{"paper_id":"8778524f-2d57-42fd-a1a5-76dd849d5306","body_text":"194 Sri Lanka Journal of Obstetrics and Gynaecology\nCase report\nA case report on cystic adenomyosis: A rare variant of uterine\nadenomyosis\nK G Hewawitharanaa, N Sarathchandrab, J Mallawarachchib, V J Meegoda c, K V H Nimana d\nCase report\nThis is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License, which\npermits unrestricted use, distribution and reproduction in any medium provided the original author and source are credited.\nSri Lanka Journal of Obstetrics and Gynaecology   2023; 45: 194-198\nDOI:  https://doi.org/10.4038/sljog.v45i4.8100\nCorrespondence: KGH, e-mail: kavi88fmas@gmail.com\nhttps://orcid.org/0009-0008-5362-5705\nReceived 07th August 2023\nAccepted 31st December 2023\na Acting Consultant Obstetrician and Gynecologist, Base Hospital, Mahaoya, Sri Lanka.\nb Medical Officer, Base Hospital, Mahaoya, Sri Lanka.\nc Medical Officer, Base Hospital, Tangalle, Sri Lanka.\nd Medical Officer, Base Hospital, Pimbura, Sri Lanka.\nKeywords: cystic adenomyosis, myometrial cysts, chronic pelvic pain\nAbstract\nBackground:-Cystic adenomyosis is a rare variant of adenomyosis that can easily be misdiagnosed, due to the non-specific\nnature of symptoms. USS and MRI are essential in making a diagnosis which is later confirmed through histopathological\nevaluation. Surgical interventions are favored over medical management, as complete resection reduces the risk of recurrences\nand alleviates the chances of malignant transformation.\nCase presentation:  A 37-year-old multipara presented with chronic abdominal pain, exacerbated by menstruation, lasting\nmost days of the month for over one year. Clinical evaluation revealed a tender, less mobile pelvic mass which mimicked an\nintramural fibroid on USS. Considering the long-standing symptoms and lack of fertility wishes, she underwent a laparotomy.\nDuring surgery, an unexpected finding of chocolate-colored exudate with a thick epithelium-lined myometrial cystic lesion\nwas noted. Histopathology evaluation revealed fragmented tissue composed of smooth muscle bundles and are lined by\nendometriotic type epithelium and stroma. There was no malignant changes. Overall findings are in favor of cystic adenomyosis.\nDiscussion and conclusions:  Cystic adenomyosis is considered to arise due to persisting mullerian epithelial cells\nstimulated by estrogen leading to periodic bleeding into the myometrium. Surgery of the myometrium can also introduce\nendometrial tissue to the myometrium. To preserve the quality of life and reduce the risk of recurrence, minimal access\nsurgery should be carried out.\nEven though rare, a young woman presenting with chronic pelvic pain, and not responding to analgesics should raise the\npossibility of cystic adenomyosis, which should warrant further investigation and treatment.\n\n195Vol. 45, No. 4, December 2023\nCase report\nBackground\nAdenomyosis is a common gynecological pathology\nwhere endometrial glands and stroma invade the uterine\nmyometrium. For descriptive purposes, it is further\ndivided into diffuse and focal adenomyosis. Cystic\nadenomyosis is a rare, sporadic disease characterized\nby chocolate-like viscous fluid-contained lesions in the\nmyometrium\n1. It represents focal adenomyosis. At\npresent, only 47 cases have been reported in the lite-\nrature, including this case report. Cystic adenomyosis\noften mimics the natural history of hemorrhagic\ndegeneration of fibroids. Apart from that, congenital\nmalformations and ovarian pathology needs to be kept\nin mind when making a diagnosis\n2.\nThis condition is predominately seen in young fertile\nage group of women and has a varied clinical presen-\ntation. Commonly these females seek medical attention\nfor dysmenorrhea, chronic pelvic pain, and abnormal\nuterine bleeding. There were cases which had been\ndiagnosed incidentally with no symptoms whatsoever.\nCase presentation\nA 37-year-old multipara female was referred by a general\npractitioner who was evaluated and treated for chronic\nlower abdominal pain for almost one year duration.