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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
a
Department of Radiology, Apollo Hospitals, Chennai, Tamil Nadu, India
b
Department of Obstetrics and Gynecology, Apollo Hospitals, Chennai, Tamil Nadu, India
c
Department of Surgery, Apollo Hospitals, Chennai, Tamil Nadu, India
1. Introduction
Leiomyomas represent the most common uterine neoplasms.
However, leiomyomas occasionally occur in unusual locations
that make their identification more challenging. Leiomyoma-
tosis peritonealis disseminata (LPD) is a benign condition and a
very rare disease. To our knowledge, there are less than 150
cases reported worldwide.1
It is suspected that this disease
originates from a metaplasia of submesothelial multipotent
mesenchymal cells.2,3
Although it is not clear if the stimulus to
smooth cell differentiation is hormonal, genetic, or both. The
disease is generally associated with high levels of exogenous
or endogenous female gonadal steroids.3
The important
practical issue with LPD is the possibility of misdiagnosing
it as a disseminated malignancy.
a p o l l o m e d i c i n e 1 2 ( 2 0 1 5 ) 1 5 2 – 1 5 4
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 from within 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
ScienceDirect
journal homepage: www.elsevier.com/locate/apme
http://dx.doi.org/10.1016/j.apme.2015.05.013
0976-0016/# 2015 Indraprastha Medical Corporation Ltd. Published by Elsevier B.V. All rights reserved.
2. Case report
A 40-year-old female with regular menstrual history presented
with vague abdominal discomfort and vulval itching. The
patient underwent a myomectomy with morcellation two
years prior. She was referred to us with pelvic ultrasound and
MRI studies done elsewhere. Both studies revealed multiple
peritoneal nodules and raised the suspicion of peritoneal
carcinomatosis by ovarian or bowel primary.
A contrast enhanced 320 slice CT scan was done by us on a
Toshiba Aquilion One, Tokyo, Japan. The intravenous contrast
used was Optiray, Mallinckrodt, USA. 0.5 mm thin sections
were performed through the abdomen and pelvis with 120 kV
and 350 mAs in the plain, arterial (bolus tracking technique),
and venous (70 s delay after start of injection) phases. The CT
scan showed innumerable well-circumscribed homogenously
enhancing peritoneal nodules with smooth convex surfaces
towards the peritoneal cavity throughout the abdomen and
pelvis. These nodules ranged in size from 5 mm to 5 cm. Few
tiny nodules were also seen on the surface of the right ovary.
Multiple small similar intramural nodules were also seen in
the uterus. Rest of the abdominal organs were unremarkable.
A diagnosis of LPD along with uterine fibroids was put forth
rather than peritoneal carcinomatosis. A diagnostic laparos-
copy of the patient was done which revealed innumerable
small nodules in the peritoneum, as described in the CT scan.
Samples were taken from the peritoneal and omental nodules
and sent for histopathological evaluation. The histopatholog-
ical examination revealed typical interlacing bundles of
smooth muscle cells arranged in a whorled pattern without
any atypia, mitosis, or stromal invasion consistent with the CT
diagnosis of LPD (Figs. 1 and 2).
3. Discussion
Discrete nodules of benign smooth muscle scattered over the
peritoneum characterize classic LPD. However, LPD may
present a protean appearance, sometimes as tiny peritoneal
nodules mimicking peritoneal carcinomatosis and at other
times as bulky masses resembling leiomyosarcoma.2
LPD is
classically seen in premenopausal patients and is frequently
seen in pregnant women, as female gonadal steroids are
considered to play an important role in the pathogenesis
resulting in multifocal metaplasia of the peritoneum.
[(Fig._1)TD$FIG]
Fig. 1 – A 40-year-old female with LPD. Post-contrast CT scan volume rendered coronal (A) and axial (B) reconstructions in the
venous phase showing innumerable well-circumscribed homogenously enhancing peritoneal nodules (arrows). Few tiny
nodules were also seen on the surface of right ovary (arrowheads).
[(Fig._2)TD$FIG]
Fig. 2 – A 40-year-old female with LPD. (A) The diagnostic laparoscopy of the patient revealed innumerable small nodules in
the peritoneum. (B) The histopathological examination revealed typical interlacing bundles of smooth muscle cells arranged
in a whorled pattern.
a p o l l o m e d i c i n e 1 2 ( 2 0 1 5 ) 1 5 2 – 1 5 4 153
However, some authors promote a disseminated origin from
a single lesion.3,4
Our patient had a history of myomectomy
with morcellation done elsewhere two years back. The
presence of peritoneal nodules was not registered at that
surgery. This suggests the possibility of dissemination.
