Article Data

  • Views 278
  • Dowloads 115

Case Reports

Open Access

Desmoplastic small round cell tumor (DSRCT) arising in the ovary: report of a case diagnosed at an early stage and review of the literature

  • G. D’Ippolito1,2
  • M.T. Huizing3
  • W.A.A. TJALMA2,*,

1Department of Obstetrics and Gynecology, University of Modena and Reggio Emilia, Modena, Italy

2Department of Gynecological Oncology, B-2650 Edegem, Belgium

3Department of Medical Oncology, University Multidisciplinary Breast and Pelvic Gynecological Oncology Clinic, Antwerp University Hospital, Edegem, Belgium

DOI: 10.12892/ejgo20120196 Vol.33,Issue 1,January 2012 pp.96-100

Published: 10 January 2012

*Corresponding Author(s): W.A.A. TJALMA E-mail: wiebren.tjalma@uza.be

Abstract

Background: Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma tumor affecting mainly young adult males. It rarely has an ovarian involvement. Case: A 29-year-old woman presented to her gynecologist for amenorrhoea. The laboratory results demonstrated a menopausal status and the ultrasound revealed a large mass of the right ovary. The right ovary was completely removed by laparoscopy. Pathology, cytology and immunochemistry revealed a DSRCT. In January 2009 a left salpingo-oophorectomy and a right salpingectomy were performed via laparoscopy. After 35 months from diagnosis there was no clinical evidence of disease recurrence. Conclusion: DSRCT is a rare ovarian tumor in adolescence with a general poor outcome. Every ovarian mass regardless of age should be approached with caution.

Keywords

Desmoplastic small round cell tumor; Ovarian neoplasm; Early stage tumor; Treatment; Good response

Cite and Share

G. D’Ippolito,M.T. Huizing,W.A.A. TJALMA. Desmoplastic small round cell tumor (DSRCT) arising in the ovary: report of a case diagnosed at an early stage and review of the literature. European Journal of Gynaecological Oncology. 2012. 33(1);96-100.

References

[1] Hill D.A., Pfeifer J.D., Marley E.F., Dehner L.P., Humphrey P.A., Zhu X., Swanson P.E.: “Laboratory of Surgical Pathology, Washington University Medical Center, St Louis, MO, USA. WT1 staining reliably differentiates desmoplastic small round cell tumor from Ewing sarcoma/primitive neuroectodermal tumor. An immunohistochemical and molecular diagnostic study”. Am. J. Clin. Pathol., 2000, 114, 345.

[2] Sesterhenn I., Davis C.J., Mostofi F.K.: “Undifferentiated malignant epithelial tumors involving serosal surfaces of scrotum and abdomen in young males”. J. Urol., 1987, 137, 241 A.

[3] Lal D.R., Su W.T., Wolden S.L., Loh K.C., Modak S., La Quaglia M.P.: “Results of multimodal treatment for desmoplastic small round cell tumors”. J. Pediatr. Surg., 2005, 40, 251.

[4] Ordonez N.G.: “Desmoplastic small round cell tumors: I. A histopathologic study of 39 cases with emphasis on unusual patterns”. Am. J. Surg. Pathol., 1998, 22, 1303.

[5] Young R.H., Eichhorn J.H., Richard Dickersin G., Scully R.E.: “Ovarian involvement by the intra-abdominal desmoplastic small round cell tumor with divergent differentiation. A report of three cases”. Hum. Pathol., 1992, 23, 454.

[6] Zaloudek C., Miller T.R., Stern J.L.: “Desmoplastic small round cell tumor of the ovary: a unique polyphenotypic tumor with an unfavorable prognosis”. Int. J. Gynecol. Pathol., 1995, 14, 260.

[7] Slomovitz B.M., Girotra M., Aledo A., Sagi A., Soslow R.A., Spigland N.A., Caputo T.A.: “Desmoplastic small round cell tumor with primary ovarian involvement. Case report and review”. Gynecol. Oncol., 2000, 79, 124.

[8] Elhajj M., Mazurka J., Daya D.: “Desmoplastic small round cell tumor presenting in the ovaries: Report of a case and review of the literature”. Int. J. Gynecol. Cancer, 2002, 12, 760.

[9] Parker L.P., Duong J.L., Wharton J.T., Malpica A., Silva E.G., Deavers M.T.: “Desmoplastic small round cell tumor: report of a case presenting as a primary ovarian neoplasm”. Eur. J. Gynaecol. Oncol., 2002, 23, 199.

[10] Fang X., Rodabaugh K., Penetrante R., Wong M., Wagner T., Sait S., Mhawech-Fauceglia P.: “Desmoplastic small round cell tumor (DSRCT) with ovarian involvement in 2 young women”. Appl. Immunohistochem. Mol. Morphol., 2008, 16, 94.

[11] Engohan-Aloghe C., Aubain Sommerhausen Nde S., Noël J.C.: “Ovarian involvement by desmoplastic small round cell tumor with leydig cell hyperplasia showing an unusual immunophenotype (cytokeratin negative, calretinin and inhibin positive) mimicking poorly differentiated sertoli leydig cell tumor”. Int. J. Gynecol. Pathol., 2009, 28, 579.

