surgeon. Other treatment options have included external beam radiation therapy, antiestrogen medications, colchicine, nonsteroidal antiinflammatory agents, and conventional tumor chemotherapy [11–14].

A preoperative biopsy was performed in 40% of our patients but failed to provide a correct diagnosis in most of the patients in whom it was performed. Moreover, attempting a biopsy preoperatively did not affect the rate of postoperative recurrence. In contrast to most chest wall tumors that should be diagnosed by excisional rather than incisional biopsy preoperatively, a preoperative biopsy in patients with DT may be reasonable when the tumor involves vital structures and potentially requires an extensive surgical procedure to achieve complete resection.

In our experience positive margins after resection are associated with a high recurrence rate. Others have reported similar findings [15, 16]. Wide radical resection, while the mainstay of therapy in most cases, may be difficult if not impossible when tumors are immediately adjacent to structures such as the brachial plexus, great vessels, and spine. Because these tumors appear to have a slow growth rate [17], a more conservative approach is reasonable in some instances.

Similar to the findings of other investigators, reoperation was associated with a significant risk of local recurrence [15]. This finding might reflect the increased complexity of the condition of these patients, as demonstrated by the fact that a significant number of reoperations occurred in patients whose initial resection was for complex tumor. Another possible explanation for the increased rate of recurrence in the reoperation group is the difficulty in differentiating both scar tissue and postradiation change from DT on gross and microscopic examination at the time of reoperation.

The role of radiation therapy remains unclear. Postoperative radiation had an adverse effect on local recurrence, but patients who had postoperative radiation were more likely to have previously undergone an incomplete resection. Although none of our patients who had a negative surgical margin and subsequently underwent postoperative radiation had a recurrence, almost a third of the patients who did not have postoperative radiation therapy had recurrent tumor. Whether postoperative radiation therapy is beneficial in all patients who undergo a complete resection cannot be determined from our nonrandomized data. Radiation might have a role in patients with unresectable DT and a need for palliative treatment of pain [13]; however, this subgroup was not evaluated in our series.

We conclude that DT involving the chest and adjacent structures is a locally aggressive tumor with a high risk of recurrence. Wide radical resection should be attempted whenever possible. Positive margins at resection, reoperation, and postoperative radiation are associated with a high risk of local recurrence.

	Discussion

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DR THOMAS D'AMICO (Durham, NC): That was very well presented and is a great contribution to the literature, because, as you said, there really are no large series, and 53 patients for this disease constitutes a very large series. I wonder if you could comment on two issues.

In your conclusion you note that a full thickness, wide radical resection is the treatment of choice and yet a number of your patients only underwent a partial thickness resection. How many of these patients recurred and why did you choose partial thickness? Was it because it was a local recurrence and the patient had already been resected and were there other factors?

And second, why do you only show five-year follow-up for a series that started in 1980? Even though on the graphs it looked like the recurrence rates plateau at five years, they probably do not, and that, like most indolent tumors, we need to follow these patients out for life. So do you think five years is adequate, or what would you recommend for follow-up?

DR ABBAS: Thank you, Dr D'Amico. The first question, why I believe a full thickness resection is required, is that just having the opportunity to review these cases and realizing that many of them were recurrent from outside hospitals, I realized that many had indeed involved the ribs, for example, and many surgeons are hesitant to perform a complete chest wall resection. When they did present to us and did undergo the appropriate therapy in those cases that included a total thickness resection, the recurrence rate was much less.

As far as how long we should follow these up, I totally agree with you, they do not plateau at five years. This was just the median length of follow-up that we had, and I suspect that these patients need to be followed up for life.

DR KAMAL MANSOUR (Atlanta, GA): I enjoyed your paper very much because it reports a large experience over 21 years.

I have two questions. First, what is the role of radiation therapy in your experience? Did you give it preoperatively or postoperatively? Would you give it only to those patients with positive margins or to all patients?

Secondly, is there any place for hormonal therapy in your experience? Any evidence of malignancy in the recurrent cases? I know it may occur but did it occur in your experience? Any sarcomatous transformation?

And finally, what do you do with lesions that are deep in the axilla? Do you perform a forequarter amputation especially in cases where there is invasion of the brachial plexus or axillary vessels?

Thank you.

DR ABBAS: Thank you, Dr Mansour. The role of radiation therapy will be described in more detail in the manuscript.

The only recurrences in the postoperative radiation group were those who had a negative margin, meaning all patients with negative margins who underwent postoperative radiation therapy developed no recurrences. Patients with negative margins who did not have radiation therapy had a 33% risk of recurrence.

As far as the role of hormonal therapy, I believe there have been some reports of an excellent effect of hormonal therapy in these tumors, however, this was not offered to any of the patients in our study.

As far as evidence for metastatic disease, I believe that any desmoid tumor that metastasizes is probably a false diagnosis and probably a low grade fibrosarcoma.

As far as involvement of the axilla, if it is only the brachial plexus, then I think a partial resection of that portion of the brachial plexus is required. If it does indeed involve the whole shoulder joint and extends into more of the upper extremity, then, yes, a four-quarter amputation can be performed and in fact was one of the procedures done for our patients.

DR EDWARD MUNNELL (Oklahoma City, OK): My friend, Kamal Mansour, did it again. He said he was going to have two questions and he had four. So he cut into my time, but I am used to that.

I thought this was a very interesting paper of a unique type of growth. Also it is my understanding most of these occur in the abdomen as far as anatomic geography. This is a historical meeting of the STSA. The Mayo Clinic has many, many firsts in its lifetime.

Your series started in 1980, but actually the first desmoid of the chest was removed in 1956 by my close friend, Frank Gatchell, who was a fellow with Dr Clagett at the time. They resected about the upper third of the chest wall somewhat like a mini Schede thoracoplasty. They had no supporting material to stabilize the chest at the time, and the patient did very well and did survive without any further operation. I thought it might be appropriate to complete your paper to point out that the Mayo Clinic was the first to resect and report a desmoid tumor of the chest. Thank you.

DR ABBAS: Thank you very much.

	References

Top Abstract Introduction Patients and Methods Results Comment Discussion References

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Abbas E. Abbas Claude Deschamps Stephen D. Cassivi Francis C. Nichols, III Mark S. Allen Peter C. Pairolero Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Abbas, A. E. Articles by Pairolero, P. C. Search for Related Content PubMed PubMed Citation Articles by Abbas, A. E. Articles by Pairolero, P. C. Related Collections Chest wall