Open AccessDesmoplastic small round-cell tumour of the peritoneal cavity: case report and literature review
Author(s) -
Nikolaos Tsoukalas,
Maria Kiakou,
Γεώργιος Νάκος,
Maria Tolia,
Michail Galanopoulos,
Konstantinos Tsapakidis,
Konstantinos Kamposioras,
Charalampos Christofyllakis,
Georgios Dimitrakopoulos,
Dimitrios Sambaziotis
Publication year - 2020
Publication title -
annals of the royal college of surgeons of england
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.39
H-Index - 63
eISSN - 1478-7083
pISSN - 0035-8843
DOI - 10.1308/rcsann.2019.0180
Subject(s) - medicine , debulking , asymptomatic , abdomen , radiation therapy , desmoplastic small round cell tumor , therapeutic approach , neoplasm , chemotherapy , constipation , surgery , peritoneum , radiology , disease , pathology , cancer , ovarian cancer
Desmoplastic small round-cell tumour is a very rare neoplasm, which usually arises from the abdominal or pelvic peritoneum of adolescents and young adults. Early diagnosis is difficult, because most tumours present with non-specific gastrointestinal symptoms after a long asymptomatic period. It is generally a very aggressive tumour, which grows rapidly with poor prognosis and an overall five-year survival rate of 15% despite multimodal treatment. Despite multiple treatment strategies, the management of desmoplastic small round-cell tumour still remains a clinical challenge and no consensus about a therapeutic protocol has been established. A 35-year-old man presented with mild abdominal pain, constipation and weight gain, and was eventually diagnosed with desmoplastic small round-cell tumour, which was shown to be limited to the abdomen. After incomplete debulking surgery, radiotherapy and chemotherapy, he developed multiple metastatic nodular foci in chest and the pleura and, unfortunately, he died due to disease progression.