Case Report Recurrent leiomyomatosis peritonealis disseminate: a case report Yaw B. Mensah1, Lawrence Buadi2, Afua Abrahams3, Andrea A. Y. Appau4 and Kwadwo Mensah5 Ghana Med J 2021; 55(2): 160-164 doi: http://dx.doi.org/10.4314/gmj.v55i2.11 1Department of Radiology, University of Ghana Medical School, Korle Bu, Accra 2Department of Obstetrics and Gynaecology, Trauma and Specialist Hospital, Winneba, Ghana 3Pathology Department, University of Ghana Medical School, Korle Bu, Accra 4Department of Radiology, Korle Bu Teaching Hospital, Korle Bu Accra 5Public Health Consultant, P. O Box GP 15533, Kumasi Corresponding author: Yaw B. Mensah E-mail: ybmensah@yahoo.com Conflict of interest: None declared SUMMARY Leiomyomatosis peritonealis disseminata (LPD), a rare and unusual condition affecting mainly women of reproduc- tive age, causes peritoneal and subperitoneal nodules formed by smooth muscle. Very few cases have been diagnosed since the disease was first described. We present a 42year old female who was managed for infertility and uterine myomata at a Municipal hospital in Ghana. Following a pelvic ultrasound diagnosis of multiple uterine myomata the patient was booked for myomectomy. At surgery to remove her myomata, the patient was found to have several peritoneal nodules some of which were attached to peritoneum, omentum and the surface of bowel loops in addition to a uterine myoma. The disease has since recurred twice after two laparotomies. The diagnosis was made by histo- pathology of ultrasound-guided biopsy of the nodules, and she has since been on GnRH analogue treatment. LPD simulates peritoneal carcinomatosis; thus, a good history, clinical evaluation, radiological imaging, and histopatho- logic analysis must be accurately diagnosed. Surgeons’ and Radiologists’ knowledge of the condition is fundamental to ensuring correct diagnosis and appropriate treatment and to minimising the probability of malignant transformation. Keywords: Leiomyomatosis peritonealis disseminata, leiomyoma, leiomyosarcoma, recurrent Funding: None declared INTRODUCTION Leiomyomatosis peritonealis disseminata (LPD) is a rare Trauma and Specialist Hospital, Winneba, Ghana. A pel- and unusual condition associated with multiple vascular vic ultrasound scan performed diagnosed multiple uterine peritoneal and subperitoneal nodules formed by smooth leiomyomata. She was thus scheduled for myomectomy muscle cells. Wilson and Peale first described it in 1952. after the routine laboratory tests were done. At surgery in Very few cases have been diagnosed since the disease June 2016, the uterus was about 12 weeks in size with a was described. This is due to its asymptomatic nature solitary leiomyoma as well as several nodules (similar to hence the possibility of being underdiagnosed.1,2,3,4,5 leiomyomata on gross examination) attached to the peri- toneum omentum and the surface of bowel loops were LPD is commonly noted in women of reproductive age noted (Figure 1). The uterine myoma, together with as and rarely in men or postmenopausal women. Patients many of the peritoneal nodules as possible, were re- usually have a history of pregnancy, oral contraceptive moved. She recovered and was discharged. use, myomectomy, hysterectomy, uterine leiomyoma, or ovarian tumours.1,2,4,5 Clinically, LPD simulates perito- The patient had previously undergone endometrial poly- neal carcinomatosis. Accurate diagnosis requires a good pectomy for abnormal uterine bleeding and anaemia sec- history, clinical evaluation and histopathologic analysis. ondary to prolapsed uterine leiomyoma in 2013 at the This will ensure appropriate treatment and minimise the Korle Bu Teaching Hospital, Accra, Ghana. She contin- probability of malignant transformation.1 ued with her infertility treatment. About eighteen months later, she presented with abdominal distension, which CASE REPORT was again diagnosed on abdominopelvic ultrasound due We present a 42-year-old married nulligravida managed to multiple uterine leiomyomata. A second laparotomy for infertility and abdominopelvic mass in 2016 at the was done in January 2018, which revealed multiple peri- toneal nodules, with some in the Pouch of Douglas and 160 www.ghanamedj.org Volume 55 Number 2 June 2021 Copyright © The Author(s). This is an Open Access article under the CC BY license. Case Report on the broad ligaments bilaterally. However, the uterus, A provisional diagnosis of LPD was made based on the which was almost the same size as previously, was fixed patient’s history. There was no family history of this con- due to previous surgery; thus, it was difficult to free it dition. She had a follow-up ultrasound-guided biopsy of completely to assess its size fully. The nodules were the lesions, which showed interlacing fascicles of smooth again removed. muscles separated by vascularised connective tissue with no evidence of necrosis or mitosis confirming the diag- nosis (Figures 3 and 4). Figure 1 Leiomyomata attached to bowel and omentum during Figure 3 LP (low power) view showing interlacing fascicles of surgery smooth muscle bundles separated by well vascularized connec- tive tissue About a year later, the abdominal distension and discom- fort recurred. The patient’s abdomen felt firm and nodu- lar. She had not lost weight. This time the patient was sent for an abdominopelvic computed tomography (CT) scan, which revealed numerous ovoid isodense enhanc- ing lesions distributed diffusely in the peritoneal cavity (Figure 2). Figure 4 HP (high power) view showing spindle cells with elon- gated nuclei and eosinophilic or occasional fibrillary cytoplasm and distinct cell membranes She was managed with 3.6mg goserelin acetate a Gonad- otropin-Releasing Hormone Analogues (GNRH) per month for three months. She is currently being followed up. A repeat CT scan four months after completing treat- Figure 2 Coronal reformatted abdominal CT scan Image ment showed an increase in the number and size of the showing multiple isodense round and oval-shaped lesions in the peritoneal cavity before GNRH therapy lesions (Figure 5). 