\nHer obstetric history was rather uncomplicated, where\nboth her children were delivered vaginally with no\ncomplications and no history of subfertility. Since the\nsecond child, she had been on intramuscular proges-\nterone for nearly two years, after which she had\ndiscontinued due to weight gain. Her menstrual cycles\nwere regular with 3-5 days of bleeding, and except\nfor dysmenorrhea outlasting cycles, no other menstrual\nsymptoms had been noted. In this background, she\nhad developed a non-specific lower abdominal pain\nwith an insidious onset, lasting for most days of month,\nwhich had progressively worsened over time and had\nnot responded to analgesics.\nOn abdominal and pelvic examination, a tender uterine\norigin pelvic mass with reduced mobility of the size of\na 16 weeks’ gravid uterus was felt. Ultrasonography\nrevealed a 8.2×7.3 cm pelvic mass, which was\nsuggestive of an intramural fibroid on the anterior\nuterine wall. Her preliminary blood investigations were\nnormal and urine HCG was negative.\nConsidering her symptoms and ultrasonic appearance\nof the mass, patient was counselled for surgical inter-\nvention. However, she insisted on uterine preservation\nbut requested a simultaneous tubal sterilization.\nDuring surgery, an unexpected finding of chocolate-\ncolored exudate with thick epithelium-lined myometrial\ncystic lesion was noted. Following drainage of altered\nblood, excision of epithelial lining was done and the\nuterine wall repaired. Intentionally the endometrial\ncavity was opened into, to exclude any concomitant\nor extension of the pathology. For histopathological\npurposes, epithelium lined myometrial segments were\nsent as per laboratory guidelines.\nHistopathology evaluation revealed fragmented tissue\ncomposed of smooth muscle bundles and are lined by\nendometriotic type epithelium and stroma. There was\nno malignant changes. Overall findings are in favor of\ncystic adenomyosis.\nFigure 1. (Image-A&B: shows chocolate colored fluid release with myometrial incision and cystic space\nwithin myometrium).\n(A) (b)\n\n196 Sri Lanka Journal of Obstetrics and Gynaecology\nCase report\nDiscussion\nAccording to age of presentation, cystic adenomyosis is further classified as primary (juvenile) or secondary\ncystic adenomyosis. Primary disease manifest about five years after menarche (around 18-years) whereas\nsecondary disease appears after 30-years of age.\nDepending on location, cystic adenomyosis is further classified\n2 as –\n• A1 subtype- submucosal or intramural cystic adenomyosis\n• A2 subtype-cystic polypoid lesions\n• B1 subtype-subserosal cystic adenomyosis\n• B2 subtype-cases of intrauterine growth\n• C subtype-similar cysts in the uterus\nMost cystic adenomyosis appears to be intra myometrial and very few are sub-serosal\n1,10.\nhttps://hysteroscopynewsletter.com/2019/08/07/hysteroscopic-treatment-of-submucous-cystic-adenomyosis)\nThe pathogenesis of cystic adenomyosis is unclear.\nThere are two proposed mechanisms are noted in\nliterature1. First hypothesized mechanism is thought\nto be due to persisting mullerian epithelial cells which\nreacts to estrogen causing periodic bleeding inside the\nmyometrium. The other method is considered to be\niatrogenic, uterine surgeries before onset of disease\nleading to dispersion of endometrial tissues into\nmyometrium.\nDiagnosis of cystic adenomyosis is supported by\nultrasonography and MRI scans. MRI being the most\nsensitive\n3. MRI will show high signal intensity on the\nT1-weighted image and significantly low signal\nintensity on the T2-weighted image3.\nSophisticated investigations apart, what is most\nimportant is the clinical suspicion that a young woman\npresenting with severe dysmenorrhea or unresolving\nchronic pelvic pain could be due to this rare yet impor-\ntant clinical entity of cystic adenomyosis. In certain\ncases, to exclude other differential diagnoses, such as\nunicornuate uterus with fluid filled rudimentary horn\nthere is a need of diagnostic hysteroscopy of hystero-\nsalpingography\n7.