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Annals of Surgical Oncology 10:669-675 (2003)
© 2003 Society of Surgical Oncology


ORIGINAL ARTICLES

Adult Spermatic Cord Sarcomas: Management and Results

Jonathan Coleman, MD, Murray F. Brennan, MD, Kaled Alektiar, MD and Paul Russo, MD

From the Departments of Urology (JC, PR), Surgery (MFB), and Radiation Oncology (KA), Memorial Sloan-Kettering Cancer Center, New York, New York.

Correspondence: Address correspondence and reprint requests to: Paul Russo, MD, The Sidney Kimmel Center for Prostate and Urologic Cancers, 353 East 68th Street, Room 525, New York, NY 10021; Fax: 212-988-0760; E-mail: russop{at}mskcc.org


    ABSTRACT
 TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
Background: We present a 20-year surgical experience with spermatic cord sarcoma, describe prognostic features, and evaluate the results of surgical and adjunctive treatments.

Methods: Forty-seven patients were identified from an institutional database. Disease-free survival was calculated, and prognostic factors such as tumor grade, size, extent of operation, and adjuvant therapy were analyzed.

Results: The median patient age was 58 years (range, 16–83 years), and the median follow-up was 51 months (range, .5–226 months). The most common tumor types included liposarcoma (51%), leiomyosarcoma (19%), embryonal rhabdomyosarcoma (13%), and malignant fibrous histiocytoma (11%). Twenty-nine (62%) patients had high-grade tumors, 21 (45%) were treated with adjuvant radiation, and 9 (19%) received chemotherapy. The overall 5- and 10-year disease-specific survival was 75% and 55%, respectively. No specific prognostic factors were identified for recurrence or disease-free survival. In 21 patients who underwent reoperative wide resection after a prior incomplete resection, a trend toward improved disease-free survival was noted (P < .059). Of these, six (29%) had residual viable sarcoma. We could not demonstrate a therapeutic effect of adjuvant radiation or chemotherapy.

Conclusions: We demonstrated that aggressive surgical strategies, including reoperative wide resection, significantly decrease local recurrence and may improve disease free-survival in select patients with spermatic cord sarcoma.

Key Words: Sarcoma • Spermatic cord • Paratesticular • Re-resection


    INTRODUCTION
 TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
Soft tissue sarcomas include a variety of unique neoplasms that arise from tissues of mesodermal origin. Sarcomas are uncommon tumors, with <8300 new cases reported in the United States in 2001.1 Sarcomas of the genitourinary tract account for <5% of these cases and <2% of all urological tumors. The spermatic cord is the most commonly involved urological site and accounts for approximately 30% of all genitourinary sarcomas.2

Significant risk factors for tumor recurrence and progression have been identified in adult sarcomas and include tumor grade, size, depth of invasion, and surgical margin status.3,4 In general, with the exception of embryonal rhabdomyosarcomas, histological subtype has not been shown to have a significant effect on prognosis independent of tumor grade. Several staging systems exist for soft tissue sarcomas; however, disagreement exists such that a comprehensive system has not been established, except for extremity lesions.

Investigation of spermatic cord sarcomas has been difficult because of their rarity. A literature review from 1845 to 1978 revealed only 212 reported cases of spermatic cord sarcomas.5 Since then, only small series from single institutions have been published, and these have had inadequate or incomplete data available for risk factor analysis.6–9 Such limitations have prevented the development of a consensus concerning optimal surgical and adjunctive treatment strategies. In this study, we critically examined all cases of adult sarcoma of the spermatic cord from a single cancer center by using data collected through a prospective sarcoma database. The objective of this study was to examine risk factors for disease recurrence and progression and to report the treatment results obtained.


    MATERIALS AND METHODS
 TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
In June 1982, a prospective database was established to enter and follow up clinical information on adult patients (>16 years) with soft tissue sarcomas who were evaluated, treated, and followed up at Memorial Sloan-Kettering Cancer Center (MSKCC). Clinical data were entered and accrued from the time of initial presentation to last follow-up or death from disease. Fifty adult patients with spermatic cord sarcomas were identified between May 1981 and September 2001. We analyzed initial tumor size, histological grade (high or low), number and extent of all surgical procedures, pathologic margin status (gross and microscopic), local recurrence, distant metastasis, adjuvant radiation and chemotherapy, and follow-up status. Pathologic evaluation of all pathologic specimens was re-reviewed at our institution at the time of patient referral. Follow-up was performed through January 2002. Actuarial disease-free and disease-specific survival were calculated with the Kaplan-Meier method. Multivariate analysis with Cox regression analysis and the log-rank test was used to compare survival between groups. Disease-specific survival was defined as death from sarcoma progression, whereas death from other causes was treated as a censored event. Disease-free survival evaluated the events of local recurrence and distant metastases as similar end points. We defined local recurrence as the presence of disease within the surgical field or contiguous retroperitoneal structures after the first documented complete resection. Patients with clinical recurrence who underwent surgical resection were documented. Patients who initially presented with distant metastases were evaluated separately.


