Peritoneal leiomyomatosis – A rare case report

Case Report

Peritoneal leiomyomatosis – A rare case report

Rochita Venkataramanan a, Pudhiavan Arunachalam a,*,Vinutha Arunachalam b, Panneer Venkat c

aDepartment of Radiology, Apollo Hospitals, Chennai, Tamil Nadu, IndiabDepartment of Obstetrics and Gynecology, Apollo Hospitals, Chennai, Tamil Nadu, IndiacDepartment of Surgery, Apollo Hospitals, Chennai, Tamil Nadu, India

1. Introduction

Leiomyomas represent the most common uterine neoplasms.However, leiomyomas occasionally occur in unusual locationsthat make their identification more challenging. Leiomyoma-tosis peritonealis disseminata (LPD) is a benign condition and avery rare disease. To our knowledge, there are less than 150

cases reported worldwide.1 It is suspected that this diseaseoriginates from a metaplasia of submesothelial multipotentmesenchymal cells.2,3 Although it is not clear if the stimulus tosmooth cell differentiation is hormonal, genetic, or both. Thedisease is generally associated with high levels of exogenousor endogenous female gonadal steroids.3 The importantpractical issue with LPD is the possibility of misdiagnosingit as a disseminated malignancy.

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a r t i c l e i n f o

Article history:

Received 6 April 2015

Accepted 13 May 2015

Available online 6 June 2015

Keywords:

Leiomyoma

Disseminated peritoneal

leiomyomatosis

Peritoneal carcinomatosis

a b s t r a c t

Leiomyomas are smooth muscle tumors that are common to the uterus. These lesions

include a range of presentations and extensions ranging fromwithin the uterus to anywhere

in the body, including parasitic leiomyoma, intravenous leiomyomatosis, disseminated

peritoneal leiomyomatosis, and benign metastasizing leiomyoma. However, these atypical

locations of these tumors present a diagnostic dilemma regarding their nature and benig-

nity. Leiomyomatosis peritonealis disseminata (LPD) is a rare benign disease of unknown

etiology of women in reproductive age group. A few reported cases of association with

endometriosis have been described, suggesting a possible origin from submesothelial

multipotent cells. Here, we present a case of a reproductive age group woman with history

of uterine fibroids, now presenting with vague abdominal symptoms and multiple benign

leiomyomas scattered throughout the peritoneal cavity. A diagnostic laparoscopy was done

and the lesions sampled,which confirmed the imaging diagnosis of disseminated peritoneal

leiomyomatosis. We stress the importance of picking up this rare benign pathology and

avoiding labeling them as malignant peritoneal disease.

# 2015 Indraprastha Medical Corporation Ltd. Published by Elsevier B.V. All rights

reserved.

* Corresponding author at: 404, Arihant Garuda Apartments, Police Manickam Street, Aynavaram, Chennai, Tamil Nadu, India.Tel.: +91 9884855174.

E-mail address: drpudhiavan@gmail.com (P. Arunachalam).

Available online at www.sciencedirect.com

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journal homepage: www.elsevier.com/locate/apme

http://dx.doi.org/10.1016/j.apme.2015.05.0130976-0016/# 2015 Indraprastha Medical Corporation Ltd. Published by Elsevier B.V. All rights reserved.

2. Case report

A40-year-old femalewith regularmenstrual history presentedwith vague abdominal discomfort and vulval itching. Thepatient underwent a myomectomy with morcellation twoyears prior. She was referred to us with pelvic ultrasound andMRI studies done elsewhere. Both studies revealed multipleperitoneal nodules and raised the suspicion of peritonealcarcinomatosis by ovarian or bowel primary.

A contrast enhanced 320 slice CT scan was done by us on aToshiba Aquilion One, Tokyo, Japan. The intravenous contrastused was Optiray, Mallinckrodt, USA. 0.5 mm thin sectionswere performed through the abdomen and pelvis with 120 kVand 350 mAs in the plain, arterial (bolus tracking technique),and venous (70 s delay after start of injection) phases. The CTscan showed innumerable well-circumscribed homogenouslyenhancing peritoneal nodules with smooth convex surfacestowards the peritoneal cavity throughout the abdomen andpelvis. These nodules ranged in size from 5 mm to 5 cm. Fewtiny nodules were also seen on the surface of the right ovary.Multiple small similar intramural nodules were also seen inthe uterus. Rest of the abdominal organs were unremarkable.

A diagnosis of LPD along with uterine fibroids was put forthrather than peritoneal carcinomatosis. A diagnostic laparos-copy of the patient was done which revealed innumerablesmall nodules in the peritoneum, as described in the CT scan.Samples were taken from the peritoneal and omental nodulesand sent for histopathological evaluation. The histopatholog-ical examination revealed typical interlacing bundles ofsmooth muscle cells arranged in a whorled pattern withoutany atypia,mitosis, or stromal invasion consistentwith the CTdiagnosis of LPD (Figs. 1 and 2).