Similar findings were also observed by Seidman et al.4
The occurrence of this tumor in postmenopausal women
and rarely in males too suggests the possibility of a second
coexistent pathogenesis involving metaplasia of submesothe-
lial multipotent mesenchymal cells.3
Imaging plays an important role in differentiating this
primarily benign condition with other sinister pathologies of
the peritoneum. On ultrasound examination, these are seen as
well-defined homogeneously hypoechoic nodules with a
smooth surface. On MRI, these appear as hypointense in
T1WI, and similarly hypo, iso, or hyperintense on T2WI
depending on the water content of the tumor.4
On post-
contrast images, they reveal a homogenous enhancement.
MDCT shows these lesions as hypodense, well-circumscribed
solid nodules with homogenous enhancement. The adjacent
peritoneum is usually normal without any thickening or
abnormal enhancement.
The chief imaging differential diagnoses for LPD are
peritoneal carcinomatosis, disseminated leiomyosarcoma,
and endometriosis.1,3
The most common feature of peritoneal
carcinomatosis is ascites.5
The other findings of peritoneal
carcinomatosis include peritoneal thickening with increased
enhancement, peritoneal nodules, omental nodules and
caking, 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 varying
degree of necrosis. While LPD is isometabolic in PET-CT,
leiomyosarcomas are hypermetabolic. Endometriosis presents
as hyperdense cystic lesions with septations and soft tissue
enhancing mass with irregular margins. MRI is more specific,
as it shows hyperintense lesions in T1W fat sat images and
hyperintense lesion with shading in T2W images.6
It has been suggested that LPD should be considered as
diagnosis during surgery, when the patient has coexisting
leiomyoma or diffuse leiomyomatosis of uterus with no
omental thickening or ascites.1,2
They have a tendency to
regress after menopause. The diagnosis is established with a
diagnostic laparoscopy and sampling of the nodules. The
histopathological examination reveals a typical interlacing
bundle of smooth muscle cells arranged in a whorled pattern
suggestive 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 unusual
presentation of pelvic leiomyomatosis misdiagnosed as
disseminated 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 possible
iatrogenic cause of leiomyomatosis peritonealis disseminata.
Gynecol Obstet Invest. 2010;69:239–244 [PMID: 20068330].
3. Fasih N, Prasad Shanbhogue AK, Macdonald DB. Leiomyomas
beyond 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 complicating
morcellation 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. CT
manifestations 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 and
MRI. Eur Soc Radiol. doi:10.1594/ecr2014/C-0537.
a p o l l o m e d i c i n e 1 2 ( 2 0 1 5 ) 1 5 2 – 1 5 4154
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Peritoneal leiomyomatosis – A rare case report

  • 1. Peritoneal leiomyomatosis – A rare case report
  • 2. Case Report Peritoneal leiomyomatosis – A rare case report Rochita Venkataramanan a , Pudhiavan Arunachalam a, *, Vinutha Arunachalam b , Panneer Venkat c a Department of Radiology, Apollo Hospitals, Chennai, Tamil Nadu, India b Department of Obstetrics and Gynecology, Apollo Hospitals, Chennai, Tamil Nadu, India c Department of Surgery, Apollo Hospitals, Chennai, Tamil Nadu, India 1. Introduction Leiomyomas represent the most common uterine neoplasms. However, leiomyomas occasionally occur in unusual locations that make their identification more challenging. Leiomyoma- tosis peritonealis disseminata (LPD) is a benign condition and a very rare disease. To our knowledge, there are less than 150 cases reported worldwide.1 It is suspected that this disease originates from a metaplasia of submesothelial multipotent mesenchymal cells.2,3 Although it is not clear if the stimulus to smooth cell differentiation is hormonal, genetic, or both. The disease is generally associated with high levels of exogenous or endogenous female gonadal steroids.3 The important practical issue with LPD is the possibility of misdiagnosing it as a disseminated malignancy. a p o l l o m e d i c i n e 1 2 ( 2 0 1 5 ) 1 5 2 – 1 5 4 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 from within 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 ScienceDirect journal homepage: www.elsevier.com/locate/apme http://dx.doi.org/10.1016/j.apme.2015.05.013 0976-0016/# 2015 Indraprastha Medical Corporation Ltd. Published by Elsevier B.V. All rights reserved.