    RESULTS
 TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
Patient Data
Fifty adult patients with spermatic cord sarcomas were identified during the study period. This represented <1% of >5000 cases of soft tissue sarcomas admitted for treatment at our institution. Three patients were lost to follow-up after initial evaluation at our institution without further treatment. Complete data were available on the remaining 47 patients, with a median follow-up time of 51 months (range, .5–226 months). Histological subtypes of tumors and associated data are listed in Table 1. The overall median patient age was 58 years (range, 16–83 years). Patients with embryonal rhabdomyosarcoma (ERMS) were significantly younger than other patient groups at the time of diagnosis (P < .0001). The median tumor size was 6.0 cm (range, 2.5–20 cm). Most tumors were high grade (29 tumors; 62%). Low-grade lesions were almost exclusively associated with liposarcoma, comprising 71% of these tumors. One leiomyosarcoma was low grade (P < .0001; Fisher’s exact test). Of the other multiple clinical variables analyzed, the only variable found to be significantly different between the two groups—high- and low-grade tumors—was the use of adjuvant radiotherapy, which was more often used for high-grade lesions (P < .05).


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TABLE 1. Clinical and histological characteristics for patients with spermatic cord sarcomas
 
Primary tumor location was equally distributed between the right and left side (53% left vs. 46% right). Seven patients (15%) had metastatic disease at the time of initial presentation, and one patient with widely metastatic ERMS died 2 weeks after diagnosis without receiving treatment. A total of 15 patients (32%) died of disease during the study period. The overall 5-year disease-specific survival was 75% (Fig. 1).



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FIG. 1. Overall disease-specific survival in a series of patients with spermatic cord sarcomas.

 
Diagnosis
All patients presented with a palpable mass in the scrotum or inguinal area. Patients with low-grade inguinal liposarcomas were frequently diagnosed incidentally at the time of inguinal hernia repair. Sonographic studies revealed a solid mass in all six patients in whom the study was performed before primary surgical resection. Ultrasound findings typically revealed solid, hyperechoic, heterogeneous lesions that were considered suggestive of malignancy.

Treatment
Surgery
Forty-six patients were initially treated surgically; 45 of these cases were treated elsewhere before referral. Forty-one patients underwent radical orchiectomy in the course of their management, and 15 of these procedures were performed at MSKCC. Thirty-one cases had documentation by the surgeon of initial complete resection. Of these, seven (23%) had positive surgical margins on pathologic analysis of the initially resected specimen. Initial treatment options were dependent on radiographical staging and tumor type (Fig. 2). Forty patients underwent a second wide local excision procedure with intent to improve local control (n = 35; 88%) or to resect suspected recurrent disease (n = 5; 12%). Fourteen patients underwent a third operation, and three patients required a fourth procedure .



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FIG. 2. Initial management options by initial stage at the time of referral. DOD, died of disease; NED, no evidence of disease; RT, radiotherapy.

 
Re-Resection
Twenty-one patients without radiographical or clinical evidence of recurrent local disease at the time of referral were treated with wide re-resection at MSKCC. This procedure was performed in cases considered at risk for residual disease, including incomplete prior resection as indicated by positive or indeterminate surgical margin status, incomplete radical orchiectomy, or high-grade tumor. Wide re-resection included scar excision with a wide margin around the inguinal canal from the internal inguinal ring to the ipsilateral hemiscrotum. Completion orchiectomy or removal of any spermatic cord remnant was performed as high as possible up to the internal inguinal ring, and the proximal extent of the cord was labeled for pathologic margin evaluation. In cases of paratesticular or testicular involvement, hemiscrotectomy was also performed en bloc with the surgical specimen. All incisions were closed primarily without the need for reconstructive techniques, and no complications were encountered during follow-up.

Characteristics of this group are listed in Table 2. Of these 21 patients, all had negative surgical margins, and 6 (29%) had residual sarcoma discovered in the specimen. Four of these six patients had high-grade sarcomas, and three patients required completion radical orchiectomy. These factors, however, did not have a statistically significant effect on the outcomes of survival or recurrence when compared with similarly treated patients who did not have residual tumor. Compared with 19 patients with no clinical evidence of disease who did not undergo re-resection (because they had low-risk disease or refused surgery), re-resection patients had less tumor recurrence (38% vs. 74%; P < .05) and had longer disease-free survival, which closely approached statistical significance (P < .059) (Fig. 3). Adjuvant radiation was used less often in the re-resection group and was primarily reserved for patients who had residual sarcoma discovered in their re-resection specimen (33% vs. 58%).


