Leydig cell tumor
Leydig cell tumor
Description, Causes and Risk Factors:
Leydig cell tumor is a rare form of testicular neoplasm. It represents only 1 to 3 percent of all testicular tumors although it is the most common form of the sex cord-mesenchyme tumors. The majority have been recognized in males between the ages of 20 and 60 years. However approximately one fourth have been reported before puberty.
The etiology of Leydig cell tumors is unknown. In contrast to germ cell tumors, there is no correlation with cryptorchidism. Causing experimental production of Leydig cell tumor in mice following chronic estrogen administration or intrasplenic testicular autografting shows its hormonal basis. In addition, estrogen and progesterone receptors were detected in about 70 per cent of the Leydig tumor cells in an immunohistologic study, though no receptor was observed in normal Leydig cells.
Leydig tumors may be hormonally active, secreting a variety of steroid hormones including primarily testosterone, together, or to a lesser degree with estrogen and its derivates. In adult patients with Leydig cell tumor of the testis, endocrinologic signs occur in 20% of cases and often precede the onset of a palpable testicular mass. Virilizing types of congenital adrenocortical hyperplasia may also produce the endocrine signs and symptoms of Leydig cell tumors. The adrenal cortex and testicle are of common mesodermal origin and the histologic pattern may overlap.
The incidence of bilateral Leydig cell tumor was reported 3-10% in some reports. 10 to 20% of Leydig cell tumors are malignant. Malignancy is likely if the tumor is greater than 5 cm in size. Histological features suggesting malignancy are: numerous and atypical mitoses, invasion of vascular channels, extension of the tumor into the spermatic cord, invasion of the capsule or the presence of marked cellular pleomorphism. However, the only true indicator of malignancy is the presence of metastases.
Generally metastatic spread occurs within two years of the primary Leydig cell tumor, and the patient dies within two years of the discovery of metastatic disease. The tumor is highly resistant to both radiation and chemotherapy. Therefore, some authors suggested to perform retroperitoneal node dissection before or after chemotherapy for staging and also for therapeutic reasons.
There may be no symptoms.When symptoms do occur, they may include:
Discomfort or pain in the testicle.
Excess development of breast tissue (gynecomastia) -- however, this can occur normally in adolescent boys who do not have testicular cancer.
Heaviness in the scrotum.
Lump or swelling in either testicle.
Pain in the lower abdomen or back.
Enlargement of a testicle or change in the way it feels.
Symptoms in other parts of the body, such as the lungs, abdomen, pelvis, back, or brain may also occur if the cancer has spread.
Macroscopically, Leydig cell tumors present as well-circumscribed, yellow-to-brown masses within the testicle.These tumors are composed of large, closely packed cells with eosinophilic cytoplasm, bland nuclei, and small nucleoli. Reinke crystals are pale-staining, cylindrical, rectangular, or rhomboid inclusions that are pathognomonic for Leydig cell tumors and are found in up to 30% of patients with such tumors. Microscopic features such as necrosis, marked pleomorphism, lymphovascular invasion, increased mitotic activity, and DNA aneuploidy are more consistent with a malignant variant.
Laboratory studies in Leydig cell tumors are usually nonspecific.
The steroid secretion of Leydig cell tumors varies. Serum testosterone levels are usually elevated; however, serum estradiol levels may also be increased, especially when feminization is evident.
Urine and serum endocrinological tests such as urine ketosteroids, plasma cortisol, or the dexamethasone suppression test may help differentiate Leydig cell tumors from other adrenocortical disorders.
Leydig cell tumor endocrine function is independent of the hypothalamus-pituitary-gonadal hormonal axis and should not demonstrate a response to adrenocorticotropic hormone stimulation or dexamethasone suppression.
Levels of testicular tumor markers such as serum alpha-fetoprotein (AFP), beta human chorionic gonadotropin (beta-HCG), and lactate dehydrogenase (LDH) should be within the reference range in pure Leydig cell tumors.
MRI can reveal small nonpalpable Leydig cell tumors not otherwise visible on sonograms.
CT scanning of the abdomen and chest radiography are indicated if malignancy is suspected.
Scrotal ultrasonography is typically performed to confirm the diagnosis, especially in patients in whom the physical examination findings are equivocal.
Immunohistochemical markers such as alpha-inhibin, calretinin, and melan-A have also been shown to be valuable in the identification of Leydig cell and other sex cord-stromal testicular tumors.
Treatment of a Leydig cell tumor depends on its stage.
Stage II: Cancer has spread to lymph nodes in the abdomen.
Stage III: Cancer has spread beyond the lymph nodes (it could have spread as far as the liver, lungs, or brain).
Stage I: Cancer has not spread beyond the testicle.
The usual treatment is surgery. The surgery usually is a fertility-sparing unilateral salpingo-oophorectomy. For malignant tumours, the surgery may be radical and usually is followed by adjuvant chemotherapy, sometimes by radiation therapy. In all cases, initial treatment is followed by surveillance. Because in many cases Leydig cell tumor does not produce elevated tumor markers, the focus of surveillance is on repeated physical examination and imaging.
The prognosis is generally good as the tumor tends to grow slowly and usually is benign: 10% are malignant. For malignant tumours with undifferentiated histology, prognosis is poor.
Chemotherapy uses drugs such as cisplatin, bleomycin, and etoposide to kill cancer cells. Because Leydig cell tumors are rare, these treatments have not been studied as well as they have for other, more common testicular cancers.
NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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