peritoneal leiomyomatosis
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#