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TABLE 2. Comparison of patient characteristics treated with or without wide re-resection for spermatic cord sarcoma
 


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FIG. 3. Actuarial disease-free survival for patients with spermatic cord sarcomas treated with or without wide re-resection.

 
Adjuvant Treatment
Twenty-four patients received adjuvant treatment with localized radiation (n = 14), doxorubicin-based chemotherapy (n = 3), or a combination of radiation and chemotherapy (n = 7). Radiation modalities included external beam radiation or brachytherapy, depending on the site and need for adjunctive use with surgery. Of the patients who received adjuvant radiation alone, the average tumor size was 6 cm (range, 4–9.5 cm). Most were high-grade tumors (n = 11; 79%). Seven adjuvant-radiation patients (50%) had recurrent disease: four at local sites and three with distant metastases. In comparison, 23 patients did not receive adjuvant therapy; 4 of these patients experienced local recurrence, and 6 developed distant metastases (P = .41). Of three adjuvant-radiation patients who had positive surgical margins before radiation treatment, one experienced local recurrence and one experienced distant recurrence. Doxorubicin-based chemotherapy was reserved for patients with ERMS treated under the T6 protocol or in selected cases of unresectable disease.

Recurrence
Of the 40 patients who did not present with metastatic disease, 22 (55%) developed recurrent disease. Eleven patients (28%) initially had local recurrences, 8 of which were liposarcomas (P < .05). Only two patients, one with low-grade and one with high-grade sarcoma, who had local recurrence went on to develop distant metastases after regional re-resection. The remaining 11 patients developed distant disease after initial resection for localized sarcoma. Sites of metastasis included discontiguous retroperitoneum (n = 5), pulmonary (n = 3), bone (n = 1), liver (n = 1), and maxillofacial (n = 1).

Eighteen patients either presented with metastatic disease or subsequently developed distant cancer after initial treatment. After a median follow-up of 58 months, four patients had no evidence of disease, whereas the remainder had either died of disease or had unresectable sarcoma after aggressive multimodality treatment. Of the four survivors, the median follow-up was 75 months (range, 40–174 months). All underwent complete surgical resection of metastatic lesions (two retroperitoneal and two pulmonary), three received adjuvant radiation treatment, and two with ERMS received doxorubicin-based chemotherapy.

Multivariate Survival Analysis
Multivariate analysis of disease-free survival in patients who did not present with metastatic disease identified a significant association with the combined variables of positive margin at first resection, wide re-resection, and positive margin at second resection (P < .001; {chi}2). Positive margin status after the performance of wide re-resection was associated with a higher chance of recurrence. Disease-free survival over time was shorter when there were positive surgical margins at first and second resection (P < .005). Clinical factors that did not significantly affect disease-free status in this series included tumor grade, size, and adjuvant radiotherapy.

Multivariate analysis of variables affecting disease-specific survival revealed that both complete first and second resection were associated with improved outcome (P < .005; {chi}2). Multiple regression analysis with time to follow-up also identified complete resection as a requirement for survival (P < .0001). Tumor grade, tumor size, and adjuvant radiation did not contribute significantly to survival.


    DISCUSSION
 TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
Spermatic cord sarcoma is a rarely encountered malignancy that may be mistakenly diagnosed as more common processes, such as inguinal hernia or cord lipoma. The unexpected discovery of a sarcoma in the scrotum or inguinal region often poses a surgical dilemma when options for complete resection, including radical orchiectomy with wide local soft tissue excision, have not been discussed with the patient before surgery. Most patients in this series (48 of 50) were initially explored and treated elsewhere before referral to our center for further treatment. Radical orchiectomy is a necessary component of surgical management and was performed in nearly one third of patients referred to our institution to achieve adequate complete resection of the cord and adjacent structures. This common presentation highlights the difficulties in treating and investigating these tumors: specifically, the variability in early surgical management and adjuvant treatment.

