Desmoplastic small round cell tumor of the ovary: A rare but devastating disease in young women

Nakayama, J.; Nassau, Sarah R.; Atkins, Kristen A.; Modesitt, Susan C.

doi:10.1016/j.gynor.2013.02.006

Cited by 15 publications

(15 citation statements)

References 10 publications

(13 reference statements)

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“…Only 15 cases previously reported in women in the English literature (3). A summary of the 15 cases is presented in Table II ( [2][3][4][5][6][7][8][9][10]. Compared with the present case with the reported cases in Table II, the majority of the characteristics of the present case were similar to the others, including the locations, which were unilateral or bilateral ovarian involved and presented as solid and/or cystic.…”

Section: Discussionmentioning

confidence: 50%

“…The patient in the present study was slightly older than this mean at 31 years old. Only 15 cases previously reported in women in the English literature (3). A summary of the 15 cases is presented in Table II ( [2][3][4][5][6][7][8][9][10].…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, to make a correct diagnosis, a combination of immunohistochemical staining and cytogenetic analysis can be useful and important. The coexpression of cytokeratins, EMA, vimentin, desmin and NSE is a unique immunophenotype of DSCRTs (3,4). By contrast, negative immunohistochemical stains for Myogenin, MyoD1, chromogranin, HMB45 and CD45 can assist in distinguishing DSRCT from the aforementioned tumors.…”

Section: Discussionmentioning

confidence: 99%

“…DSCRT is very uncommon and only 15 cases in the English literature were reported until now (2)(3)(4)(5)(6)(7)(8)(9)(10). The DSRCT primarily affects children and young adults, particularly young men, with a reported male to female ratio of 4:1 (11).…”

Section: Introductionmentioning

confidence: 99%

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Ovarian involvement of a desmoplastic small round cell tumor of unknown primary origin with lymph node and lung metastases: A case report

Xie¹,

Shen

2015

Oncology Letters

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Abstract. Desmoplastic small round cell tumors (DSRCTs) were initially characterized as exhibiting divergent differentiation and were extremely aggressive, belonging to the family of 'small round blue cell tumors'. Due to a male predominance, to date, only 15 cases in women have been reported in the English literature. The present study describes a case of DSRCT in a young woman who initially presented with ovarian masses accompanied with lymph node and lung metastases. A correct diagnosis was reached by combining the hematoxylin and eosin, and immunohistochemical staining results. Following surgery, the patient underwent the chemotherapy and, 3 months later, is in a good condition. The study also provides an overview of this uncommon disease. IntroductionDesmoplastic small round cell tumor (DSRCT) was originally described and reported in a study by Gerald and Rosai in 1989 (1). DSCRT is very uncommon and only 15 cases in the English literature were reported until now (2-10). The DSRCT primarily affects children and young adults, particularly young men, with a reported male to female ratio of 4:1 (11). The initial presenting symptoms are associated with the tumor involvement, such as pain and abdominal distention, however, the majority of the patients present with widespread intra-abdominal and pelvic involvement when first examined. The mass was characterized as exhibiting divergent differentiation and was an extremely aggressive tumor belonging to the family of 'small round blue cell tumors ' (12-15). Despite the aggressive nature of DSRCT, it has low overall survival rates; the overall progression-free 5-year survival rate of patients is 18% (2,16). Since there are no symptoms at the early stage it is very difficult to make correct diagnosis at the early stage. The present study describes a case of DSRCT in a young woman who initially presented with ovarian masses accompanied with lymph node and lung metastases. Case reportClinical manifestation. On November 11, 2013, a 30-year-old female (gravida 3, para 1) initially presented to West China Second University Hospital (Chengdu, China) with abdominal fullness. Pelvic examination revealed bilateral adnexal masses. The patient's past medical history was unremarkable. Transabdominal and transvaginal ultrasonography showed the adnexal masses were irregular, complex and predominantly solid, but partially cystic. Chest, abdominal and pelvic computed tomography (CT) scans demonstrated multiple, bilateral intrapulmonary nodules, bilateral ovarian masses and pleural involvement. The level of the tumor marker serum carbohydrate antigen-125 (normal, <35 U/ml) was slightly increased at 50.8 U/ml.Treatment. The patient underwent an exploratory laparotomy. Intraoperatively, large, irregular, bilateral ovarian masses accompanying metastatic nodules that varied in size adhering to the diaphragm, peritoneum, omentum, stomach surface and uterine surface were observed. Analysis of the frozen sections revealed that the tumor was composed of small and round tumor cells growing in...

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Section: Discussionmentioning

confidence: 50%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Ovarian involvement of a desmoplastic small round cell tumor of unknown primary origin with lymph node and lung metastases: A case report

Xie¹,

Shen

2015

Oncology Letters

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“…Hattori et al suggested that prominent cytoplasmic vacuoles and cohesion in serous effusions of DSRCT may resemble metastatic adenocarcinoma [7]. DSRCT occurs rarely in females and can masquerade ovarian cancer [8]. Zeeshanud-din et al reported two cases of intra-abdominal desmoplastic small cell tumour resembling adenocarcinoma of colon [9].…”

Section: Clinical History and Examinationmentioning

confidence: 99%

Intraabdominal Desmoplastic Small Round Cell Tumour Mimicking Metastatic Adenocarcinoma

Kataria¹

2017

MOJS

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Submit Manuscript | http://medcraveonline.com complaints of something moving in the abdomen for the past two months. On physical examination, there was a palpable lump in the right periumbilical area about 5 x 4 cm in size, which was non tender, mobile, firm in consistency and smooth margins. Rest of abdominal examination was normal. The routine haematological and biochemistry investigations were within normal limits.

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Ovarian Mucinous, Brenner Tumors, and Other Epithelial Tumors

Matrai

Jenkins

Baranov

et al. 2019

Gynecologic and Obstetric Pathology, Volume 2

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Desmoplastic small round cell tumor of the ovary: A rare but devastating disease in young women

Cited by 15 publications

References 10 publications

Ovarian involvement of a desmoplastic small round cell tumor of unknown primary origin with lymph node and lung metastases: A case report

Ovarian involvement of a desmoplastic small round cell tumor of unknown primary origin with lymph node and lung metastases: A case report

Intraabdominal Desmoplastic Small Round Cell Tumour Mimicking Metastatic Adenocarcinoma

Ovarian Mucinous, Brenner Tumors, and Other Epithelial Tumors

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