Giant well-differentiated liposarcoma of the spermatic cord: Case report

Abstract

Liposarcomas represent less than 5% of genitourinary sarcomas and account for approximately 21%–51% of all paratesticular sarcomas, with the spermatic cord being the most in-volved structure. Diagnosis is confirmed by histopathology, and the mainstay of treatment is complete surgical resection, which can be supplemented with radiotherapy if surgical margins are positive. The role of chemotherapy remains controversial. We present the case of an 86-year-old male with a well-differentiated liposarcoma of the spermatic cord.

Highlights

• •Giant spermatic cord liposarcoma in an 86-year-old patient.
• •Clinical presentation mimicked a slowly growing benign scrotal mass.
• •Complete resection with high cord ligation achieved negative margins.
• •Long-term follow-up is essential due to the risk of late recurrence.

1. Introduction

Liposarcomas are malignant mesenchymal tumors of adipose tissue that most often arise in the thigh or retroperitoneum; paratesticular localization is exceedingly rare.¹ Even though liposarcoma comprises about 20% of all soft tissue sarcomas,¹ fewer than 200 cases of spermatic cord (paratesticular) liposarcoma have been reported in the literature worldwide.² The spermatic cord is the most favored site for paratesticular liposarcomas, accounting for only ∼3–7% of all liposarcoma cases by primary location.³ Notably, liposarcoma is reported to be the single most common histologic subtype of adult paratesticular sarcomas (comprising roughly 39%–51% of cases),⁴ underscoring the need to consider this entity in patients with intrascrotal tumors.

Paratesticular liposarcoma typically presents in older adult males, most frequently during the fifth to seventh decades of life,⁵ though cases have been documented from adolescence up to the ninth decade (patients in their 80s).⁵ They can attain very large sizes if neglected; for example, cases of paratesticular liposarcoma measuring over 50 cm and weighing 30 kg have been reported.¹ Such an enlarging scrotal mass often causes a feeling of heaviness and discomfort. Due to their indolent growth and nonspecific presentation, paratesticular liposarcomas are frequently misdiagnosed initially as benign conditions, including inguinal hernias, hydroceles, epididymal cysts, lipomas, or even testicular tumors.⁴ This potential for misdiagnosis highlights the importance of thorough evaluation (with ultrasound or other imaging) of any atypical scrotal mass.

There are five recognized histological subtypes of liposarcoma⁶: well-differentiated, myxoid, round cell, dedifferentiated, and pleomorphic.^7,8 Well-differentiated liposarcoma is also termed atypical lipomatous tumor in surgically accessible locations, and it includes adipocytic [lipoma-like], sclerosing, and inflammatory variants. The last three subtypes, round cell, dedifferentiated, and pleomorphic, are considered high-grade tumors and are associated with significantly worse prognoses.⁹ In contrast, well-differentiated liposarcoma (WDLPS) is a low-grade, locally aggressive neoplasm with a relatively favorable outcome. Indeed, most reported paratesticular liposarcomas are of the well-differentiated (lipoma-like) type, which carries a better prognosis than the dedifferentiated or pleomorphic variants.⁴ We present the case of an elderly patient with a long-standing giant paratesticular liposarcoma, and we discuss its management in light of the current literature.

2. Case report

An 86-year-old male presented to the urology department of our hospital with a chief complaint of a left scrotal mass that had been gradually increasing in size over the past three years. He had a history of a left radical orchiectomy 18 years prior at another institution (no pathology report available), and no other significant medical history. The new mass had grown slowly and painlessly, eventually limiting his daily activities due to its bulk. On physical examination, a very large, firm, non-tender scrotal mass was noted in the left hemiscrotum, measuring approximately 30 × 20 cm with irregular contours. The penis was partially buried within the mass, and the right testis could not be palpated separately on exam (presumably due to displacement by the mass) (Fig. 1).

Fig. 1.

Physical exam of patient with paratesticular liposarcoma. A. Coronal view of scrotal tumor that displaces penis laterally. B. Sagittal view with displacement of buried penis to the right. C. Transverse view shows real size of scrotal tumor.

Scrotal ultrasound and an abdomino-pelvic CT scan demonstrated an encapsulated, predominantly fat-density intrascrotal tumor measuring about 30 × 23 × 12.5 cm (Hounsfield units ∼ −98, consistent with adipose tissue). There was no evidence of inguinal or retroperitoneal lymphadenopathy on imaging. Serum tumor markers, including alphafetoprotein (AFP), beta-human chorionic gonadotropin (β-hCG), and lactate dehydrogenase (LDH), were within normal limits.

A traction suture is placed on the glans to facilitate manipulation and identification of the penis, which is buried within the tumor. The affected scrotal area is then demarcated, and an incision is made using a scalpel and electrocautery. LigaSure forceps are advanced around the mass to achieve better hemostatic control. The incision is extended until the preputial area is identified and preserved intact. The contralateral, unaffected testicle is also identified and carefully isolated from the tumor. The mass is excised en bloc, avoiding any residual disease. The spermatic cord is ligated, and sufficient skin is preserved to allow adequate closure, ensuring satisfactory oncologic and cosmetic outcomes,(Fig. 2).

