In an earlier report from the current study center regarding surgical treatment for patients with soft tissue sarcoma (STS) of the hand, it was concluded that repeat resection or amputation improves outcomes. Since then, the authors have aggressively sought to achieve negative resection margins, using standard or modified amputations when needed, and performing repeat resections to negative surgical margins when they were not achieved at the time of initial surgery. The current review was conducted to determine whether this approach resulted in better outcomes.
A retrospective review of 53 patients with STS of the hand who were treated between 1996 and 2005 was performed. Recurrence-free survival (RFS) and functional outcome of hand-preserving procedures were assessed according to the Musculoskeletal Tumor Society (MSTS) system.
The median RFS was not reached at the time of last follow-up. Of 53 patients, 6 (11%) had positive microscopic resection margins. Three patients underwent repeat resection to negative surgical margins, whereas another 3 patients did not. All 3 patients with positive microscopic surgical margins that were not re-excised developed local disease recurrence; 2 patients also developed distant metastases. Two of the 50 patients with negative resection margins developed distant metastases. All 5 patients who developed local and/or distant disease recurrence had deep tumors. The median MSTS score was 29 (interquartile range, 27-30). Patients who underwent more extensive resections, such as double ray amputations, had lower MSTS scores.
The results of surgical treatment for soft tissue sarcoma (STS) at our center between 1982 and 1990 were reported by Brien et al in 1995.1 The authors noted the high rate of positive resection margins and determined that adjuvant radiotherapy could not compensate for the positive surgical margins with a high rate of local disease recurrence noted in their study, and recommended repeat resection or amputation for patients with positive resection margins after surgical treatment for STS of the hand to improve the oncologic outcomes. Pradhan et al2 and Lin et al3 reported similar findings and also emphasized the importance of negative surgical margins in achieving good local tumor control.
Since 1995, we have been more aggressive in our efforts to achieve negative surgical margins, using standard or modified amputations when needed. If negative surgical margins were not achieved during the first attempt at definitive treatment, our default position was to perform additional wide excision or amputation to achieve negative resection margins. Exceptions were made on an individual case basis.
The purpose of the current study was to review the oncologic results with this approach to the definitive surgical treatment of STS of the hand. In addition, we assessed the functional outcome of hand-preserving resections and the factors that were associated with differences in outcome using the Musculoskeletal Tumor Society (MSTS) scoring system.4
MATERIALS AND METHODS
An upper extremity surgical registry was created in 1995. All patients treated by a single surgeon (E.A.A.) for STS of the hand were entered and data recorded, including demographics, tumor characteristics, and presentation. On the basis of whether the biopsy incision could be incorporated into a standard or modified limb salvage or amputation incision, the treating surgeon assessed whether an optimal biopsy had been performed, as well as the type of biopsy. The surgery performed, surgical margin status, use of adjuvant therapies, local disease recurrence, metastasis, functional status, and disease status were recorded and updated at the time of each follow-up. After institutional review board approval, data for patients treated between 1996 and 2005 were retrospectively reviewed, tabulated, and statistically analyzed.
All patients were staged preoperatively with a computed tomography (CT) scan of the chest and magnetic resonance imaging (MRI) of the hand, unless medically contraindicated (as was the case in 1 patient). In addition, 10 of 11 patients with epithelioid sarcoma and 1 patient with rhabdomyosarcoma underwent sentinel lymph node biopsy as part of their staging. The American Joint Committee on Cancer (AJCC) staging system5 was used to stage the tumors. For patients treated between 1996 and 2002 (when the latest AJCC staging system was developed), AJCC stage was assigned retrospectively. All patients were treated with definitive wide excision, combined partial hand amputation with wide excision, or standard hand amputation.
Patients were typically discharged the day after surgery, or 5 days after for those who underwent flap wound coverage. Range-of-motion therapy was initiated by the surgeon the day after surgery and continued under the supervision of a hand occupational therapist. Patients were evaluated on the 10th postoperative day when stitches were removed; then at 6 weeks, 3 months, and every 3 months during the first year; every 4 months for the next 2 years; and every 6 months during the 4th and 5th years. Local recurrence was assessed clinically during outpatient visits, with MRI scans ordered when local disease recurrence was suspected for patients treated in the earlier half of this study. Yearly surveillance MRI scans of the hand were performed in addition for patients treated in the later half of this study to assess for local disease recurrence. An open biopsy was performed when a suspicious lesion was identified. The presence of distant metastases was assessed during each follow-up visit, with a chest x-ray and a CT scan of the chest performed annually until 5 years postoperatively, after which annual chest x-rays were performed. Hand function was assessed for all patients, except those who had undergone complete hand amputations, using the MSTS system. This was done when function was believed to have stabilized, usually within 5 months to 6 months for the majority of patients. The MSTS score is used to assess the outcomes after tumor resection and reconstruction. In the upper extremity, the measures included are pain, function, emotional acceptance, hand positioning, dexterity, and lifting ability. Each factor is assigned a value of 0 to 5 and added together, with the final score ranging from 0 to 30.
