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Disseminated peritoneal leiomyomatosis

Orpha number ORPHA71274
Synonym(s) DPL
Diffuse peritoneal leiomyomatosis
LPD
Leiomyomatosis peritonealis disseminate
Prevalence <1 / 1 000 000
Inheritance Unknown
Age of onset Adult
ICD-10
  • D20.1
ICD-O -
OMIM -
UMLS -
MeSH -
MedDRA -
SNOMED CT
  • 62557001

Summary

Disseminated peritoneal leiomyomatosis (DPL) is characterized by the proliferation of multiple benign smooth muscle cell-containing nodules in the peritoneal cavity. Fewer than 150 cases have been reported in the literature to date. DPL manifests during adulthood and is predominantly found in women. Most patients are asymptomatic but clinical features such as abdominal and pelvic pain, rectal or vaginal bleeding and, more rarely, gastrointestinal disorders have been reported. Malignant transformation is rare and in a few cases, metastases have been found in the liver and lungs. Etiology is unknown but DPL seems to be a multifactorial disease with a genetic or hormonal component (high levels of estrogen and progesterone) leading to metaplasia of peritoneal mesenchymal cells. In some women, DPL seems to originate from proliferation of fragments of uterine leiomyoma in the abdominal cavity after laparoscopic surgery. Imaging techniques such as ultrasound examination are required for detection of DPL. Diagnosis is confirmed by biopsy of the nodules which reveals the presence of smooth-muscle cells with no atypia or necrosis, fibroblasts and myofibroblasts. Differential diagnosis includes parasitic leiomyoma, intravenous leiomyomatosis and other primitive or secondary peritoneal carcinomatoses. Depending on the extent of the disease, first-line treatment for DPL is surgical excision or cytoreductive surgery. Hormone intake (e.g. oral contraceptives) must be discontinued. Systemic chemotherapy (off-label use) has been suggested as a treatment option for the rare cases of unresectable or metastatic tumors. Prognosis is usually good. Death has however been observed in some cases presenting unresectable or metastatic tumors.

Expert reviewer(s)

  • Pr Olivier GLEHEN

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