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CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 1  |  Page : 131-133
Uterine tumor resembling ovarian sex cord tumor: Histomorphological features


1 Department of Histopathology, BLK Superspeciality Hospital, New Delhi, India
2 Department of IVF and Reproductive Medicine, BLK Superspeciality Hospital, New Delhi, India

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Date of Web Publication22-Mar-2018
 

   Abstract 


Uterine tumors resembling ovarian sex cord tumors are rare neoplasms with varied histological and immunophenotypic profile, uncertain histiogenesis and biological behavior. A critical evaluation of histological features is essential for diagnosis and management of these cases.

Keywords: Biological behavior, histological features, immunohistochemical profile, uterine tumors resembling ovarian sex cord tumor

How to cite this article:
Thakur A, Malhotra V, Sachan S, Aggarwal A. Uterine tumor resembling ovarian sex cord tumor: Histomorphological features. Indian J Pathol Microbiol 2018;61:131-3

How to cite this URL:
Thakur A, Malhotra V, Sachan S, Aggarwal A. Uterine tumor resembling ovarian sex cord tumor: Histomorphological features. Indian J Pathol Microbiol [serial online] 2018 [cited 2019 Dec 15];61:131-3. Available from: http://www.ijpmonline.org/text.asp?2018/61/1/131/228187





   Introduction Top


Uterine tumors resembling ovarian sex cord tumors are rare tumors with distinct morphological and immunohistochemical features. Their biological behavior depends on the histological features but still remains uncertain. We report a case of this rare tumor, highlighting the diagnostic features.


   Case Report Top


A 37-year-old-female presented to the hospital with secondary infertility. She had a history of one living child 8 years of age. Her physical examination did not reveal any abnormality. Ultrasound examination showed bulky uterus with endometrial thickness of 8 mm. Two small lesions possibly fibroids measuring 11 and 10 mm on the left wall of lower segment of uterus were seen. Right and left ovary showed small follicles. Patient underwent laparohysteroscopic resection of the lesions.

Microscopic examination showed a neoplasm made up of small- to medium-sized monomorphic cells seen as anastomosing cords, resulting in retiform appearance [Figure 1]a. Individual tumor cells had nucleus with mildly dense chromatin, inconspicuous nucleolus, and scanty cytoplasm. Neoplastic stromal tissue was scanty. Tumor was seen in continuity with endometrium, which showed proliferative glands and normal stromal tissue. Few normal endometrial glands were caught in the tumor. Tumor was invading myometrium. Immunohistochemistry revealed tumor cells to be diffusely positive for Calretinin and CD99 [Figure 1]b and [Figure 1]c. Tumor cells were negative for synaptophysin and inhibin. Focal positivity was seen for cytokeratin, WT-1, and CD117. CD10 positive stromal element was minimal. Ki-67 proliferative index was up to 3/100 cells in focal areas. The tumor was diagnosed as uterine tumor resembling ovarian sex cord tumor (UTROSCT).
Figure 1: (a) Monomorphic tumor cells lining tubular spaces resulting in retiform appearance. Stromal tissue is fibrovascular (H and E, ×200). (b) Positive staining for calretinin (IHC, ×200). (c) Positive staining for CD99 (IHC, ×200)

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   Discussion Top


Clement and Scully in 1976 reported 14 uterine tumors with sex cord differentiation and classified these neoplasms into two subgroups. Group I represented endometrial stromal tumors with focal sex cord-like elements (ESTSCLEs) and Group II with predominant sex cord like morphology, i.e., 50% to 100% and therefore are called UTROSCTs.[1] The term UTROSCTs is now used exclusively for Type II tumors by most of the authors. ESTSCLEs are associated with recurrences and metastasis, whereas UTROSCTs have relatively benign behavior. In 2014, WHO classification Type II tumors are placed in the miscellaneous category of tumors of uterine corpus.[1] They are more common than ESTSCLEs. UTROSCTs present as vaginal bleeding, pain abdomen, or as fibroids on ultrasonography.[2] Our case was being investigated for secondary sterility, when ultrasonography showed two small submucosal polypoidal masses considered to be fibroids.

These tumors can present as polypoidal masses projecting into uterine cavity or intramural or subserosal masses. The size of tumor can range from 2 to 24 cm (mean 6 cm). Cut surface is fleshy. Areas of hemorrhage and necrosis are unusual.[2] Histologically sex cord elements are seen as small cells in anastomosing cords, retiform appearance, tubules, or glandular appearance along with stromal cells showing varying degrees of mitosis replacing endometrium and myometrium. In our case, tumor did not have significant neoplastic stromal elements. Thus, the tumor was UTROSCT.

Immunohistochemistry helps in confirming the nature of the tumor. Tumor cells show positive staining for sex cord markers, i.e., calretinin, CD99, inhibin, and Melan-A. Most of the studies have indicated that all these markers may not be positive. Calretinin is most consistent maker in these tumors. Immunoreactivity for at least one more sex cord marker is required for diagnosis. Besides these, positive staining with epithelial markers such as pancytokeratin, smooth muscle actin, and desmin has also been reported.[3] CD10 positivity is seen in endometrial stromal component. CD117 has also shown positivity in up to 33.3% cases.[2] Estrogen and progesterone receptors are present in tumor cells. Our case besides characteristic morphological features showed positive staining for calretinin and CD99. Tumor cells were negative for inhibin. Only scanty CD10 positive stromal elements were present, thus the tumor was labeled as UTROSCT or Group II tumor without features of ESTSCLE. Proliferative index (Ki-67 labeling) was <3/100 cells, indicating possible benign behavior of the tumor. Extent of CD10 positive morphologically abnormal stromal elements determines the biological behavior of these tumors.

