SCIENTIFIC ARTICLE
Desmoid Tumors of the Upper Extremity Matthew T. Houdek, MD, Peter S. Rose, MD, Sanjeev Kakar, MD
Purpose To determine the outcomes of surgical excision with or without adjuvant treatment in the management of desmoid tumors of the upper extremity. Methods We retrospectively reviewed 52 patients with a histologically confirmed desmoid tumor in the upper extremity that was managed surgically. All patients presented between 1970 and 2011 and had a minimum 2-year follow-up. Results There were 25 males and 27 females with an average age of 37 17 years. The most common location was the shoulder (n ¼ 27). The most common symptom was a painful mass (n ¼ 30). Average tumor size was 189 371 cm3. Negative margins (wide or marginal resection) were achieved in 43 patients. The 5-year disease-free interval was 57%. Patients with recurrence were younger than those without (31 vs 43 y). Postoperative radiotherapy increased the time to recurrence (2.6 vs 1.6 y) but ultimate disease-free interval at 5 years was similar in patients who did and did not receive radiotherapy. Compared with the preoperative evaluation, there was a significant reduction in patients reporting moderate or severe pain postoperatively. Conclusions Desmoid tumors are locally aggressive fibrous tumors. Recurrence after surgical excision of a desmoid tumor in the upper extremity is common, especially in younger patients. Adjuvant radiation therapy tended to increase time to recurrence but not rate of recurrence. (J Hand Surg Am. 2014;39(9):1761e1765. Copyright Ó 2014 by the American Society for Surgery of the Hand. All rights reserved.) Type of study/level of evidence Therapeutic IV. Key words Desmoid tumors.
E
XTRA-ABDOMINAL DESMOID
tumors are benign fibroblastic tumors that arise from the fascia or deep muscle and aponeurosis.1e4 Described by MacFarlane in 1832, desmoid tumors are rare, with an estimated 900 new cases diagnosed in the United States each year.2 Although desmoids are considered benign, they are locally aggressive with an infiltrative growth pattern that can hamper the efforts of local control.2,5,6 Mortality from these tumors is rare but
From the Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN. Received for publication October 9, 2013; accepted in revised form June 11, 2014. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Sanjeev Kakar, MD, Department of Orthopedic Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905; e-mail: [email protected]. 0363-5023/14/3909-0015$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2014.06.015
morbidity from the tumors and from treatment can be profound.2 Primary treatment options include surgical resection, radiation therapy, or both. Chemotherapy, nonsteroidal anti-inflammatories, and anti-estrogen agents may provide benefit.1,2,5,7e9 Even with treatment, local recurrence remains common, occurring in up to 68% of patients.10 Previous studies examining extra-abdominal desmoids combine tumors arising in both the upper and lower extremity.1,5,6,9e12 The purpose of this study was to compare the clinical outcomes of extraabdominal desmoid tumors located in the upper extremity treated surgically with or without adjuvant radiation. MATERIALS AND METHODS After we obtained institutional review board approval, we retrospectively reviewed the medical records of
Ó 2014 ASSH
r
Published by Elsevier, Inc. All rights reserved.
r
1761
1762
DESMOID TUMORS
TABLE 1.
Desmoid Tumors of the Upper Extremity
Location
All Upper Extremity
Shoulder
Arm
Forearm
Hand
Total number of patients
52
27
7
15
3
Males
25
14
3
7
1
Females Average age, y Tumor size, cm
2
13
4
8
2
38
38
49
34
28
7
8
5
7
4
Dominant extremity
29
16
2
10
1
Painful mass
30
17
3
7
1
Mass increasing in size
13
4
3
6
0
Surgery alone
25
11
4
8
2
Surgery plus radiation
27
16
3
7
1
Recurrence (%)
43
39
14
63
33
Treatment
Pain score
Preoperative
Postoperative
Severe or moderate
31
0
None or mild
21
52
57 patients with pathologically confirmed desmoid tumor of the upper extremity who were treated surgically between 1970 and 2011. Three patients were lost to follow-up and 2 were removed from the study because they received chemotherapy in addition to radiation therapy. This resulted in a study population of 52 patients. Patients were treated with either surgery (n ¼ 25) or surgery plus postoperative radiation (n ¼ 27). All surgery was performed by staff orthopedic surgeons at our institution. Staff pathologists reviewed all pathology slides. Likewise, radiation protocols instituted were at the discretion of staff radiation oncologists. All patients had a minimum 2 years of follow-up (average, 9 y; range, 2e30 y), which included a focused physical examination and imaging. Recurrence was defined as the return of a mass on physical examination or on imaging studies. Our current practice is to observe these patients every 4 months for the first 2 years with a physical examination and cross-sectional imaging (magnetic resonance imaging or computed tomography). After that, we observe the patients every 6 months indefinitely. Patient pain level was recorded at the preoperative and postoperative visits based on a numeric (1e10) rating system (Table 1). A pain score of 1 to 4 was considered mild, 5 to 7 was moderate, and greater than 7 was severe. We performed statistical analysis of categorical data using Fisher exact test and analyzed nominal data using Student t test employing 2-tailed statistics with P ¼ .05 considered statistically significant. J Hand Surg Am.
