Uterine adenosarcoma: a case report and review of the literature.

Am J Nucl Med Mol Imaging 2023;13(2):70-76 www.ajnmmi.us /ISSN:2160-8407/ajnmmi0149134 Case Report Uterine adenosarcoma: a case report and review of the literature Qiyue Wang*, Si Sun*, Jing Cai, Lu Yang, Gang Lv, Qiang Yang Department of Obstetrics and Gynecology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei, P. R. China. *Equal contributors. Received January 14, 2023; Accepted April 25, 2023; Epub April 25, 2023; Published April 30, 2023 Abstract: Uterine adenosarcoma is a rare gynecological malignancy with no specific symptoms, and the optimal management is still inconclusive. Herein we present a case of uterine adenosarcoma in a 38-year-old woman with a good prognosis and review of literatures. The patient presented with abnormal vaginal bleeding with no special medical history. Sonographic scan revealed a heterogeneous echoic mass in the cavity, indicating a polypus or a submucous myoma. The pathology based on the specimen after the hysteroscopic tumor excision suggested diagnosis of uterine adenosarcoma. Subsequently, the patient received pelvic MRI scan before surgery. MRI identified a patchy lesion at the cervix-lower endometrial cavity with low signal in T1WI and a mixed high T2 signal in T2WI, with no sign of metastasis. Then total abdominal hysterectomy with bilateral salpingo-oopherectomy plus pelvic lymph node dissection was performed and 6 cycles of chemotherapy were administered. The patient remains disease-free on follow-up to date, more than 15 months after chemotherapy. Keywords: Müllerian adenosarcoma, sarcoma, uterine cancers, gynecological malignancy, Müllerian malignancy Introduction Uterine adenosarcoma is a rare malignancy, accounts for 5%-10% of all uterine sarcomas [1, 2]. It was first reported by Clement and Scully in 1974, characterized by components of both sarcoma and benign epithelial glands [3]. The morbidity was hard to estimate worldwide but it only has an age-adjusted incidence of 2-3 per 1,000,000 in the US population [4]. Due to the rarity and the inconsistent of clinical management, here we present a case of uterine adenosarcoma in a 38-year-old woman with a good prognosis. Case A 38-year-old woman presented with abnormal vaginal bleeding for 1 year and a prolonged 10-day menstruation compared to the original 3-5 days with no other complaints such as menorrhagia, watery discharge, or abdominal fullness etc. She had no other medical history but caesarean section for one childbirth and she took no specific medications. The patient did not go to the clinic in the past year until her last menstrual period lasted for 15 days. In the outpatient department, no significant signs were found during the pelvic exam while transvaginal 3D ultrasound scan revealed a heterogeneous echoic mass, 3.8*2.2*2.1 cm in size with a cystic region of approximately 1.6*1.5*1.3 cm, started from the uterine cavity and descended to the cervical canal. An endometrial polypus or a submucous myoma was suspected, the patient was admitted and hysteroscopy was performed. During the hysteroscopy, the endocervical canal was visually normal, but from the middle of endometrial cavity to the internal os, a soft, partly cystic, sessile mass about 4*2*2 cm in size was seen. It was difficult to specify whether the mass was a submucous myoma with cystic degeneration or an endometrial polypus intraoperatively, so a hysteroscopic tumor excision was done. The pathological examination found that the tumor was composed of benign glandular elements and malignant stromal components. The epithelial glands were papillary or slit-like while the stro- A case report of uterine adenosarcoma Figure 1. The tumor was composed of benign glandular elements and malignant stromal components. A. The slitlike epithelial glands were widely distributed in the stroma. B. The stromal cells forming intraglandular protrusions (blue arrow) and peri-glandular cuffs around the glands (black arrow). mal cells were spindle-shaped, with increased mitotic activity. The stromal cells formed intraglandular protrusions and in some areas, formed peri-glandular cuffs around the glands, which was the typical microscopic characteristic of adenosarcoma (Figure 1). The final pathological diagnosis was uterine adenosarcoma, with the malignant stromal component was low grade endometrial stromal sarcoma. In order to understand the characteristics of the tumor and whether there was myometrial invasion or distant metastasis, pelvic Magnetic Resonance Image (MRI) with contrast enhanced was performed before reoperation. It can be observed a patchy lesion at the cervixlower endometrial cavity with low signal in T1 Weighted Image (T1WI) and a mixed high T2 signal in T2 Weighted Image (T2WI). No obvious diffusion limitation was observed in Diffusion Weighted Image (DWI), and uneven tissue enhancement was shown in T1WI with contrastenhanced (Figure 2). There were no obvious enlarged lymph nodes in pelvic cavity and no sign of myometrial invasion on MRI. cervix-lower uterus. The tumor is mainly exogenous, with focal invasion to the superficial myometrium (<½ myometrial thickness), and no lymphovascular space invasion (LVSI), no involvement on surgical margins, bilateral parametria, bilateral uterine horns, bilateral fallopian tubes and ovaries, and 24 resected lymph nodes were negative. Given that the risk of recurrence increased while the presence of myometrial invasion, we conducted 6 cycles of chemotherapy, with regimen of pegylated liposomal doxorubicin and carboplatin. The patient has been followed up for more than 15 months after chemotherapy, and has no signs of recurrence so far. Discussion Uterine adenosarcoma is a biphasic neoplasm consisting of benign or occasionally atypical glandular component and malignant stromal component. It accounts for about 5%-10% of uterine sarcomas, while uterine sarcomas are only 1% of all gynecological malignancies and 3% of all uterine cancers [1, 2, 4, 5]. Macroscopic examination found a protruding adenosarcoma sized 1.8*1.2*0.8 cm at the Uterine adenosarcoma occurs more frequently in postmenopausal women. According to three retrospective analyses, the median ages of patients diagnosed with uterine adenosarcoma were 54 [6], 56 [7] and 58 [8] respectively, ranging from 14 to 89 years old. Of 544 cases from the SEERs database, 51.5% of the patients were between 40 and 65 years old, and less than 10% were younger than 40 years old 71 Am J Nucl Med Mol Imaging 2023;13(2):70-76 A subsequent laparoscopy was scheduled. There were no visible tumor and no enlarged lymph nodes detected in the surgery, then total abdominal hysterectomy with bilateral salpingo-oopherectomy (TAH with BSO) and pelvic lymphadenectomy was performed. A case report of uterine adenosarcoma Figure 2. MRI revealed a patchy lesion (arrow) of 17*9 mm at the cervix-lower endometrial cavity. A. A mixed high T2 signal in T2WI. B. Uneven tissue enhanceme (...truncated)