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Genetic and Rare Diseases Information Center (GARD)

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Your Question

How many cases of polyembryoma have been reported worldwide?  What is the prognosis for polyembryoma?  How might this condition be treated?  What causes polyembryoma?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is polyembryoma?

Polyembryoma is a type of tumor that develops from the cells of the gonads (testes in men or ovaries in women).  Such tumors are called germ cell tumors.  Polyembryomas have a distinctive look because they are composed of many parts that are shaped like embryos, one of the earliest stages of a developing human during pregnancy.[1]  Symptoms of a polyembryoma may include an unusual bump or mass in the abdomen which can cause pain in some individuals; puberty at an unusually young age (known as precocious puberty); or irregularities in a female's menstruation.  Treatment begins with surgery and may be followed by chemotherapy and/or radiation therapy.[2][3]  The cause of polyembryoma is not yet known.[3]
Last updated: 3/20/2012

How might polyembryoma be treated?

Because polyembryomas are quite rare, there are no established guidelines for treating this condition.  However, the first step for treating a polyembryoma is often surgery to remove as much of the tumor as possible.  Chemotherapy, and sometimes radiation therapy, have also been used after surgery to destroy any cancer cells that may remain.[2][3]
Last updated: 3/20/2012

What is the prognosis for polyembryoma?

The prognosis for individuals diagnosed with a polyembryoma is difficult to determine because of the rarity of this disease.  One study of eleven women with a polyembryoma found that half died within the first year following diagnosis.  The authors of this study suggest that prognosis might vary depending on the precise make-up of each tumor.[3] 
Last updated: 3/20/2012

How many cases of polyembryoma have been reported worldwide?

Polyembryomas are extremely rare.[2]  The exact number of cases diagnosed worldwide is unknown.  One article published in 1994 stated that only 11 cases of polyembryoma of the ovaries had been reported in the medical literature.[3]
Last updated: 3/20/2012

  • Ulbright TM. Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues. Modern Pathology. 2005; 18 Suppl 2:S61-S79. Accessed 3/19/2012.
  • Beresford L, Fernandez CV, Cummings E, Sanderson S, Ming-Yu W, Giacomantonio M. Mediastinal polyembryoma associated with Klinefelter syndrome. Journal of Pediatric Hematology/Oncology. 2003; 25:321-323. Accessed 3/19/2012.
  • Chapman DC, Grover R, Schwartz PE. Conservative management of an ovarian polyembryoma. Obstetrics and Gynecology. 1994; 83:879-882. Accessed 3/19/2012.