Unraveling Cystic Adenomyosis: Diagnostic Odyssey and Surgical Resolution in a Multiparous Woman

In August 2023, a 37-year-old multiparous female from a lower socioeconomic background, with a history of prior cesarean sections, sought help at the outpatient department due to heavy menstrual bleeding. This complaint was not new to her; it had been troubling her since August 2021. Despite being on medical therapy, her symptoms persisted, prompting a detailed evaluation at our center. Her clinical examination, particularly the bimanual examination, revealed an enlarged uterus, regular in shape, corresponding to a size of 10–12-week gravid uterus. Ultrasonography uncovered a heterogeneous lesion in the posterior myometrium, measuring 4 cm × 6 cm with calcifications and peripheral vascularity, pushing the myometrium anteriorly. Further investigation with endometrial sampling revealed secretory endometrium without atypical cells, leading us to start with medical management using tablet ormeloxifene (60 mg twice weekly). For a while, everything seemed under control. However, in December 2023, she returned with a sense of heaviness in her lower abdomen. Her uterine size had increased, comparable to 14–16-week gravid uterus. A repeat ultrasound revealed a well-defined, round-to-oval hypoechoic lesion measuring 7.2 cm × 8.7 cm in the posterior myometrium, pushing the endometrium anteriorly, and containing cystic areas. Despite these findings, we opted to continue with tablet ormeloxifene (60 mg weekly) as she had no episodes of heavy menstrual bleeding, severe dysmenorrhea, or pressure symptoms. In March 2024, the situation escalated with the patient presenting with acute AUB, despite having no such episodes between September 2023 and February 2024. The acute AUB was accompanied by a palpable abdominal mass, lower abdominal pain, and pressure symptoms. Her general physical examination revealed pallor. On further examination, her uterine size had now increased comparable to 26-week gravid uterus, and tenderness was evident. A follow-up ultrasound painted a concerning picture: the uterus was bulky with a heterogeneous echo pattern, the endometrial–myometrial interface was indistinguishable, and a heterogeneous isoechoic mass with cystic areas, measuring 9.6 cm × 12.8 cm, exhibited internal vascularity. The differential diagnosis included a fibroid with cystic degeneration or a neoplastic lesion. To get a clearer picture, a magnetic resonance imaging (MRI) of the pelvic organs was performed. It showed a large, well-defined, multiloculated ovoid mass, heterogeneous on both T1 and T2 images, measuring 9 cm anteroposterior, 13.9 cm craniocaudal, and 13 cm transverse, located in the posterior myometrium. The mass extended superiorly to the epigastrium and anteriorly to the rectus abdominis, encroaching the prevertebral space and psoas muscle, abutting the aorta and common iliac vessels, with preserved fat planes. The features were indicative of a degenerative fibroid [ Figure 1 ]. Magnetic resonance imaging image - T2-weighted fat-saturated - Contrast-enhanced - Sagittal section of the abdomen and pelvis. The red arrow shows enlarged uterus Given that the patient had completed her family, and her symptoms were not being managed effectively with medication, we planned for surgical intervention. Preoperatively, she developed severe anemia with a hemoglobin level of 6.9 g/dL, necessitating transfusion of two packed red blood cells. Furthermore, serum lactate dehydrogenase (S. LDH) was notably elevated at 600 IU/L (114–240 IU/L). Subsequently, she underwent a total abdominal hysterectomy. The surgery was challenging due to adhesions from her previous two cesarean sections and the large size of the uterus. Intraoperative findings included a uterus with a regular surface, increased vascularity on the posterior surface, and a soft consistency [ Figure 2b ]. The initial plan was to conserve the ovaries, which was altered due to bleeding from the left tubo-ovarian pedicle, leading to left salpingo-oophorectomy. The right tube was also partially removed, including the fimbriae end, but a complete salpingectomy could not be performed due to extensive adhesions. (a) Anterior surface of the uterus - Cut section - depicting myohyperplasia of the uterus walls and apparently normal-looking endometrial cavity. (b) Intraoperative picture of the posterior surface of the uterus depicting increased vascularity. (c) Specimen after formalin fixation - Cut section of the posterior wall depicting cysts filled with dark-colored altered blood On gross examination, the uterus displayed myometrial hyperplasia in the anterior wall, while the endometrial cavity appeared normal [ Figure 2a ]. The posterior wall cut section revealed cystic spaces filled with dark altered blood within the posterior myometrium [ Figure 2c ]. Microscopic examination confirmed the presence of adenomyosis with cystic spaces, explaining her persistent symptoms and leading to a definitive diagnosis [ Figure 3 ]. Histopathology section of the uterus - (a) Hematoxylin and eosin stain showing endometrial glands and stroma along with cysts [red arrow] in the myometrium. (b) Magnified view showing cyst with absence of epithelial lining at some places