Factors that can affect local control rates include tumor biology, adequacy of surgical resection, and adjuvant treatment. The current treatment strategy for all soft tissue sarcomas begins with a complete local resection. The importance of adequate surgical margins has been well documented.10 Adjuvant radiotherapy decreases local recurrence but not long-term survival in extremity lesions.6,11–13 When concern for vital anatomical structures during resection is an issue (i.e., major blood vessels and nerves), a close surgical margin may provide reasonable local control when adjuvant or neoadjuvant radiation is used.12–14 The risks, in this case, associated with local recurrence and subsequent long-term survival are unclear but may be intermediate between the outcome for patients who undergo complete resection and for those with positive margins.15,16 Optimal initial surgical treatment of soft tissue sarcomas entails early wide local excision that includes all nonvital structures. In the case of a spermatic cord sarcoma, radical orchiectomy and wide local resection of surrounding soft tissues should be performed.

In this series, our reoperative strategy of wide re-resection and obtaining a negative margin status significantly influenced disease-free survival, supporting the value of aggressive complete surgical resection. These findings are consistent with other investigations of soft tissue sarcomas, in which positive surgical margins and incomplete resection have been shown to correlate with early local recurrence and subsequent mortality.3,15,17,18 In a study of wide local excision for spermatic cord sarcoma in a limited series of patients, Rabbani et al.19 demonstrated the ominous prognostic significance of positive surgical margins. In this study, the relatively high rate of positive margins (19%) found in the specimens after initial local excision and the percentage of patients with positive pathology after wide re-resection (29%) further documents the problem with intraoperative assessment of complete excision. Namely, local excision does not provide sufficient correlation with adequate complete resection. Of the patients without clinically apparent disease who underwent wide re-resection at MSKCC, residual tumor was discovered in nearly a third of patients, and negative margins were obtained in all cases, suggesting that wide resection provides the best chance for local surgical control.

We perform wide re-resection in circumstances in which complete previous resection has not been performed or cannot be assessed, such as in the case of inguinal orchiectomy alone. Completion radical orchiectomy is performed in all cases when the ipsilateral cord and testis are still present. Resection is performed widely around the inguinal canal and previous scar to include surrounding muscle layers, all soft tissues of the cord remnant, and soft tissue just deep to the internal inguinal ring. For sarcomas involving the scrotum or in patients presenting with a scrotal scar, hemiscrotectomy is performed. In the case where a cord remnant exists, the aponeurosis of the external oblique is opened, and the cord is taken at the internal inguinal ring. These same principles are followed in the few cases in which patients have been treated by primary resection.

The effectiveness of re-resection for spermatic cord sarcomas has not heretofore been evaluated. A recent review of 88 male and female patients treated at MSKCC for groin sarcomas identified margin status, patient age, and tumor grade as significant predictors of recurrence and progression.20 The institutional experience with wide re-resection has been shown to be beneficial in treating soft-tissue sarcomas of the extremity.4 This approach has been adapted for the treatment of spermatic cord sarcomas with the understanding that, despite anatomical differences, the principles of resection in these sites are essentially the same. The customary surgical practice of treating primary testicular tumors with inguinal orchiectomy and high ligation of the spermatic cord alone seems to be insufficient surgical management for spermatic cord sarcomas. Patterns of local dissemination in spermatic cord sarcomas are different, extending locally into soft tissues around the cord and inguinal canal in addition to hematological and, very rarely, lymphatic routes. Indeed, patterns of tumor detection and spread in the extremity and spermatic cord are comparable, in contrast to other genitourinary sarcomas. Spermatic cord sarcomas may arise from any mesodermally derived cord structures, including cremasteric and interstitial cells that lie outside of, or are components of, the fascial layers of the cord itself.21 Thus, they may not be limited by this anatomical boundary.

Routes of spread for soft tissue sarcomas may vary depending on the sarcoma histological type. Rhabdomyosarcoma can spread through lymphatic routes and carries an increased risk of retroperitoneal involvement. Historically, retroperitoneal lymphadenectomy (RPLND) had been suggested as treatment for all high-grade sarcomas.22 At our institution, we generally reserve RPLND for ERMS, for which it was performed in three of six cases. Hematogenous dissemination is also a significant path for tumor metastasis, as demonstrated by the number of recurrences in this series at distant sites. Excluding patients with rhabdomyosarcoma, four lymph node dissections of high-grade spermatic cord sarcomas were performed. Three patients underwent regional lymphadenectomy, with negative findings on pathology; one patient underwent extended RPLND for a retroperitoneal mass, with positive pathology for high-grade sarcoma. This patient remained disease-free after 2 years of follow-up. If RPLND had been performed in all 23 patients with high-grade tumors without rhabdomyosarcoma, 21 patients (87%) would have undergone unnecessary operations. RPLND should be reserved for cases of identifiable lymphatic involvement or ERMS.

