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SECTION II: ORIGINAL ARTICLES: Tumor

Epithelioid Sarcoma of the Hand

Herr, Mark J MD*; Harmsen, William S; Amadio, Peter C MD*; Scully, Sean P MD, PHD*‡

Author Information
Clinical Orthopaedics and Related Research: February 2005 - Volume 431 - Issue - p 193-200
doi: 10.1097/01.blo.0000150317.50594.96
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Abstract

Tumors in the hand are common, and fortunately, most are benign. In the 30-year experience of one hand surgery referral center handling 2161 cases, only 2.2% of the tumors in the hand were malignant. A ganglion, not a true neoplasm, was the most common tumor, accounting for more than ½ of all tumors seen. Of the malignant tumors seen, 70.8% involved the skin.46 Soft tissue sarcomas in the hand are less common than malignant lesions involving the skin. Unfortunately, on initial evaluation benign and malignant conditions frequently may present as a painless mass, creating the potential for errors in diagnosis and management. Among the largest reviews of soft tissue sarcomas in the hand is the report by Bryan et al,7 involving 31 cases at the Mayo Clinic, finding epithelioid sarcoma to be the most common lesion.7 In a more recent review involving 23 cases, Brien et al6 again found epithelioid sarcoma to be one of the most common soft tissue sarcomas in the hand. This later study compared survival in patients with hand sarcomas to survival of patients with extremity sarcomas, showing that patients with sized-matched deep sarcomas in the hand had a lower survival rate.6 In one large review of 12,370 malignant soft tissue tumors,25 epithelioid sarcoma accounted for 1.4% of all tumors and more than 15% of all tumors seen in the hand. Forty-one percent of all epithelioid sarcomas evaluated were found in the hand. Only malignant fibrous histiocytoma was diagnosed more frequently in the hand view.25

Enzinger was the first to describe epithelioid sarcoma in 1970.14 His initial series included 62 patients.14 He found that the tumor tended to grow slowly and frequently was misdiagnosed as other malignant and nonmalignant conditions. Enzinger found that this tumor tended to occur in males in their second and third decades of life, located most commonly in the distal upper extremity. Twenty-one of the 62 cases reviewed occurred in the hand. In this series, there was a high local recurrence rate (87%) and frequent late metastasis (30%).14

Since Enzinger’s original series there have been more than 600 cases of epithelioid sarcoma described in the literature.4,7,8,10,14,19,21,26,30,37,42,44,50,60 Numerous of these series have confirmed some of the findings of Enzinger and identified predictors of outcome, including size of tumor, local recurrence, metastasis, age at first diagnosis, necrosis, vascular invasion, location on the extremity, number of mitotic figures, and depth, among others.6,8,10,21,26,37,44,50 Some investigators also have reviewed the experience with soft tissue sarcomas of the hand, including cases of epithelioid sarcoma.6,26,31,43,54,55 To date, however, few sizable series have been dedicated to epithelioid sarcoma in its most common location, the hand.1,2,5,7,11,15,18,20,23,34,35,38,40,45,48,49,51,53,56–58

Our goal was to review treatment of 28 consecutive cases of epithelioid sarcoma of the hand, including presentation, patient factors, efficacy of preoperative imaging, experience with adjuvant therapy, survival, recurrence after different resection types, and function after different resection types.

MATERIAL AND METHODS

A review of institutional files from January 1, 1975 to January 1, 2000 revealed 88 patients with soft tissue sarcomas of the hand. Ten of the 88 patients were seen but not treated at this institution; therefore, they were eliminated, including three patients with epithelioid sarcoma. In the remaining 78 patients, there were 28 with epithelioid sarcomas. All cases were retrospectively reviewed, including hospital notes, clinical notes, pathology records, operative reports, radiology records, and tumor registry files. Data extracted and reviewed included: side, gender, age at presentation, presenting symptoms, duration of symptoms, number of previous biopsies or surgeries, location of primary lesion, history of trauma, radiographic studies, type of surgery, type of adjuvant therapy, date of recurrence, location of recurrence, date of metastasis, location of metastasis, type of subsequent surgeries, size of tumor in terms of maximal dimension, and area defined by pathology reports. Vascular invasion and depth of tumor in relation to the fascia were not reproducibly found in the charts.

