//A CASE OF LEIOMYOMATOSIS PERITONEALIS DISSEMINATA

A CASE OF LEIOMYOMATOSIS PERITONEALIS DISSEMINATA

Alfonso Rossetti*,Ornella Sizzi*, Giuseppe Florio*,Rosario F. Grasso#

*Gynecological Endoscopic Division Nuova Villa Claudia, Rome
#Radiological Department Campus Biomedico, Rome 

The paper reports the case of a 30 years old patient, nulliparous, without any previous surgeries, who underwent a rapid abdominal swelling. The gynaecological examination showed an abdominal mass occupying the left quadrant from pouch of Douglas to the spleen.
Cancer markers were negative.
Sonographic findings: nonspecific solid and complex mass with intralesional vegetations. Areas with high vascularity with low Resistance Index were present.

ecografia

MR findings:

On unenhanced T-1 weighted imagines: masses similar in signal intensity to skeletal muscle or uterine parenchyma. A variable degrees of enhancement following the administration of gadolinium was visualized. On T-2 weighted imagines the masses remained low in signal because of their smooth muscle components.

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A Diagnostic Laparoscopy was performed:
The optic was introduced on the umbilicus using a visual cannula: Ternanian’s ENDOTIP without previous pneumoperitoneum. Laparoscopy showed a mass (with a smooth surface without vegetations) arising from the uterus developed into the left abdominal cavity. On the uterine surface and on the anterior abdominal wall, irregular vegetations with an aspect of vesicles were present. Peritoneal washing and multiple biopsies were performed.

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Histological examination failed to make the correct diagnosis although orienting toward a possible LEIOMYOMATOSIS PERITONEALIS DISSEMINATA with high invasive potentially, while lacking necrosis and atypias. A laparotomy was then performed, the pelvic mass turned out to be the left tube completely invaded by hundreds of irregular vesicles. The irregular vegetations on the uterine surface continued with a fibromatous nodule of the uterine fundus (no cleavage plane was present and with an invasion of the left broad ligament. Left salpingectomy, nodulectomy and removal of peritoneal implants was performed.

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Hystological findings:
LPD with high invasive potentiality. Negative immunoistochemical staining for p53 and estrogen and progesteron receptors was found.

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Post-operative period was uneventful. The patient conceived after sixteen months and delivered a healthy baby with a C-section at 34 weeks for premature rupture of membrains.

Leyomiomatosis Peritonealis Disseminata is a rare disorder (100 cases reported in the literature).

It is characterized by multiple subperitoneal muscle nodules. It is prevalent in women of reproductive age, particularly in the fourth decade. Often is associated with pregnancy or with the use of oral contraceptives.

Has been diagnosed also in postmenopausal women and in a man.

LPD has a correlation with Hormonal influence and with a hyperestrogenic state because of its association with pregnancy, prolonged oral contraceptive use,subserosal uterine leiomyomata, functional granulosa cells tumors.

LPD is related to a smooth muscle metaplasia of the subcoelomic mesenchymal stem cells,or to a fibrosing reaction to ectopic omental deciduosis (induced by steroid hormonal imbalance or excess) or to Genetics.

Malignant trasformation has an incidence is of about 10% and it represents the 30% of cases occurred in postmenopausal women.

The interval between the LPD diagnosis and the development of sarcoma varies from synchronous diagnosis to up to 8 years.

An increase of Ca 125 levels could be noted.

Cases of LPD without exogenous or increased endogenous estrogen exposure, without uterine leyomyomas, without ER and PR expression in the nodules may represent a different entity and should be regarded as cases with a high malignant potential.

The risk of malignant degeneration justifies a close follow up based on radiologic and ultrasonographic evaluations and periodic laparoscopic evaluations to identify any eventual sarcomatous degeneration of disease. Leyomiomatosis Peritonealis Disseminata can be asymptomatic or symptomatic.

The symptoms can be: irregular uterine bleeding, pain in the lower abdomen, weight in the lower abdomen, discomfort, urinary frequency due to mass effect on the bladder, gastrointestinal bleeding following erosion of the LPD implant into the bowel wall. When an hormonal stimulus is identified, several authors advise a conservative approach without extensive surgery. Despite this fact, many patients with LPD had been treated with surgical debulking combined with total abdominal hysterectomy, with bilateral salpingo-oophorectomy.

References:

  • Al-Talib A, Al-Farsi AR, Stanimir G. Leiomyomatosis Peritonealis Disseminata with Features of Carcinomatosis on Laparoscopy: A case report. Sultan Qaboos Univ Med J. 2009;9:315-8. PMID:Abstract
  • Al-Talib A, Tulandi T. Pathophysiology and possible iatrogenic cause of leiomyomatosis peritonealis disseminata. Gynecol Obstet Invest. 2010;69:239-44. Review. PMID:Abstract
  • Marwah N, Duhan A, Aggarwal G, Sen R. An unusual presentation of pelvic leiomyomatosis misdiagnosed as disseminated malignancy. Case Rep Pathol. 2012;2012:394106. PMID: 23198231
  • Meneni RD, Farrow AJ, Fawzi H. Leiomyomatosis peritonealis disseminata: an unusual presentation. J Obstet Gynaecol. 2010;30:210-1. PMID:Abstract
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2017-12-12T11:36:15+00:00