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Year : 2008  |  Volume : 51  |  Issue : 3  |  Page : 445-447
Sclerosing stromal tumor: Unusual histologic features of a rare ovarian tumor


Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

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How to cite this article:
Arora R, Gupta R, Dinda A K. Sclerosing stromal tumor: Unusual histologic features of a rare ovarian tumor. Indian J Pathol Microbiol 2008;51:445-7

How to cite this URL:
Arora R, Gupta R, Dinda A K. Sclerosing stromal tumor: Unusual histologic features of a rare ovarian tumor. Indian J Pathol Microbiol [serial online] 2008 [cited 2019 Dec 11];51:445-7. Available from: http://www.ijpmonline.org/text.asp?2008/51/3/445/42520


Sir,

We are reporting a case of sclerosing stromal tumor of the ovary, as reported by Pai et al, in your journal. [1] A 25-year-old female, mother of two children, presented with complaints of dull aching constant pain in the lower abdomen for the past 3 years. On examination, a left adnexal mass measuring 5 5 cm in size was felt. Ultrasonography (USG) of the abdomen and pelvis revealed a left ovarian mass, 6 5 cm, with heterogeneous echotexture. Scattered echogenic foci with shadows suggestive of calcifications were seen. With a pre-operative diagnosis of left ovarian tumor, the patient underwent exploratory laparotomy and left salpingo-oophorectomy.

The specimen consisted of the left ovary, measuring 7 5 2 cm, with attached  Fallopian tube More Details 6cm in length. The ovary was occupied by a tumor, which was predominantly solid, pale yellow and homogeneous. Few cystic areas were noted and the mass was focally gritty on sectioning. Representative sections showed a tumor composed of hypocellular areas with extensive sclerosis, myxoid change and spiculate calcifications [Figure 1]. Careful search revealed few cellular foci composed of fibroblast-like cells with hemangiopericytoma-like vascular areas. No nuclear pleomorphism or mitotic activity was noted. Immunohistochemically, the tumor cells showed positivity for smooth muscle actin [Figure 2] and CD99 while being negative for desmin, S-100 protein and epithelial membrane antigen. Staining with CD34 highlighted the rich vascularity within the cellular foci. Considering the pathological features, a final diagnosis of sclerosing stromal tumor of the left ovary with extensive calcification was rendered. The patient has been well in 10 months of follow-up at the time of this writing.

Sclerosing stromal tumor (SST) of the ovary is a rare, distinct subtype of sex cord-stromal tumor. Since its initial description, fewer than 100 cases have been reported. [2] Histologically, SSTs reveal a distinctive pseudo lobular pattern of growth with cellular and hypocellular areas. The cellular nodules show a disorganized mixture of fibroblasts, vacuolated luteinized theca-like cells and a rich network of thin-walled vessels. These nodules are separated by densely collagenized or edematous connective tissue. [2] Occasionally, the vacuolated cells may assume a signet-ring like appearance and simulate Krukenberg tumor; however, the cells in the latter contain mucin rather than lipid. [2] Immunohistochemically, the cells of SSTs are positive for inhibin, CD99 and actin; and are negative for desmin, S-100 protein and epithelial markers. [2]

The main differential diagnoses of SST of ovary include other sex cord-stromal tumors like fibroma and thecoma. Other sex cord-stromal tumors, including fibroma, may be differentiated from SST on the basis of histologic findings of lobulation, hemangiopericytoma-like pattern and positivity for actin in SSTs. [2] In the presence of calcification, which is an unusual feature in SST as seen in our case, certain entities like "burned-out" gonadoblastoma, serous epithelial tumors, idiopathic calcification and hyalinized fibroma also need to be considered. "Burned-out" gonadoblastoma shows smooth, rounded calcific masses with scarce or absent tumor cells. This appearance, especially in a dysgenetic gonad, is considered to be suggestive of gonadoblastoma and urges the pathologist to carefully search the tumor cells. [3] Since gonadoblastoma can act as a source of malignant germ cell tumors, bilateral oophorectomy is advocated, [3] unlike unilateral ovariectomy or cystectomy in SSTs. [2] A rare form of serious carcinoma, psammocarcinoma, is composed of numerous psammoma bodies with scant tumor cells showing nuclear atypia and this needs to be excluded in ovaries with extensive calcifications. [4] Extensive idiopathic calcification has been described in one case where both ovaries, though of normal size, were calcified into stony-hard consistency. Microscopy showed numerous spherical calcific foci without accompanying epithelial cells. [5] Ovarian fibromas, rarely, show extensive hyalinization and stromal edema with dystrophic spiculate calcification, [6] similar to that seen in our case. However, careful search in our case yielded the cellular foci with features of SST. The tumor in the present case may represent a late calcifying stage of SST and needs to be recognized to avoid misdiagnosis and inappropriate management.

In conclusion, sclerosing stromal tumor is a rare ovarian neoplasm, which can show extensive dystrophic calcification in unusual cases. Such a phenomenon in this rare tumor should be kept in mind when confronted by a paucicellular hyalinized ovarian tumor with extensive calcification. In such cases, careful examination of multiple sections and immunohistochemical expression of tumor cells help in rendering an accurate diagnosis.

 
   References Top

1.Pai RR, Shaktawat SS, Khadilkar UN, Lobo FD, Rao VS, Philipose R. Sclerosing stromal tumor of the ovary: A clinicopathologic spectrum. Indian J Pathol Microbiol 2005;48:370-2.  Back to cited text no. 1    
2.Chang W, Oiseth SJ, Orentlicher R, Agarwal G, Yahr LJ, Cayten CG. Bilateral sclerosing stromal tumor of the ovaries in a premenarchal girl. Gynecol Oncol 2006;101:342-5.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Scully RE. Gonadoblastoma: A gonadal tumor related to dysgerminoma (seminoma) and capable of sex hormone production. Cancer 1953;6:455.  Back to cited text no. 3    
4.Powell JL, McDonald TJ, White WC. Serous psammocarcinoma of the ovary. South Med J 1998;91:477-80.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Clement PB, Cooney TP. Idiopathic calcification of the ovarian stroma. Arch Pathol Lab Med 1992;116:204-5.  Back to cited text no. 5  [PUBMED]  
6.Thecoma-fibroma group of tumors. In : Russell P, Bannatyne P, editors. Surgical Pathology of the ovaries. Melbourne: Churchill Livingstone; 1989. p. 343.  Back to cited text no. 6    

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Correspondence Address:
Ruchika Gupta
162, Pocket-B, Sarita Vihar, New Delhi - 110 076
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.42520

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