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Diffuse peritoneal leiomyomatosis: An exceptional entity in a male patient


1 Department of Internal Medicine, Faculty of Medicine and Pharmacy of Rabat, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco
2 Department of Medicine, Mohamed V Regional Hospital, Al Houceima, Morocco
3 Department of Biophysics and Clinical MRI Methods, Faculty of Medicine of Fez, Fez, Morocco
4 Department of Internal Medicine, Faculty of Medicine and Pharmacy of Fez, Hassan II University Hospital, University Sidi Mohamed Ben Abdallah, Fez, Morocco

Correspondence Address:
Saïd Boujraf,
Department of Biophysics and Clinical MRI Methods, Faculty of Medicine and Pharmacy of Fez, BP. 1893, Km 2.200, Sidi Hrazem Road, Fez 30000
Morocco
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_688_17

 > Abstract 


Diffuse peritoneal leiomyomatosis is a benign peritoneal tumor which develops from smoother muscular fibers. It is a rare entity in females undergoing hormonal effects. This pathological entity is exceptional in male patients. Hence, we report a unique case of diffuse peritoneal leiomyomatosis that occurred in a male patient without hormonal stimulations. The etiopathology and genesis are not completely elucidated. Besides, the clinical symptoms are not specific. The positive diagnosis is based on sets of imaging argument. The anatomicopathological studies allow confirming the diagnosis and reject any tumoral origin. The evolution of the pathology is often favorable. The malignant transformation is exceptional. The treatment is based on abolishing any of the hormonal stimulations. The surgical exeresis is indicated in case of higher peritoneal mass. In case of recurrence or progression, luteinizing hormone-releasing hormone analogs or surgical castrations are indicated.

Keywords: Benign peritoneal tumor, benign tumors with fusiform cells, diffuse peritoneal leiomyomatosis



How to cite this URL:
Khibri H, Bousseaden A, Alami B, Boujraf S, Rabhi S. Diffuse peritoneal leiomyomatosis: An exceptional entity in a male patient. J Can Res Ther [Epub ahead of print] [cited 2019 Nov 18]. Available from: http://www.cancerjournal.net/preprintarticle.asp?id=257727




 > Introduction Top


Peritoneal leiomyomatosis is a rare benign tumor which originates from the smoother muscle cells. It occurs mostly in young females that undergo hormonal effects and exceptionally arises in males.

Diffuse peritoneal leiomyomatosis represents a diffuse nodular entity at the peritoneal level. The etiopathology and genesis are not completely elucidated. The evolution is mostly favorable while the treatment is based on abolishing the hormonal stimuli.

We report a unique male case that presented a diffuse peritoneal leiomyomatosis without hormonal stimulations.


 > Case Report Top


We report a patient wile the consent form has been obtained. The patient was 40-year-old, single male without children and without a medical or surgical history. This includes an exclusion of tuberculosis and contagious tuberculosis and exclusion of abdominal and pelvic surgery. The patient did not report any previous use of hormonal treatment. The patient reported that there was never similar case in his family.

The patient reported diffuse intermittent abdominal pains associated with dyspepsia, while the patient did not report any intestinal transit disorders, bleeding, and digestive or tumoral syndrome.

The initial clinical examination was negative and did not show any hepatosplenomegaly or noticeable mass or peripheral adenopathy.

The investigation did not reveal declivitous Matt effect. The rectal touch was normal. The cutaneous, neurological, osteoarticular, or genital examination did not show any tumoral mass.

The initial abdominal ultrasound highlighted an echogenic-free ascites of low abundance; it was without deep adenopathy, nor a tumoral mass, or a digestive thickening.

The cytochemistry and bacteriological studies of ascites fluid highlighted a sterile ascites containing exudative (proteins: 60 g/l, lymphocytes: 65%).

Considering this clinical profile consisting of associated dyspepsia with exudative ascites, we investigated pulmonary or extra lung tuberculosis which was negative.

The gastroesophageal duodenal endoscopy and colonoscopy investigated carcinomatous origins. The anatomicopathological study of the digestive biopsies discarded malignant neoplastic site and highlighted gastritis and a moderated subacute colitis.

