Leiomyomatosis Peritonei Disseminata Following Total Abdominal Hysterectomy: A Case Report and Literature Review

Case Report

Austin J Womens Health. 2016; 3(1): 1020.

Leiomyomatosis Peritonei Disseminata Following Total Abdominal Hysterectomy: A Case Report and Literature Review

Natalia A¹*, Usman AU¹, Zainab DA¹ and Ali BU²

¹Obstetrics and Gynaecology Department, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria

²Pathology Department, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria

*Corresponding author: Natalia A, Obstetrics and Gynaecology Department Bayero University/Aminu Kano Teaching Hospital, Kano, Zaria road P.M.B.3452, Nigeria

Received: March 01, 2016; Accepted: May 09, 2016; Published: May 11, 2016

Abstract

Uterine leiomyoma is a benign neoplasm that arises from uterine smooth muscles and the most common tumour of the reproductive tract. Very rare leiomyomas present with unusual behavior. Disseminated peritoneal leiomyomatosis presents as multiple small nodules on the peritoneum. We present a case of Mrs. K who is a 60 years old grand multipara, ten years postmenopausal. She had total abdominal hysterectomy for multiple uterine fibroids a year earlier. Abdomino-pelvic scan and CT scan revealed a large multi lobulated heterogeneous lesion in the pelvis. The lesion measured approximately 13.5x11.5cm in size. The tumour was resected. On immunohistochemistry tumour cells were positive for alpha-smooth muscle actin, which is characteristic for smooth muscle tumour. A nuclear staining for progesterone receptor was positive, but negative for oestrogen receptors. Due to rarity of condition different modalities in the management are proposed. In view of possible recurrence and malignant transformation patients need clinical and radiological follow up? Leiomyomatosis peritonei disseminate is a rare condition. Hormonal treatment may be effective in recurrent cases especially in hormone receptor positive tumor or when temporary relief of symptoms is necessary.

Keywords: Leiomyoma; Leiomyomatosis Peritonei Disseminate; Total Abdominal Hysterectomy; Nigeria

Introduction

Uterine leiomyoma is a benign neoplasm that arises from uterine smooth muscle. It is the most common tumour of the reproductive tract in the women of reproductive age, occurring in 20-25% of women over 30 years of age, especially the nulliparous ones. The lifetime risk of fibroids in nulliparous women over the age of 45 years is more than 60% [1]. The incidence of these tumours is several times higher in black women than in white women [2]. Studies indicate that leiomyomas are monoclonal, originated from somatic mutations in myometrial cells, resulting in progressive loss of growth regulation. The key regulators of fibroid growth are ovarian steroids (oestrogen, progestogen) and growth factors [3]. The environment within the leiomyomas is hyperestrogenic. Estrogen may induce the proliferation of leiomyoma cells in part by activating Adenosine Triphosphate (ATP) channel. Progesterone upregulate the level of antiapoptotic protein Bcl-2, which influences cell proliferation [2,3]. The effects of oestrogens and progestins on leiomyomas are mediated by growth factors. Epidermal growth factor, Insuline like growth factor I and II are over expressed in myomas [2,3]. Fibroblast growth factor and vascular endothelial growth factor are also over expressed in myomas, leading to an abnormal vasculature, characterized by a dilated venous plexuses [2]. Oncogene and tumour suppressor genes may also have a role in fibroid development (p-53, p-16) [2,3]. The most frequent cytogenetic changes are translocation and deletion of chromosome 7, which are found in up to 58% of fibroid specimen [2]. Heredity, nulliparity, obesity, polycystic ovary syndrome, diabetes and hypertension are associated with increased risk of fibroid [3]. Reported recurrence rates after myomectomy was up to 50% at five years [3]. Very rare leiomyomas present with unusual behavior. These include parasitic leiomyomas, retroperitoneal leiomyomas, and benign metastasizing leiomyoma and disseminated peritoneal leiomyomatosis [2,4,5]. Disseminated peritoneal leiomyomatosis presents as multiple small nodules on the peritoneum that composed of whorled bundles of smooth muscle cells [4]. It is considered as benign condition and occurs in women predominantly of reproductive age [5]. A few cases were reported in postmenopausal women and even in men [6]. The aetiology and pathogenesis of disseminated peritoneal leiomyomatosis is still controversial. Progesterone receptors, oestrogen receptor and luteinizing hormone receptor expression were detected in cells of disseminated peritoneal leiomyomatosis [6,7]. We present a case of disseminated peritoneal leiomyomatosis in a postmenopausal woman following total abdominal hysterectomy for multiple uterine fibroids a year earlier.

Case Report

Mrs. K was a 60 year old P 12+0 A 11, ten years postmenopausal Nigerian lady (Hausa) who’s last child birth was16 years prior to presentation. She presented to the gynaecological clinic on 22/04/2015 with complaints of heaviness and progressive swelling in her lower abdomen about six months duration following total abdominal hysterectomy for multiple uterine fibroids a year earlier. There was no associated weight loss, pressure symptoms, pains, dysuria, bleeding per vaginam, nausea and vomiting or diarrhea. She had total abdominal hysterectomy and bilateral salpingo-ophorectomy in March 2014 on account of multiple uterine fibroids. The size of the tumour was up to 22 week of gestation with histopathology conformation of leiomyoma. She had never practiced contraception. There was no history of hormone replacement therapy. She had cervical cancer screening prior to the hysterectomy and it was negative. She is a known diabetic and hypertensive patient for the past five years and was on Metformin/ Glibenclomide; Amlodipine and Lisinopril respectively.

Clinical examination revealed an elderly woman, who was not pale and had no peripheral lymphadenopathy. There was no pedal oedema. She had no thyroid gland enlargement. Her BMI was 35.4kg/ m². Her pulse rate was 84 beats per minutes and blood pressure was 130/80 mm Hg.

On abdominal examination a firm, multi nodular, non tender and fixed suprapubic mass about 20 weeks gestation size was felt. The liver, spleen and kidneys were not enlarged. There was no ascites. Pelvic examination revealed atrophic vulva and vagina. No cervix was palpable. The mass approximately of 20 weeks of gestation was felt. It was fixed and multi nodular.

A diagnosis of recurrent pelvic mass was made; she was evaluated and had basic investigations which were within normal limits. Her Fasting blood sugar was 5.2mmol/l. Ca 125 was 7u/ml, oestrogen level was 0.598pg/ml and progesterone level was 23.5ng/l. Preoperative electrocardiogram and chest radiograph revealed no abnormality.

Abdomino-pelvic scan revealed a mass containing mixed echoes of varying sizes with largest measuring 3.6x5.1 cm and no ascites (Figure 1).