Paratesticular liposarcoma

What is it?

Liposarcoma is a malignant tumor of fat tissue (adipose tissue). It belongs to sarcomas, more specifically soft tissue sarcomas, a large and heterogeneous group of rare tumors that form from muscles, connective tissues, vessels, ligaments, and, indeed, adipose tissue. Liposarcoma of the spermatic cord arises at the soft tissues of the spermatic cord, usually at the paratesticular site and, in some cases, may extend to the inguinal canal. Cancer can occur at any stage of an individual's life; however, it is very rare in children and adolescents and mostly affects people between the ages of 40 and 60.

Microscopic analysis of a tissue specimen (histologic examination) makes it possible to distinguish different types of liposarcoma, characterized by different degrees of malignancy. According to the World Health Organization (WHO) classification, the main forms of liposarcoma are: 

  • Well-Differentiated Liposarcoma (WDLS, from Well-Differentiated Liposarcoma/Atypical lipomatous tumor): this is the most common; it is a low-grade tumor that rarely gives rise to metastasis or recurs after treatment (recurrence) and is the most common form in this location;
  • De-Differentiated liposarcoma (DDLS, from De-Differentiated Liposarcoma) which usually evolves from well-differentiated liposarcoma, grows faster than the other and can form distant metastases. 

The other types of liposarcoma in the paratesticular site are of rare occurrence.

Causes and risk factors

Well-differentiated liposarcoma at its various sites of origin may associate with Li Fraumeni syndrome, but at the level of the funiculus and paratesticular areas almost all forms are sporadic, and the etiology of these forms, and associated risk factors, are unknown.

Which are the symptoms?

Liposarcoma can remain asymptomatic for a long time. Consequently, it often happens that it is discovered when it is already in an advanced stage. It appears as a solid, elastic mass, noticeable to the touch that causes a sense of turgor and heaviness of the scrotal sac. Liposarcomas pushing into the abdomen can cause:

  • abdominal bloating;
  • abdominal pain;
  • early feeling of fullness at meals;
  • constipation;
  • difficulty urinating. 

Because liposarcoma is a rare tumor, the diagnosis and choice of treatment pathway can be complex, so it is important for patients to go to centers with specific experience in this type of tumor early on.

How is it diagnosed?

The diagnosis of liposarcoma is based on the use of imaging (diagnostic imaging) techniques, mainly ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI). A tissue sampling (biopsy) allows for histological examination by which it is possible to define which subtype of liposarcoma is involved.

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How is it treated?

As with other types of sarcomas, the standard treatment of liposarcoma is surgery to remove the tumor. After surgery, patients may receive radiation therapy to reduce the risk of tumor recurrence. Tumors that tend to recur may undergo dedifferentiation and possibly have poor prognosis as a result of uncontrolled local growth or, more rarely, as a result of systemic spread following dedifferentiation. If the liposarcoma has spread throughout the body, forming metastases, chemotherapy, with anthracyclines, is used; while trabectedin, a drug originally derived from a marine mollusk (Ecteinascidia turbinata), is reserved for myxoid forms (myxoid liposarcoma) rarely found in paratesticular sites.

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