r
Spearman rank correlation coefficients were used to calculate correlations between variables. Odds ratios were calculated to determine predictive features of recurrence. RESULTS There were 27 females (52%) and 25 males in our cohort, presenting at an average age of 37 17 years (range, 12e85 y). Tumors averaged 189 371 cm3 in size (range, 2e2,400 cm3) and involved the dominant extremity in 56% of the patients (n ¼ 32). There was a minimal negative correlation (r ¼ e0.12) between patient age and size of the tumor. The most common presenting problem was a painful mass. A total of 38 patients remembered a previous traumatic event (bruise, broken bone, etc) to the extremity or had previous surgery in the region in which the desmoid tumor was located. Thirty patients underwent preoperative magnetic resonance imaging; however, in all patients the diagnosis of a desmoid tumor was not made with this imaging. Preoperative biopsy (either open or needle core) was obtained in 65% of patients (n ¼ 34). At the time of surgical excision, the tumor was found to abut the periosteum in 52% of patients (n ¼ 27) and was in intimate contact with a neurovascular bundle in 38% (n ¼ 20). Thirty-six patients underwent a wide excision, 12 underwent a marginal surgical excision, and 3 underwent tumor debulking. Five patients with a marginal excision had a positive Vol. 39, September 2014
DESMOID TUMORS
1763
FIGURE 1: Disease-free interval analysis of surgically treated desmoid tumors of the upper extremity. The overall 5-year disease-free interval was 57%. The best disease-free interval rate was obtained in desmoid tumors of the arm, whereas the worse rate occurred in the forearm.
surgical margin. Four required a free tissue transfer (free fibula, n ¼ 3; or free latissimus, n ¼ 1) to reconstruct and provide soft tissue coverage in the forearm. At the time of the tumor excision, in an attempt to achieve a negative surgical margin, multiple margins were taken in 37% of patients (n ¼ 19). In 83% (n ¼ 43), the ultimate surgical margin was negative either at the initial margin (n ¼ 27) or at the subsequent margin after further excision (n ¼ 16). Postoperative complications included symptomatic nonunions of the free fibula fixation requiring revision (n ¼ 2), postoperative hematoma requiring irrigation and debridement (n ¼ 1), and an elbow capsulotomy resulting from a flexion contracture (n ¼ 1). The 5-year disease-free interval was 57% (Fig. 1). Mean time to local recurrence was 25 months (range, 2 mo to 5.3 y) after surgery. Treatment for recurrence included repeated wide excision (n ¼ 14), marginal excision (n ¼ 3), tumor debulking (n ¼ 1), amputation (n ¼ 1), chemotherapy and radiation therapy (n ¼ 2), and observation alone (n ¼ 2). Further local recurrence occurred in 35% of these patients (n ¼ 9). A total of 83% (n ¼ 19) of recurrent tumors were histologically confirmed as desmoid tumors at the time of re-excision. In the remaining 17% of patients with recurrences the mass was not rebiopsied or excised, and the decision was made to treat the mass either with medical therapies or observation. In these J Hand Surg Am.
