A Giant Paratesticular Liposarcoma: Case Report and Literature Review

Sarcomas are a heterogeneous group of malignant neoplasms arising from mesenchymal cells. Genitourinary (GU) sarcomas account for <5% of all soft-tissue sarcomas and <2% of malignant urologic tumours.

Initially possibly misdiagnosed as inguinal, scrotal hernia, or hydrocele [1, 2], they may arise from the spermatic cord, inguinal canal, testicular tunic, or epididymis. The spermatic cord is the most involved urologic site and is thought to comprise 30–90% of all GU sarcomas.

At the moment, in literature are reported less than 350 cases. Primary treatment is therefore only surgical [3]. Here, we present a case report of a giant paratesticular liposarcoma of the spermatic cordon and a comprehensive literature review with the objective of providing useful information on this malignancy.

A 64-year-old man, without a medical history, came to our attention for enlarging left scrotum since about 18 months. Physical examination showed a giant, firm inguinoscrotal mass that reached down to the knees (Fig. 1a1, a2). Serum levels of tumour markers (HCG, α-FP, LDH) were normal.

A thoraco-abdomino-pelvic computed tomography scan confirmed a voluminous 30 cm scrotal lesion, within which a 20 cm solid component of polycystic and lipid-dense content was recognized, associated with macrocalcifications inside, suggestive of a liposarcoma (Fig. 1b). In addition, two inguinal adenopathies were identified. No pelvic or retroperitoneal lymphadenomegaly or distant lesions suspicious for repetitive lesions were identified.

The patient underwent left-sided orchiectomy with hemiscrotectomy (Fig. 1c) towards an incision of the inguinal region until with the deep inguinal orifice. It was needed to incise the fascia of the external oblique muscle because of diffuse infiltration of the cord.

A circumferential skin incision is made to remove the mass with the scrotal skin, keeping a scrotal flap for a scrotal plasty at the end of the operation. It was necessary to make a scrotal plasty for the repositioning of the penis in its physiological position with a shark’s mouth plasty.

The total operating time was of 1 h and 40 min and the blood loss of about 200 mL. Pathological analysis revealed a specimen of about 8 kg of weight and measured approximately 21 × 30 × 20 cm.

The tumour section found out a poly-lobed mass with areas of varying degrees of adipose tissue, whitish or fleshy, with necrotic areas representing approximately 30% of the tumour volume. At its tip (near the surgical margin), the spermatic cord contains small whitish nodules.

A fragment of the tumour was frozen confirming 95% tumour cells. On histological examination, the tumour corresponded to a sarcoma, the architectural, and morphological aspects of which were very variable from one territory to another. Indeed, the architecture was sometimes and mostly fasciculated, with spindle cells with moderate atypia and little mitotic.

Elsewhere, the architecture was diffuse and the tumour cells were epithelioid or rhabdoid or pleomorphic. In these areas, they are characterized by abundant eosinophilic or clarified foamy cytoplasm and a large, rounded, nucleated, often peripheral nucleus.

In minority areas, a well-differentiated, lipoma-like liposarcoma contingent is seen. On immunohistochemical examination, the tumour cells in all these areas expressed MDM2 and HMGA2 with partial positivity for CD34. No significant positivity of PS100, CKAE1/AE3, smooth muscle actin, myogenin, desmin, CD45, CD30, caldesmone were found. Desmin marked rare cells.

The mitotic index approached to about 20 mitoses per 10 fields at high magnification in the most densely cellular tumour areas. The testis and rete testis were embedded in the tumour without any significant intrinsic histological abnormality. The CARE Checklist has been completed by the authors for this case report, attached as online supplementary material (for all online suppl. material, see www.karger.com/doi/10.1159/000528221).

The LSC is rare tumour which usually affects adults between the ages of 50 and 70 [3]. The diagnosis is usually very difficult and is mostly made on histopathology.