Fig. 2.

Final outcome of reconstruction of genital area. A. Coronal view shows reconstructed scrotus and penis. B. Sagital view of penile trunk and glan. C. Transverse view of glan and base penis.

On gross pathological examination, the resected specimen was a multinodular, yellow tan mass with interspersed fibrous septa of varying thickness. It measured 30 × 23 × 12.5 cm and weighed 4.375 kg (Fig. 3). Microscopically, the tumor was composed of a proliferation of adipocytic cells with scattered atypical lipoblasts, consistent with a well-differentiated liposarcoma. Most areas showed features of a lipoma-like (well-differentiated) liposarcoma, while focal regions contained increased cellularity with pleomorphic lipoblasts. In the non-lipogenic (dedifferentiated) areas, sheets of pleo-morphic spindle cells were present, indicating progression towards a higher-grade component. According to the French FNCLCC grading system, the tumor was classified as histologic grade 3 (owing to the presence of significant cellular atypia and pleomorphic lipoblasts), with <50% tumor necrosis observed. Immunohistochemical staining was positive for S-100 and CD34 in the tumor cells, supporting the adipocytic lineage; MDM2 amplification testing was not available at our institution, but the overall morphology was consistent with a well-differentiated (lipoma-like) liposarcoma with focal dedifferentiated/pleomorphic areas. Surgical margins were negative for tumor.

Fig. 3.

Surgical piece of approximately 30 cm in its largest diameter.

The postoperative course was uneventful. At the 1-year follow-up mark, the patient was alive and well, with no evidence of local recurrence or new masses on clinical exam and imaging.

3. Discussion

Paratesticular liposarcoma is an infrequent tumor of the genitourinary tract. The typical age at presentation is in the sixth to seventh decade of life, although cases have been reported in patients as old as the late 80s⁵ – our patient being an example at 86 years. Given the rarity of paratesticular sarcomas, population-based data are limited; however, in one series of testicular/paratesticular sarcomas (n = 230), the median age was around 58, and a substantial proportion of patients were over 65 years old at diagnosis.⁵ Clinically, paratesticular liposarcomas usually present as a slowly enlarging, painless scrotal or inguinal mass. They can reach massive sizes (over 50 cm in diameter in extreme cases) due to their indolent growth¹ Patients may experience a sensation of heaviness or notice progressive scrotal enlargement. Because these tumors are so rare and often asymptomatic aside from the bulk, they are often initially mistaken for more common benign conditions. Indeed, paratesticular liposarcoma can be confused with inguinal hernia, hydrocele, cord lipoma, epididymal cyst, or even a primary testicular neoplasm on initial evaluation.⁴ This underscores the importance of careful imaging and consideration of uncommon diagnoses when evaluating any atypical groin or scrotal mass. In our patient's case, the extreme size and slow growth over years exemplify how these tumors can remain undetected or misdiagnosed until they become very large.

Histologically, our patient's tumor was a well-differentiated (lipoma-like) liposarcoma, which is the most common subtype of paratesticular liposarcoma.⁴ Well-differentiated tumors tend to have the best prognosis among liposarcoma subtypes, with a low metastatic potential; however, they are locally aggressive and can recur if not completely excised.⁵ In contrast, the dedifferentiated and pleomorphic subtypes (and the round cell variant, which is now considered part of the high-grade myxoid spectrum) are associated with a much higher risk of recurrence and distant metastasis.⁷ It is noteworthy that prolonged, untreated growth of a well-differentiated liposarcoma can lead to dedifferentiation – the emergence of a higher grade non-lipogenic sarcomatous component within the tumor.⁴ This phenomenon has serious implications: a de-differentiated liposarcoma (DDLPS) behaves more aggressively and can become unre-sectable or life-threatening. In one reported case, a paratesticular WDLPS that was initially misdiagnosed and observed transformed over time into a high-grade dedifferentiated tumor that metastasized, ultimately leading to the patient's death.⁴ Such reports highlight the importance of early diagnosis and complete surgical management of even “well-differentiated” liposarcomas to prevent malignant progression.

Currently, there is no standardized treatment protocol for paratesticular sarcomas due to their rarity, but the consensus is that surgery is the definitive primary treatment. A high inguinal radical orchiectomy with wide excision of the surrounding affected soft tissue and a high ligation of the spermatic cord is considered the gold-standard approach.² This method removes the tumor in continuity with the testis and cord structures, aiming to achieve clear margins. In practice, organ sparing resection (simple tumorectomy) is generally avoided because it may lead to inadequate margins and higher local recurrence rates.² A recent systematic review of 265 reported cases of paratesticular liposarcoma supports this approach: patients who underwent radical orchiectomy had significantly better recurrence-free survival compared to those who had a partial scrotal excision of the tumor.⁵ In that analysis, the 3-year recurrence-free survival rate was about 80% for those treated with orchiectomy and wide excision.⁵ Additionally, tumor size and histological subtype emerged as important prognostic factors – tumors larger than 10 cm, as well as tumors with dedifferentiated or round cell features, were associated with a higher risk of recurrence and worse outcomes.¹⁰ These findings reinforce that aggressive initial surgical management is warranted, especially for large or higher-grade tumors.