r
patients the recurrent mass abutted a critical structure (nerve or blood vessels), which the patient or surgeon thought would lead to notable morbidity if treated surgically. In patients with recurrent tumors, the mean age of surgical excision was 31 years, whereas for those without evidence of recurrence the average age was 43 years; this was statistically significant (P ¼ .01). There was no significance between patient’s sex and tumor size (Table 2). Recurrence was not affected by the use of adjuvant radiation, a positive surgical margin, re-excision of a positive margin, or a tumor that was in intimate contact with a neurovascular bundle or abutted the periosteum. There was no difference in 5-year disease-free interval for surgery alone versus surgery with addition of adjuvant therapy (58% vs 57%; P ¼ .70). However, among patients who had recurrence, those who underwent adjuvant postoperative radiation therapy tended to have a longer time to recurrence than patients undergoing surgical excision alone (3 vs 2 y; P ¼ .14). Table 3 details comparisons between patients who received adjuvant radiation therapy and those who underwent surgery alone. There was no significant difference in age, sex, location, margin status, or relationship to a neurovascular structure (Table 3). Patients in the adjuvant radiation treatment group tended to have larger tumors; however, this was not Vol. 39, September 2014
1764
TABLE 2.
DESMOID TUMORS
Factors That Could Influence Recurrence of Upper Extremity Desmoid Tumors
Total Patients, n Average age, y Size of tumor, cm3
Recurrence
No Recurrence
23
29
Odds Ratio
95% Confidence Interval
P Value
31
43
.01
267
125
.17
6
3
0.32
0.07e1.48
.16
History of trauma or previous surgery
Positive surgical margin
18
19
0.52
0.15e1.84
.36
Intimate contact with neurovascular bundle
11
9
0.49
0.15e1.52
.26
Multiple margins taken
10
9
0.58
0.18e1.82
.39
Periosteal invasion
13
14
0.71
0.23e2.15
Adjuvant radiation treatment
12
15
0.98
0.33e2.94
TABLE 3. Comparison of Patients Undergoing Surgery Alone Versus Surgery and Radiation
Average age, y Males Size of tumor, cm3 Shoulder mass
Surgery Alone (n ¼ 25)
Surgery Plus Radiation (n ¼ 27)
P Value
41
34
.11 .40
11
14
102
274
.09
11
14
.40
Forearm mass
8
7
.76
Positive margin
3
6
.46
Periosteal abutment
9
18
.02
Close to neurovascular bundle
9
11
.77
Multiple margins taken
11
8
.56
statistically significant. Likewise, periosteal abutment of the desmoid tumor was significantly more common in the adjuvant radiation group compared with the group treated only surgically. There was a significant reduction in the number of patients reporting moderate or severe pain between the preoperative and postoperative settings. At last follow-up all patients reported either mild or no pain (Table 1). DISCUSSION Recurrence after surgical excision of upper extremity desmoid tumors with or without adjuvant postoperative treatment in this study was high and similar to studies reporting tumors from both the upper and lower extremities. In previous studies, patients with J Hand Surg Am.
r
.59 1.0
primary extremity desmoid tumors had 5-year diseasefree intervals ranging from 53% to 72% compared with 57% for our study.1,10,13,14 There were several limitations to this study. Its retrospective nature limited the amount of data we were able to collect from the medical record and constrained the analysis. All of the lesions in this study were surgically excised; as such, we were unable to comment on the natural history of desmoid tumors in the upper extremity treated nonsurgically or with other modalities alone. Although all of these tumors were treated at one institution, the surgeries and adjuvant therapies were performed by multiple providers, and there was no standardized protocol for their treatment or follow-up at our institution. These differences in surgical and adjuvant treatment protocols could potentially influence outcomes. Likewise, not all patients received the same preoperative and postoperative imaging. Previous studies examining the prognostic factors of disease-free intervals have not been able to adequately predict recurrence.15 Within our study, patients with recurrence were younger than those without recurrence. In the upper extremity the ability to achieve adequate surgical margins can be associated with patient morbidity owing to the removal of nerves, tendons, and other critical structures.9,15,16 Margin status has been debated as a factor in the recurrence of desmoid tumors.12,13,15,17e19 The infiltrative nature of desmoids tumors and the fact that they frequently lack a pseudocapsule make it difficult to determine the true extent of disease at the time of excision.15,17 A positive surgical margin did not increase the odds of recurrence in our study. Likewise, having to excise multiple biopsy samples to achieve a negative surgical margin did not increase the odds of recurrence. Vol. 39, September 2014