Most of these tumours manifest as painless, slow-growing scrotal, or inguinal masses. It is easily confused with an inguinal hernia or a hydrocele, and it is possible that the diagnosis, as evidenced by the literature, may be delayed also for years [4, 5].

However, they rarely reach the size of the patient reported here. Imaging, both with ultrasound, CT and MRI, can provide useful information regarding location, size, and consistency of the mass.

At ultrasound, some shapes are displayed as homogeneous and isoechoic, others as inhomogeneous. Furthermore, the echo-density is quite variable; therefore, it cannot provide a differential diagnosis between benign and malignant neoplasms.

Liposarcomas, on CT, are usually well delimited and low density. However, there is no pathognomonic feature to differentiate benign from malignant lesions. MRI can also be useful; however, it also does not allow a preoperative differential diagnosis [6, 7].

Thus, this is a rare condition; the indications for the treatment come only from case reports or small series [3]. The standard therapeutic management has been radical inguinal orchiectomy with wide local resection of surrounding soft tissues [3, 8].

Very poor data on lymphatic spread are available and invasion of the retroperitoneal lymph nodes, even in large masses, and remains rare. Therefore, the indications for lymphadenectomy are very limited [9, 10].

According to the World Health Organization’s International Classification of Disease, the commonest histologic subtypes of sarcoma are liposarcoma (20–32%), leiomyosarcoma (19–32%), and rhabdomyosarcoma (11–24%) [11, 12]. Low-grade tumours are well-differentiated and appear to do not have metastatic potential; on the contrary, they tend to recur locally. The literature reports a local recurrence rate after surgery alone of up to 30%.

On the other hand, highly elevated diseases (round and pleomorphic cells) are rarer but have a marked metastatic capacity (lungs and bones) and are associated with a higher relapse rate [13]. The main risk factor for early local recurrence and distant metastasis it seems to be a positive surgical margin. Wide excision has demonstrated microscopic residual disease in 27% of apparently complete excision [14]. In case of positive margins, a second resection, even wide, is recommended [13, 14].

Instead, the role of adjuvant therapies (radiotherapy and chemotherapy) remains controversial [10]. Regarding radiotherapy, liposarcomas are the most radiosensitive of all sarcomas, and in some cases, remission has been achieved with radiotherapy alone [15].

Two randomized trials showed a reduction in local recurrence and DFS but no increase in OS was shown [15]. Therefore, radiotherapy can be recommended when, at the definitive histological examination, an aggressive high-grade tumour, or lymphovascular invasion or positive margins are found, or it can be useful for control in case of local recurrences. At the moment, however, there are no data on the role of neo- or adjuvant chemotherapy in the treatment of LSC.

In conclusion LSCs, and in general the tumours of paratesticular tissues, remain rare entities and with a rather complex differential diagnosis, especially in the initial stages. Due to their rarity, it is difficult to find consensus on the best pre-, intra-, and postoperative management.

The data in the literature derive only from low-level evidences. In any case, it is always difficult to obtain high statistical standards in such infrequent diseases. Radical orchiectomy with wide resection in order to obtain microscopic negative margins is the most recommended treatment. As a matter of fact, the role of adjuvant therapies still remains controversial. Long-term follow-up is absolutely necessary because local recurrence is common even after several years.

Ethical approval is not required for this study in accordance with local or national guidelines. This retrospective review of patient data did not require ethical approval in accordance with local/national guidelines. Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

The authors have no conflicts of interest to declare.

The authors declare that they have received no funding for the article in question.

Dr Caviglia and Dr Beverini contributed to writing this paper. Dr Pacchetti contributed to the writing and literature search Dr Branger and Dr Pignot contributed to the writing and research of the patient’s clinical and follow-up data Prof Bertucci and Prof Walz contributed to the compilation and revision of this article, as well as to the literature search necessary for the drafting.

If required clinical case data are available, after approval of the patient for data sharing to third parties. All data that support the findings of this study are included in this article. Further enquiries can be directed to the corresponding author.