The ultrasound superficial exploration of the abdomen highlighted diffuse peritoneal nodules with well-delineated peritoneal hypoechogenic fat, and the size was infracentimetric [Figure 1]. These nodules were associated with hyperechogenic granulations which were partially calcified [Figure 2].
Figure 1: Ultrasound image showing hypoechogenic peritoneal nodules

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Figure 2: Ultrasound image showing partially calcified peritoneal granulations

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This ultrasound objectified a thicker anterior epiploic fat [Figure 3] as well as a peritoneal hypoechogenic irregular mass measuring 20 mm × 14 mm [Figure 4].
Figure 3: Ultrasound image showing an irregular epiploic thickening of the fat

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Figure 4: Ultrasound image showing a peritoneal mass measuring 20 mm × 14 mm

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The abdominal computed tomography (CT) scan showed an infiltration of the anterior mesenteric and epiploic fat [Figure 5] and [Figure 6] with an aspect of epiploic cake [Figure 7]. The metastatic form was also discarded other intra-abdominal organs and the lung.
Figure 5: Computed tomography scan showing an epiploic infiltration

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Figure 6: Computed tomography scan showing an infiltration of the mesenteric fat

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Figure 7: Computed tomography scan showing the aspect of an epiploic cake

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The surgical exploration using a minilaparotomy allowed achieving exeresis biopsies' peritoneal nodules measuring 15 mm × 10 mm × 6 mm and 12 mm × 8 mm × 6 mm, both nodules weighed 2 g. Their peroperating macroscopic aspects evoked a peritoneal carcinosis [Figure 8].
Figure 8: Peroperating macroscopic aspect of the diffuse peritoneal leiomyomatosis of our patient. The surgical exploration showed shrinkage of the big epiplon, stomach, and cross-colon

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The anatomicopathological study objectified smooth muscle cells arranged in beams crossed in the right angle. These muscle cells included a fusiform cytoplasm and oval nuclei that are not atypical, figures mitoses were not visible. These muscle fascicles were separated by fibrous tissues. This histological aspect was compatible with a peritoneal leiomyoma.

The histological study discarded the presence of epithelio-giganto-cellular granuloma and caseous necrosis.

A tumoral origin such as carcinomatosis was eliminated by the absence of tumoral cells in the ascites fluid and by the immunohistochemistry study biopsied samples. The antibody anti-keratin AE1/AE3 (DaKo) study was negative.

The patient did not report earlier hormone intake nor drugs impacting on the hormonal secretion status such as estrogen-progesterone, neuroleptics, antidepressants, corticosteroids, and proteins for rapid bodybuilding.

Indeed, the patient did report neither erectile dysfunction nor a reduced sexual drive. The clinical examination targeting endocrine disorders showed normal secondary sexual aspects including facial and pubic pilosity and well set testicles with normal size; the clinical examination allowed to exclude any gynecomastia or goiter.

The biological endocrine assessment with minima has demonstrated that a free rate of testosterone was normal at 102 ng/l and the prolactin was normal at 10 ng/l. The thyroid-stimulating hormone was 1.5 mUI/l, and the follicle-stimulating hormone (FSH) was 9 UI/l.

Based on assessment findings, the diagnosis of a diffuse peritoneal leiomyomatosis was approved in our young male patient without hormonal impact.

The indicated therapeutic approach consisted of a symptomatic treatment including antispasmodic, analgesic, and regulators of the transit. Considering the diffuse form of leiomyomatosis nodules of small sizes, the surgical exeresis of the tumoral masses was not indicated. The surgical or chemical castration by luteinizing hormone-releasing hormone (LHRH) analogs was not suggested to the patient, and this allows protecting the sexual functions.

The clinical and ultrasound follow-up of the patient was done each trimester. Along 1-year follow-up, the patient did not reveal any increase of the size of peritoneal nodules nor occurrence of tumoral or metastasis signs. The volume of the ascites remained stable within small quantity. The follow-up continues at the same intervals, and by the end of the 2nd year, the follow-up will be at rate of 6-month interval.


 > Discussion Top


The peritoneal leiomyoma is a rare pathological entity, which corresponds to a proliferation of smoother muscles in the peritoneal cavity. The peritoneal leiomyoma belongs to a group of benign tumors with fusiform cells. Only 150 cases were described in the literature. The peritoneal leiomyoma is mostly occurring in young female adults with age ranging between 35 and 45 years old. The peritoneal leiomyoma might occur in 20%–25% of females undergoing hormonal influences including pregnancy and estrogen-progesterone medicines.[1],[2]

Two similar cases are described in the literature in male patients, therefore our third case worth to be documented as original observation.[3]

The pathogenesis of this pathology is mostly developed from a uterine myoma. This entity might occur as an isolated leiomyoma, benign metastatic leiomyoma, and of diffuse peritoneal leiomyomatosis.[4]

Indeed, the isolated leiomyoma involves the parasitic theory which explains the pathophysiological mechanisms, while the etiopathology is not yet elucidated in metastatic benign leiomyoma and the peritoneal diffuse leiomyomatosis. The pathophysiological hypotheses suggest the hormone as main impact factor including exposition to endogenous or exogenous estrogens.[5]

The diffuse peritoneal leiomyomatosis can be clinically asymptomatic and might express nonspecific symptoms including abdominal or pelvic pains; vaginal or rectal bleedings; intestinal transit disorders; and nervous, vascular, or urinary effects. The diffuse peritoneal leiomyomatosis is revealed by etiological assessment of abdominal or pelvic mass,[2],[3],[4] while the clinical examination is mostly insignificant.

