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peritoneal leiomyomatosis

Wednesday 1 June 2005

Definition: Leiomyomatosis peritonealis disseminata (LPD) is a rare disease presenting as multiple peritoneal nodules of smooth muscle cells, mimicking peritoneal carcinomatosis. This disease usually pursues a benign course.

Disseminated peritoneal leiomyomatosis (DPL, leiomyomatosis peritonealis disseminata) is a rare condition in which multiple histologically benign smooth muscle tumorlets diffusely stud peritoneal and omental surfaces in females, predominantly of reproductive age. Although the distribution of these lesions suggests a metastatic process, DPL generally has a benign clinical course.

Female gonadal steroids play the major role in pathogenesis, but reported cases in postmenopausal women suggests other factors. A few cases in the literature with coexisting endometriosis confirm the hypothesis that both originate in submesothelial multipotential mesenchymal cells. (#17583244#)

Synopsis

- smooth-muscle cell proliferation
- no mitosis
- no atypia
- no necrosis
- possible intravascular extension (#17583244#)
- possible familial clustering (#16223449#)
- possible malignant change (#15581996#, #12608900#)
- possible recurrence (#14597256#)
- in man (#12608900#)
- in postmenopausal woman (#11045333#)

Associations

- endometriosis (#17583244#, #11444203#)
- caudal regression syndrome (Currarino syndrome) (#16686944#)
- leiomyoma of the uterine body (#16341539#)
- oestrogen secreting ovarian fibrothecoma (#11426907#)

Immunohistochemical analysis

- diffuse and strong staining for progesterone receptors
- partial expression of estrogen receptors
- smooth muscle actin
- desmin, h-caldesmon
- calretinin
- WT1
- CD117 (KIT)
- 5% of cells being positive for MIB-1

Cytogenetics

- cytogenetic abnormalities involving chromosomes 7, 12, and 18 (#9176406#)

- t(12; 14)(q15;q24) (12q15 and 14q24) (#11904348#)

  • three copies of HMGIC (alias HMGA2), two on the normal chromosomes 12 at 12q15, as well as another on the der(14) in the breakpoint region, suggesting that the 12q breakpoint occurred 5’ (centromeric) to HMGIC (HMGA2) (#11904348#)

- monosomy 22 (-22) (#11904348#)

Molecular biology

- monoclonality assessed by HUMARA methylation study (#9176406#)

See also

- smooth muscle tumors

  • leiomyomas
  • leiomyomatosis

References

- Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC. Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case. Mod Pathol. 2002 Mar;15(3):351-6. PMID: #11904348#

- Quade BJ, McLachlin CM, Soto-Wright V, Zuckerman J, Mutter GL, Morton CC. Disseminated peritoneal leiomyomatosis. Clonality analysis by X chromosome inactivation and cytogenetics of a clinically benign smooth muscle proliferation. Am J Pathol. 1997 Jun;150(6):2153-66. PMID: #9176406#

- Mark J, Havel G, Dahlenfors R, Wedell B. Cytogenetics of multiple uterine leiomyomas, parametrial leiomyoma and disseminated peritoneal leiomyomatosis. Anticancer Res. 1991 Jan-Feb;11(1):33-9. PMID: #2018368#