Liposarcoma of the Spermatic Cord: A Case Report and Review of Management

ABSTRACT

Malignant lesions of the spermatic cord are rare; among them, sarcomas are the most frequent. The present article is a case report of a well-differentiated liposarcoma of the spermatic cord involving a 58 year-old male who presented with a painless scrotal mass. The mainstay of management is excision with radical orchiectomy. The most important factors for prognosis are the histologic subtype and surgical margin status. Adjuvant treatment may include radiation, depending on the presence of poor prognostic indicators. The role of chemotherapy is not clearly defined, and experience is limited to cases where recurrence has occurred. Patients with a well-differentiated liposarcoma of the spermatic cord have a good prognosis if surgical margins are negative and do not routinely require adjuvant therapy. Long-term surveillance is critical, because reports in the literature have demonstrated recurrences after 20 years.

KEYWORDS: Liposarcoma; Spermatic cord; Paratesticular tumor

CORRESPONDENCE: Joshua E. Logan, M.D., 6333 Center Drive, Building 16, Interstate Corporate Center, Norfolk, VA 23502, USA ().

CITATION: UroToday Int J. 2010 Aug;3(4). doi:10.3834/uij.1944-5784.2010.08.08

ABBREVIATIONS AND ACRONYMS: CT, computed tomography; FNCLCC, Fédération Nationale des Centres de Lutte Contre le Cancer; MRI, magnetic resonance image; NCI, National Cancer Institute.

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INTRODUCTION

Reports regarding paratesticular tumors have appeared in the literature for over 100 years [1]. Malignant lesions of the spermatic cord are rare; among them, sarcomas are the most frequent [2,3]. Liposarcomas are the most common of the soft tissue sarcomas, accounting for approximately 20% of all mesenchymal malignancies. Liposarcomas are most commonly diagnosed in the lower extremities and the retroperitoneum [4,5]. Involvement of the spermatic cord is much less common.

The following is a case report of a well-differentiated liposarcoma of the spermatic cord. A review of diagnostic and treatment issues is included.

CASE REPORT

A 58-year-old male presented to his primary care physician with a 9-month history of an enlarging left hemiscrotal mass. His past medical history included hypertension, hyperlipidemia, and type 2 diabetes mellitus. He had not undergone any previous surgeries. Family medical history was negative for cancer. Social history was negative for tobacco and alcohol. Alpha-fetoprotein, ß-human chorionic gonadotropin (ß-hCG), and lactate dehydrogenase (LDH) isoenzymes were all normal. A scrotal ultrasound indicated a left hemiscrotal vascular soft tissue mass. Computed tomography (CT) demonstrated a fat-containing mass with nodular enhancement in the anterior medial aspect of the left hemiscrotum (Figure 1a; Figure 1b). The mass was clearly separate from the testicle. There was no radiographic evidence of nodal or metastatic disease. The patient underwent a left radical orchiectomy.

There was a clear plane of dissection around the mass, which was entirely contained within the tunica vaginalis. Pathology confirmed the 16 cm x 3.5 cm x 4.5 cm well-circumscribed, encapsulated mass that was seen on gross inspection. It had a cut surface that was yellow and homogenous, with a focal nodular appearance. The relationship of the tumor to adjacent anatomic structures demonstrated that the tumor was grossly growing around but not invading the epididymis. There was no involvement of the tunica vaginalis. Microscopic examination revealed a lipomatous neoplasm consisting of variably sized adipocytes, rare multinucleated lipoblasts, and numerous atypical stromal cells. In addition, rare stromal mitoses were seen. Necrosis was not present. There was invasion into (but not through) the tunica albuginea of the testis. The spermatic cord resection margin was free of sarcoma. Two representative photomicrographs of the hematoxylin and eosin (H&E) stained tissues are shown in Figure 2a and Figure 2b. Examination by soft-tissue pathologists revealed a well-differentiated liposarcoma with mixed features of inflammatory and adipocytic subtypes.

The patient has been seen in follow-up at regular intervals and evaluated with imaging. He has had no adjuvant therapy to date. He has no evidence of disease recurrence.

DISCUSSION

Liposarcomas of the spermatic cord are rare. The differential diagnosis of a scrotal mass includes inguinal hernia, hydrocele, epididymitis, varicocele, teratoma, spermatocele, testicular tumor, lipoma, adenomatoid tumor, and sarcoma. The majority of these diagnoses are benign. In fact, most paratesticular lesions are benign cystic lesions of the epididymis, scrotal fluid collections, inflammatory lesions, or hernias.

