Cystic endometriosis in a degenerated sub-serous myoma in a sub-fertile woman: A case report

Amrit Bhusal: literature review, follow up the patient, writing the manuscript, and final approval of the manuscript Prashant Rai: literature review, follow up the patient, writing the manuscript, and final approval of the manuscript Dr. Anju Pradhan: literature review, histopathology of the specimen and final approval of the manuscript Dr. Ajay Agrawal: Supervisor, literature review, surgery of the patient and final approval of the manuscript

Ethical approval was provided by the author' institution.

No funding received from external source.

Written consent was obtained from the patient for the publication of this case report and accompanying images. A copy of written consent is available for review by the Editor-in-chief of this journal on request.

Leiomyoma is the most common gynaecological tumor in women [1] . Leiomyomas are rarely found in post-menopausal women as their growth is thought to be estrogen dependent and most of them regress after menopause [5] . There are various types of leiomyoma degenerations, most common one being, hyaline degeneration(60 %), while myxoid, calcific, mucoid, cystic, red and fatty degeneration being the others [2] , [3] . Cystic degeneration could be sequel of edema reported to be seen in 4 % of all leiomyomas [6] , [7] .The key regulators of fibroid growth are ovarian steroids, estrogen and progesterone, growth factors, angiogenesis and process of apoptosis [8] . The most common site of endometriosis is ovaries followed fallopian tube, pelvic peritoneum and utero-sacral ligaments with atypical sites being gastrointestinal tract, urinary tract, soft tissue and chest, nose and incisional sites [4] . It can be asymptomatic (discovered incidentally) or may present with pelvic pain, dysmenorrhea, dyspareunia and infertility [4] . The patient reported in this article had dysmenorrhic pain of progressively increasing intensity not limited to around menstrual period. Common imaging modalities include Ultrasonography, CT scan and MRI for evaluating uterine and ovarian pathology which can be utilized depending upon patient's clinical history and presentation [2] . In our patient, just clinical evaluation and Ultrasonography was done with no other lab investigations (CA-12-5 and CEA) and the patient was prepared for laparoscopic surgery. The implantation of the endometriosis into the degenerated myoma is thought to be due to the inoculation of endometriotic cells by follicular aspirations during operations [1] , [6] but it is not the case in our article as there is no history of operations in the past. In rare disease like Leiomyomatosis Peritonealis Disseminata (LPD), endometriosis can co-exist with leiomyoma which is hypothesized to be possible due to origin of sub-mesothelial multipotent stem cells also called secondary mullerian system and endometrium from the same epithelium [1] , [6] . A study in 2021 showed strong association existing between uterine leiomyoma and endometriosis which was hypothesized by the fact that uterine leiomyoma could predispose to retroverted uterus or distorted uterine cavity, increasing the risk of retrograde menstruation and thus increasing the risk of endometriosis [9] . Our case reports uncommon diagnosis and management of a fibroid that underwent cystic degeneration. Upon review of case reports and case series of degenerative cystic leiomyomas, the first line intervention is commonly the exploratory laparotomy followed by definitive hysterectomy. In few cases, minimally invasive approaches have been used which only provides temporary measure as the fluid re-accumulates. Our case was successfully treated with fertility-sparing laparoscopic removal avoiding a laparotomy and definitive hysterectomy.

None.

Amrit Bhusal.

Though Leiomyoma is the most common gynecologic tumor seen in women, degeneration in leiomyoma is rare. Endometriosis can be seen at rare locations. And also, cystic degeneration is rarer than the commonly occurring hyaline degeneration. Degenerated Leiomyomas can be misdiagnosed as other adnexal mass both clinically and radiologically. So, there must be high degree of suspicion among the gynaecological consultants for the early diagnosis and management of the patient. Cystic endometriosis in a degenerated subserous leiomyoma is an extremely rare endometriotic implantation and to the best of our knowledge is the first case of its kind to be reported from our country Nepal.

Not commissioned and not externally peer reviewed.

Leiomyoma, being the most common gynaecological tumor seen in women, is classified as sub-mucosal, intramural and sub-serosal according to its origin with the symptoms and signs differing according to the location [1] . Symptoms classically present in women aged thirty to forty, consistent with hormonal changes that occur during reproductive years [2] . Leiomyoma degenerations, mostly being symptomatic can sometimes be asymptomatic while subserous leiomyomas are pedunculated and symptoms can be seen due to torsion of the myoma pedicle. It may commonly present with abnormal uterine bleeding, bulk related symptoms or infertility [2] . Fibroid degeneration typically occurs when a fibroid outgrows the vascular supply. The most common degeneration is hyaline degeneration, accounting for about 60 % of all degenerating fibroids. Other types of degeneration are myxoid, cystic, calcific, mucoid, red and fatty degeneration [2] , [3] . Endometriosis is the common gynaecological disease affecting 10 % to 15 % of reproductive age women being associated with various degrees of fertility problems [2] . Endometriosis is the presence of endometrial glands and stroma at extrauterine sites. It is a benign, chronic, estrogen-dependent disorder. It can be associated with many distressing and debilitating symptoms such as pelvic pain, severe dysmenorrhea, dyspareunia and infertility or it may be asymptomatic and incidentally discovered at laparoscopy or exploratory surgery. These ectopic endometrial implants are usually located in the pelvis but can occur anywhere in the body, the common locations being the ovaries, fallopian tube, pelvic peritoneum and the utero-sacral ligaments while the atypical sites of endometriosis include the gastrointestinal tract, urinary tract, soft tissues and chest, nose and incisional sites [4] . We report a case of a cystic endometriosis in a degenerated subserous myoma in a sub-fertile woman. Our case report has been reported in line with SCARE criteria 2020 [10] .

