| Abstract|| |
Ovarian fibroma with minor sex cord element (MSCE) is a rare tumor. The increased estrogen production due to the presence of MSCE and/or luteinized thecal cells within fibroma can be a risk factor for endometrial hyperplasia or carcinoma.
Keywords: Luteinized thecal cells, minor sex cord element, ovarian fibroma
|How to cite this article:|
Mathur A, Seth A, Pant L. Ovarian fibroma with luteinized thecal cells and minor sex cord element: A rare case report. Indian J Pathol Microbiol 2018;61:264-7
|How to cite this URL:|
Mathur A, Seth A, Pant L. Ovarian fibroma with luteinized thecal cells and minor sex cord element: A rare case report. Indian J Pathol Microbiol [serial online] 2018 [cited 2021 Mar 4];61:264-7. Available from: https://www.ijpmonline.org/text.asp?2018/61/2/264/230551
| Introduction|| |
Ovarian stromal tumor with minor sex cord element (MSCE) is a rare entity, first described in 1983 by Young and Scully. Fibroma is the most common sex cord–stromal tumor of the ovary accounting for almost 1%–5% of all tumors of the ovary. These tumors are typically nonfunctioning, but the concomitant presence of luteinized thecal cells and/or MSCE cells can cause hyperestrogenic, and in rare cases, androgenic manifestations in these patients. Recognition of these accompanying elements in an otherwise inactive ovarian stromal tumor can help explain various clinical features as well as histopathological endometrial findings in such patients.
We report a case of a 52-year-old female diagnosed to have ovarian fibroma with luteinized thecal cells as well as MSCE cells. The patient had simple hyperplasia of endometrium without atypia and adenomyosis in the myometrium.
| Case Report|| |
A 52-year-old female presented to the Outpatient Department of Gynaecology and Obstetrics with a chief complaint of irregular bleeding per vaginam for 1½ years. Physical examination did not show any feature of hormonal imbalance. Ultrasonography of the abdomen showed uterus size 12.0 cm × 7.0 cm × 4.0 cm and a right-sided tubo-ovarian mass measuring 6.0 cm × 4.0 cm × 2.0 cm. Hysterectomy with right-sided salpingo-oophorectomy was done, and the specimen was sent for histopathological examination.
Specimen of a partially cut open uterus with cervix with the right-sided tube and ovary was received, with the uterus and cervix measuring 11.0 cm × 6.5 cm × 4.0 cm. Cut section of the uterus showed endometrium measuring 16 mm in thickness and tiny foci of hemorrhage in the myometrium. The ovary measured 5.0 cm × 3.5 cm × 2.5 cm and its external surface was smooth and lobulated. Cut section of the ovary was grayish-white and firm, showing whorled appearance [Figure 1]a.
|Figure 1: (a) Gross photograph showing whorled appearance of ovarian mass; (b) composite photomicrograph of tumor showing fibroma with luteinized thecal component (arrow) and minor sex cord element cells (H and E, ×100); (c) fibroma with distinct luteinized thecal cell island (arrow) (H and E, ×400); (d) foci of minor sex cord element cells showing well-formed Sertoli cell tubules (H and E, ×400)|
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Microscopic examination revealed simple hyperplasia of endometrium without atypia and adenomyosis in the myometrium.
Microscopic examination of sections from the ovarian mass showed a well-circumscribed tumor tissue comprising of sheets and interlacing fascicles of elongated spindle-shaped cells having cigar-shaped single nuclei with inconspicuous nucleoli. A varying amount of collagen was present interspersed focally in between these fascicles. Occasional aggregates of polygonal luteinized thecal cells with abundant eosinophilic cytoplasm and single rounded nuclei were seen in between the fibrous stromal element. Furthermore, dispersed among the stromal tumor cells were tiny foci of closely packed cuboidal or columnar cells, arranged mostly as solid or hollow tubules, cords, and at some places, poorly defined nests. These foci occupied <10% of the area of tumor tissue on any slide. The presence of few well-formed hollow tubules and absence of nuclear grooves suggested these foci to be of sex cord element cell type [Figure 1]b, [Figure 1]c, [Figure 1]d.