The history of a patient with a paratesticular sarcoma is often a painless scrotal swelling that has progressed in size over a period ranging from months to years. Physical examination should focus on determining the local extent of the lesion and assessing for palpable inguinal adenopathy. Imaging in these patients helps characterize the lesion and assists with ruling out metastatic disease. Initially, inguinal and scrotal masses are commonly evaluated by ultrasound in order to answer the two most important questions: (1) is the mass intratesticular or extratesticular, and (2) is the lesion cystic or solid. Scrotal ultrasound can distinguish between intratesticular and extratesticular lesions with 98-100% sensitivity [6]. Scrotal ultrasound can also be helpful in evaluating scrotal extension for local staging [7]. However, these findings can often be variable and nonspecific, making definitive characterization difficult. With the use of CT and magnetic resonance imaging (MRI), aspects such as tumor location, morphologic features, and tissue characteristics can narrow the differential diagnosis [8].

Once the lesion is radiographically defined as solid, it is considered malignant until proven otherwise. This is clinically sound reasoning according to a report by Beccia et al [9], who showed that 56% of cord tumors are malignant (excluding cord lipomas). From a staging perspective, the purpose of additional imaging is to characterize the full extent of the lesion. This is particularly important because there have been multiple reports of retroperitoneal liposarcomas extending into the inguinal canal [10,11]. The present authors recommend contrast-enhanced CT or MRI of the abdomen and pelvis for this assessment.

Surgical therapy should include a radical orchiectomy, with early control of the spermatic cord at the internal ring to reduce the risk of tumor dissemination. One of the most common routes of tumor dissemination is by regional lymph node spread, according to Fagundes et al [12]. This is in clear distinction to soft tissue sarcomas of other locations where regional lymph node spread is uncommon. There may be some rationale to performing a regional lymphadenectomy at the time of orchiectomy; however, no data exist demonstrating improved outcomes. Intraoperative frozen sections should be obtained. If a sarcoma is diagnosed, a radical orchiectomy with high ligation of the cord should be performed.

According to the World Health Organization's International Classification of Disease, Tenth Revision (ICD-10) [13], there are seven recognized classifications of liposarcoma: (1) well differentiated, (2) myxoid, (3) round cell, (4) pleomorphic, (5) mixed, (6) dedifferentiated, and (7) not otherwise specified. The histological type of liposarcoma does not always provide sufficient information to stratify tumors into those with a favorable prognosis and those with a poor prognosis. Therefore, grading based on specific histologic features determines the probability of local and distant recurrence and overall survival. However, the results of most studies support the quality of surgical margins as the most important factor for predicting recurrence [14].

There are 2 commonly used soft tissue sarcoma grading systems: the National Cancer Institute (NCI) system and the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) system [15,16]. Both are 3-grade systems that are well established and widely accepted. However, according to Guillou et al [17] the FNCLCC grading system has a better correlation with overall and metastasis-free survival. In this system, a score is attributed independently to mitotic activity, differentiation, and necrosis; the grade is obtained by adding the 3 scores.

Both the NCI and FNCLCC grading systems assigned a grade to each of the histologic types of liposarcoma. The well-differentiated type is a grade 1. Historically, grade 1 lesions do not metastasize, but have a propensity for local recurrence [4]. Microscopically, the tissue consists of mature fat that is mixed with atypical hyperchromatic cells and rare lipoblasts. Over time, these lesions may progress histologically toward dedifferentiation, a process by which the tissue gains metastatic potential. Dedifferentiation is a time-dependent phenomenon [18]. This is typically a late-complication of grade 1 disease. The majority of dedifferentiated lesions present de novo (ie, not as a progression from a well-differentiated type) and are described as high-grade lesions [19]. An additional histologic type that is designated a grade 3 (high-grade) lesion is pleomorphic liposarcoma. This lesion is rare and accounts for less than 5% of all liposarcomas [4,20]. A key feature of pleomorphic liposarcoma is necrosis, which correlates with poorer prognosis for overall survival [20]. The remaining 2 histologic subtypes are myxoid and round cell liposarcomas, which together comprise 40% of liposarcomas. The myxoid variant is a tumor of varying behavior, but it is traditionally classified as a low-grade lesion. However, the myxoid variant could be considered a grade 2 lesion in the FNCLCC grading system with the presence of mitoses and necrosis. Round cell liposarcomas are high-grade tumors. These 2 variants can be thought of as being on a spectrum: the larger the numbers of round cells, the worse the behavior of the lesion [4,18].