[12] Ota S., Ushijima K., Fujiyoshi N., Fujimoto T., Hayashi R., Murakami F. et al.: “Desmoplastic small round cell tumor in the ovary: Report of two cases and literature review”. J. Obstet. Gynaecol. Res., 2010, 36, 430.

[13] Chang F.: “Desmoplastic small round cell tumors: cytologic, histologic, and immunohistochemical features”. Arch. Pathol. Lab. Med., 2006, 130, 728.

[14] Li H., Smolen G.A., Beers L.F., Xia L., Gerald W., Wang J. et al.: “Adenosine transporter ENT4 is a direct target of EWS/WT1 translocation product and is highly expressed in desmoplastic small round cell tumor”. PLoS One, 3, e2353.

[15] Sang Hwa Lee, Wan Seop Kim, Ji Hoon Kim, Hye Seung Han, So Dug Lim, Sang Yoon Kim, Tae Sook Hwang: “Desmoplastic small round cell tumor with ovarian involvement - a case report”. Korean J. Pathol., 2009, 43, 185.

[16] Ordóñez N.G., Sahin A.A.: “CA 125 production in desmoplastic small round cell tumor: report of a case with elevated serum levels and prominent signet ring morphology”. Hum. Pathol., 1998, 29, 294.

[17] Doros L., Kaste S.C., Rodriguez-Galindo C.: “Sister Mary Joseph’s nodule as presenting sign of a desmoplastic small round cell tumor”. Pediatr. Blood Cancer, 2008 eb, 50, 388.

[18] Albano E.A., Kanter J.: “Images in clinical medicine. Sister Mary Joseph’s nodule”. N. Engl. J. Med., 2005, 352, 1913.

[19] Schwarz R.E., Gerald W.L., Kushner B.H., Coit D.G., Brennan M.F., La Quaglia M.P.: “Desmoplastic small round cell tumors: prognostic indicators and results of surgical management”. Ann. Surg. Oncol., 1998, 5, 416.

[20] Church D.N., Bailey J., Hughes J., Williams C.J.: “Desmoplastic small round cell tumor: obstetric and gynaecological presentations”. Gynecol. Oncol., 2006, 102, 583.

[21] De Lena M., Caruso M.L., Marzullo F. et al.: “Complete response to chemotherapy in intra-abdominal desmoplastic small round cell carcinoma. A case report”. Tumori, 1998, 84, 412.

[22] Kushner B.H., LaQuaglia M.P., Wollner N., Meyers P.A., Lindsley K.L., Ghavimi F. et al.: “Desmoplastic small round-cell tumor: Prolonged progression-free survival with aggressive multimodality therapy”. J. Clin. Oncol., 1996, 14, 1526.

[23] Ordóñez N.G.. “Desmoplastic small round cell tumor: I: a histopathologic study of 39 cases with emphasis on unusual histological patterns”. Am. J. Surg. Pathol., 1998, 22, 1303.

[24] Quaglia M.P., Brennan M.F.: “The clinical approach to desmoplastic small round cell tumor”. Surg. Oncol., 2000, 9, 77.

Abstracted / indexed in

Science Citation Index Expanded (SciSearch) Created as SCI in 1964, Science Citation Index Expanded now indexes over 9,500 of the world’s most impactful journals across 178 scientific disciplines. More than 53 million records and 1.18 billion cited references date back from 1900 to present.

Biological Abstracts Easily discover critical journal coverage of the life sciences with Biological Abstracts, produced by the Web of Science Group, with topics ranging from botany to microbiology to pharmacology. Including BIOSIS indexing and MeSH terms, specialized indexing in Biological Abstracts helps you to discover more accurate, context-sensitive results.

Google Scholar Google Scholar is a freely accessible web search engine that indexes the full text or metadata of scholarly literature across an array of publishing formats and disciplines.

JournalSeek Genamics JournalSeek is the largest completely categorized database of freely available journal information available on the internet. The database presently contains 39226 titles. Journal information includes the description (aims and scope), journal abbreviation, journal homepage link, subject category and ISSN.

Current Contents - Clinical Medicine Current Contents - Clinical Medicine provides easy access to complete tables of contents, abstracts, bibliographic information and all other significant items in recently published issues from over 1,000 leading journals in clinical medicine.

BIOSIS Previews BIOSIS Previews is an English-language, bibliographic database service, with abstracts and citation indexing. It is part of Clarivate Analytics Web of Science suite. BIOSIS Previews indexes data from 1926 to the present.

Journal Citation Reports/Science Edition Journal Citation Reports/Science Edition aims to evaluate a journal’s value from multiple perspectives including the journal impact factor, descriptive data about a journal’s open access content as well as contributing authors, and provide readers a transparent and publisher-neutral data & statistics information about the journal.

Submission Turnaround Time

Conferences

Top