DESMOID TUMORS
To increase the disease-free interval, adjuvant radiotherapy is used.9,11,13,20 The addition of radiotherapy increases the disease-free interval compared with surgery alone.9,11,13,20 In this study, the use of radiotherapy increased the time to recurrence but the 5-year disease-free interval was similar between patients who did and did not receive radiotherapy, although we recognize the inherent limitations of the current study methodology for considering this conclusion. Previous work has demonstrated that the use of radiation therapy as an adjuvant is especially useful in cases where complete excision of the desmoids tumor would cause substantial morbidity.9 Although there was no difference in the ultimate recurrence rate between these patients, we think the addition of radiation therapy should be considered in cases where it would be difficult to obtain adequate surgical margins. Unfortunately, recurrence is a common problem despite aggressive surgical treatment. We think that younger patients in particular should be counseled regarding their increased risk for recurrence. Although adjuvant radiation treatment did not reduce the rate of recurrence, it tended to increase the disease-free interval in surgically treated desmoids. REFERENCES 1. Chew C, Reid R, O’Dwyer PJ. Evaluation of the long term outcome of patients with extremity desmoids. Eur J Surg Oncol. 2004;30(4): 428e432. 2. Hosalkar HS, Torbert JT, Fox EJ, et al. Musculoskeletal desmoid tumors. J Am Acad Orthop Surg. 2008;16(4):188e198. 3. Micke O, Seegenschmiedt MH. Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national Patterns of Care study. Int J Rad Oncol. 2005;61(3):882e891. 4. Pakos EE, Tsekeris PG, Goussia AC. Desmoid tumours of the extremities and trunk: a review of the literature. Int Orthop. 2005;29(4): 210e213.
J Hand Surg Am.
r
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5. Rudiger HA, Ngan SY, Ng M, Powell GJ, Coong PF. Radiation therapy in the treatment of desmoid tumours reduces surgical indications. Eur J Surg Oncol. 2010;36(1):84e88. 6. Pritchard DJ, Nascimento AG, Petersen IA. Local control of extraabdominal desmoid tumors. J Bone Joint Surg Am. 1996;78(6): 848e854. 7. Lim CL, Walker MJ, Mehta RR, Das Gupta TK. Estrogen and antiestrogen binding sites in desmoid tumors. Eur J Cancer Clin Oncol. 1986;22(5):583e587. 8. Okuno SH, Edmonson JH. Combination chemotherapy for desmoid tumors. Cancer. 2003;97(4):1134e1135. 9. Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT III. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer. 2000;88(7): 1517e1523. 10. Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66(9): 1369e1374. 11. Sorensen A, Keller J, Nielsen OS, Jensen OM. Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1-27 years. Acta Orthop Scand. 2002;73(2):213e219. 12. Reitamo JJ. The desmoid tumor. IV. Choice of treatment, results, and complications. Arch Surg. 1983;118(11):1318e1322. 13. Gronchi A, Casali PG, Mariani L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 2003;21(7):1390e1397. 14. Shido Y, Nishida Y, Nakashima H, et al. Surgical treatment for local control of extremity and trunk desmoid tumors. Arch Orthop Trauma Surg. 2009;129(7):929e933. 15. Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999;86(10):2045e2052. 16. Goy BW, Lee SP, Fu YS, Selch MT, Eilber F. Treatment results of unresected or partially resected desmoid tumors. Am J Clin Oncol. 1998;21(6):584e590. 17. Posner MC, Shiu MH, Newsome JL, et al. The desmoid tumor: not a benign disease. Arch Surg. 1989;124(2):191e196. 18. Ballo MT, Zagars GK, Pollack A. Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 1998;42(5):1007e1014. 19. Miralbell R, Suit HD, Mankin HJ, et al. Fibromatoses: from postsurgical surveillance to combined surgery and radiation therapy. Int J Radiat Oncol Biol Phys. 1990;18(3):535e540. 20. Jelinek JA, Stelzer KJ, Conrad E, et al. The efficacy of radiotherapy as postoperative treatment for desmoid tumors. Int J Radiat Oncol Biol Phys. 2001;50(1):121e125.