161 www.ghanamedj.org Volume 55 Number 2 June 2021 Copyright © The Author(s). This is an Open Access article under the CC BY license. Case Report The aetiology and pathophysiology of LPD is not well understood. Aetiological factors documented are hormo- nal, subperitoneal mesenchymal stem cell metaplasia, ge- netic and iatrogenic.1,6 Lesions are also thought to develop from an unusual and selective sensitivity of mesothelial, submesothelial, mul- tipotential mesenchymal stem cells to hormonal stimula- tion leading to metaplasia. This phenomenon is believed to be potentiated by the presence of oestrogen receptors (ER) and progesterone receptors (PR) on lesions. The hormonal influence is deduced from pregnancy, pro- longed oral contraceptives use, and occasionally, ovarian tumours. In men and postmenopausal women, the condi- tion is attributed partly to the increased responsiveness of tumour cells to normal hormone levels.1,3,5,6,8,10,11 Myo- mectomy and hysterectomy, which fall in the iatrogenic category, are believed to produce leiomyoma fragments that disseminate and implant on the peritoneum and later grow into LPD nodules.1,5 The history of the index pa- Figure 5 Coronal reformatted abdominal CT scan Images show- tient make her condition lean more towards this aetiolog- ing multiple isodense round and oval-shaped lesions in the per- ical model than the others aforementioned. This is sup- itoneal cavity after GNRH therapy ported by the fact that she had not received any hormonal therapy before both surgeries. DISCUSSION This disease was designated as LPD by Taubert et al in The LPD nodules are often noted on the mesentery, 1965.1,5,6 Till date, very few cases have been diagnosed omentum, peritoneum, Douglas' pouch, the serosal sur- because the disease is asymptomatic and possibly being face of the small and large intestine and rarely involve underdiagnosed.1,4 Patients are often diagnosed as an in- the entire muscular layer of the colon. 6 Consistent with cidental finding during a caesarean section or laparotomy what has been documented, the patient also had nodules for some other indication. She was initially diagnosed attached to the peritoneum, omentum, and the bowel's with multiple uterine leiomyomata but was noted to have surface. multiple nodules not linked to the uterus during myomec- tomy.1,4,7 Pre-operatively, patients are often identified incidentally during imaging for other conditions or because of some Like the index patient, most of the reported cases of LPD of the symptoms above. Imaging modalities like Mag- are between 20 years and 55 years. Some LPD patients netic resonance Imaging (MRI), Computed Tomogra- also have other conditions like leiomyosarcoma, liver phy(CT) scan and ultrasound scan can generally detect leiomyoma, steroid hormone-secreting ovarian tumours the lesions of LPD, which tend to have the same features 1,2 and endometriosis;1,4,7,8,9 she had LPD and leiomyoma. as uterine leiomyoma (Figures 2 and 3). However, it is The disease is believed to occur sporadically, and only not always easy to distinguish LPD lesions from malig- one family cluster with inherited autosomal dominant in- nant lesions like leiomyosarcoma and peritoneal dissem- heritance has been reported. The patient denied a family inated metastasis on imaging, requiring direct sampling history of LPD, thus supporting the sporadic occurrence with image-guided biopsies for histological diagno-1,6,12 theory.1,8 sis. As has been documented, the sonographers who initially performed the ultrasound examinations misdiag- Though often asymptomatic, some patients may manifest nosed the nodules as myomata. The surgical findings are nonspecific symptoms like abdominal pain and discom- in agreement with the reported difficulty in distinguish- fort, nausea, vomiting, rectal bleeding, vaginal bleeding, ing LPD from leiomyomata. The abdominopelvic CT abdominal distention, abdominal masses and intestinal scan diagnosed the condition, but it still had to be con- obstruction.1 Similarly, she also complained about ab- firmed histologically, keeping with what has been docu- dominal distension with discomfort and prolonged bleed- mented in the literature. ing per vaginum. 162 www.ghanamedj.org Volume 55 Number 2 June 2021 Copyright © The Author(s). This is an Open Access article under the CC BY license. Case Report Unlike the malignant lesions, LPD is histologically made such aggressive treatment is bilateral salpingo-oophorec- of spindle-shaped smooth muscle cells in interdigitating tomy which is believed to provide a cure in patients with or whorled arrangement with or without mitotic figures. symptomatic LPD with worrisome gross or histopatho- The index patient had a similar pattern, which lacked the logical features.1,3,8 nuclear polymorphism, hyperchromasia, tumour cell ne- crosis and cellular atypia typically noted in malignant le- CONCLUSION sions.1,8 In conclusion, LPD is a rare benign condition which mimics peritoneal carcinomatosis and other malignan- LPD must also be distinguished from other benign tu- cies. Accurate diagnosis requires a good history, clinical mours like benign metastasizing leiomyoma (BML), evaluation, radiological imaging, preoperative image fbromatosis (desmoid tumour), and gastrointestinal stro- guided-biopsy and sometimes post-operative histopatho- mal tumour (GIST), but this is sometimes difficult. Im- logic analysis. Surgeons’ and Radiologists’ knowledge munohistochemistry can help with this differentiation in of the condition is fundamental to ensuring correct diag- most cases. LPD nodules tend to show positivity for des- nosis and appropriate treatment and to minimising the min, actin, caldesmon , Ki-67, vimentin, oestrogen recep- probability of malignant transformation. tor (ER), progesterone receptor (PR), and negativity c- kit.1,8,12 Not all the nodules express both the ER and PR REFERENCES simultaneously. Some express only one, while others do 1. Psathas G, Zarokosta M, Zoulamoglou M, et al. not express any of them. 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