A giant symptomatic primary ovarian leiomyoma with prominent necrosis and degenerative changes in a 75-year-old female: a case report.

A 75-year-old gravida 5 para 5 Syrian female presented to the gynecology department with recurrent abdominal pain, a persistent sensation of discomfort and heaviness, intermittent mild vaginal bleeding, and frequent urination. Her body mass index (BMI) was 32.6 kg/m 2 . She denied smoking or alcohol consumption, and she had a family history of hypertension (her medical history was remarkable for well-controlled hypertension on the medications listed in Table  1 ), as well as three cesarean deliveries. Table 1 The patient’s medications at presentation Medication Dosage Valsartan 80 mg once daily Nebivolol 5 mg once daily Furosemide 40 mg once daily Clopidogrel 75 mg once daily The patient’s medications at presentation Her vitals were all within normal ranges, and the physical examination identified a large palpable abdominopelvic mass. Subsequent gynecological examination revealed mild vaginal bleeding. Complete blood count results were all within reference ranges except for red blood cells (3.09 × 10 6 /µL), hemoglobin (8.6 g/dL), and hematocrit (25.8%). In addition, she had mildly impaired renal function (creatinine 1.6 mg/dL), with normal liver enzymes and tumor markers (CA-125 and CA-19-9). Imaging via ultrasound (US) and computed tomography (CT) demonstrated a complex right adnexal mass measuring approximately 24 cm × 15.5 cm with notable cystic components, exerting pressure on the ureters, contributing to bilateral grade II hydronephrosis (Fig.  1 ). Fig. 1 Abdominopelvic computed tomography scan. Sagittal view ( A ) demonstrating the mass with bilateral grade II hydronephrosis (red arrow). Coronal view ( B ) and transverse view ( C ) demonstrating a complex right adnexal mass measuring approximately 24 cm × 15.5 cm (green arrow in B ). The cystic component is evident (blue arrow in C ) Abdominopelvic computed tomography scan. Sagittal view ( A ) demonstrating the mass with bilateral grade II hydronephrosis (red arrow). Coronal view ( B ) and transverse view ( C ) demonstrating a complex right adnexal mass measuring approximately 24 cm × 15.5 cm (green arrow in B ). The cystic component is evident (blue arrow in C ) Unfortunately, magnetic resonance imaging (MRI) was not performed in our case because the device was undergoing maintenance in our hospital, and the patient refused to have an MRI conducted at another hospital owing to financial reasons. Following adequate preoperative assessment, exploratory laparotomy was performed through Pfannenstiel incision under general anesthesia, along with total hysterectomy and bilateral salpingo-oophorectomy. Intraoperative findings included a large right ovarian mass with prominent necrosis, along with mild bowel adhesion and minimal free intraperitoneal fluid (Fig.  2 ). Fig. 2 Postoperative images showing the resected uterus ( A ), and a large complex right adnexal mass with apparent necrosis ( B ) Postoperative images showing the resected uterus ( A ), and a large complex right adnexal mass with apparent necrosis ( B ) A specimen was collected from the free intraperitoneal fluid and was subjected to histopathological examination along with the resected mass and uterus. A smear of the collected fluid showed normal blood elements, with few reactive mesothelial cells, but no atypical or malignant cells were noted. In addition, a microscopic study of the mass revealed findings suggestive of primary OL with no cell atypia (Fig.  3 ). Fig. 3 Histopathological examination of the resected mass with hematoxylin and eosin staining. A 10× view ( A ) and 40× view ( B ) reveal bundles of smooth muscle cells, which are uniform in size and shape and have oval nuclei with intervening collagen along with wide areas of ischemic necrosis, hyalinized transformation, focal areas of hemorrhage, and cystic degeneration ( C ). No cell atypia is noted Histopathological examination of the resected mass with hematoxylin and eosin staining. A 10× view ( A ) and 40× view ( B ) reveal bundles of smooth muscle cells, which are uniform in size and shape and have oval nuclei with intervening collagen along with wide areas of ischemic necrosis, hyalinized transformation, focal areas of hemorrhage, and cystic degeneration ( C ). No cell atypia is noted Subsequent immunohistochemistry (IHC) corroborated the diagnosis, exhibiting intense positivity with α-smooth muscle actins (α-SMA) and diffuse positivity with desmin. The mitotic activity was scant (< 3/10 high-power fields), thereby excluding malignancy, and the diagnosis of primary OL was established. Postoperatively, the creatinine became 1.2 mg/dL, and hydronephrosis regressed to grade I within 48 hours. The patient completed the postoperative course uneventfully, and the follow-up for the next 6 months indicated no further recurrence.