\nDiagnostic criteria for cystic adenomyosis includes4\n(adapted from Zhao CZ et al)\n• Isolated lesion\n• No abnormalities in the uterus, fallopian tubes,\nand ovaries\n• Post-operative lesions with pathological reports\nof cystic adenomyosis\n• Excised lesions with endometrial glands and\ninterstitium\n\n197Vol. 45, No. 4, December 2023\nCase report\n• Lesions that contain viscous chocolate-like liquid\n• Small lesions of adenomyosis, such as\nadenomyoma, adjacent to cystic lesion\nApart from pain and bleeding symptoms affecting\nquality of life, there is a risk of secondary malignant\ntransformation causing risk to life5. This signifies the\nneed of active intervention once condition is diagnosed.\nAge, symptoms, size of cyst, malignancy status and\nfertility wishes influence management options. The\nmain aims of treatment are to remove the cyst, to\nimprove fertility, prevent recurrences and to minimize\nthe risk of malignancy.\nConservative / medical management is suitable for\nwomen with mild symptoms and smaller cysts.\nTherapeutic options include, standard analgesics\ntherapy, hormonal suppression using COCP, LNG-IUS\nor GnRH agonists. The downside for medical\nmanagement include the possibility of relapsing once\ntreatment is halted and the interference with fertility\nwishes.\nSurgery remains the mainstay of management. Open,\nlaparoscopic or hysteroscopic approaches can be used\nand whenever feasible, minimal access surgical inter-\nventions must be considered as it has many advantages\nover open surgery\n9. Hysteroscopy avoids abdominal\nincisions, serosal and outer myometrial incisions. But\nlarger lesions are difficult to manage by this route\n6.\nWhen no fertility wishes and if patient consented for\nhysterectomy, surgery itself is curative. Otherwise,\ncomplete resection of cyst with wall and adjacent\nmyometrium is the option. There are many other\ndescribed interventions and attempted interventions\nincluding high frequency ultrasound ablations\n8 and\nradio-frequency ablations with alternating efficacy.\nWe chose the traditional open surgical approach as\ncomplete excision is more promising and post\noperatively patient was placed on continuous oral\nprogestogen with 6 weeks review plan.\nConclusions\nCystic adenomyosis is a distinct uterine pathology that\nneeds to be kept in mind when evaluating a myometrial\ncyst in a younger woman. Though rare, it has a\npotential for malignant transformation. Surgical\nmanagement is preferred over medical management in\nmost instances. However lesion size, location, patient\nage and fertility wishes should factor-in when deciding\nthe treatment modality.\nAcknowledgements\nWe acknowledge the Anesthesia Team and Surgical\nTheater Staff of Base Hospital, Mahaoya for their\nsupport.\nConflicts of interests\nNone.\nReferences\n1. Xu T, Li Y , Jiang L, Liu Q, Liu K. Subserous Cystic\nAdenomyosis: A Case Report and Review of the\nLiterature.Sec.Obstetric and Gynecological\nSurgery 2022; 9.  https://doi.org/10.3389/fsurg.\n2022.807676\n2. Brosens I, Gordts S, Habiba M, Benagiano G.\nUterine Cystic Adenomyosis: A Disease of Younger\nWomen. Journal of Pediatric and Adolescent\nGynecology 2015; 28(6): 420-6.doi; 10.1016/\nj.jpag.2014.05.008\n3. Tamai K, Koyama T, Umeoka S, Saga T, Fujii S,\nTogashi K. Spectrum of MR features in\nadenomyosis. Best Pract Res Clin Obstet\nGynaecol. 2006; 20: 583. doi: 10.1016/j.bpobgyn.\n2006.01.009\n4. 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Efficacy of high intensity focused ultrasound\ntreatment for cystic adenomyosis: a report of four\ncases. Ann Palliat Med. 2020; 9: 3742-9.\ndoi: 10.21037/apm-20-1599\n9. Takeda A, Sakai K, Mitsui T, Nakamura H.\nLaparoscopic management of juvenile cystic\nadenomyoma of the uterus: Report of two cases\nand review of the literature. J Minim Invasive\nGynecol. 2007; 14: 370.\n10. Neri A, Bahary C, Eckerling B, Lurie M. Serosal\n(subperitoneal) cysts of the uterus. Am J Obstet\nGynecol. 1968; 102: 612.","source_license":"CC0","license_restricted":false}