Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report with a New Modality of Treatment and Review of the Literature

Vujić, Goran; Mikuš, Mislav; Matak, Luka; Bonevski, Aleksandra; Babić, Ivan; Planinić, Pavao; Babić, Damir; Ćorušić, Ante

doi:10.1055/s-0040-1710350

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Revista Brasileira de Ginecologia e Obstetrícia

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Brasil

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Sumário

Case Report • Rev Bras Ginecol Obstet. 42 (5) • May 2020 • https://doi.org/10.1055/s-0040-1710350 copy

Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report with a New Modality of Treatment and Review of the Literature

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Objective

Desmoplastic small round cell tumor (DSRCT) is a rare intraabdominal neoplasm that grows along serosal surfaces and is primarily found in young men. To Keywords date, only 16 cases of ovarian DSRCT have been previously reported in women in the English literature, and no large population-based studies on this topic exist.

Case Report

We report the case of a 19-year-old virgo with unremarkable past medical history, initially presented with abdominal fullness. After being treated with the optimal treatment modality (primary and secondary surgical debulking, unique chemotherapy, protocol and adjuvant radiotherapy), the patient has remained without tumor disease for 40 months.

Conclusion

Although the best therapy for patients with DSRCT has yet to be determined, combining complete surgical resection, adjuvant chemotherapy, and radiotherapy is required to prolong survival and to achieve proper quality of life.

Keywords:
desmoplastic small; round cell tumor; ovary; surgical debulking; adjuvante; chemotherapy; radiotherapy; immuno-histochemistry

Case (n)	Reference	Age (yrs)	Ovarian involvement	Treatment modalities	Chemotherapy protocol	Follow-up
1	Young et al²⁴24 Young RH, Eichhorn JH, Dickersin GR, Scully RE. Ovarian involvement by the intra-abdominal desmoplastic small round cell tumor with divergent differentiation: a report of three cases. Hum Pathol. 1992;23(04):454-464. Doi: 10.1016/0046-8177(92) 90094-j https://doi.org/10.1016/0046-8177(92) 90...	15	Unknown laterality	PDS +SDS + CHT	Multi-agent protocol including carboplatin	Succumbed at 4 months
2	Young et al²⁴24 Young RH, Eichhorn JH, Dickersin GR, Scully RE. Ovarian involvement by the intra-abdominal desmoplastic small round cell tumor with divergent differentiation: a report of three cases. Hum Pathol. 1992;23(04):454-464. Doi: 10.1016/0046-8177(92) 90094-j https://doi.org/10.1016/0046-8177(92) 90...	15	Bilateral	PDS + SDS	Not used	Unknown
3	Young et al²⁴24 Young RH, Eichhorn JH, Dickersin GR, Scully RE. Ovarian involvement by the intra-abdominal desmoplastic small round cell tumor with divergent differentiation: a report of three cases. Hum Pathol. 1992;23(04):454-464. Doi: 10.1016/0046-8177(92) 90094-j https://doi.org/10.1016/0046-8177(92) 90...	14	Right	PDS	Unknown, ifused	Unknown
4	Zaloudek et al²⁵25 Zaloudek C, Miller TR, Stern JL. Desmoplastic small cell tumor of the ovary: a unique polyphenotypic tumor with an unfavorable prognosis. Int J Gynecol Pathol. 1995;14(03):260-265. Doi: 10.1097/00004347-199507000-00011 https://doi.org/10.1097/00004347-1995070...	22	Bilateral	PDS + CHT	BEP	Succumbed at 18 months
5	Slomovitz et al²⁶26 Slomovitz BM, Girotra M, Aledo A, Saqi A, Soslow RA, Spigland NA, Caputo TA. Desmoplastic small round cell tumor with primary ovarian involvement: case report and review. Gynecol Oncol. 2000;79(01):124-128. Doi: 10.1006/gyno.2000.5829 https://doi.org/10.1006/gyno.2000.5829...	11	Right	PDS + CHT + ASCS	Modified P6 + myeloablative chemotherapy	Succumbed at 11 months
6	Parker et al²⁷27 Parker LP, Duong JL,Wharton JT, Malpica A, Silva EG, Deavers MT. Desmoplastic small round cell tumor: report of a case presenting as a primary ovarian neoplasm. Eur J Gynaecol Oncol. 2002;23(03):199-202	23	Right	PDS + CHT	Platinum and taxol chemotherapy	Unknown
7	Elhajj et al²³23 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(06):760-763. Doi: 10.1046/j.1525-1438.2002.01132.x https://doi.org/10.1046/j.1525-1438.2002...	27	Bilateral	PDS + CHT	Delayed C/E followed by CAV	Succumbed at 42 months
8	Ota et al¹1 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 (02):430-434. Doi: 10.1111/j.1447-0756.2009.01126.x https://doi.org/10.1111/j.1447-0756.2009...	26	Bilateral	PDS + CHT + SDS + RT	P6	Succumbed at 23 months
9	Ota et al¹1 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 (02):430-434. Doi: 10.1111/j.1447-0756.2009.01126.x https://doi.org/10.1111/j.1447-0756.2009...	19	Bilateral	PDS + CHT + SDS	BEP	Succumbed at 11 months
10	Fang et al¹⁴14 Fang X, Rodabaugh K, Penetrante R, Wong M, Wagner T, Sait T, Mhawech-Fauceglia P. Desmoplastic small round cell tumor (DSRCT) with ovarian involvement in 2 young women. Appl Immunohistochem Mol Morphol. 2008;16(01):94-99. Doi: 10.1097/01.pai.0000213158.71934.9c https://doi.org/10.1097/01.pai.000021315...	13	Left	PDS + CHT + RT	BEP	Succumbed at 21 months
11	Fang et al¹⁴14 Fang X, Rodabaugh K, Penetrante R, Wong M, Wagner T, Sait T, Mhawech-Fauceglia P. Desmoplastic small round cell tumor (DSRCT) with ovarian involvement in 2 young women. Appl Immunohistochem Mol Morphol. 2008;16(01):94-99. Doi: 10.1097/01.pai.0000213158.71934.9c https://doi.org/10.1097/01.pai.000021315...	23	Bilateral	PDS + CHT	Myeloablative chemotherapy	Alive at 7 months
12	Engohan- Aloghe et al¹²12 Engohan-Aloghe C, Aubain Sommerhausen N de S, Noël JC. 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(06):579-583. Doi: 10.1097/pgp.0b013e3181aae8dc https://doi.org/10.1097/pgp.0b013e3181aa...	21	Bilateral	PDS + CHT	unknown	Alive at 7 months
13	Nakayama et al²⁸28 Nakayama J, Nassau S, Atkins K, Modesitt SC. Desmoplastic small round cell tumor of the ovary: A rare but devastating disease in young women. Gynecol Oncol Case Rep. 2013;7:16-18. Doi: 10.1016/j.gynor.2013.02.006 https://doi.org/10.1016/j.gynor.2013.02....	6	Bilateral	PDS + CHT + RT + CHT	P6 + IMC-A12 trial + temsirolimus	Succumbed at 28 months
14	Nakayama et al²⁸28 Nakayama J, Nassau S, Atkins K, Modesitt SC. Desmoplastic small round cell tumor of the ovary: A rare but devastating disease in young women. Gynecol Oncol Case Rep. 2013;7:16-18. Doi: 10.1016/j.gynor.2013.02.006 https://doi.org/10.1016/j.gynor.2013.02....	28	Right	CHT + SDS	Neoadjuvant P6 (with the removal of adriamycin) + topotecan after surgery	Succumbed at 40 months
15	Nakayama et al²⁸28 Nakayama J, Nassau S, Atkins K, Modesitt SC. Desmoplastic small round cell tumor of the ovary: A rare but devastating disease in young women. Gynecol Oncol Case Rep. 2013;7:16-18. Doi: 10.1016/j.gynor.2013.02.006 https://doi.org/10.1016/j.gynor.2013.02....	17	Right	PDS + CHT + SDS + RT	P6	Alive at 11 months
16	Xie and Shen²2 Xie YP, Shen YM. Ovarian involvement of a desmoplastic small round cell tumor of unknown primary origin with lymph node and lung metastases: A case report. Oncol Lett. 2016;11(02): 1125-1129. Doi: 10.3892/ol.2015.4012 https://doi.org/10.3892/ol.2015.4012...	30	Right	PDS + CHT	VAC	Alive at 15 months
17	Present case	19	Bilateral	PDS + CHT + SDS + RT + ASCS	CWS + Bu-Mel	Alive at 40months

