|Year : 2018 | Volume
| Issue : 12 | Page : 1209-1212
Uterine tumors resembling ovarian sex-cord tumor: A case report and literature review
Li-li Fan1, Yang Shen1, Kenneth Chanda2, Mu-Lan Ren1
1 Department of Obstetrics and Gynaecology, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009, China
2 Department of Obstetrics and Gynaecology, University Teaching Hospital, University of Zambia, Lusaka, Zambia
|Date of Web Publication||11-Dec-2018|
Department of Obstetrics and Gynecology, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009
Source of Support: None, Conflict of Interest: None
Uterine tumors resembling ovarian sex-cord tumor (UTROSCT) are a rare, multi-phenotype sex-cord tumors, containing a structure which is characteristic with the trabecular, cord, nests, and false adenoid arrangement. In addition, CD99-positive was a basis for diagnosis of the disease. With uncertain malignant potential and relapse, these patients should be closely followed up. This article is to summarize clinical and pathological features, diagnosis and differential diagnosis, treatment, and prognosis of UTROSCT.
Keywords: Clinical, diagnosis, pathology, treatment outcome, uterine tumors resembling ovarian sex-cord tumors
|How to cite this article:|
Fan Ll, Shen Y, Chanda K, Ren ML. Uterine tumors resembling ovarian sex-cord tumor: A case report and literature review. J Can Res Ther 2018;14, Suppl S5:1209-12
| > Introduction|| |
Similar to ovarian sex cord-derived tumors of the uterus, uterine tumors resembling ovarian sex-cord tumors (UTROSCTs) are very similar to some of the sex-cord tumors that occur in the ovary. This tumor was first proposed in 1976 by Clement and Scully. Our hospital has a case of UTROSCT. The clinical characteristics were analyzed. Its pathological features, diagnosis, differential diagnosis, treatment, and prognosis were further analyzed and compared with related literature to raise awareness of the tumor.
| > Clinical Case|| |
The patient was a 62-year-old postmenopausal woman who had a history of irregular, abnormal vaginal bleeding for 26 days. Histopathology of the curettings showed a large number of densely packed small-shaped cells while immunohistochemistry results revealed epithelial malignancies (cancer) with neuroendocrine differentiation.
The patient accepted and underwent a laparoscopic extended hysterectomy with pelvic plus para-aortic lymph node dissection. In addition, she received anti-inflammatory, rehydration and anticoagulation therapy conventionally after surgery. After 2 weeks, the patient improved and was discharged.
| > Pathological Features|| |
Macroscopic features of the whole uterus and bilateral salpingo-oophorectomy specimens
The uterus was 7.5 cm × 6 cm × 5 cm in size with endometrial thickness of 0.15 cm. On section, the mass was soft with clear boundaries and appeared pale yellow. The left ovary was 2 cm × 1 cm × 0.3 cm in size with no obvious abnormalities on section while the left Fallopian tube More Details was 6 cm long, 0.3 cm in diameter, and the fimbrial end was visible. The right ovary was 1.5 cm × 1 cm × 0.3 cm in size with no obvious abnormalities on section while the right fallopian tube measured 6.5 cm long, 0.5 cm in diameter, and the fimbrial end was visible [Figure 1].
Uterine tumor tissue had a trabecular, cord, nests, and false adenoid arrangement [Figure 2], [Figure 3], [Figure 4], [Figure 5]. The tumor cells were polygonal, oval-shaped with abundant eosinophilic cytoplasm. They had a central nucleus which was round or oval with a clear nuclear membrane. The boundary of tumor and surrounding tissue was unclear as both the myometrium and endometrium were affected by tumor invasion. However, endometrial glands surface was intact.
Molecular pathological markers
CD99 (+), Pan CK (2+), estrogen receptor (ER) (+), progesterone receptor (PR) (3+), P53 (1+), CD10 (focal +), Vim (+), WT-1 (part +), PTEN (2+), Syn (x special treatment +), CgA (−), CR (+) [Figure 6], CD56 (3+) [Figure 7], Ki67 (about 10% +), Pax-8 (−), NapsinA (−), inhibin-a (−), SMA (+), EMA (±), Desmin (+), Melan (−).