The Kaplan-Meier survival method was used to assess risk factors for association with adverse outcomes, defined as the first occurrence of metastasis, recurrence, or death. Ninety-five percent confidence intervals for these estimates are reported at 5 and 10 years.24 Significance tests of association were done using a log-rank test.36 However, power for these significance tests was low because of only 10 events. Therefore, a nonsignificant result should not be interpreted as evidence that no association exists. The alpha level was set at 0.05 for statistical significance.

RESULTS

The only statistically significant finding in the data set was that affected women were younger (p = 0.001) at presentation than the affected males. Although not significant statistically because of lack of power, there were some trends observed in the data. These trends will be reviewed in the descriptive results section. Table 1 summarizes the statistical analysis of our data, and displays these trends numerically. The 5-year disease-free survival rate indicates the patient was free of any event during that period. An event included death, metastasis, or recurrence. These were grouped for subsequent analysis.

Table 1
Table 1:
Disease-free Survival at 5 Years

This tumor occurred more commonly in males than females; although, the females tended to be younger. There were 18 males and 10 females. The average age of the patients was 31 years, with a range of 14–50 years at presentation. The median was 29 years. Male patients were an average of 34 years old at presentation, with a range of 18–50 years. However, female patients were an average of 23 years at presentation, with a range of 14–33 years. This difference in age was found to be statistically significant between males and females. Not only were the females younger, but they also tended to have better outcomes. At 5 years, 90% of females were alive and free of recurrence, metastasis, or death. By comparison, 65% of males were alive without evidence of an event at 5 years. The 10-year data show that 77% of females were alive without evidence of an event compared with 59% of males. There also was a tendency for younger patients to have better event-free survival.

The presenting symptom in 23 of 28 (82%) patients was a painless mass. The right hand was affected in 15 patients and the left hand in 13 patients; this did not affect survival. The tumor occurred in the web space in three patients, the digit in 12, the dorsum of the hand in two, the palm in four, the thenar eminence in one, and the ulnar border in six patients. Three patients had a painful mass. One patient reported symptoms of ulnar nerve compression, with numbness in the 5th digit and ulnar half of the 4th digit. Finally, one patient presented with a draining wound thought to be a chronic infection. The duration of symptoms in this group averaged 41 months, with a median of 24 months and a range of 4–180 months. Six of the 28 patients (21%) stated that trauma preceded the mass on the hand. Duration of symptoms did not influence survival.

Because of the presence of new imaging modalities, evaluation for the presence of metastatic disease in these patients evolved during the 25-year period of this study. Twenty of 28 patients had chest radiographs at presentation. In 18 patients, the chest radiograph was normal. Nodules were identified in two patients. One patient had a nodule evident on on the chest radiograph and chest CT scan. This patient’s hand tumor previously was treated elsewhere, with tissue sent to our institution for review and confirmation of the diagnosis. This patient was treated with chemotherapy only and was followed up for more than 18 years without evidence of additional local or systemic disease. A second patient had numerous nodules seen on CT scans and plain film. This patient was treated with a distal third forearm amputation and axillary node dissection without adjuvant therapy. This patient was alive and well 24 years after presentation. Only one patient had a normal chest tomogram. This patient is alive and well without evidence of disease at the last followup. One patient also had a bone scan that was normal.

With the advent of magnetic resonance imaging (MRI), images showed accuracy with improved negative and positive predictive values even in patients with prior surgery. Of the 28 patients, 11 have no record of having plain films. Among the 17 patients who did have plain films, 12 were normal. One plain film showed a previous amputation that was done at an outside institution. Four patients had evidence of soft tissue swelling or a mass. Three of these patients had pathologic confirmation of residual tumor during surgery. Four patients had CT of the hand. One CT scan was normal and one was interpreted as having postoperative changes from a previous excision, but at the time of surgery was found to have residual tumor. Two patients were found to have a mass in the hand, and one was found to have residual tumor at surgery. Fourteen of the 28 patients were evaluated with MRI of the hand. In all eight patients in whom a mass was identified on MRI, it was confirmed at the time of surgery. Six patients had findings consistent with postoperative changes attributable to previous biopsies or excisions. One patient in this group of six patients had residual tumor at the time of surgical intervention. Therefore, the positive predictive value of MRI of the hand in a patient with a previous history of tumor and previous surgery is 100%, and the negative predictive value is 83% in this small series.