Cystic adenomyosis is a particularly rare and intriguing variant of adenomyosis, with <50 cases documented in medical literature.[ 1 ] It involves ectopic endometrial glands forming cysts within the myometrium, which bleed cyclically.[ 2 3 4 ] These cysts, 15–30 mm in size, contain chocolate-brown fluid. Symptoms include abnormal uterine bleeding (AUB) such as menorrhagia, severe dysmenorrhea, and chronic pelvic pain.[ 5 6 ] A variant of this is juvenile cystic adenomyosis, seen in younger women/adolescents; it refers to accessory and cystic uterine masses.[ 7 ] Notably, in older women, the condition often involves widespread adenomyosis throughout the myometrium, with cysts typically lacking an epithelial lining also known as diffuse cystic adenomyosis.[ 8 ] This distinct clinical and pathological profile of cystic adenomyosis necessitates a thorough understanding for accurate diagnosis and effective management.

Diffuse cystic adenomyosis typically presents in patients aged 30–45 years.[ 1 6 ] Our patient fits within this age range. She exhibited symptoms of AUB, a palpable abdominal mass, and progressive, severe, dysmenorrhea not responding to medical treatment.[ 2 6 8 ] A significant risk factor in her case was her history of two cesarean sections, consistent with findings in the literature. For instance, Koga et al .[ 9 ] reported a case of giant cystic adenomyosis measuring 17 cm × 11 cm × 8 cm in a 37-year-old female with a history of multiple uterine surgeries, including myomectomy, cesarean section, and hydatidiform mole evacuation, which was also associated with squamous metaplasia. Similarly, Fan et al .[ 5 ] documented a 39-year-old female who developed cystic adenomyosis following myomectomy and experienced worsening symptoms despite medical management. The rarity of this condition complicates diagnosis, often leading to confusion with degenerations in fibroids, as observed in our case. MRI, particularly T1-weighted images, typically shows a brighter cystic lesion compared to T2-weighted images.[ 10 ] Circulating protein biomarkers, including vascular endothelial growth factor, basic fibroblast growth factor, interleukins, cancer antigen 125, S. LDH, and growth differentiation factor 15, are noted in the literature as potential tools for distinguishing between benign and malignant uterine pathologies.[ 11 ] In our case, the only biomarker we were able to assess was S. LDH, which was elevated at 600 IU/L. While an elevated S. LDH is nonspecific, it can be associated with rapidly growing or large uterine masses, which may be indicative of either benign or malignant pathology.[ 11 ] Various management strategies are described in the literature, including high-intensity focused ultrasound radiofrequency ablation, endoscopic resection, and hysterectomy.[ 1 6 ] However, given the size of the lesion in our patient and family completed, we opted for a conventional exploratory laparotomy to ensure complete excision. Previous reports of large uterine masses often suggest malignancy. For example, Ohta et al .[ 12 ] described a 54-year-old female with an 11 cm × 11 cm × 10 cm uterine mass with solid areas in it, which was diagnosed as clear cell adenocarcinoma arising from cystic adenomyosis requiring chemotherapy. Mori et al .[ 13 ] reported a 67-year female with an 11 cm mass initially diagnosed as cystic degeneration of leiomyoma, which on histopathology later revealed to be endometrioid adenocarcinoma arising from cystic adenomyosis, also necessitating chemotherapy. Definitive diagnosis hinges on histopathological examination to distinguish between benign and malignant lesions. In our case, her relatively young age and the absence of solid areas likely contributed to the benign histopathological outcome. The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. There are no conflicts of interest.