A Rare Variant of Ovarian Fibroma With Minor Sex Cord Elements: A Unique Expression of Stromal Differentiation.

A 56‐year‐old postmenopausal woman presented with a history of intermittent postmenopausal bleeding and lower abdominal discomfort. Her obstetric history included primary infertility for 20 years. Menarche had occurred at the age of 14 with a normal 21–35 days regular cycle. Clinical examination revealed no palpable abdominal mass.

Saira Rathore: conceptualization, data curation, visualization, writing – original draft, writing – review and editing. Kanwal Babar: writing – original draft, writing – review and editing. Noreen Javaid: writing – original draft, writing – review and editing. Aalaa Saleh: writing – original draft, writing – review and editing.

Ethical approval was not required because it was a case report.

The authors have nothing to report.

Tumors of this type, including fibromas, are generally considered to have benign behavior and favorable outcomes. After 2 months follow‐up of surgery, our patient remains healthy and free of symptoms.

Ovarian stromal tumors with minor sex cord elements are exceptionally uncommon. This type of tumor was initially identified by Young and Scully, who described it as primarily fibromatous or thecomatous neoplasms with scattered foci of sex cord elements occupying less than 10% of the tumor volume [ 1 ]. Histologically, these minor components typically appear as small nests, cords, or tubules of cells resembling granulosa cells, Sertoli‐like cells, or undifferentiated sex cord elements [ 2 , 4 ]. Additionally, the individual clusters of these elements should not exceed 0.45 mm in size [ 5 ]. According to available literature, only 20 cases of ovarian fibroma with minor sex cord differentiation have been reported globally to date [ 3 , 6 ]. We report the 21st case of ovarian fibroma with minor sex cord‐like elements, along with a review of literature. The average age at presentation ranges from 16 to 70 years, with a median age of 59 years [ 4 , 7 ]. The most frequently reported clinical manifestations include abdominal pain, abnormal vaginal bleeding, postmenopausal bleeding [ 5 ], and the presence of an adnexal mass [ 1 , 7 ]. In most reported cases, patients have a history of live births [ 8 , 9 ]. In contrast, our patient had an obstetric history of primary infertility for 20 years. The ovarian pathology identified in the present case may represent a possible contributing factor to her infertility which may be unrelated to the current condition given the limited available data. To the best of our knowledge, this is the first reported case in association with infertility, indicating a potentially relevant and noteworthy clinical correlation. Ultrasonographic findings in reported cases have included a unilateral solid adnexal mass, increased endometrial thickness, and the presence of an endometrial polyp or adenomyosis [ 5 , 9 ]. The adnexa, particularly the ovary, is the most common site of presentation. Bilateral ovarian involvement and extraovarian occurrence are exceptionally rare. Only one case of bilateral ovarian fibromas containing minor sex cord elements [ 10 ] and one case from the sigmoid colon [ 11 ] are reported. In our case, imaging demonstrated additional findings of endometrial polyp and intramural fibroid. The significance of identifying minor sex cord elements lies in the potential diagnostic challenges they present. Importantly, such elements may be hormonally active, particularly capable of estrogen secretion, which can lead to endometrial stimulation and subsequent pathological changes including endometrial hyperplasia without or with atypia or even endometrial carcinoma [ 6 , 12 ]. Most cases reported in the literature have exhibited estrogenic effects, though only two have been associated with virilization [ 13 , 14 ]. In the present case, the tumor demonstrated estrogenic activity, as clinically evidenced by postmenopausal bleeding and endometrial polyp, although hormonal assays were within normal limits. These minor sex cord components may occasionally be misinterpreted as other ovarian neoplasms, such as Brenner tumor, thecoma, adenofibroma, adult granulosa cell tumor, other sex cord‐stromal tumors, or fibrosarcoma [ 9 , 15 ]. These tumors are close histological differentials to our case entity both clinically as well as microscopically. However, sex cord elements can be reliably identified by their strong immunoreactivity for inhibin and calretinin. Accurate diagnosis requires meticulous histopathological examination to identify these minor components. Reporting such cases contributes to better understanding of their clinical behavior, hormonal activity, and implications for follow‐up, especially in postmenopausal women.

Written informed consent was taken from the patient for this case report prior to publishing it. Final manuscript was reviewed by all the authors, and consent was taken before publishing it.

Transabdominal ultrasonography revealed a well‐defined intramural fibroid in the uterus, an endometrial polyp on the left side of the endometrial cavity, and a left adnexal mass measuring 30 × 30 × 25 mm. Serum tumor markers including CA‐125, carcinoembryonic antigen (CEA), and inhibin were all within normal limits. The patient subsequently underwent a total abdominal hysterectomy with bilateral salpingo‐oophorectomy. The surgical specimen was submitted for histopathological evaluation. On gross examination, the uterus with cervix measured 90 × 70 × 50 mm, bilateral fallopian tubes measured 40 mm in length, left ovary measured 35 × 35 × 20 mm and right ovary measured 20 × 20 × 10 mm. Serial slicing of the uterus reveals an endometrial polyp (15 × 5 mm) and intramural fibroid (30 × 25 × 25 mm) having a firm, whorled white cut surface. The left ovary was entirely replaced by a solid, firm, white mass with a whorled cut surface. The right ovary and fallopian tubes were unremarkable. Microscopic examination of the left ovarian mass revealed features characteristic of a spindle cell neoplasm. The tumor was predominantly composed of intersecting fascicles of bland spindle cells, consistent with an ovarian fibroma (Figure  1 ). Scattered within the fibrous stroma were seen small nests and cords of cells exhibiting sex cord‐like differentiation, resembling granulosa or Sertoli cells, accounting for less than 10% of the tumor volume. These cells displayed uniform round to oval nuclei with scant cytoplasm. No evidence of nuclear atypia or mitotic activity was observed (Figure  2 ). (A) Fibroma with intersecting fascicles of spindle cells and minor component of sex cord tubules in the lower part of image. (B) Ovarian fibroma showing minor sex cord elements comprising of tubular structures lined by Sertoli cells. (A) SMA expression in spindle cells of ovarian fibroma, sparing the sex cord component. (B) Sex cord component highlighted by Inhibin. Extensive sampling of the tumor was performed to exclude any malignant component, but no features of malignancy were identified. Immunohistochemical findings supported the diagnosis: the sex cord elements were positive for calretinin and inhibin, while the spindle cell component demonstrated positivity for smooth muscle actin (SMA) (Figure  3 ). (A) Inhibin highlighting sex cord tubules (B) Negative CK expression in sex cord tubules.

Ovarian stromal tumors containing minor sex cord components are an uncommon variant within the broader category of sex cord‐stromal neoplasms. First described by Young and Scully in 1983, these tumors predominantly consist of fibroma or thecoma‐like tissue, with less than 10% of the tumor volume made up of scattered sex cord elements [ 1 ]. To date, approximately 20 such cases have been documented in the literature [ 1 , 2 ]. Their clinical significance lies in the potential for hormonal activity and the difficulty in preoperative diagnosis, as imaging and clinical findings are often nonspecific. Histopathological examination remains essential for accurate identification, given the subtle presence of sex cord elements. While often hormonally inactive, some cases may exhibit estrogen‐producing sex cord elements such as granulosa or Sertoli‐like cells, potentially leading to endometrial changes like hyperplasia, polyp, or carcinoma [ 3 ]. We present the case of a 56‐year‐old postmenopausal woman with an incidentally detected pelvic mass, subsequently diagnosed as an ovarian fibroma with minor sex cord elements following surgical excision. This case is presented due to its rarity and typically silent clinical presentation.

The authors declare no conflicts of interest.