Cases
A 32-year-old woman, P2L2A0, previous full-term normal vaginal deliveries, was admitted with progressive distension abdomen and weight loss for 1 year. Her menstrual cycles were regular with normal flow and no dysmenorrhea. There was no history of pain abdomen, fever, urinary or bowel complaints, jaundice, or any previous surgery. Her general physical examination was normal. The abdomen was uniformly distended up to xiphisternum, soft doughy in consistency with palpable multiple intraabdominal firm nodules. The mass was nontender, diffuse margins, and not well defined. On per speculum examination, cervix was normal pointing toward the left and the vagina was normal. On pelvic examination, the uterus was normal size, deviated to left, right fornix was full of nodules, and the left fornix was shortened. On per rectal examination, firm nodules were palpable anteriorly in a pouch of Douglas and rectal mucosa was free. A provisional diagnosis of Koch’s abdomen was made.
On investigations, tumor markers, namely, CA-125, Lactate dehydrogenase (LDH), Alfa feto protein (AFP), human chorionic gonadotropin (hCG), were negative. Ultrasound abdomen showed a large mass of heterogeneous echogenicity occupying the whole abdomen and pelvis, extending up to the epigastrium, and uterus and ovaries were normal. Computed tomography scan of the abdomen and pelvis revealed a large 22.8 cm × 23.2 cm × 15 cm unencapsulated solid, heterogeneous soft tissue mild enhancing lesion, provisionally arising from peritoneum or omentum or mesentery, and reaching up to inferior hepatic margins. There were small cystic changes and areas of necrosis. Mass was abutting the anterior abdominal wall and partially encasing small bowel loops, uterus, adnexa, and urinary bladder. There was mild ascites, diffuse peritoneal thickening, and omental nodularity. A provisional diagnosis of peritoneal mesothelioma or a lymphoma was made. Fine-needle aspiration cytology of mass was inconclusive and showed hemorrhage and few blood vessels.
An exploratory laparotomy was planned in view of the large size of the mass and suspected malignancy under general anesthesia. Intraoperatively, there was a large heterogeneous solid cystic mass measuring 20 cm × 25 cm, attached to the right posterolateral wall of the uterus and adherent to omentum and small-bowel extending retroperitoneally into the leaves of broad ligaments up to the pelvic floor. The uterus was normal size; bilateral tubes and ovary were normal. There were no palpable pelvic and para-aortic nodes. Intraoperative diagnosis of uterine leiomyosarcoma or a degenerated uterine leiomyoma was made. The facility of frozen section was not available at the time of surgery and the decision was taken for hysterectomy along with the removal of the lesion after consent. Total abdominal hysterectomy with complete excision of tumor with ligation of anterior division of right internal iliac artery was performed. The cut section of the uterus revealed normal myometrium and endometrium, and the tumor was a soft-tissue mass with cysts filled with dark altered blood [ Figure 1 ]. Three units of packed red blood cells were transfused, postoperative period was uneventful and she was discharged in stable condition. Gross examination revealed a large mass 15 cm × 18 cm with cystic areas, irregular surface with hemorrhagic contents. It was attached to the uterus with a 2-cm stalk. On microscopic examination, the cyst wall was lined with endometrial glands. There were areas of hemorrhage; chronic inflammation and hemosiderin laden macrophages were also present. There was no evidence of atypia or necrosis. Along with the endometrial tissue, there were focal areas of squamous epithelium with sebaceous glands. Histopathology was reported as extrauterine adenomyosis with elements of mature cystic teratoma. The myometrial sections where the tumor was attached to the uterus did not show any evidence of adenomyosis. On follow-up after 6 months, the patient is asymptomatic and is disease free.
Large mass 15 cm × 18 cm with cystic spaces filled with dark-colored blood attached to the uterus with a 2 cm stalk. Uterus and cervix normal size
Intro
Adenomyosis is a common condition in the reproductive age group and etiopathogenesis of uterine adenomyomas includes invagination of basalis layer of the endometrium into the myometrium or origin from embryologically misplaced pluripotent Mulleria remnants.[ 1 ]
The presence of uterine-like masses outside the uterus or extrauterine adenomyoma is a rare entity and is usually located at the ovary, uterosacral ligaments, and rectovaginal septum.[ 2 ] The exact incidence is not reported and less than 100 cases have been reported in literature. Cases have also reported adenomyomas present at multiple sites in the ileum, colon, peritoneum, and pelvic walls, but these were mainly post morcellation or after surgery for endometriosis or hysterectomy.[ 3 4 ] We describe a unique case which presented as a large abdominal tumor without any previous surgery, with elements of mature teratoma.
Discussion
Extrauterine adenomyoma is a very rare entity and only 66 cases have been reported in literature till date after its first description by Cozzutto in 1981.[ 5 ] Various theories such as mesenchymal metaplasia, proliferation of Mullerian elements, and stem cell differentiation have been proposed. Considering the presence of two elements, in this case, the theory of metaplastic transformation of the subcelomic mesenchyme can be considered appropriate.[ 6 ] The totipotent cells of the subcelomic tissue undergo proliferation and differentiation into endometrial stromal cells, decidua, or smooth muscle under extrogenic stimulus. Batt proposed three criteria for extrauterine adenomyosis including the absence of pelvic endometriosis, no communication of adenomyoma with endocervix, endometrium and endosalpinx, and the absence of surgeries on reproductive organs. All three criteria were met in the present case.[ 7 ]
To our knowledge, this is the first reported case in literature of an extrauterine adenomyoma with coexistent features of a mature cystic teratoma. Another unique feature is the extensive and widespread disease in the absence of any previous surgery.
Differential diagnoses include diffuse leiomyomatosis peritonealis disseminata, sarcoma, and carcinomatosis in such cases of widespread lesions with retroperitoneal extension. Abdominopelvic pain is the most common presentation and there can be the previous history of surgery, endometriosis, or infertility.[ 2 ] Surgical treatment followed by long-term GnRH agonist therapy is the treatment of choice.[ 2 ]
To conclude, this is a rare and interesting case with extensive extrauterine adenomyosis with associated mature teratoma and this entity must be considered in differential diagnosis of unexplained intra-abdominal masses.
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Nil.
There are no conflicts of interest.