The type of surgical resection did not tend to affect 10-year survival. All but the one patient previously mentioned who was treated with chemotherapy had at least one biopsy or excision before referral for additional evaluation; therefore, 26 of 27 patients or 96% had prior surgery, and 39% had multiple procedures before referral. The average number of prior surgeries was 1.9, with a range of 0–6. The 5-year and 10-year survival rates for patients having one or no surgeries before treatment at this institution are 74% and 74%. By comparison, patients with greater than one surgery have a 5-year survival free of events rate of 73% and a 10-year rate of 49%.

In the surgically treated group, the procedures were divided into three main categories: forearm amputation, partial amputation of the hand, or partial amputation of the finger. This includes digit amputation, ray resection, and multiple ray resection (with and without portions of the carpus), and wide excision. Three patients were treated with a distal third forearm amputation. The 5- and 10-year event-free survival rates were 67% and 67%, respectively. Three patients who were treated with a wide excision have 5- and 10-year event-free survival rates of 67% and 67%. Twenty-one patients had a partial amputation of the hand. In this group, the 5-year event-free survival rate was 76%, and the 10-year survival rate was 65%. Three patients had an axillary node dissection at the time of surgery. Two of the three patients had negative nodes at the time of surgery. One of the three patients had one of 15 nodes sampled, which indicated a tumor. All three patients were alive without evidence of disease after axillary node dissections at an average of 188 months after surgery (169 months, 155 months, and 241 months, respectively).

Adjuvant therapy also was used in the treatment of these patients. Six patients were treated with radiation therapy, including five of six treated with preoperative radiation therapy, four of six treated with intraoperative radiation, and two of six treated with postoperative radiation. The 5- and 10-year event-free survival rates of patients receiving any kind of radiation were 83% and 42% respectively. The survival rates of patients who did not receive any radiation therapy were 72% at 5 years and 72% at 10 years. Two patients had chemotherapy, one preoperatively and one as the only intervention. Both patients were alive and well at the 5-year followup, and one had an event before 10 years.

Patients with no residual neoplasm found at time of resection tended to have the best 5- and 10-year survival rates. When a tumor was present, patients with smaller tumors tended to have a better survival rate. Twenty-seven patients had specimens obtained for pathologic examination during surgery at our institution. Fifteen patients had residual neoplasms at the time of surgery. The 5-year and 10-year event-free survival rates of patients with tumors at the time of surgery were 65% and 49%, respectively. This compares with 83% and 83% survival rates in patients with no residual neoplasms at the time of surgery. The tumor size also was recorded. The maximal measured dimension was the parameter used. The average tumor was 2.6 cm, with a range of 0.1–5 cm when excised. Looking at the event-free survival rate by tumor size showed that patients with smaller tumors had better outcomes. The 5-year and 10-year event-free survival rates in patients with lesions smaller than 2.5 cm were 71% and 57% respectively, compared with 57% and 38% in patients with lesions larger than 2.5 cm. Vascular invasion and depth of lesion were not consistently documented; therefore, these two parameters were not used in the analysis (Fig 1).

Fig 1.
Fig 1.:
The photograph shows the palmar and dorsal surfaces of the left hand after ray amputation for epithelial sarcoma.

Only one patient in our series of 28 consecutive patients had a local recurrence. This patient was a 40-year-old woman presenting with a 10-year history of a painless mass in her palm, previously excised three times. Results of pathologic examinations from outside institutions are not known after the first two excisions. After the third excision, the pathologic diagnosis of epithelioid sarcoma was made by pathologists at our institution. The patient was treated with a wide excision and radiotherapy. She had a local recurrence 42 months after her initial procedure. On diagnosis of recurrence, she was treated with a distal third forearm amputation and axillary node dissection secondary to a palpable node. There were no positive nodes. At short-term followup of less than 1 year, she is alive without evidence of disease.