Vol. 39, September 2014
Desmoid Tumors of the Upper Extremity Matthew T. Houdek, MD, Peter S. Rose, MD, Sanjeev Kakar, MD
Purpose To determine the outcomes of surgical excision with or without adjuvant treatment in the management of desmoid tumors of the upper extremity. Methods We retrospectively reviewed 52 patients with a histologically confirmed desmoid tumor in the upper extremity that was managed surgically. All patients presented between 1970 and 2011 and had a minimum 2-year follow-up. Results There were 25 males and 27 females with an average age of 37 17 years. The most common location was the shoulder (n ¼ 27). The most common symptom was a painful mass (n ¼ 30). Average tumor size was 189 371 cm3. Negative margins (wide or marginal resection) were achieved in 43 patients. The 5-year disease-free interval was 57%. Patients with recurrence were younger than those without (31 vs 43 y). Postoperative radiotherapy increased the time to recurrence (2.6 vs 1.6 y) but ultimate disease-free interval at 5 years was similar in patients who did and did not receive radiotherapy. Compared with the preoperative evaluation, there was a significant reduction in patients reporting moderate or severe pain postoperatively. Conclusions Desmoid tumors are locally aggressive fibrous tumors. Recurrence after surgical excision of a desmoid tumor in the upper extremity is common, especially in younger patients. Adjuvant radiation therapy tended to increase time to recurrence but not rate of recurrence. (J Hand Surg Am. 2014;39(9):1761e1765. Copyright Ó 2014 by the American Society for Surgery of the Hand. All rights reserved.) Type of study/level of evidence Therapeutic IV. Key words Desmoid tumors.
E
XTRA-ABDOMINAL DESMOID
tumors are benign fibroblastic tumors that arise from the fascia or deep muscle and aponeurosis.1e4 Described by MacFarlane in 1832, desmoid tumors are rare, with an estimated 900 new cases diagnosed in the United States each year.2 Although desmoids are considered benign, they are locally aggressive with an infiltrative growth pattern that can hamper the efforts of local control.2,5,6 Mortality from these tumors is rare but
From the Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN. Received for publication October 9, 2013; accepted in revised form June 11, 2014. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Sanjeev Kakar, MD, Department of Orthopedic Surgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905; e-mail: [email protected]. 0363-5023/14/3909-0015$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2014.06.015
morbidity from the tumors and from treatment can be profound.2 Primary treatment options include surgical resection, radiation therapy, or both. Chemotherapy, nonsteroidal anti-inflammatories, and anti-estrogen agents may provide benefit.1,2,5,7e9 Even with treatment, local recurrence remains common, occurring in up to 68% of patients.10 Previous studies examining extra-abdominal desmoids combine tumors arising in both the upper and lower extremity.1,5,6,9e12 The purpose of this study was to compare the clinical outcomes of extraabdominal desmoid tumors located in the upper extremity treated surgically with or without adjuvant radiation. MATERIALS AND METHODS After we obtained institutional review board approval, we retrospectively reviewed the medical records of
Ó 2014 ASSH
r
Published by Elsevier, Inc. All rights reserved.
r
1761
1762
DESMOID TUMORS
TABLE 1.
Desmoid Tumors of the Upper Extremity
Location
All Upper Extremity
Shoulder
Arm
Forearm
Hand
Total number of patients
52
27
7
15
3
Males
25
14
3
7
1
Females Average age, y Tumor size, cm
2
13
4
8
2
38
38
49
34
28
7
8
5
7
4
Dominant extremity
29
16
2
10
1
Painful mass
30
17
3
7
1
Mass increasing in size
13
4
3
6
0
Surgery alone
25
11
4
8
2
Surgery plus radiation
27
16
3
7
1
Recurrence (%)
43
39
14
63
33
Treatment
Pain score
Preoperative
Postoperative
Severe or moderate
31
0
None or mild
21
52
57 patients with pathologically confirmed desmoid tumor of the upper extremity who were treated surgically between 1970 and 2011. Three patients were lost to follow-up and 2 were removed from the study because they received chemotherapy in addition to radiation therapy. This resulted in a study population of 52 patients. Patients were treated with either surgery (n ¼ 25) or surgery plus postoperative radiation (n ¼ 27). All surgery was performed by staff orthopedic surgeons at our institution. Staff pathologists reviewed all pathology slides. Likewise, radiation protocols instituted were at the discretion of staff radiation oncologists. All patients had a minimum 2 years of follow-up (average, 9 y; range, 2e30 y), which included a focused physical examination and imaging. Recurrence was defined as the return of a mass on physical examination or on imaging studies. Our current practice is to observe these patients every 4 months for the first 2 years with a physical examination and cross-sectional imaging (magnetic resonance imaging or computed tomography). After that, we observe the patients every 6 months indefinitely. Patient pain level was recorded at the preoperative and postoperative visits based on a numeric (1e10) rating system (Table 1). A pain score of 1 to 4 was considered mild, 5 to 7 was moderate, and greater than 7 was severe. We performed statistical analysis of categorical data using Fisher exact test and analyzed nominal data using Student t test employing 2-tailed statistics with P ¼ .05 considered statistically significant. J Hand Surg Am.