This article is reported in line with the CARE criteria for case reports [ 9 ].

Adnexal masses represent a prevalent gynecological condition that may affect females of any age, although particularly those of reproductive age. These masses can be classified as either physiological or neoplastic, with the majority of adnexal neoplasms being benign [ 1 ]. Although there are many considerable classifications for benign tumors of the ovary, the most predominantly noted benign tumors in patients are ovarian mature cystic teratomas, luteal cysts, benign ovarian epithelial tumors, and ovarian endometriosis [ 1 ]. Primary ovarian leiomyoma (OL) is an exceedingly rare type of extrauterine leiomyoma, with fewer than 100 cases of primary OLs reported in literature. It accounts for approximately 0.5–1% of all benign smooth muscle tumors found in the ovaries. Its development can be traced to several potential origins, as it may arise from the undifferentiated germ cells within the ovarian ligament and stroma, as well as the smooth musculature of hilar vessels and the remnants of the Wolffian body or the stroma associated with endometriosis [ 2 ]. Primary OLs are mostly asymptomatic, unilateral, noncancerous tumors, typically measuring less than 3 cm in size, and tend to be reported in women aged 20–65 years [ 3 , 4 ]. In addition, primary OLs exhibit a higher incidence in premenopausal women compared with postmenopausal women [ 3 ]. These tumors lack distinctive clinical features, intermixing with a variety of differential diagnoses such as leiomyosarcomas and sex cord stromal tumors, such as sclerosing stromal tumor, cellular fibroma, and ovarian fibroma, thereby prompting thorough preoperative and postoperative investigations to avoid confusion with other differential diagnoses [ 3 , 4 ]. In this article, we present a case of a giant primary OL measuring approximately 24 cm in size and exhibiting apparent necrosis with cystic degeneration in a 75-year-old female.

This case underscores the importance of considering ovarian tumors in elderly patients complaining of abdominal pain and heaviness with a palpable mass on physical examination. US and CT assist in demonstrating the extent and the components of the mass, even when MRI is not available. Primary OL diagnoses should not be excluded from patients older than the typical age groups. The postoperative histological evaluation remains irreplaceable in the diagnostic process of primary OLs. Clinical practitioners and histopathologists must remain vigilant regarding the similarity between OLs and ovarian leiomyosarcoma to guide the appropriate management plan. Leiomyosarcoma must be ruled out in cases of large ovarian tumors, particularly in postmenopausal women, relying on the established criteria, irrespective of the benign appearance on microscopic examination.

