What are the other Names for this Condition? (Also known as/Synonyms)

• Diffuse Peritoneal Leiomyomatosis
• DPL (Disseminated Peritoneal Leiomyomatosis)
• Leiomyomatosis Peritonealis Disseminata (LPD)

What is Disseminated Peritoneal Leiomyomatosis? (Definition/Background Information)

• Disseminated Peritoneal Leiomyomatosis (DPL) is a rare condition which is characterized by nodules or small lumps of smooth muscle cells located on the peritoneum (lining of the abdominal wall) and abdominal organs. The condition is usually benign (non-cancerous), but in rare cases has become cancerous
• Although it can be seen in post-menopausal women and very rarely in men, DPL occurs most often in women of childbearing age. Most women with DPL are pregnant, taking the birth control pill, or have uterine leiomyomas or estrogen-secreting tumors
• Some people with DPL have no signs or symptoms of the condition. When present, symptoms may include abdominal and pelvic pain; rectal or vaginal bleeding; and less commonly constipation
• The cause of Disseminated Peritoneal Leiomyomatosis is unknown, but may be linked to hormonal and genetic factors. Some cases of DPL resolve when hormone levels are returned to normal. However, surgery may be suggested based on the size and location of the tumor

(Source: Disseminated Peritoneal Leiomyomatosis; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Disseminated Peritoneal Leiomyomatosis? (Age and Sex Distribution)

• Disseminated Peritoneal Leiomyomatosis is a rare disorder
• Although both genders may be affected, the condition is more common in women of child-bearing age 
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Disseminated Peritoneal Leiomyomatosis? (Predisposing Factors)

The risk factors for Disseminated Peritoneal Leiomyomatosis may include the following:

• Having an affected family member (family history of the condition)
• Female gender, especially women of child-bearing age
• Having a tumor that secretes estrogen, which may cause a hormonal imbalance
• Presence of uterine fibroids

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Disseminated Peritoneal Leiomyomatosis? (Etiology)

• The cause of Disseminated Peritoneal Leiomyomatosis (DPL) is unknown, but medical researchers believe it is influenced by both hormonal and genetic factors. The cause of the condition is considered multifactorial
• Not all cases are related to hormone levels, as some cases have occurred in men and in post-menopausal women not receiving hormone replacement therapy
• DPL is often associated with uterine leiomyomas but the connection is unclear
• Most cases occur sporadically in people with no family history of the condition; however, more than one family member can be affected
• Although this suggests that genetic factors may play a role in the development of DPL in some families, researchers have not identified any specific gene changes known to cause the condition

(Source: Disseminated Peritoneal Leiomyomatosis; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

What are the Signs and Symptoms of Disseminated Peritoneal Leiomyomatosis?

Disseminated Peritoneal Leiomyomatosis is often asymptomatic. If signs and symptoms are present, they may vary among affected individuals in type and severity, and include:

• Abdominal and pelvic pain which is often associated with abnormal menstrual bleeding (dysmenorrhea)
• Rectal bleeding
• Abnormally heavy bleeding during menstruation (menorrhagia)
• Constipation
• Intestinal obstruction

(Source: Disseminated Peritoneal Leiomyomatosis; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How is Disseminated Peritoneal Leiomyomatosis Diagnosed?

• Disseminated Peritoneal Leiomyomatosis may be diagnosed incidentally during a physical exam when masses may be felt in the abdomen
• Since DPL usually does not produce any symptoms, the condition may also be unexpectedly found during a cesarean section (C-section) or abdominal surgery of another reason
• An ultrasound may reveal the presence of nodules (lumps) which may indicate Disseminated Peritoneal Leiomyomatosis (DPL)
• However, DPL can only be confirmed by a biopsy of the nodule
  • The nodules should contain smooth muscle cells with no atypia (no abnormal structure) or necrosis (dead cells)
  • The cells usually have both progesterone and estrogen receptors, but this is not always the case
  • The cells usually have a low mitotic index (meaning they are not dividing at a high rate)

(Source: Disseminated Peritoneal Leiomyomatosis; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Disseminated Peritoneal Leiomyomatosis?

The complications of Disseminated Peritoneal Leiomyomatosis may include:

• Anemia, due to loss of blood
• The tumor becoming cancerous

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Disseminated Peritoneal Leiomyomatosis Treated?

• Presently there are no treatment guidelines for Disseminated Peritoneal Leiomyomatosis (DPL)
• DPL is considered a benign condition and some cases of DPL resolve after the baby is delivered (if pregnant), hormone treatment is stopped (including both birth control pill and hormone replacement therapy), or a hormone producing tumor is removed
• However, surgery may be suggested based on the size and location of the tumor

(Source: Disseminated Peritoneal Leiomyomatosis; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How can Disseminated Peritoneal Leiomyomatosis be Prevented?

• Disseminated Peritoneal Leiomyomatosis may not be preventable, since it is a disorder which may be multifactorial, and all the factors contributing to the development of the condition are not known
• Active research is currently being performed to explore the possibilities for treatment and prevention of conditions such as Disseminated Peritoneal Leiomyomatosis
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Disseminated Peritoneal Leiomyomatosis? (Outcomes/Resolutions)

• The prognosis of Disseminated Peritoneal Leiomyomatosis is good, since it is a benign condition that can be effectively treated
• However, in rare cases, the benign tumors can become cancerous, and the prognosis is then determined by additional factors, such as the stage of the cancerous tumor, size, and whether it is localized or metastasized

Additional and Relevant Useful Information for Disseminated Peritoneal Leiomyomatosis:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/