r
Spearman rank correlation coefficients were used to calculate correlations between variables. Odds ratios were calculated to determine predictive features of recurrence. RESULTS There were 27 females (52%) and 25 males in our cohort, presenting at an average age of 37 17 years (range, 12e85 y). Tumors averaged 189 371 cm3 in size (range, 2e2,400 cm3) and involved the dominant extremity in 56% of the patients (n ¼ 32). There was a minimal negative correlation (r ¼ e0.12) between patient age and size of the tumor. The most common presenting problem was a painful mass. A total of 38 patients remembered a previous traumatic event (bruise, broken bone, etc) to the extremity or had previous surgery in the region in which the desmoid tumor was located. Thirty patients underwent preoperative magnetic resonance imaging; however, in all patients the diagnosis of a desmoid tumor was not made with this imaging. Preoperative biopsy (either open or needle core) was obtained in 65% of patients (n ¼ 34). At the time of surgical excision, the tumor was found to abut the periosteum in 52% of patients (n ¼ 27) and was in intimate contact with a neurovascular bundle in 38% (n ¼ 20). Thirty-six patients underwent a wide excision, 12 underwent a marginal surgical excision, and 3 underwent tumor debulking. Five patients with a marginal excision had a positive Vol. 39, September 2014
DESMOID TUMORS
1763
FIGURE 1: Disease-free interval analysis of surgically treated desmoid tumors of the upper extremity. The overall 5-year disease-free interval was 57%. The best disease-free interval rate was obtained in desmoid tumors of the arm, whereas the worse rate occurred in the forearm.
surgical margin. Four required a free tissue transfer (free fibula, n ¼ 3; or free latissimus, n ¼ 1) to reconstruct and provide soft tissue coverage in the forearm. At the time of the tumor excision, in an attempt to achieve a negative surgical margin, multiple margins were taken in 37% of patients (n ¼ 19). In 83% (n ¼ 43), the ultimate surgical margin was negative either at the initial margin (n ¼ 27) or at the subsequent margin after further excision (n ¼ 16). Postoperative complications included symptomatic nonunions of the free fibula fixation requiring revision (n ¼ 2), postoperative hematoma requiring irrigation and debridement (n ¼ 1), and an elbow capsulotomy resulting from a flexion contracture (n ¼ 1). The 5-year disease-free interval was 57% (Fig. 1). Mean time to local recurrence was 25 months (range, 2 mo to 5.3 y) after surgery. Treatment for recurrence included repeated wide excision (n ¼ 14), marginal excision (n ¼ 3), tumor debulking (n ¼ 1), amputation (n ¼ 1), chemotherapy and radiation therapy (n ¼ 2), and observation alone (n ¼ 2). Further local recurrence occurred in 35% of these patients (n ¼ 9). A total of 83% (n ¼ 19) of recurrent tumors were histologically confirmed as desmoid tumors at the time of re-excision. In the remaining 17% of patients with recurrences the mass was not rebiopsied or excised, and the decision was made to treat the mass either with medical therapies or observation. In these J Hand Surg Am.