Nine of the 28 patients had metastasis with five of the nine having died of disease. Four patients had metastasis but were alive at the last followup; two had no evidence of disease, and two were alive with disease. The average duration from the time of surgery at this institution until metastasis was 53 months, with a range of 9–140 months. The average time from metastasis to death of the five patients who died of disease was 16 months, with a range of 1–46 months. The average duration between surgery and death in patients with metastasis was 48 months, with a range of 14–117 months. The location of metastasis was regional in four patients, involving the elbow lymph nodes in one patient and axillary lymph nodes in three patients. Five patients had distant metastatic disease occurring in the brain in one patient, the scalp in one patient, the lungs in two patients, and in the lungs and scalp in one patient. The overall 5- and 10-year Kaplan-Meier survival rates were 85% and 85%.

Four patients had metastasis to regional lymph nodes; two of these patients died of disease, and two are alive without evidence of disease at the last followup. Regional metastases occurred in 14% of all patients and are accountable for 40% of all treatment failures. One patient experienced metastasis to the epitrochlear nodes about the elbow. This patient was a 47-year-old man; he had surgical dissection of the epitrochlear nodes and resection of the epitrochlear mass. At the last followup 4 years after surgery, he is alive without evidence of disease. Three patients had metastasis to the axillary lymph nodes of the ipsilateral limb. Two patients, a 28-year-old man and 15-year-old girl, were treated with surgical dissection and resection plus adjuvant radiation therapy. One patient is alive without evidence of disease at 19 years. The 15-year-old girl died of disease 4 years after discovery of metastasis. A 35-year-man with axillary metastasis was treated with surgical resection and chemotherapy. He died of disease at 18 months after discovery of his metastasis.

Five patients had distant metastasis with three patients dying of disease and two patients alive with disease at the last followup. Distant metastases occurred in 18% of the patients and account for 50% of all recurrences. Two patients had pulmonary metastasis. Both were treated with resection plus adjuvant chemotherapy. One of the patients is deceased at 2 years from the diagnosis of pulmonary metastasis; the other patient is alive with disease at 4 years. Two patients experienced the first sign of disease as a metastatic lesion to the scalp. One patient was treated with surgical resection alone and is deceased at 10 months secondary to disease. The second patient was treated with surgery plus adjuvant chemotherapy and is alive with disease, in the form of pulmonary disease, at 2 years. The final patient with distant metastasis experienced metastasis to the brain. This patient was treated with surgical excision in addition to radiation therapy and systemic chemotherapy and is deceased secondary to disease at 1 year.

Wide excision, digit amputation, chemotherapy only, and ray amputation all tended to provide satisfactory function. In the chart review process, any time the examining surgeon noted that patient’s function was satisfactory on examination or the patient made this statement in the documented history, this patient was noted to have satisfactory function. All patients with forearm amputations had abnormal function; although, one of these three patients was fitted with a myoelectric prosthesis that assisted with activities of daily living. Three patients were treated with wide excision; all three had their function rated as satisfactory. One patient did not have surgery and was treated with only chemotherapy. This patient had satisfactory hand function. Eight patients had a hemi-amputation of the hand, including multiple digits or rays or parts of the carpus. In this group, seven of eight patients had abnormal function. One patient was treated with a finger amputation, having satisfactory function at followup. Twelve patients were treated with ray amputations. Nine of 12 patients had satisfactory function at last followup.

Of the 28 patients in our series, 20 (71%) had no evidence of disease at last followup. Three patients were alive with evidence of disease: one with local recurrence and two with distant metastasis. The patient with a local recurrence was treated with radical amputation. This patient had no signs of systemic disease at the last followup. Three patients had evidence of advanced disease at one point, but have survived for extended periods without additional recurrence. The overall survival, as estimated by the Kaplan-Meier method, was 85% at 5 and 10 years. This analysis was done with death as an end point.

Factors that showed a tendency to negatively affect outcome included: male gender, advanced age, increased tumor size at definitive resection, having more than one prior biopsy or surgery, or having residual neoplasm at definitive resection. Factors that did not show tendencies to influence outcome included: side, function, symptom duration, location, and adjuvant therapy (Fig 2).