Reference	Des	AE1/AE3	CK7	Inh	Vim	EMA	NSE	Chr	Actin	WT1	EWS/WT1 (PCR)
1	+ +	+ +	-	N/A	N/A	+ +	N/A	-	-	+	NP
1	+ +	+ +	+	N/A	N/A	+ +	N/A	-	-	+	P
2	+ + (*)	+ +	N/A	-	+ +	+ +	-	N/A	N/A	N/A	N/A
12	+ + (*)	-	N/A	+	N/A	-	+ +	-	+	N/A	P
18	+	N/A	N/A	N/A	+	N/A	+	N/A	+	N/A	N/A
19	+	N/A	N/A	N/A	+	+	+	-	-	N/A	N/A
19	+	+ +	N/A	N/A	+ +	+ +	-	-	+ +	N/A	N/A
19	+	+ +	N/A	N/A	+	+ +	+ +	-	-	N/A	N/A
21	+	+	+	N/A	N/A	N/A	N/A	N/A	N/A	N/A	N/A
23	+	N/A	N/A	N/A	N/A	+	+	N/A	N/A	N/A	P
23	+ +	+ +	+ +	N/A	N/A	N/A	N/A	N/A	N/A	N/A	NP
23	+ +	+ +	+ +	-	N/A	N/A	N/A	N/A	N/A	-	P
Present case	+	+ +	-	+	+ +	+ +	-	-	-	+	P

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[1] Address for correspondence Mislav Mikuš, Department of Obstetrics and Gynecology, University Hospital Center Zagreb, Zagreb, Croatia (e-mail: m.mikus19@gmail.com).