Primary OL is an exceptionally rare type of extrauterine leiomyoma, often detected incidentally through pelvic examination or during surgical procedures [ 2 , 3 ]. OLs are simultaneously seen with uterine leiomyomas in 78% of cases, which suggests induction by the same hormonal factors [ 4 ]. According to literature, estrogen is believed to contribute to the growth of leiomyomas in general, and some studies suggest that tamoxifen, an adjuvant hormonal therapy used for breast cancer, can induce the proliferation of uterine leiomyomas as well as extrauterine leiomyomas [ 3 , 5 ]. On the contrary, multiparity and progestin-only contraception are believed to reduce the risk of developing leiomyomas [ 6 ]. Our patient was multiparous (gravida 5 para 5). She denied any history of contraceptive medication use, and she had no concomitant uterine leiomyomas. While OLs typically measure less than 3 cm in size [ 3 , 4 ], cases of OLs larger than 3 cm have been documented [ 5 ]. Furthermore, cases of giant OLs larger than 20 cm have rarely been reported in literature [ 5 ]. Consequently, the prevalence of large OLs remains uncertain [ 5 ]. Our patient had a giant OL measuring approximately 24 cm. OLs primarily occur in women aged 20–65 years, though the majority of documented cases have been reported in premenopausal women, while primary OLs in postmenopausal women have been identified in only 16% of cases [ 3 , 4 ]. Our patient was a 75-year-old postmenopausal woman, which is noteworthy. In most instances, OLs are asymptomatic; however, larger OLs have been associated with a variety of symptoms, including a palpable mass in the abdominopelvic region, abdominal pain, ascites, hydronephrosis, hydrothorax, and polymyositis, along with increased levels of CA-125 [ 4 ]. Our patient presented with complaints of recurrent abdominal pain, an incessant sensation of heaviness, and intermittent mild vaginal bleeding. She also reported frequent urination, possibly due to the pressure exerted on the bladder by the large mass. CA-125 levels were within the reference range. Establishing a definitive diagnosis for primary OL is challenging prior to surgical excision, primarily owing to the lack of unique symptoms or distinct findings on imaging [ 4 ]. In addition, OLs are frequently mistaken for other pelvic masses, such as ovarian fibromas and thecomas, ovarian endometriomas, and pedunculated uterine fibroids [ 4 ]. This uncertainty in the clinical picture prompts comprehensive preoperative and postoperative investigations to rule out other differential diagnoses [ 3 , 4 ]. Preoperative imaging modalities such as CT, US, and MRI are very advantageous in the evaluation process of ovarian masses, providing insights into the extent and the components of the mass, and assessing the adjacent organs [ 7 ]. However, the postoperative histological evaluation is irreplaceable to affirm the diagnosis of primary OLs. Remarkably, though abdominopelvic US and CT in our case identified a unilateral complex right adnexal mass measuring approximately 24 cm × 15.5 cm with notable cystic components, our patient had bilateral grade II hydronephrosis, which can be attributed to the large size of the tumor. Surgical management remains the treatment of choice, indicated for symptomatic ovarian masses, or if the ovarian mass exceeds 6 cm in diameter [ 1 ]. Our patient underwent exploratory laparotomy with total hysterectomy and bilateral salpingo-oophorectomy, and the total resected specimen was subjected to histopathological examination for further inspection. On histopathological examination, OLs are characterized by fusiform cells organized into interlacing spindles or bundles. The nuclei are typically oval, with scant mitotic activity and minimal atypia, akin to the features commonly observed in uterine leiomyomas. In addition, in OLs, hyaline degeneration and myxomatous changes might also be evident [ 3 , 4 ]. Histopathological examination of the resected mass revealed bundles of smooth muscle cells, which were uniform in size and shape and had oval nuclei with intervening collagen, along with wide areas of ischemic necrosis, hyalinized transformation, focal areas of hemorrhage, and cystic degeneration. No cell atypia was noted. These findings were suggestive of primary OL. However, IHC tests were crucial to distinguish OL from other ovarian spindle cell tumors. On IHC staining, primary OLs demonstrate intense positivity for α-SMA and widespread positivity for desmin, while fibromas exhibit positivity for α-SMA and negativity or only focal positivity for desmin. On the contrary, thecomas exhibit negativity for α-SMA but are positive for calretinin and α-inhibin [ 3 , 4 ]. IHC staining of the mass in our case revealed intense positivity with α-SMA and diffuse positivity with desmin. Considering the large size of the tumor and the age of our patient, it was essential to rule out malignancy, despite the benign appearance on microscopy, particularly since both OL and leiomyosarcoma demonstrate similar positivity for α-SMA and desmin on IHC analysis [ 4 , 8 ]. For differentiation between OL and leiomyosarcoma, histopathological criteria encompassing the presence of tumor necrosis, cytological atypia, and mitotic activity were established [ 4 ]. Leiomyosarcoma should be considered if two of the following criteria are evident: Tumor necrosis. Moderate-to-severe cytological atypia. High mitotic activity [ 8 ]. Tumor necrosis. Moderate-to-severe cytological atypia. High mitotic activity [ 8 ]. The mass in our patient had prominent ischemic necrosis with cystic degeneration and hyaline transformation, but the mitotic activity was scant (< 3/10 high power fields), and no cell atypia was noted, thereby confirming the diagnosis of primary OLs and excluding malignancy. The patient’s symptoms improved after surgery, and no recurrence was noted during the follow-up of 6 months.