The diffuse peritoneal leiomyomatosis could extend over several intraperitoneal organs such as neighboring the uterus and the abdominal surgery scars, in the Omentum and mesenterium.[6] Indeed, our patient showed extension the epiplon and mesenterium levels.

The imaging explorations are essential for achieving positive diagnosis of the diffuse peritoneal leiomyomatosis, while it is not sufficient for confident diagnosis.[4] The imaging allows to rule out the signs of malignancy and to specify the extent of the peritoneal leiomyomatosis.

The literature reports that the ultrasound signs of diffuse peritoneal leiomyomatosis are not specific; the technique might reveal nonspecific masses of hypo-, iso-, or hyperechogenic signals compared to the myometrium.[7],[8] In our patient, the superficial ultrasound showed various aspects with different features such as size and aspect; this includes partially calcified hyperechogenic granulations and diffuse peritoneal nodules in peritoneal fat with hypoechogenic aspect; it presents well-delineated shape of centimetric and infracentimetric size; it presents also an anterior epiploic thickening fat, with a hypoechogenic peritoneal irregular mass of 20 mm × 14 mm.

The CT scan could show typical but not specific lesions. They are hypodense, well confined, and hyperdense after contrast enhancement.[9] The CT scan could confirm the presence of metastatic forms, especially in the lung. In our patient, the CT scan showed an infiltration of the mesenteric fat and anterior epiploic presenting an aspect of epiploic cake.

The magnetic resonance imaging (MRI) of the diffuse peritoneal leiomyomatosis is characterized by peritoneal nodules measuring from few millimeters to few centimeters.

They present a hyposignal to intermediate signal in T1-weighted images and hyposignal in T2-weighted images, these signal levels are similar to recorded signals in the pelvic wall muscles, and they present a homogeneous gadolinium contrast enhancement. Sometimes, it is difficult to distinguish malignant tumors from diffuse peritoneal leiomyomatosis in MRI, therefore the anatomicopathological study peritoneal nodules much more interesting.[10],[11]

The histological study of leiomyomatous nodules demonstrates the presence of smooth muscular fibers that might contain myofibroblasts and decidual cell. They are similar to normal smooth muscle cells such as eosinophil cytoplasm and nucleus with rounded extremities, without nuclear polymorphism; they are arranged in beams that are crossing perpendicularly.

Often, these cells not show active mitoses, and sometimes, they have a low mitosis index since being lower than three mitoses per ten fields. Besides, any mitotic activity within a smooth muscular tumor of deep smooth tissues has to generate reserves on the evolutionary potential of the tumor; then, the presence of more than two mitoses for ten fields in the strong magnification as well as modifications of the chromatic texture or an increase of the cellular density or the nuclear pleomorphism could be symptomatic expression of leiomyosarcoma.[12],[13]

In our patient, the anatomicopathological study meets the histological and immunohistochemistry criteria of benign leiomyoma and allows discarding a possible malignant proliferation. The differential diagnosis includes leiomyosarcoma, gastrointestinal primitive stromal tumor of the peritoneum, and the malignant peritoneal mesothelioma.

Leiomyosarcoma is a malignant tumor occurring generally in old patients. It originates lung metastasis before the peritoneal diffusion. The histological investigations demonstrate a high mitotic index and a positive reaction to CD117 markers and caldesmon. The gastrointestinal primitive stromal tumor represents an important subentity of mesenchymal tumors of the gastrointestinal tracts. They are mainly developing in the stomach, bowel, rectum, esophagus, and colon. They mark CD117, CD34, and h-caldesmon, sometimes focally in the actine but rarely in the desmine. The malignant mesothelioma peritoneal is initially in nodular form of the small size. This tumor is positive for calretinin, Wilm's Tumor 1 (WT1), Epithelial Membrane Antigen (EMA), the actine, and the desmine.[14],[15]

In opposite to malignant tumors, the diffuse peritoneal leiomyomatosis has a favorable prognosis. The total regression is possible after abolition of the hormonal stimuli. There are no predictive factors of second recurrence or transformation. In addition, a transformation was described in eight patients within 3–18 months. This deadline could delay to 8 years.