The current study included 53 patients (26 men and 27 women) who were treated for STS of the hand between 1996 and 2005. The median age of the patients at the time of disease presentation was 36 years (interquartile range [IQR], 28-46 years). A total of 45 patients were treated for primary tumors, whereas the remaining 8 were treated after at least 1 local recurrence. Of the 53 patients, 11 patients (21%) had epithelioid sarcoma, 11 (21%) had malignant fibrous histiocytoma, 8 (15%) had synovial sarcoma, 4 (8%) had leiomyosarcoma, and 4 (8%) had malignant peripheral nerve sheath tumors; the remaining 15 patients had 1 of 9 other types of STS (Table 1).Twenty-eight patients (53%) had tumors centered in the palm, 18 (34%) had tumors that were over the dorsum of the hand, and another 7 patients (13%) had tumors that were over the digits. The median size of the tumor was 2.5 cm (IQR, 2-4.5 cm), and 47 of the tumors (89%) were deep, whereas the remaining 6 tumors (11%) were superficial. There were 17 (32%) low-grade tumors and 36 (68%) high-grade tumors. One patient with an alveolar rhabdomyosarcoma had axillary lymph node involvement identified during sentinel lymph node biopsy. None of the other patients had distant metastases. There were 17 patients with AJCC stage I disease, 29 with stage II disease, 6 with stage III disease, and 1 patient (with alveolar rhabdomyosarcoma with axillary lymph node involvement) with stage IV disease. Approximately half of the population (24 patients; 45%) received radiotherapy within 3 months before or after surgery, whereas only the 1 patient with rhabdomyosarcoma received chemotherapy (Table 2).
Table 1. Summary of Histologic Diagnoses
No. of Patients
Malignant fibrous histiocytoma
Malignant peripheral nerve sheath tumor
Acral myxoinflammatory fibroblastic sarcoma
Ossifying fibromyxoid tumor
Clear cell sarcoma
Table 2. Patient Characteristics (n = 53)
No. of Patients (%)
IQR indicates interquartile range; MSKCC, Memorial Sloan-Kettering Cancer Center.
Median age at disease presentation [IQR], y
Median follow-up for survivors [range], y
Median tumor size [IQR], cm
Positive surgical margin
Wide local excision
Partial hand amputation
Biopsy performed at MSKCC
Optimal biopsy incision
Type of biopsy
Function score, median [IQR]
Only 6 patients underwent biopsy at our center, whereas the other 47 had undergone biopsies before referral to our center. Thirteen patients (25%) underwent incision biopsies, whereas 40 patients (75%) underwent marginal excision biopsies performed for presumed benign lesions, often with significant contamination. A total of 24 biopsy incisions (45%) were deemed suboptimal and required at least a modification of the limb salvage incision, a wider resection, or more extensive amputation than would have been required with an optimal biopsy.
Twenty-nine patients (55%) underwent wide local excisions, 22 (42%) underwent partial hand amputations (digit amputations, single or double ray amputations), and 2 patients (3%) underwent hand amputations. Twenty-six patients (49%) required flap wound coverage. Six patients (11%) had positive microscopic resection margins, 3 of whom underwent repeat resections to negative surgical margins, whereas the remaining 3 patients did not. Of the 3 patients who did not undergo repeat resection, 1 patient had an ossifying fibromyxoid tumor, another had a low-grade myxofibrosarcoma, and the final patient had a high-grade malignant fibrous histiocytoma. All 3 of these patients were aged >70 years and it was believed that the morbidity of a repeat resection (which would have required a ray amputation in 2 patients and a below-the-elbow amputation in the last patient) would be too great given their advanced age.
Recurrence-free survival (RFS) was calculated from the date of surgery to the date of death or local disease recurrence or the date of distant disease recurrence, whichever occurred first. The RFS at 2 years was estimated using the Kaplan-Meier product-limit method. Correlations between MSTS score, age, and tumor size were estimated by the Spearman rank correlation coefficient (r_s_). MSTS score, according to categorical patient characteristics, was compared using the Wilcoxon rank sum test. All analyses were performed using SAS statistical software (version 9.2; SAS Institute Inc, Cary, NC).
The median follow-up for survivors was 7 years. Three patients (6%) developed local tumor recurrence. All 3 were patients who had positive microscopic resection margins and did not undergo repeat resection. None of the patients with negative resection margins (47 patients with negative surgical margins at the time of initial surgery and 3 patients who underwent repeat resection to negative surgical margins) had developed local recurrence of the tumor at the time of last follow-up. Four patients developed distant lung metastases, among them 2 of the patients with positive microscopic resection margins (the patient with the ossifying fibromyxoid tumor and another with a high-grade malignant fibrous histiocytoma), for whom repeat resection was not performed. Only 2 of the 50 patients with negative resection margins (1 patient with a clear cell sarcoma and another with a high-grade myxoid liposarcoma) developed distant lung metastases. At the time of last follow-up, 47 patients had no evidence of disease, 3 were alive with disease, 2 had died of unknown causes, and 1 patient had died of disease. The median RFS was not reached in this cohort because there were a total of 5 events (disease recurrence or death) in this cohort, and the 2-year RFS was 90% (95% confidence interval,81%-97%]) (Fig. 1). All 5 events occurred in patients who had deep-depth tumors, had undergone biopsy outside the study center, and had undergone a suboptimal biopsy. Comparison of survival curves for all factors were not performed because of the small number of events (n = 5).
