excerpts from VHL Handbook
VHL lesions in the reproductive tract are classified as cystadenomas. In males, epididymal cystadenomas may occur in as many as 50% of men with VHL. Similarly, women with VHL may have cystadenomas of the broad ligament near the fallopian tube, the embryological counterpart to the epididymis. Both lesions are benign although they may sometimes cause pain.
The epididymis is a small coiled conduit that lies above and behind the testicle on the path to the vas deferens, the tube that carries the sperm from the testicle to the prostate gland. The epididymis is as long as the testicle, lying in a flattened C shape against one side of the testicle. It is a complex tubular system that gathers the sperm and stores them until needed. After having been stored in the epididymis, sperm then moves through the vas deferens to the prostate where they are mixed with seminal fluid from the seminal vesicles and move through the prostate into the urethra during ejaculation.
A small number of cysts are found in the epididymis of about 25% of men in the general population. By themselves, cysts are not an occasion for concern and are not even particularly noteworthy. However, one specific type of cyst is significant in VHL A cystadenoma is a benign tumor with one or more cysts inside it, having more density than a simple cyst. Papillary cystadenomas of the epididymis are a rare occurrence in the general population. In VHL, these cysts can occur on one or both testes. When they occur on both sides, they almost always mean a definite diagnosis of VHL. They range in size from 1 to 5 cm (0.3 to 1.7 inches). The man may feel a “pebble” in the scrotum. They are usually not painful and do not continue to enlarge.
Papillary cystadenomas of the epididymis may arise during the teenage years or later in life. It is not unusual for them to occur for the first time in men in their forties. The cysts can be removed if they are annoying. Removal is much the same operation as a vasectomy and may result in the disabling of the delivery of sperm from the operated side.
These cysts do not interfere with sexual function. In most cases, the only “problem” associated with cystadenomas is the minor annoyance of knowing it is there. Occasionally, depending on their position, cystadenomas may block the delivery of sperm and cause infertility. If a cystadenoma is painful, check with a doctor, since on rare occasions they can become inflamed and even rupture.
In some cases, they may cause atrophy of the vas deferens, which will also cause infertility. Men who wish to keep their childbearing options open may want to bank some sperm in their teens or twenties for possible later use.
The best way to keep track of epididymal cysts is to do a Testicular Self-Exam (TSE) monthly, as recommended for all men in the general population. VHL does not increase the risk of testicular cancer. A TSE helps you become familiar with the size and shape of any epididymal cystadenomas, and make sure there are no unusual bumps or lumps in the testicles.
1) Check yourself right after a hot shower. The skin of the scrotum is then relaxed and soft.
2) Become familiar with the normal size, shape, and weight of your testicles.
3) Using both hands, gently roll each testicle between your fingers.
4) Identify the epididymis. This is a rope-like structure on the top and back of each testicle. This structure is NOT an abnormal lump, but epididymal cystadenomas may occur in this structure. Note their size and shape; keep a record for comparison in the future.
5) Be on the alert for a tiny lump under the skin, in front or along the sides of either testicle. A lump may remind you of a piece of uncooked rice or a small cooked pea.
6) Report any swelling to your health care provider.
If there are lumps or swellings, it does not necessarily mean that you have testicular cancer, but you must be checked by your healthcare provider.
A corresponding tumor occurs in women, called an Adnexal Papillary Cystadenoma of Probable Mesonephric Origin (APMO). A cystadenoma is a benign tumor with one or more cysts inside it making it denser than a simple cyst. Papillary cystadenoma of the broad ligament are a rare occurrence in the general population.
The broad ligament is a folded sheet of tissue that drapes over the uterus, fallopian tubes and the ovaries. Cells in this area are from the same origin in the development of the embryo as the epididymis in males.
Cysts in this area are very common in the general population. However, if an “unusual” cyst or tumor is seen in the area of the broad ligament or fallopian tubes, a cystadenoma associated with VHL should be considered. Ask the doctor to do a careful differential diagnosis to prevent over-treatment of benign tumors, which are sometimes confused with ovarian cancer.
Many physicians recommend that a woman with VHL limit her contraceptive choices to those that are non-hormonal or very low in progesterone. The rationale is that hemangioblastomas associated with VHL may be sensitive to the progestin contained in birth control pills, patches, rings, implants, and long-acting injections. Some IUDs contain copper and others have a low dose progestin. The copper IUDs are a non-hormonal contraceptive. The progestin IUD has a low dose of progestin and may also be considered.
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