Malignant mesothelioma of the tunica vaginalis testis is a very rare type of mesothelioma affecting males. Mesothelioma is a disease of the layers lining the organs. In pleural mesothelioma, it is the pleural layer lining the lung, in peritoneal mesothelioma it is the perineal lining the abdominal cavity, cardiac mesothelioma it is the pericardial lining around the heart. The lining of the testicles is known as the tunica vaginalis. As the tumor develops the lining starts to thicken and produce fluid buildup. The reason this happens is not well understood. Less than 1% of all malignant mesothelioma cases are malignant mesothelioma of the testis.
What is Testicular Mesothelioma
Mesothelioma is a very rare cancer; testicular mesothelioma is the rarest of the four types of mesothelioma. It is a primary testicular tumor; the tumor is not found outside of the testis, and is specific to the testicles. It develops from the mesothelium covering the tunica albuginea, (connective tissue with smooth muscle cells and elastic fibers coated on the surface of the visceral layer that wraps around the testicle), as well as parietal and visceral sheets of the tunica vaginalis of the testis, epididymis, and spermatic cord, providing protection and support of this part of the body.
History of Testicular Mesothelioma
The first case was described in the literature in 1957. There are currently about 100 cases that have been reported to date in the literature. It was first reported by Barbara and Rubino in a published article. No racial or ethnic group has been identified as being more susceptible to this form of mesothelioma. There are reports from all over the world about this rare tumor.
Symptoms of Testicular Mesothelioma
The symptoms of malignant mesothelioma of testis are nonspecific, such as painless scrotal mass associated with hydrocele. Hydrocele is a type of swelling in the scrotum that occurs when fluid collects in the sheath surrounding a testicle. Malignant mesothelioma of the testicle is most often seen in male patients that are 55-75 years old. Although it is estimated that 10% of the patients that are affected are younger than 25. There have been reports of patients with testicular pain, low grade fever and profuse sweating, fatigue, fainting, dizziness, swelling in the lower legs, and weight loss.
There may also be palpable invasive masses or large tumors in the testicles. The tumor usually affects one testis, it can spread and involve both the testis and the scrotum.
Types and Stages of Testicular Mesothelioma
There are two types of testicular mesothelioma, benign mesothelioma- non-cancerous-and malignant-cancerous. This is based on the cell type from a biopsy.
Testicular mesothelioma is classified as epithelial tissue, cystic and mixed.
Treatment
The diagnosis of this very rare cancer is challenging, often it is done by excluding other diagnosis. Scrotal ultrasound is used in addition to CT scans, MRI’s and biopsies.
A multi-modality approach can be used for treatment depending on the patient’s functional status, stage of disease, and wishes of the patient. Surgery is used to help relieve the symptoms and aid in the diagnosis. A radical orchiectomy- removal of the testicles- may be recommended, or an embolization – cutting off the blood supply to the tumor, may provide relief for the patient. Chemotherapy has also had some success. One of the goals of therapy is to prevent the spread of the cancer to nearby organs. In this particular rare cancer, the cell growth rate factor, ki-67 value- based on protein found in cells is a good indicator of how fast the tumor is progressing.
The success of treatment is individualized. With prompt, multidisciplinary treatment the mean survival has been approximately 2 years, with some living many years longer.
Options
This rare cancer is best evaluated and treated at a major academic mesothelioma center, where a multidisciplinary team of experts can collaborate on the latest evidenced based treatment. Due to the rarity of the disease there is no established standardized treatment and treatment must be personalized to the patient. Factors that will influence treatment options are general health, type of the tumor, and stage of the disease.
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