Even with optimal surgery, paratesticular liposarcomas are prone to local recurrence. Reported overall recurrence rates range from about 25% to over 50%, depending on tumor grade and margin status.⁵ In one series, even after orchiectomy and seemingly adequate resection, roughly 25–37% of patients experienced local recurrence.⁵ Long-term surveillance is therefore critical – recurrence has been documented many years after the initial resection.² There are reports citing 3-year recurrence-free survival of ∼76% and 5-year overall survival around 75% in patients with paratesticular liposarcoma.⁵ Fortunately, distant metastasis is relatively uncommon in well-differentiated and even de-differentiated liposarcomas, with the lungs being the most typical site if it occurs. Unlike testicular germ cell tumors, routine retroperitoneal lymph node dissection is not recommended in these cases, as paratesticular sarcomas usually spread by local extension or via the bloodstream rather than through lymphatic channels. Any clinically suspicious lymph nodes can be evaluated with imaging and biopsied if needed, but prophylactic lymphadenectomy offers little benefit in the absence of proven metastasis.

The role of adjuvant therapy in paratesticular liposarcoma is not well defined. Radiation therapy may be considered on a case-by-case basis – for instance, in situations of positive surgical margins or in certain high-grade tumors – with the goal of improving local control. Some retrospective analyses and case reports have suggested that adjuvant radiotherapy can reduce local recurrence rates.⁵ However, robust evidence is lacking, and radiotherapy must be balanced against the risk of morbidity in the scrotal/inguinal region (such as wound complications or radiation-induced damage to surrounding structures). In our patient, negative margins were achieved, and the tumor was mostly low-grade, so adjuvant radiation was not pursued. Chemotherapy has generally shown limited efficacy in liposarcoma, particularly for well-differentiated and myxoid types, and thus is not routinely used in localized disease. The use of systemic chemotherapy is typically reserved for unresectable or metastatic cases, or for certain high-grade histologies like pleomorphic liposarcoma. Overall, both adjuvant radiotherapy and chemotherapy remain of uncertain benefit in this disease and their use is controversial.^11,12 Management guidelines largely emphasize surgery and vigilant follow-up.^13,14

Our patient's case illustrates several key points. First, even an extremely large liposarcoma can be successfully resected with careful surgical technique, yielding good outcomes – in this case, the patient has no recurrence at one year and has preserved endocrine/testicular function on the contralateral side. Second, it underlines the importance of considering rare tumors in the differential diagnosis of longstanding scrotal masses. Had this tumor been recognized and addressed earlier, the growth (and areas of dedifferentiation) might have been more limited; nonetheless, prompt surgical intervention upon diagnosis prevented further progression. Long-term follow-up is planned for this patient, given the known risk of late recurrence.

4. Conclusions

This case report enhances our understanding of the clinical behavior of paratesticular liposarcoma and emphasizes the need to include this rare entity in the differential diagnosis of any atypical scrotal or inguinal mass. Early diagnosis is important, as timely surgical management can prevent the tumor from achieving massive size or higher-grade transformation. The cornerstone of initial treatment is surgical – specifically, radical orchiectomy with high cord ligation and wide excision of surrounding fatty tissue – as this approach offers the best chance for cure and the lowest risk of local recurrence. Adjuvant therapies may be considered in select cases, but their benefits are not clearly established. Close, long-term surveillance of the patient is mandatory, given the tumor's propensity for late recurrences.

CRediT authorship contribution statement

José Antonio Arias-Delgado: Writing – review & editing, Writing – original draft, Supervision, Resources, Investigation, Formal analysis, Data curation, Conceptualization. Mario Eduardo Garrido-Toribio: Writing – review & editing, Writing – original draft, Methodology, Investigation, Conceptualization. Jesús Barahona-Mendoza: Writing – review & editing, Writing – original draft, Methodology, Investigation, Conceptualization. José Luis Barrantes-Ticlla: Writing – review & editing, Writing – original draft, Methodology, Investigation, Conceptualization. Christhian Manuel Cabeza-Luján: Writing – review & editing, Writing – original draft, Methodology, Investigation, Conceptualization. Fernando Imán-Izquierdo: Writing – review & editing, Writing – original draft, Investigation, Conceptualization. Rodrigo Alonso Fribourg-Liendo: Writing – review & editing, Writing – original draft, Methodology, Investigation, Conceptualization. Johana Martins-Luna: Writing – review & editing, Writing – original draft, Resources, Investigation, Conceptualization. Wilmer Silva-Caso: Writing – review & editing, Writing – original draft, Resources, Project administration, Investigation, Formal analysis, Conceptualization.

Informed consent statement

Informed consent was obtained from subject involved in the case report.” Written informed consent has been obtained from the patient(s) to publish this case report.

Institutional review board statement

Not applicable.

Data availability statement

All available data have been published in this case report.

Funding

This research received no external funding.

Conflicts of interest

The authors declare no conflicts of interest.