Fig 2.
Fig 2.:
The Kaplan-Meier survival estimate of survival is shown for patients treated for epithelial sarcoma.

DISCUSSION

Soft tissue sarcomas are rare with 6000 new cases being reported each year.55 This accounts for less than 1% of all malignancies reported yearly.33 Only approximately 1000 of these 6000 soft tissue sarcomas occur in the upper extremity, with a minority occurring in the hand.55 In the current series, epithelioid sarcoma accounted for 28 of 78 soft tissue sarcomas treated in a 25-year period at a tertiary referral institution for surgery of the hand and orthopaedic oncology, totaling 36% of all hand sarcomas encountered. Synovial sarcoma was second most common, accounting for 14% of all cases. Despite being common in this location in our series of patients, epithelioid sarcomas are relatively uncommon, accounting for only 1.4% all soft tissue sarcomas treated at a large referral center.25 Other investigators of soft tissue sarcomas also have found epithelioid sarcomas to be the most common or one of the most common to occur in the hand.4,6,14,42,55

The patients in our series had many previous surgeries ranging from 0–6, with an average of 1.9 per individual. The number of procedures was not significant in terms of event-free survival; although, the 10-year event-free survival was 73% for the group with one or no surgeries and 49% in patients with greater than one biopsy. This trend supports the notion of early diagnosis and appropriate treatment at centers that offer orthopaedic oncology and surgery for the hand, both experienced in reconstruction and resection. Duration of symptoms did not show a tendency to influence outcomes in this data set.

There were numerous cases of missed diagnoses, resulting in multiple surgeries in 39% of patients. Epithelioid sarcoma has been mistaken for numerous conditions ranging from benign growths to rheumatoid nodules to infection at initial presentation. Typically, the presentation was with a slow-growing, painless mass in 82% of our patients. Many mistaken diagnoses of epithelioid sarcoma have been described in the literature including granulomata, fungal infections, warts, rheumatoid nodules, Dupuytren’s disease, or palmar fibrosis, among others.12,15,18,23,27,28,32,34,47,49 The typical presentation of a long duration of symptoms, male predominance, and young age have become associated with the condition.10,14,21,37,59 Our data supported the trend for females, who as a group presented at a younger age, and younger patients to have better outcomes.10

In reviewing our radiographic evaluation of this patient group, the accuracy of MRI in differentiating residual tumor from postoperative disease was confirmed as has been reported.22 All patients with a mass seen preoperatively on MRI scans did have tissue confirming residual epithelioid sarcoma. Six patients who had previous surgeries were thought to have only postoperative findings seen on preoperative MRI scans; unfortunately, one of the patients had a tumor as confirmed during surgery. As expected, CT scans and plain films were less useful in the diagnosis. Our institution has powerful, high-Tesla body coils, creating high-quality MRI scans, and the images are read by musculoskeletal radiologists. Another interesting study would be to assess size of mass by MRI in a patient with a previous resection and compare the size with the pathologic specimen. This data were not available. None of the plain films showed the calcification that had been seen in as much as 10% of cases in other series.14,42,61

Because of a lack of statistical power, we were unable to determine a statistical difference in outcome between patients treated with wide excision and amputation. Some authors advocate radical resection with this kind of tumor,43,51 but because of small numbers, it is difficult to prove a survival benefit with radical resection, specifically middle or distal forearm amputation.26,44,60 Patients with wide excision, finger amputation, and ray amputation had good outcomes in terms of function and survival. All patients with axillary node dissections at the time of their index operation survived long term (average, 188 months); although, only one of these patients had positive nodes at the time of surgery. Fong et al16 found radical lymphadenectomy in addition to surgical resection to increase survival in patients with extremity soft tissue sarcomas. The use of adjuvant therapy was inconsistent in our patient group; patients who received either chemotherapy or radiation did better in terms of 5-year event-free survival. The role for adjuvant therapy is most clear in the case of metastatic disease, where its use is advocated.44 Chemotherapy and radiation for treatment of nonmetastatic soft tissue sarcoma in the hand or epithelioid sarcoma in the hand has not been beneficial for patients as reported by some investigators.3,4,6,13,43 Chang et al,9 however, reported a 5-year survival benefit in patients with high-grade extremity soft tissue sarcomas treated with systemic chemotherapy, finding a 5-year survival rate of 83% in the group with chemotherapy versus 60% in the group without chemotherapy. It is difficult to extrapolate this finding to this specific anatomic location and this particular tumor.