r
patients the recurrent mass abutted a critical structure (nerve or blood vessels), which the patient or surgeon thought would lead to notable morbidity if treated surgically. In patients with recurrent tumors, the mean age of surgical excision was 31 years, whereas for those without evidence of recurrence the average age was 43 years; this was statistically significant (P ¼ .01). There was no significance between patient’s sex and tumor size (Table 2). Recurrence was not affected by the use of adjuvant radiation, a positive surgical margin, re-excision of a positive margin, or a tumor that was in intimate contact with a neurovascular bundle or abutted the periosteum. There was no difference in 5-year disease-free interval for surgery alone versus surgery with addition of adjuvant therapy (58% vs 57%; P ¼ .70). However, among patients who had recurrence, those who underwent adjuvant postoperative radiation therapy tended to have a longer time to recurrence than patients undergoing surgical excision alone (3 vs 2 y; P ¼ .14). Table 3 details comparisons between patients who received adjuvant radiation therapy and those who underwent surgery alone. There was no significant difference in age, sex, location, margin status, or relationship to a neurovascular structure (Table 3). Patients in the adjuvant radiation treatment group tended to have larger tumors; however, this was not Vol. 39, September 2014
1764
TABLE 2.
DESMOID TUMORS
Factors That Could Influence Recurrence of Upper Extremity Desmoid Tumors
Total Patients, n Average age, y Size of tumor, cm3
Recurrence
No Recurrence
23
29
Odds Ratio
95% Confidence Interval
P Value
31
43
.01
267
125
.17
6
3
0.32
0.07e1.48
.16
History of trauma or previous surgery
Positive surgical margin
18
19
0.52
0.15e1.84
.36
Intimate contact with neurovascular bundle
11
9
0.49
0.15e1.52
.26
Multiple margins taken
10
9
0.58
0.18e1.82
.39
Periosteal invasion
13
14
0.71
0.23e2.15
Adjuvant radiation treatment
12
15
0.98
0.33e2.94
TABLE 3. Comparison of Patients Undergoing Surgery Alone Versus Surgery and Radiation
Average age, y Males Size of tumor, cm3 Shoulder mass
Surgery Alone (n ¼ 25)
Surgery Plus Radiation (n ¼ 27)
P Value
41
34
.11 .40
11
14
102
274
.09
11
14
.40
Forearm mass
8
7
.76
Positive margin
3
6
.46
Periosteal abutment
9
18
.02
Close to neurovascular bundle
9
11
.77
Multiple margins taken
11
8
.56
statistically significant. Likewise, periosteal abutment of the desmoid tumor was significantly more common in the adjuvant radiation group compared with the group treated only surgically. There was a significant reduction in the number of patients reporting moderate or severe pain between the preoperative and postoperative settings. At last follow-up all patients reported either mild or no pain (Table 1). DISCUSSION Recurrence after surgical excision of upper extremity desmoid tumors with or without adjuvant postoperative treatment in this study was high and similar to studies reporting tumors from both the upper and lower extremities. In previous studies, patients with J Hand Surg Am.
r
.59 1.0
primary extremity desmoid tumors had 5-year diseasefree intervals ranging from 53% to 72% compared with 57% for our study.1,10,13,14 There were several limitations to this study. Its retrospective nature limited the amount of data we were able to collect from the medical record and constrained the analysis. All of the lesions in this study were surgically excised; as such, we were unable to comment on the natural history of desmoid tumors in the upper extremity treated nonsurgically or with other modalities alone. Although all of these tumors were treated at one institution, the surgeries and adjuvant therapies were performed by multiple providers, and there was no standardized protocol for their treatment or follow-up at our institution. These differences in surgical and adjuvant treatment protocols could potentially influence outcomes. Likewise, not all patients received the same preoperative and postoperative imaging. Previous studies examining the prognostic factors of disease-free intervals have not been able to adequately predict recurrence.15 Within our study, patients with recurrence were younger than those without recurrence. In the upper extremity the ability to achieve adequate surgical margins can be associated with patient morbidity owing to the removal of nerves, tendons, and other critical structures.9,15,16 Margin status has been debated as a factor in the recurrence of desmoid tumors.12,13,15,17e19 The infiltrative nature of desmoids tumors and the fact that they frequently lack a pseudocapsule make it difficult to determine the true extent of disease at the time of excision.15,17 A positive surgical margin did not increase the odds of recurrence in our study. Likewise, having to excise multiple biopsy samples to achieve a negative surgical margin did not increase the odds of recurrence. Vol. 39, September 2014
