Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 

 Table of Contents  
Year : 2018  |  Volume : 14  |  Issue : 12  |  Page : 1209-1212

Uterine tumors resembling ovarian sex-cord tumor: A case report and literature review

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 Publication11-Dec-2018

Correspondence Address:
Yang Shen
Department of Obstetrics and Gynecology, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.204893

Rights and Permissions
 > Abstract 

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

How to cite this URL:
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 [serial online] 2018 [cited 2020 Feb 19];14:1209-12. Available from: http://www.cancerjournal.net/text.asp?2018/14/12/1209/204893

 > Introduction Top

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.[1] 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 Top

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 Top

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].
Figure 1: The whole uterus and bilateral salpingoophrectomy specimens

Click here to view

Microscopic features

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.
Figure 2: Microscopic features (×100)

Click here to view
Figure 3: Microscopic features (×100)

Click here to view
Figure 4: Microscopic features (×400)

Click here to view
Figure 5: Microscopic features (×400)

Click here to view

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 (−).
Figure 6: CR (+) (×100)

Click here to view
Figure 7: CD56 (+++) (×100)

Click here to view

Pathological diagnosis

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 Top

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 Top

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.[2]

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.[3] 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.[4]

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.[5]

Differential diagnosis

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.[6]

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.[7] 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[8] 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 Top

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.[9]

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 > References Top

Clement PB, Scully RE. Uterine tumors resembling ovarian sex-cord tumors. A clinicopathologic analysis of fourteen cases. Am J Clin Pathol 1976;66:512-25.  Back to cited text no. 1
Pradhan D, Mohanty SK. Uterine tumors resembling ovarian sex cord tumors. Arch Pathol Lab Med 2013;137:1832-6.  Back to cited text no. 2
Hashmi AA, Faridi N, Edhi MM, Khan M. Uterine tumor resembling ovarian sex cord tumor (UTROSCT), case report with literature review. Int Arch Med 2014;7:47.  Back to cited text no. 3
Symmonds RE, Dockerty MB, Pratt JH. Sarcoma and sarcoma-like proliferations of the endometrial stroma. III. Stromal hyperplasia and stromatosis (stromal endometriosis). Am J Obstet Gynecol 1957;73:1054-70.  Back to cited text no. 4
Liu CY, Shen Y, Zhao JG, Qu PP. Clinical experience of uterine tumors resembling ovarian sex cord tumors: A clinicopathological analysis of 6 cases. Int J Clin Exp Pathol 2015;8:4158-64.  Back to cited text no. 5
Liang SX, Patel K, Pearl M, Liu J, Zheng W, Tornos C. Sertoliform endometrioid carcinoma of the endometrium with dual immunophenotypes for epithelial membrane antigen and inhibin alpha: Case report and literature review. Int J Gynecol Pathol 2007;26:291-7.  Back to cited text no. 6
Aziz O, Giles J, Knowles S. Uterine tumours resembling ovarian sex cord tumours: A case report. Cases J 2009;2:55.  Back to cited text no. 7
O'Meara AC, Giger OT, Kurrer M, Schaer G. Case report: Recurrence of a uterine tumor resembling ovarian sex-cord tumor. Gynecol Oncol 2009;114:140-2.  Back to cited text no. 8
Croce S, de Kock L, Boshari T, Hostein I, Velasco V, Foulkes WD, et al. 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.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


Similar in PUBMED
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  >Abstract>Introduction>Clinical Case>Pathological Fea...>Follow-Up>Discussion>Conclusion>Article Figures
  In this article

 Article Access Statistics
    PDF Downloaded89    
    Comments [Add]    

Recommend this journal