MSTS score was assessed for 49 patients. We excluded the 2 patients who underwent primary hand amputations and 2 others who underwent amputation after local recurrence of the tumor. The median MSTS score was 29 of a possible 30 (IQR, 27-30), indicating a good functional outcome. There was a moderately negative correlation noted between the size of the tumor and MSTS score (Spearman rank correlation coefficient [r_s_] = −0.43; P <.01) and a weak negative correlation was noted between age and MSTS score (r = −0.21; P = .14). Patients who received radiotherapy also had lower MSTS scores compared with patients who did not (median score, 27 vs 30; P <.01). Finally, patients who underwent double ray amputation were found to have lower MSTS scores than those who underwent other procedures (P <.001). There was a broad association noted between MSTS score and the requirement for flap wound coverage (P = .05). Other factors, such as tumor site and biopsy parameters, were not found to be associated with differences in the MSTS score (Table 3).
Table 3. MSTS Score According to Patient Characteristics (n = 49)
Our previous experience, the results of which were published in 1995,1 led us to take a more aggressive approach in our efforts to achieve negative surgical margins when treating patients with STS of the hand. Our subsequent 10-year experience appears to confirm this approach. All 3 patients for whom we did not achieve negative resection margins developed local recurrence of the tumor, and 2 of 3 patients developed distant lung metastases. None of the other 50 patients with negative resection margins (including 47 patients with negative surgical margins at the time of the initial resection and another 3 who initially had positive resection margins and subsequently underwent repeat resection to negative surgical margins) had developed local tumor recurrence at the time of last follow-up, and only 2 of the 50 patients with negative resection margins had developed distant lung metastases. The results of the current study support the imperative to achieve negative surgical margins when treating patients with STS of the hand. Other centers have reached these same conclusions when treating patients with STS of the hand2 and of the hand and foot.3 In addition, all 5 patients with either local and/or distant disease recurrence had deep tumors and suboptimal biopsy incisions.
The current study had 2 primary limitations. First, the finding that there were few events (local disease recurrence and/or distant metastases or death) limited our ability to identify factors known to be associated with poorer outcomes in patients with STS such as tumor size and grade5; however, the results of the current study may suggest that deep tumors, suboptimal biopsy incisions, and positive resection margins are associated with poorer outcomes. In addition, the current study represents the experience of a single surgeon and therefore may be difficult to extrapolate elsewhere, although it does suggest that specialized care may improve outcomes for patients with STS of the hand.
Studies have demonstrated that it is possible to perform an en bloc resection of STS of the hand with good oncologic outcomes while preserving a functional hand.6-8 However, the limited anatomical confines of the hand make achieving negative surgical margins difficult. Thus, some authors argue that it is acceptable to have a positive resection margin and use adjuvant radiotherapy to achieve local control because positive microscopic margins do not always result in local disease recurrence,9, 10 and reserve amputation for patients with recurrent tumors. However, our previous experience1 and the experience reported at other centers2, 3 suggest the critical importance of achieving negative resection margins for patients with STS of the hand. The results of the current study underscore this point.
STS of the hand was initially believed to confer a poorer prognosis than extremity STS of similar size.1 More recent studies have indicated that the opposite may actually be true.11 Our surgical efforts since 1995 have resulted in fewer positive surgical margins, local disease recurrence, distant disease recurrence, and disease-specific deaths. One possible explanation may be the difference in the patient population, because the original study included more patients with rhabdomyosarcomas. The results of the current study are those of a single surgeon, ensuring consistency in technique and approach. The availability of MRI to aid in surgical planning, the frequent use of preoperative radiotherapy to downstage tumors, and a deliberate attempt to achieve negative surgical margins have been important components of our management strategy. Despite this aggressive surgical approach, we managed to avoid performing complete hand amputation in all but 2 patients.
The functional outcome of the resection as measured by the MSTS score was correlated with the amount of native tissue we could preserve, with the best outcomes noted for wide resections without digit sacrifice and the poorest for double ray amputations. Large tumor size and the need for flap wound coverage, both of which are indicative of the extent of resection required, were also found to be associated with a lower MSTS score, as was the use of perioperative radiotherapy.
All patients who developed local disease recurrence or distant metastases had suboptimal biopsy incisions, although this did not appear to affect the functional outcome in the entire cohort. The hazards of a poorly performed biopsy have been well documented,12, 13 and the high proportion of suboptimal incisions or marginal excisions of unsuspected STS can be at least partly attributed to the rarity of malignant tumors of the hand .14 Despite this, care must be exercised when evaluating tumors of the hand.
The results from the current study cohort suggest that aggressive surgical treatment of STS of the hand yields better clinical outcomes, and underscore that care must be taken when planning biopsies of tumors in the hand.
CONFLICT OF INTEREST DISCLOSURES
Supported by the Memorial Sloan-Kettering Cancer Center Hand Sarcoma Research Fund and The Maynard Limb Preservation Fund.