DESMOID TUMORS
To increase the disease-free interval, adjuvant radiotherapy is used.9,11,13,20 The addition of radiotherapy increases the disease-free interval compared with surgery alone.9,11,13,20 In this study, the use of radiotherapy increased the time to recurrence but the 5-year disease-free interval was similar between patients who did and did not receive radiotherapy, although we recognize the inherent limitations of the current study methodology for considering this conclusion. Previous work has demonstrated that the use of radiation therapy as an adjuvant is especially useful in cases where complete excision of the desmoids tumor would cause substantial morbidity.9 Although there was no difference in the ultimate recurrence rate between these patients, we think the addition of radiation therapy should be considered in cases where it would be difficult to obtain adequate surgical margins. Unfortunately, recurrence is a common problem despite aggressive surgical treatment. We think that younger patients in particular should be counseled regarding their increased risk for recurrence. Although adjuvant radiation treatment did not reduce the rate of recurrence, it tended to increase the disease-free interval in surgically treated desmoids. REFERENCES 1. Chew C, Reid R, O’Dwyer PJ. Evaluation of the long term outcome of patients with extremity desmoids. Eur J Surg Oncol. 2004;30(4): 428e432. 2. Hosalkar HS, Torbert JT, Fox EJ, et al. Musculoskeletal desmoid tumors. J Am Acad Orthop Surg. 2008;16(4):188e198. 3. Micke O, Seegenschmiedt MH. Radiation therapy for aggressive fibromatosis (desmoid tumors): results of a national Patterns of Care study. Int J Rad Oncol. 2005;61(3):882e891. 4. Pakos EE, Tsekeris PG, Goussia AC. Desmoid tumours of the extremities and trunk: a review of the literature. Int Orthop. 2005;29(4): 210e213.
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5. Rudiger HA, Ngan SY, Ng M, Powell GJ, Coong PF. Radiation therapy in the treatment of desmoid tumours reduces surgical indications. Eur J Surg Oncol. 2010;36(1):84e88. 6. Pritchard DJ, Nascimento AG, Petersen IA. Local control of extraabdominal desmoid tumors. J Bone Joint Surg Am. 1996;78(6): 848e854. 7. Lim CL, Walker MJ, Mehta RR, Das Gupta TK. Estrogen and antiestrogen binding sites in desmoid tumors. Eur J Cancer Clin Oncol. 1986;22(5):583e587. 8. Okuno SH, Edmonson JH. Combination chemotherapy for desmoid tumors. Cancer. 2003;97(4):1134e1135. 9. Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT III. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer. 2000;88(7): 1517e1523. 10. Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66(9): 1369e1374. 11. Sorensen A, Keller J, Nielsen OS, Jensen OM. Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1-27 years. Acta Orthop Scand. 2002;73(2):213e219. 12. Reitamo JJ. The desmoid tumor. IV. Choice of treatment, results, and complications. Arch Surg. 1983;118(11):1318e1322. 13. Gronchi A, Casali PG, Mariani L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 2003;21(7):1390e1397. 14. Shido Y, Nishida Y, Nakashima H, et al. Surgical treatment for local control of extremity and trunk desmoid tumors. Arch Orthop Trauma Surg. 2009;129(7):929e933. 15. Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999;86(10):2045e2052. 16. Goy BW, Lee SP, Fu YS, Selch MT, Eilber F. Treatment results of unresected or partially resected desmoid tumors. Am J Clin Oncol. 1998;21(6):584e590. 17. Posner MC, Shiu MH, Newsome JL, et al. The desmoid tumor: not a benign disease. Arch Surg. 1989;124(2):191e196. 18. Ballo MT, Zagars GK, Pollack A. Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 1998;42(5):1007e1014. 19. Miralbell R, Suit HD, Mankin HJ, et al. Fibromatoses: from postsurgical surveillance to combined surgery and radiation therapy. Int J Radiat Oncol Biol Phys. 1990;18(3):535e540. 20. Jelinek JA, Stelzer KJ, Conrad E, et al. The efficacy of radiotherapy as postoperative treatment for desmoid tumors. Int J Radiat Oncol Biol Phys. 2001;50(1):121e125.
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