In the current series, only one patient had a local recurrence at 42 months after surgery. In other series, the local recurrence rate has been as much as 87% to as few as 35%.14,50 Nine of 28 patients in the current series had distant metastasis. In this group, the average number of prior surgeries was 2.3, with a range of 1–6 surgeries. The average duration of symptoms was 28 months, with a range of 4–72 months. Patients in this group who died of disease tended to have more prior surgeries and a shorter duration of symptoms, possibly indicating more aggressive biologic factors of their tumors. This represents 32% of patients; a wide range of regional and distant metastases with rates of distant metastasis ranging from 30–58% have been reported.14,37 The location of metastasis in our series was regional in four patients, accounting for 44% of all metastases and 14% of all patients. This implicated that the spread is through lymphatic flow. Mazeron and Suit29 reviewed 183 patients who had soft tissue sarcomas, finding that only 8.2% had regional lymph node metastases. In that series, epithelioid sarcomas had 28% regional lymph node metastases.29 Rates of regional spread ranging from 17–80% have been reported.17,29 Five of our patients had spread to distant sites including the brain, the scalp, and the lungs, implicating hematogenous metastasis. The literature supports these findings with descriptions of pulmonary, cardiac, abdominal, central nervous system, and scalp metastases secondary to epithelioid sarcoma.7,35,39,41,49,52,53,58 These events may, as in our patients, occur very late.

Our look at function was global and involved ability to complete activities of daily living.Talbert et al54 reviewed function and reported that 50% of patients with soft tissue sarcomas of the hand and wrist treated with surgery and radiation had normal function. We found that patients treated with ray amputation, finger amputation, or wide excision had good function as a rule. Patients with a hemi-amputation of the hand tended to have poor function. It would be interesting to pursue a more systematic analysis of function of patients after aggressive treatment of hand tumors.

Because of the rare nature of this tumor, despite reviewing its most common location, our study lacked significant power for any of our variables to be statistically significant. Our study is a retrospective review of a series of patients who, except for one, had previous excision of the tumor before referral. This bias may make our data more difficult to interpret. Again, prospective studies of extremely uncommon conditions can be difficult. Our findings help to confirm previous descriptions of the behavior of this tumor. This lesion typically presents as a slow growing mass that may be associated with trauma in ⅓ of cases. Females have better results than males, and younger patients tend to do better than older patients. With time, patients with earlier diagnosis, meaning fewer biopsies or surgeries, tend to have better outcomes. Regional and distant metastases are common and may occur late. The role of specific adjuvant therapy or radical lymphadenectomy is unclear. Surgical management with wide margins can lead to a cure in a majority of patients. In our series, the overall survival rate was 85% at 5 years and 10 years.

Based on our experience, we recommend wide resection of primary epithelioid sarcomas of the hand. Based on our data, survival did not change with resection type, however, function did change. Resection should be chosen to allow negative surgical margins with optimal remaining function. This was achieved in a majority of patients even with numerous prior excisions treated with wide excision, ray resection, or digit amputation.

The role of chemotherapy and radiation are not clear. All patients treated with regional lymph node dissection are alive without disease at long-term followup. Routine use of lymph node dissection or sentinel node biopsy is not supported by the data in the current study or the literature. However, routine sentinel node biopsy had been advocated because of the frequent regional failures and is used at our institution.

Treatment of soft tissue sarcomas is optimized at tertiary referral centers by musculoskeletal oncologists. This is a slow growing tumor that occurs in young patients. Twenty percent of patients reported previous trauma. In a majority of the patients in this series, a benign diagnosis of either inflammatory or infectious nature was made at presentation. Patients with fewer prior surgeries and complete resections had superior survival. Any patient with an unusual bump should be evaluated preoperatively, and the patient should be referred if there is suspicion of malignancy.

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