Endometrioid carcinoma with sex cord features, metastatic ovarian sex cord tumor, and epithelial and vascular plexiform leiomyoma have to be differentiated from UTROSCT. Endometrioid carcinoma with sex cord features shows typical areas of endometrioid carcinoma, whereas metastatic ovarian sex cord tumor can be differentiated on clinical and imaging studies. Epithelioid leiomyoma and vascular plexiform leiomyoma have distinctive morphology and immune profile and can be easily differentiated.

UTROSCTs are polyphenotypic neoplasms showing both epithelial and sex cord-like features on ultrastructure.[3] Cytogenetic studies have revealed t(7.17)(p15q21) translocation, resulting in fusion of two normal genes such as JA2FI and SUZ12 in ESTSCLE but are not detected in UTROSCT.[4] Thus, this feature is helpful in differentiating UTROSCT from ESTSCLE. Wang et al.[5] have described t(x: 6)(p22.3; q23.1) and t(4:18)(q 21.1; q21.3) in a case of UTROSCT. Tumor - associated genes bcl2, MALT-1, DCC at 18q21 RAPI at 4q: 21 and a gene related to embryogenesis of gonads such as H-Y regulator gene at xp22.3 have been studied in these tumors.

Tumor is considered to arise from pluripotent uterine mesenchymal cells or endometrial stromal cells with secondary sex cord differentiation. In view of polyphenotypic profile, few reports have suggested origin from uncommitted stem cell.

Aggressive biological behavior correlates with tumor size >10 cm, lymphovascular invasion, presence of solid cell nests, epithelioid morphology, clear cells, and spindle cells.[6] UTROSCT is generally considered to have relatively benign behavior in comparison to ESTSCLE. Recurrences have not been reported on long-term follow-ups.[7] However, few cases even with low proliferative index have been reported to metastasize.[8] Thus, the behavior of these tumors is uncertain and these should be considered as potentially malignant tumors.

The management of UTROSCT is either by hysterectomy or hysteroscopy mass resection by minimally invasive surgery.[7],[9] A good response with gestagen has been observed and a few reports indicate that conservative treatment may be adequate.[9],[10] In view of uncertain behavior of these tumors, patients desirous of fertility conservation are advised to complete family at the earliest so that hysterectomy can be done later. Our patient was unwilling for hysterectomy as she wanted to complete her family.

In view a rarity of this tumor, it is essential that a correct diagnosis is made for proper management of these cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
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
    
2.
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
    
3.
de Leval L, Lim GS, Waltregny D, Oliva E. Diverse phenotypic profile of uterine tumors resembling ovarian sex cord tumors: An immunohistochemical study of 12 cases. Am J Surg Pathol 2010;34:1749-61.  Back to cited text no. 3
    
4.
Staats PN, Garcia JJ, Dias-Santagata DC, Kuhlmann G, Stubbs H, McCluggage WG, et al. Uterine tumors resembling ovarian sex cord tumors (UTROSCT) lack the JAZF1-JJAZ1 translocation frequently seen in endometrial stromal tumors. Am J Surg Pathol 2009;33:1206-12.  Back to cited text no. 4
    
5.
Wang J, Blakey GL, Zhang L, Bane B, Torbenson M, Li S, et al. Uterine tumor resembling ovarian sex cord tumor: Report of a case with t(X; 6)(p22.3;q23.1) and t(4;18)(q21.1;q21.3). Diagn Mol Pathol 2003;12:174-80.  Back to cited text no. 5
    
6.
Blake EA, Sheridan TB, Wang KL, Takiuchi T, Kodama M, Sawada K, et al. Clinical characteristics and outcomes of uterine tumors resembling ovarian sex-cord tumors (UTROSCT): A systematic review of literature. Eur J Obstet Gynecol Reprod Biol 2014;181:163-70.  Back to cited text no. 6
    
7.
Bakula-Zalewska E, Danska-Bidzinska A, Kowalewska M, Piascik A, Nasierowska-Guttmejer A, Bidzinski M, et al. Uterine tumors resembling ovarian sex cord tumors, a clinicopathologic study of six cases. Ann Diagn Pathol 2014;18:329-32.  Back to cited text no. 7
    
8.
Umeda S, Tateno M, Miyagi E, Sakurai K, Tanaka R, Tateishi Y, et al. Uterine tumors resembling ovarian sex cord tumors (UTROSCT) with metastasis: Clinicopathological study of two cases. Int J Clin Exp Pathol 2014;7:1051-9.  Back to cited text no. 8
    
9.
Berretta R, Patrelli TS, Fadda GM, Merisio C, Gramellini D, Nardelli GB, et al. Uterine tumors resembling ovarian sex cord tumors: A case report of conservative management in young women. Int J Gynecol Cancer 2009;19:808-10.  Back to cited text no. 9
    
10.
Blinman P, Tattersall MH. A case of uterine tumour resembling ovarian sex cord tumour responding to second-line, single agent anastrazole. Intern Med J 2009;39:617-9.  Back to cited text no. 10
    

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Correspondence Address:
Veena Malhotra
Department of Histopathology, BLK Hospital, Pusa Road, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_702_16

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