3. Discussion

Discrete nodules of benign smoothmuscle scattered over theperitoneum characterize classic LPD. However, LPD maypresent a protean appearance, sometimes as tiny peritonealnodules mimicking peritoneal carcinomatosis and at othertimes as bulky masses resembling leiomyosarcoma.2 LPD isclassically seen in premenopausal patients and is frequentlyseen in pregnant women, as female gonadal steroids areconsidered to play an important role in the pathogenesisresulting in multifocal metaplasia of the peritoneum.

[(Fig._1)TD$FIG]

Fig. 1 – A 40-year-old female with LPD. Post-contrast CT scan volume rendered coronal (A) and axial (B) reconstructions in thevenous phase showing innumerable well-circumscribed homogenously enhancing peritoneal nodules (arrows). Few tinynodules were also seen on the surface of right ovary (arrowheads).

[(Fig._2)TD$FIG]

Fig. 2 – A 40-year-old female with LPD. (A) The diagnostic laparoscopy of the patient revealed innumerable small nodules inthe peritoneum. (B) The histopathological examination revealed typical interlacing bundles of smooth muscle cells arrangedin a whorled pattern.

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However, some authors promote a disseminated origin froma single lesion.3,4 Our patient had a history of myomectomywith morcellation done elsewhere two years back. Thepresence of peritoneal nodules was not registered at thatsurgery. This suggests the possibility of dissemination.Similar findings were also observed by Seidman et al.4

The occurrence of this tumor in postmenopausal womenand rarely in males too suggests the possibility of a secondcoexistent pathogenesis involving metaplasia of submesothe-lial multipotent mesenchymal cells.3

Imaging plays an important role in differentiating thisprimarily benign condition with other sinister pathologies ofthe peritoneum. On ultrasound examination, these are seen aswell-defined homogeneously hypoechoic nodules with asmooth surface. On MRI, these appear as hypointense inT1WI, and similarly hypo, iso, or hyperintense on T2WIdepending on the water content of the tumor.4 On post-contrast images, they reveal a homogenous enhancement.MDCT shows these lesions as hypodense, well-circumscribedsolid nodules with homogenous enhancement. The adjacentperitoneum is usually normal without any thickening orabnormal enhancement.

The chief imaging differential diagnoses for LPD areperitoneal carcinomatosis, disseminated leiomyosarcoma,and endometriosis.1,3 The most common feature of peritonealcarcinomatosis is ascites.5 The other findings of peritonealcarcinomatosis include peritoneal thickening with increasedenhancement, peritoneal nodules, omental nodules andcaking, thickening of bowel wall, and adenopathy. Dissemi-nated leiomyosarcoma presents as peritoneal nodules.Though smaller lesions can reveal homogenous enhance-ment, larger lesions more than 3 cm in size show varyingdegree of necrosis. While LPD is isometabolic in PET-CT,leiomyosarcomas are hypermetabolic. Endometriosis presentsas hyperdense cystic lesions with septations and soft tissueenhancing mass with irregular margins. MRI is more specific,as it shows hyperintense lesions in T1W fat sat images andhyperintense lesion with shading in T2W images.6

It has been suggested that LPD should be considered asdiagnosis during surgery, when the patient has coexistingleiomyoma or diffuse leiomyomatosis of uterus with noomental thickening or ascites.1,2 They have a tendency toregress after menopause. The diagnosis is established with adiagnostic laparoscopy and sampling of the nodules. Thehistopathological examination reveals a typical interlacingbundle of smooth muscle cells arranged in a whorled patternsuggestive of a leiomyoma.

Conflicts of interest

The authors have none to declare.

r e f e r e n c e s

1. Marwah N, Duhan A, Aggarwal G, Sen R. An unusualpresentation of pelvic leiomyomatosis misdiagnosed asdisseminated malignancy. Case Rep Pathol. 2012;2012:. http://dx.doi.org/10.1155/2012/394106 [Article ID 394106, 3 pp.,PMCID: PMC3502812].

2. Al-Talib A, Tulandi T. Pathophysiology and possibleiatrogenic cause of leiomyomatosis peritonealis disseminata.Gynecol Obstet Invest. 2010;69:239–244 [PMID: 20068330].

3. Fasih N, Prasad Shanbhogue AK, Macdonald DB. Leiomyomasbeyond the uterus: unusual locations, rare manifestations.Radiographics. 2008;28:1931–1948 [PMID: 19001649].

4. Seidman MA, Oduyebo T, Muto MG, Crum CP, Nucci MR,Quade BJ. Peritoneal dissemination complicatingmorcellation of uterine mesenchymal neoplasms. PLoS ONE.2012;7(11):e50058. http://dx.doi.org/10.1371/journal.pone.0050058 [PMID: 23189178].

5. Walkey MM, Friedman AC, Sohotra P, Radecki PD. CTmanifestations of peritoneal carcinomatosis. Am J Roentgenol.1988;150(5):1035–1041 [PMID: 3258703].

6. Granados Palacio LF, Pernas JC, Menso MM, Hernández JA.Endometriosis: different locations and faces seen by CT andMRI. Eur Soc Radiol. doi:10.1594/ecr2014/C-0537.

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