A 40-years old woman with P1L1A2, previous normal vaginal delivery 7 years back, presented with history of secondary sub fertility for last 5 years. She was diagnosed to have fibroid uterus and was under conservative management for last one year when she presented to us. She also had complaints of cyclic, congestive dysmenorrhea of moderate intensity which used to get relieved on taking analgesic for one year. She had regular menstrual cycle with average amount of flow. But since last one month the intensity of her abdominal pain was increasing and not limited to around menstrual period. On abdominal examination, there was abdomino-pelvic mass of 16 weeks size with irregular surface contour and variegated consistency. On pelvic exam, uterus felt to be enlarged to 16 weeks of pregnant uterus size with firm mass of around 10 cm felt in right fornix which had irregular contour and firm consistency with mild tenderness. She was admitted with provisional diagnosis of complex ovarian mass probably endometrioma. Her ultrasound reported as a large multiseptated solid cystic lesion seen in both adnexal region measuring 10.6 × 6 cm in left and 5.6 × 4.8 cm in right adnexae respectively, with another thick walled cystic mass measuring 4.5 × 4.3 cm is also seen in right adnexal region with homogenous internal echoes. So, with clinical diagnosis of bilateral adnexal mass, she was prepared for laparoscopic surgery. Upon laparoscopy, uterus was found to be enlarged with a mass arising from left cornufundal region ( Fig. 1 ). The base of the mass was around 4 cm thick, however the mass was huge of around 15 cm occupying the pouch of Douglas and the right lateral pelvic wall. Flimsy adhesion of the mass with omentum, bowel and posterior surface of uterus was present. Bilateral fallopian tubes and ovaries were grossly normal and tubes were patent. After adhesiolysis the pedicle of uterine mass was coagulated, cut and repaired. The mass was removed by manual morcellation ( Fig. 2 ). During morcellation there were multiple cystic spaces within the mass filled with mucinous and chocolate coloured fluid. The specimen was sent for histopathology which was consistent with cystic endometriotic degenerated myoma. The patient did well in post-operative period and was discharged on second post-operative day with the advice to plan for conceiving after 3 months. Fig. 1 Large fibroid (laparoscopic view). Fig. 1 Fig. 2 Manual morcellation being performed. Fig. 2 Large fibroid (laparoscopic view). Manual morcellation being performed. Pathology report: 1. Macroscopy: Macroscopy: Multiple fragmented bits of grey-white to grey-brown soft tissue measuring together 17 ∗ 15 ∗ 5.5 cm ( Fig. 3 ). Fig. 3 Multiple fragmented bits of soft tissues. Fig. 3 Multiple fragmented bits of soft tissues. Cut surface is solid, cystic, grey-white to grey-brown to focally blackish. Cysts range in size from 4 ∗ 3 ∗ 1 cm to 0.3 ∗ 0.2 ∗ 0.1 cm, expelling clear fluid with areas of haemorrhage. Solid area measures 4 ∗ 3 ∗ 4.5 cm. Cut surface also revealed whorling pattern. 2. Microscopy: Microscopy: Sections from solid area revealed proliferation of spindle cells arranged in intersecting fascicles and whorls ( Fig. 4 ). These cells have elongated nuclei with blunt end, fine dispersed chromatin and abundant fibrillary cytoplasm. Fig. 4 Section from solid area revealing proliferation of spindle cells arranged in intersecting fascicles and whorls. Fig. 4 Section from solid area revealing proliferation of spindle cells arranged in intersecting fascicles and whorls. Sections from cystic area revealed cyst wall lined by endometrial epithelium and stroma ( Fig. 5 ) with areas lined by haemorrhage and hemosiderin laden macrophages. Fig. 5 Section from cystic area revealing cyst wall lined by endometrial epithelium and stroma. Fig. 5 Section from cystic area revealing cyst wall lined by endometrial epithelium and stroma. The overall histological features are those of: Leiomyoma with Cystic Endometriosis.

There are no conflicts of interest.