Desmoplastic Small Round Cell Tumor (DSRCT) With Ovarian Involvement in 2 Young Women

Author(s):  
Xueping Fang ◽  
Kerry Rodabaugh ◽  
Remedios Penetrante ◽  
Michael Wong ◽  
Timothy Wagner ◽  
...  
2016 ◽  
Vol 8 (3) ◽  
pp. 239-242
Author(s):  
K Harish ◽  
G Nandini ◽  
K Padma ◽  
ACV Swamy ◽  
Murali Subramanian

ABSTRACT Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm. It is primarily found in young men, with a reported male to female ratio of 4:1. The tumor typically develops in the abdominal cavity, invading the omentum with multiple peritoneal implants involving the diaphragm, splenic hilum, mesentery of the small and large bowel, and the pelvic peritoneum. Most of them have widespread disease at presentation, with an organ of origin difficult to ascertain. We report a case of desmoplastic round cell tumor in a 17-year-old teenage girl. Immunohistochemistry helped in the diagnosis. She received multimodality treatment. How to cite this article Nandini G, Harish K, Swamy ACV, Subramanian M, Padma K. Desmoplastic Small Round Cell Tumor: A Rare but Aggressive Tumor in Young Women. J South Asian Feder Obst Gynae 2016;8(3):239-242.


2002 ◽  
Vol 12 (6) ◽  
pp. 760-763 ◽  
Author(s):  
M. Elhajj ◽  
J. Mazurka ◽  
D. Daya

Desmoplastic small round cell tumor is a recently recognized clinical entity with specific morphologic, immunocytochemical, and genetic features. Though this tumor is mostly described to involve serosal surfaces, we report a case with ovarian involvement. The clinical presentation and differential diagnoses as well as the treatment including aggressive surgical debulking and multiagent chemotherapy are discussed.


1992 ◽  
Vol 23 (4) ◽  
pp. 454-464 ◽  
Author(s):  
Robert H. Young ◽  
John H. Eichhorn ◽  
G. Richard Dickersin ◽  
Robert E. Scully

2014 ◽  
Vol 7 ◽  
pp. 16-18 ◽  
Author(s):  
John Nakayama ◽  
Sarah Nassau ◽  
Kristen Atkins ◽  
Susan C. Modesitt

2000 ◽  
Vol 79 (1) ◽  
pp. 124-128 ◽  
Author(s):  
Brian M. Slomovitz ◽  
Monica Girotra ◽  
Alexander Aledo ◽  
Anjali Saqi ◽  
Robert A. Soslow ◽  
...  

2009 ◽  
Vol 43 (2) ◽  
pp. 185
Author(s):  
Sang Hwa Lee ◽  
Wan Seop Kim ◽  
Ji Hoon Kim ◽  
Hye Seung Han ◽  
So Dug Lim ◽  
...  

2020 ◽  
Vol 53 (3) ◽  
pp. 177-182
Author(s):  
Atsushi Kihara ◽  
Kazuya Takahashi ◽  
Ayataka Ishikawa ◽  
Yusuke Amano ◽  
Daisuke Matsubara ◽  
...  

2015 ◽  
Vol 45 (9) ◽  
pp. 1308-1315 ◽  
Author(s):  
Austin Ostermeier ◽  
M. Beth McCarville ◽  
Fariba Navid ◽  
Scott E. Snyder ◽  
Barry L. Shulkin

ORL ◽  
2021 ◽  
pp. 1-6
Author(s):  
Qingjiao Li ◽  
Xiaolu Yuan

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressively malignant tumor mostly occurring in the abdominal and pelvic cavity of young patients. However, few cases had been reported concerning DSRCT occurring in the head and neck region. We presented a rare case of DSRCT of the right submandibular in a 25-year-old man. MRI revealed a 3 × 2-cm solid nodule located in the right submandibular, and physical examination showed no other occupying lesion elsewhere. Histologically, the tumor was composed of various-sized small round cell nests, embedded in an abundant desmoplastic stroma. Immunohistochemically, the tumor cells were typically positive for epithelial (CK and EMA), mesenchymal (vimentin and desmin), and neuroendocrine (CD56, NSE, Syn, and CgA) markers, but negative for WT1. Fluorescence in situ hybridization revealed the presence of a break apart involving the <i>Ewing sarcoma</i> (<i>EWS</i>) gene. The patient received chemotherapy and radiotherapy and relapsed after 19 months of follow-up. DSRCT of the submandibular gland is rare, and the diagnosis of this tumor in an uncommon location relies on the histomorphology, immunophenotype, and <i>EWS</i> gene translocation detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.


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