The uterine tumor had a diameter of 3.8 cm, and immunohistochemistry results showed that it was similar to ovarian sex-cord tumors. Some areas of the tumor grew within the uterine muscle, but there was neither cervical nor neurovascular tissue invasion of the cancer cells. The cervical and uterine margins had no residual tumor though the cervix showed chronic cervicitis. Both ovaries and fallopian tubes had atrophic changes. The sampled lymph nodes showed reactive changes. Therefore, the disease diagnosis, in this case, is UTROSCTs.
| > Follow-Up|| |
The patient was readmitted 20 days after surgery for incomplete ileus. She was on nil per oral, and decompression was done. She received fluid infusion and anti-inflammatory therapy. Tumor markers and pelvic CT scan showed no abnormality. Following insertion of a right ureter double “J” catheter at ureteroscopy, the patient improved and discharged.
| > Discussion|| |
The clinical and pathological features
The tumor often occurs in reproductive and postmenopausal women, and the average age is 45 years old. A common clinical symptom is abnormal vaginal bleeding or lower abdominal discomfort or pain though there are also cases without symptoms. The boundaries of the tumor and surrounding tissues are usually clear. The cut surfaces are fleshy and grey-yellow to white, but necrosis and hemorrhage are unusual.
Microscopically, a UTROSCT displays a variety of architectural patterns, including anastomosing cords of 1-2 cells wide, broad trabeculae, small nests, and sertoliform or retiform tubular structures. Call-Exner-like bodies and diffuse sheets of uniform granulosa cell tumor-like areas are also seen. The intervening stroma shows hyalinization with a sparse lymphocytic infiltrate accompanied by foamy histiocytes, a few multinucleated giant cells, hemosiderin deposition, and/or cholesterol crystals.
Although immunohistochemical markers showed no specific targets, studies have shown that all UTROSCT are positive for at least two kinds of sex-cord-labeled antibodies (CD99, calretinin, melan A, and inhibin) often accompanied by smooth muscle (SMA, desmin, calponin), endometrial stromal (CD10), and other antibodies (vimentin, ER, PR) with varying degrees of expression.
Although UTROSCT is a distinct histopathologic entity, several benign and malignant neoplasms can cause a diagnostic dilemma. Some of these distinctions are of morphologic importance, and others are important from a prognostic standpoint. Endometrioid carcinomas are diffusely immunoreactive for epithelial membrane antigen and ER, whereas calretinin and WT1 are usually negative. However, they usually display areas with architecture and morphology typical of endometrioid carcinoma and behave like the parent tumor in a malignant fashion.
Treatment and prognosis
A UTROSCT is generally discovered only after hysterectomy and most of the patients reported so far were managed with hysterectomy with or without bilateral salpingo-oophorectomy. The UTROSCTs are generally considered tumors of low malignant potential although most of them behave in a benign fashion. These tumors recur in very few cases although to our knowledge, no deaths have been reported. Patients treated with hysterectomy for well-circumscribed tumors with bland features typically have a benign course.
| > Conclusion|| |
The UTROSCTs are a unique group of uterine neoplasms. It is important for the surgical pathologist to recognize this rare entity and differentiate it from other lesions, particularly tumors of intermediate malignant potential through sampling, use of a proper IHC panel, and awareness of the patient's complete clinical picture.
In future, in tumors with atypical histomorphology, novel molecular markers of sex-cord differentiation, such as forkhead box L2 (FOXL2), splicing factor 1 (SF1), dicer 1 and ribonuclease type III (DICER1) may be used to arrive at a definitive diagnosis, and further subclassify the tumor.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| > References|| |
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Uterine tumor resembling ovarian sex cord tumor (UTROSCT) commonly exhibits positivity with sex cord markers FOXL2 and SF-1 but lacks FOXL2 and DICER1 mutations. Int J Gynecol Pathol 2016;35:301-8.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]