  • 3. 2. Case report A 40-year-old female with regular menstrual history presented with vague abdominal discomfort and vulval itching. The patient underwent a myomectomy with morcellation two years prior. She was referred to us with pelvic ultrasound and MRI studies done elsewhere. Both studies revealed multiple peritoneal nodules and raised the suspicion of peritoneal carcinomatosis by ovarian or bowel primary. A contrast enhanced 320 slice CT scan was done by us on a Toshiba Aquilion One, Tokyo, Japan. The intravenous contrast used was Optiray, Mallinckrodt, USA. 0.5 mm thin sections were performed through the abdomen and pelvis with 120 kV and 350 mAs in the plain, arterial (bolus tracking technique), and venous (70 s delay after start of injection) phases. The CT scan showed innumerable well-circumscribed homogenously enhancing peritoneal nodules with smooth convex surfaces towards the peritoneal cavity throughout the abdomen and pelvis. These nodules ranged in size from 5 mm to 5 cm. Few tiny nodules were also seen on the surface of the right ovary. Multiple small similar intramural nodules were also seen in the uterus. Rest of the abdominal organs were unremarkable. A diagnosis of LPD along with uterine fibroids was put forth rather than peritoneal carcinomatosis. A diagnostic laparos- copy of the patient was done which revealed innumerable small nodules in the peritoneum, as described in the CT scan. Samples were taken from the peritoneal and omental nodules and sent for histopathological evaluation. The histopatholog- ical examination revealed typical interlacing bundles of smooth muscle cells arranged in a whorled pattern without any atypia, mitosis, or stromal invasion consistent with the CT diagnosis of LPD (Figs. 1 and 2). 3. Discussion Discrete nodules of benign smooth muscle scattered over the peritoneum characterize classic LPD. However, LPD may present a protean appearance, sometimes as tiny peritoneal nodules mimicking peritoneal carcinomatosis and at other times as bulky masses resembling leiomyosarcoma.2 LPD is classically seen in premenopausal patients and is frequently seen in pregnant women, as female gonadal steroids are considered to play an important role in the pathogenesis resulting in multifocal metaplasia of the peritoneum. [(Fig._1)TD$FIG] Fig. 1 – A 40-year-old female with LPD. Post-contrast CT scan volume rendered coronal (A) and axial (B) reconstructions in the venous phase showing innumerable well-circumscribed homogenously enhancing peritoneal nodules (arrows). Few tiny nodules were also seen on the surface of right ovary (arrowheads). [(Fig._2)TD$FIG] Fig. 2 – A 40-year-old female with LPD. (A) The diagnostic laparoscopy of the patient revealed innumerable small nodules in the peritoneum. (B) The histopathological examination revealed typical interlacing bundles of smooth muscle cells arranged in a whorled pattern. a p o l l o m e d i c i n e 1 2 ( 2 0 1 5 ) 1 5 2 – 1 5 4 153
  • 4. However, some authors promote a disseminated origin from a single lesion.3,4 Our patient had a history of myomectomy with morcellation done elsewhere two years back. The presence of peritoneal nodules was not registered at that surgery. This suggests the possibility of dissemination. Similar findings were also observed by Seidman et al.4 The occurrence of this tumor in postmenopausal women and rarely in males too suggests the possibility of a second coexistent pathogenesis involving metaplasia of submesothe- lial multipotent mesenchymal cells.3 Imaging plays an important role in differentiating this primarily benign condition with other sinister pathologies of the peritoneum. On ultrasound examination, these are seen as well-defined homogeneously hypoechoic nodules with a smooth surface. On MRI, these appear as hypointense in T1WI, and similarly hypo, iso, or hyperintense on T2WI depending on the water content of the tumor.4 On post- contrast images, they reveal a homogenous enhancement. MDCT shows these lesions as hypodense, well-circumscribed solid nodules with homogenous enhancement. The adjacent peritoneum is usually normal without any thickening or abnormal enhancement. The chief imaging differential diagnoses for LPD are peritoneal carcinomatosis, disseminated leiomyosarcoma, and endometriosis.1,3 The most common feature of peritoneal carcinomatosis is ascites.5 The other findings of peritoneal carcinomatosis include peritoneal thickening with increased enhancement, peritoneal nodules, omental nodules and caking, 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 varying degree of necrosis. While LPD is isometabolic in PET-CT, leiomyosarcomas are hypermetabolic. Endometriosis presents as hyperdense cystic lesions with septations and soft tissue enhancing mass with irregular margins. MRI is more specific, as it shows hyperintense lesions in T1W fat sat images and hyperintense lesion with shading in T2W images.6 It has been suggested that LPD should be considered as diagnosis during surgery, when the patient has coexisting leiomyoma or diffuse leiomyomatosis of uterus with no omental thickening or ascites.1,2 They have a tendency to regress after menopause. The diagnosis is established with a diagnostic laparoscopy and sampling of the nodules. The histopathological examination reveals a typical interlacing bundle of smooth muscle cells arranged in a whorled pattern suggestive 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 unusual presentation of pelvic leiomyomatosis misdiagnosed as disseminated 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 possible iatrogenic cause of leiomyomatosis peritonealis disseminata. Gynecol Obstet Invest. 2010;69:239–244 [PMID: 20068330]. 3. Fasih N, Prasad Shanbhogue AK, Macdonald DB. Leiomyomas beyond 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 complicating morcellation 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. CT manifestations 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 and MRI. Eur Soc Radiol. doi:10.1594/ecr2014/C-0537. a p o l l o m e d i c i n e 1 2 ( 2 0 1 5 ) 1 5 2 – 1 5 4154