Bekkers et al. suggested that diffuse peritoneal leiomyomatosis cases that occurred outside any hormonal stimulation such without associated uterine leiomyomas and without expression of the hormone receiving correspond to a fully independent entity except for cases with a high malignancy potential.[12] Therefore, it is important to follow up these patients clinically and radiologically. This patient was planned a clinical and ultrasound follow-up while measuring the peritoneal nodule size every trimester. In case of doubt, a histological investigation should be conducted in case of doubt of malignant transformation.

The therapeutic approach consists of eliminating any hormonal stimulation, in particular the estrogen-progesterone. In woman, stopping any hormonal contraception is recommended.

In case of recurrence or progress, LHRH analogs are suggested together with surgical castration. The surgical exeresis is indicated in case of higher volume mass.[12]

Considering the young age, the desire of paternity, and the absence of peritoneal mass, our patient underwent a simple symptomatic treatment including antispasmodic.


 > Conclusion Top


Diffuse peritoneal leiomyomatosis is a rare benign smooth muscle tumor, which is affecting mostly females undergoing estrogen-progesterone stimulations. The occurrence in males is exceptional. Both anatomicopathological and imaging investigations allow differentiating between benign and malignant forms. The evolution was favorable using the treatment based on abolishing the hormonal stimuli.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

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Bachert BJ, Patel RS. Disseminated Peritoneal Leiomyomatosis. Ultrasound Q 2017;33:305-7.  Back to cited text no. 2
    
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Bekkers RL, Willemsen WN, Schijf CP, Massuger LF, Bulten J, Merkus JM, et al. Leiomyomatosis peritonealis disseminata: Does malignant transformation occur? A literature review. Gynecol Oncol 1999;75:158-63.  Back to cited text no. 3
    
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Roue A, Laboisse C, Winer N, Darnis E, Bouquin R, Lopes P, et al. Extra-uterine pelvic leiomyoma: Diagnosis and practical management. J Gynecol Obstet Biol Reprod (Paris) 2007;36:403-8.  Back to cited text no. 4
    
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Julien C, Bourgouin S, Boudin L, Balandraud P. Disseminated Peritoneal Leiomyomatosis. J Gastrointest Surg 2018. doi: 10.1007/s11605-018-3841-7.  Back to cited text no. 5
    
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Thiry T, Dohan A, Naneix AL, Pocard M, Guerrache Y, Fazel A, et al. Diffuse abdominopelvic leiomyomatosis: CT and MR imaging findings with histopathological correlation. Diagn Interv Imaging 2014;95:105-8. doi: 10.1016/j.diii.2013.07.007.  Back to cited text no. 6
    
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Taourel P, Camus C, Lesnik A, Matti-Gazzgnes M, Gallix B, Pujol J, et al. The imaging of normal and pathological peritonitis. Encycl Méd Chir (Elsevier, Paris), Radiodiagnostic-Appareil Digestif 33-482-A-10, 1999. p. 29.  Back to cited text no. 7
    
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Darbois Y, Wacrenier A. Benign y uterine pathology. Imagerie clinique en gynécologie. Paris: Ed. Vignot; 1993. p. 73-100.  Back to cited text no. 8
    
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Warshauer DM, Mandel SR. Leiomyoma of the extraperitoneal round ligament: CT demonstration. Clin Imaging 1999;23:375-6.  Back to cited text no. 9
    
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Tanaka YO, Tsunoda H, Sugano M, Satoh T, Yagi H, Minami R, et al. MR and CT findings of leiomyomatosis peritonealis disseminata with emphasis on assisted reproductive technology as a risk factor. Br J Radiol 2009;82:e44-7.  Back to cited text no. 10
    
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Robert Y, Launey P. IRM en gynécologie. J Gynecol Obstet Biol Reprod (Paris) 2002;31:471-539.  Back to cited text no. 11
    
12.
Bekkers RL, Willemsen WN, Schijf CP, Massuger LF, Bulten J, Merkus JM, et al. Leiomyomatosis peritonealis disseminata: Does malignant transformation occur? A literature review. Gynecol Oncol 1999;75:158-63.  Back to cited text no. 12
    
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Fletcher CD, Kilpatrick SE, Mentzel T. The difficulty in predicting behavior of smooth-muscle tumors in deep soft tissue. Am J Surg Pathol 1995;19:116-7.  Back to cited text no. 13
    
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Miettinen M, Lasota J. Gastrointestinal stromal tumors (GISTs): Definition, occurrence, pathology, differential diagnosis and molecular genetics. Hum Pathol 2003;34:369-74.  Back to cited text no. 14
    
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Horvai AE, Li L, Xu Z, Kramer MJ, Jablons DM, Treseler PA. c-Kit is not expressed in malignant mesothelioma. Mod Pathol 2003;16:818-22.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

 
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