Historically, treatment with surgical extirpation alone has been the primary mode of therapy. Surgical therapy alone appears to be appropriate for those patients with small tumors and wide negative margins. Baldini et al [21] described positive results from surgery in 74 patients with soft tissue sarcomas of the extremities and trunk and an average tumor size of 4 cm. Local control at 10 years was 93%. Adequate resection with negative margins is critical to outcome. Zagars et al [22] reported the experience of 666 patients who underwent gross total resection before referral to MD Anderson Cancer Center. In this series, 295 patients underwent resection for positive or uncertain margins. Resection improved local control, metastasis-free survival, and disease-specific survival. Montgomery and Fisher [23] reported on 30 patients with resected paratesticular liposarcomas, 60% of which were low-grade. Only 2 patients in this group received adjuvant therapy. Eight recurrences were observed over highly variable time courses ranging from 2 years to over 20 years. These results underscore the necessity of long-term evaluation of these patients.

Radiation therapy has an important role in the management of soft tissue sarcomas. The role of adjuvant radiation therapy for soft tissue sarcomas of the extremities or superficial trunk has been evaluated in randomized prospective trials. Two studies concluded that adjuvant radiation therapy improved local recurrence, but not overall survival [24,25]. Timing of preoperative or postoperative adjuvant radiation was evaluated in a randomized controlled trial [26] and there was no difference in local control or survival. However, there were increased wound complications in those patients undergoing neoadjuvant radiation. Fagundes et al [12] studied 18 patients with resected spermatic cord sarcoma, including 7 patients with liposarcoma. Nine patients received adjuvant radiation consisting of a median dose of 4610 cGy to the regional ipsilateral iliac and inguinal lymph nodes. At 5 years, local recurrence was seen in 5 of the 9 patients in the surgery-only group. No local recurrences occurred in those patients receiving adjuvant radiation. Data regarding which histological types of sarcoma recurred in the surgery-only group were not provided. Thus, the only conclusion that can be made is that surgery combined with radiation appears to provide superior locoregional control as compared to surgery alone for spermatic cord sarcomas, in general.

Some investigators advocate adjuvant radiation for all patients with malignant tumors of the spermatic cord. Blitzer et al [27] reported on 10 patients with tumors of the spermatic cord during a 37-year period. Seven patients had at least 5 years of follow-up; 5 of the 7 patients had a recurrence. On the basis of this retrospective data, the authors modified their standard approach to include postoperative radiation therapy. However, Coleman et al [28] reported their experience with spermatic cord sarcomas in 47 patients over 24 years. Of the 47 tumors, 24 were liposarcomas. Fourteen patients received adjuvant radiation, 3 patients received doxorubicin-based chemotherapy, and 7 patients received a combination of radiation and chemotherapy. The patients that received adjuvant treatment were not reported by histology. The authors concluded that a therapeutic effect with adjuvant radiation or chemotherapy was not demonstrated. They did show that patients with inadequately resected disease who underwent a reoperative procedure, including wide resection and negative margins, had significantly less tumor recurrence and longer disease-free survival; this result approached statistical significance.

Liposarcoma of the spermatic cord is relatively rare, leading to a paucity of data. Therefore, it is difficult to make evidence-based decisions about adjuvant treatment. However, the larger experience with extremity and trunk soft tissue sarcomas can be applied to genitourinary sarcomas. Adjuvant radiation therapy improves local control in the majority of patients.

The role of adjuvant chemotherapy in the treatment of liposarcomas of the spermatic cord is even less clear than the role of radiation therapy. In the very few cases reported, chemotherapy has been used for treatment of recurrent disease with only limited follow-up. Adjuvant doxorubicin-based chemotherapy for resected localized soft tissue sarcomas was evaluated in a large meta-analysis. Patients with liposarcoma comprised 9% of the study population. Time to local and distant recurrence was improved with chemotherapy. Overall, recurrence-free survival was improved and a trend toward increased overall survival was observed [29].

A multidisciplinary approach to patients with paratesticular liposarcoma is critical, given the rarity of the tumor and the controversy surrounding postoperative therapy. These patients can have recurrences exceeding 2 decades from their surgery and thus require long-term follow-up.

ACKNOWLEDGEMENT

The authors thank Mary L. Blumberg, MD for her assistance with the pathology portion of this manuscript.

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