Reticulin staining showed strong positivity around individual fibroma and thecal cells. The sex cord element cells also showed pericellular reticulin positivity, ruling out these foci to be of granulosa cell origin [Figure 2]c. Immunohistochemistry (IHC) pattern for different cell types is shown in [Table 1] and [Figure 2]a, [Figure 2]b, [Figure 2]d.
|Figure 2: (a) Immunohistochemistry, inhibin cytoplasmic staining in Sertoli cell component (×400); (b) immunohistochemistry, calretinin-intense cytoplasmic staining in Sertoli cell components; weak and focal staining in fibromatous cells (×400); (c) reticulin positivity, encircling individual fibromatous, and Sertoli cell component (×400); (d) immunohistochemistry, cytokeratin focal cytoplasmic staining in Sertoli cell tubules (×400)|
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|Table 1: Immunohistochemistry findings of different components of the tumor*|
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On the basis of histological morphology, special staining, and IHC, the final diagnosis of ovarian fibroma having luteinized thecal cells and MSCE of Sertoliform cell type was made.
The patient is asymptomatic 4 months after surgery.
| Discussion|| |
Young and Scully, in 1983, were the first to describe “Ovarian stromal tumors with MSCEs” as ovarian tumors, predominantly of fibromatous or thecomatous type along with sex cord elements comprising of <10% of the tumor area. Their importance lies in the diagnostic differentiation from other histologically similar tumor and tumor-like conditions such as ovarian fibromatosis, Brenner tumor, adenofibroma, endometrioid carcinoma, and metastatic carcinoid; and in their potential hormonal activity.
Ovarian fibromatosis is characterized by the proliferation of collagen-producing spindle cells that typically surround normal follicular structures, while in ovarian fibroma normal follicular structures are not seen.
Fibromas are shown to be mostly negative or weakly positive for inhibin, while most of them show positivity for calretinin (1+ or 2+)., Sex cord element cells show strong positivity for inhibin and calretinin unlike ovarian epithelial tumors and metastatic carcinoids., Brenner tumor, adenofibroma, and endometrioid carcinoma show EMA positivity, while sex cord stromal tumors are negative for EMA. Sertoli cells More Details tend to be positive for cytokeratin. Our case supports these IHC findings.
The ovarian fibromas are typically inactive. However, fibromas with the presence of luteinized thecal cells have been found to be estrogenic in 50% cases and androgenic in 11% cases. The presence of sex cord elements may also be an additional source of hormone; Sertoli cell tumors have been reported to secrete estrogen., Rare cases of ovarian stromal tumor with MSCE associated with endometrial hyperplasia or carcinoma have been reported in the literature., One case of virilizing ovarian fibrothecoma with MSCE has also been reported in a 13-year-old girl. In our patient, the presence of luteinized thecal cells along with MSCE in ovarian fibroma could explain the source of estrogen hormone leading to endometrial hyperplasia. [Table 2] enumerates similar cases in the literature with salient features of these cases.
|Table 2: Case reports of ovarian fibroma (with or without luteinized thecal cells) with minor sex cord elements|
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In the latest WHO classification scheme for ovarian sex cord–stromal tumors (2014), mixed sex cord–stromal tumors other than Sertoli–Leydig cell type are classified as sex cord–stromal tumors NOS. “Luteinized thecoma” is no more categorized as a separate entity unless associated with “sclerosing peritonitis.” However, as seen in literature and in our case report too, identification of other elements present within fibroma can help explain the clinical presentation of the patient and also assess any further risk of endometrial carcinoma.
| Conclusion|| |
Potential hormone-secreting cells such as luteinized thecal cells and/or MSCEs in an otherwise hormonally inactive fibroma can be a risk factor for endometrial hyperplasia or carcinoma and can also lead to feminizing or androgenic clinical features. Hence, the specific diagnosis of these entities has prognostic significance.
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Conflicts of interest
There are no conflicts of interest.
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Department of Pathology, Kasturba Hospital, MCD (North) Daryaganj, New Delhi
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]
[Table 1], [Table 2]