One half of patients experienced tumor recurrence; this underscores the need for thorough radiographical evaluation and intensive, long-term follow-up. Relatively few patients received adequate perioperative radiographic staging before their initial surgery or referral, and this emphasizes the rarity of the lesion and the difficulties with misdiagnosis. A number of case histories also indicated that several patients with palpable solid lesions in the scrotum were followed up clinically for several months before surgical removal. The radiographical diagnosis of a solid lesion was made with sonography in all cases in which it was used, confirming its value as an initial study to evaluate any mass of the spermatic cord or scrotum. Other series, however, have not supported this degree of reliability with scrotal ultrasound.23

Our experience with spermatic cord sarcomas has several limitations. The data set is small, and adjuvant therapies were delivered on an individual basis; thus, their potential therapeutic effect cannot be fully judged. The potential bias in the selection of cases with unfavorable pathologic features for adjuvant therapy may be partly responsible for the lack of a significant observed benefit. Despite these limitations, an emerging pattern of disease progression is apparent that is consistent with the management of soft tissue sarcoma arising from other sites and renders our approach of repeat wide local resection most reasonable. In this series, patients treated with adjunctive radiation were not significantly different in the parameters of patient age or tumor histology, grade, or size. Still, a significant advantage for local recurrence or overall survival was not seen. Radiotherapy has demonstrated efficacy in larger series of sarcomas in decreasing the rates of local recurrence, so its use in this setting is also rational.


    CONCLUSION
 TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
Sarcoma of the spermatic cord commonly presents as a firm, palpable mass of the inguinal canal or scrotum. Any palpable mass of the cord structures should be evaluated before surgery by scrotal ultrasound. Solid masses should be excised by using an inguinal incision and sent for frozen sectioning. If the pathology is suggestive of sarcoma, radical orchiectomy with high cord ligation and wide excision of surrounding soft tissue structures within the inguinal canal should be performed. The risk of recurrence is high and, in this data set, was not clearly affected by tumor grade, tumor size, or use of adjuvant radiation. Rhabdomyosarcomas have a different pattern of metastases, with more likely involvement of retroperitoneal nodes; thus, RPLND is appropriate in that setting. RPLND is also appropriate for those rare cases in which retroperitoneal nodal metastasis is identified on imaging studies. Liposarcomas are frequently low-grade lesions; however, their risk of local recurrence is comparable to that of high-grade lesions, and they should be aggressively treated. Patients with inadequately resected disease should undergo a reoperative procedure for wide inguinal re-resection, including completion orchiectomy or removal of the cord remnant to the internal inguinal ring with surrounding soft tissue and scar excision adequate to obtain a negative margin. The presence of high-grade sarcoma in these re-resected cases represents a greater risk of recurrent and metastatic disease. The definitive value of adjunctive radiation treatment or chemotherapy cannot be assessed in this small data set.


    FOOTNOTES
 
Aggressive surgical strategies, including reoperative wide resection, significantly decrease local recurrence and may improve disease free-survival in select patients with spermatic cord sarcoma.

Received for publication November 22, 2002. Accepted for publication March 19, 2003.


    REFERENCES
 TOP
 ABSTRACT
 INTRODUCTION
 MATERIALS AND METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 

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  10. Beech DJ, Pollack RE. Surgical management of soft tissue sarcoma. Hematol Oncol Clin North Am 1995; 9: 707–18.[Medline]
  11. Pisters PWT, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 1996; 14: 859–68.[Abstract/Free Full Text]
  12. Sadoski C, Suit HD, Rosenberg A, Mankin H, Efird J. Preoperative radiation, surgical margins and local control of extremity sarcomas in soft tissues. J Surg Oncol 1993; 53: 223–30.
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  14. Yang JC, Chang AE, Baker AR, et al. Randomized prospective study of the benefits of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 1998; 16: 197–203.[Abstract/Free Full Text]
  15. Heslin MJ, Woodruff J, Brennan MF. Prognostic significance of a positive microscopic margin in high-risk extremity soft tissue sarcoma: implications for management. J Clin Oncol 1996; 14: 473–8.[Abstract/Free Full Text]
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  17. Lewis JJ, Leung D, Casper ES, Woodruff J, Hajdu SI, Brennan MF. Multifactorial analysis of long-term follow-up (more than 5 years) of primary extremity sarcoma. Arch Surg 1999; 134: 190–4.[Abstract/Free Full Text]
  18. Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 1996; 14: 1679–89.[Abstract/Free Full Text]
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  20. Brooks AD, Bowne WB, Delgado R, et al. Soft tissue sarcomas of the groin: diagnosis, management, and prognosis. J Am Coll Surg 2001; 193: 130–6.[CrossRef][Medline]
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