Desmoplastic Small Round Cell Tumor: Imaging Pattern of Disease at Presentation

Morani, Ajaykumar C.; Bathala, Tharakeswara K.; Surabhi, Venkateswar R.; Yedururi, Sireesha; Jensen, Corey T.; Huh, Winston W.; Prasad, Srinivasa R.; Hayes‐Jordan, Andrea

doi:10.2214/ajr.18.20179

Cited by 39 publications

(74 citation statements)

References 59 publications

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“…DSRCT showed a higher rate of occurrence on serosal surfaces, where it appeared as single or multiple nodular masses on the surface of intra-abdominal organs. The characteristic imaging feature of DSRCT was multiple soft-tissue masses implicating the peritoneal cavity in the omentum or mesentery without a definite organ of origin as described in the literatures [16,17]. On CT images, tumor mass was usually patchy hypodense and mild heterogeneous enhancement on contrast enhanced images.…”

Section: Discussionmentioning

confidence: 89%

Primary desmoplastic small round cell tumor of the tibia: PET/CT and MRI presentation of a rare case and review of the literature

Xiang-ke

Guo

Zhang

2020

Journal of Bone Oncology

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Desmoplastic small round cell tumor (DSRCT) was a soft tissue sarcoma of mesenchymal cell origin that typically exhibited a multi-phenotypic pattern of immunohistochemical staining. DSRCT mainly presented in the abdomen sites and primary occurrence in bone was exceptional. In this study, we reported a new case of primary DSRCT of the tibia in a 33-year-old man who had intermittent pain in the left tibia. Radiographs showed transparent lesions in the left upper tibial. MRI revealed a lobular, lytic and ill-identified lesion with adjacent soft tissues swelling of the upper left tibia. CT confirmed notable destruction and wormlike osteolysis of the bone cortex. PET/CT showed a mass of high uptakes, indicating the malignance. He accepted surgical resection with followed multi-agent chemotherapy, containing vincristine, doxorubicin, ifosfamide and etoposide. Clinically and radiologically, the patient did not show any evidence of recurrence or metastasis at 30 months after surgical treatment. Primary osteogenic DSRCT was extremely rare and should be considered in differential diagnosis of bone tumors.

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Section: Discussionmentioning

confidence: 89%

Primary desmoplastic small round cell tumor of the tibia: PET/CT and MRI presentation of a rare case and review of the literature

Xiang-ke

Guo

Zhang

2020

Journal of Bone Oncology

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“…Las masas variaban en tamaño de 2 a 27 cm en su dimensión máxima (mediana de 12 cm); 32 pacientes (34 %) tenían metástasis hepáticas, 3 pacientes manifestaron hidronefrosis bilateral causada por una gran masa o masas pélvicas obstructivas, 49 pacientes (52 %) tuvieron afectación retroperitoneal en forma de implantes, extensión tumoral o afectación nodal. 15 En el estudio histológico presenta un patrón de células pequeñas, redondas, con núcleos hipercromáticos, escaso citoplasma y abundante mitosis, que se disponen en nidos, cordones y áreas solidas bien delimitadas, sobre un estroma desmoplásico. 16 El estudio inmunofenotípico se caracteriza por presentar un patrón con expresión para marcadores epiteliales, mesenquimales y neuronales.…”

Section: Discussionunclassified

Tumor abdominal desmoplásico de células pequeñas y redondas

Chavarría-Caldas

Terán-Vásquez

Páucar-Pérez

et al. 2019

spmi

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“…Lymph nodes, lungs, brain, and bones can also be affected. Computed tomography (CT) scan, magnetic resonance imaging (MRI), and positron emission tomography (PET) scan with fluorodeoxyglucose (FDG) are used as preoperative diagnostic modalities revealing multiple soft-tissues masses [5]. Final diagnosis can only be revealed by tissue biopsy following complex immunohistochemical and molecular analysis.…”

Section: Introductionmentioning

confidence: 99%

Presentation of a rare, highly aggressive peritoneal disease: desmoplastic small round cell tumor and its therapeutic options

et al. 2021

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Summary Background Desmoplastic small round cell tumor is a rare highly aggressive peritoneal disease (sarcoma) with mortality rates up to 70% in the first 3 years after diagnosis. It mainly affects young men. Patients clinically complain about diffuse abdominal pain. Methods This case report describes the clinical course of a 69-year-old man who presented with recurrent abdominal pain. Physical examination, laboratory testing, imaging, and gastroscopy were performed. Intra-abdominal peritoneal biopsies were taken during diagnostic laparoscopy. Results Physical examination was unremarkable. Laboratory results showed elevated white blood cells, C‑reactive protein, and negative tumor markers. Computed tomography and positron emission tomography scan revealed extensive peritoneal metastases with diffuse intra-abdominal signal intensities and ascites. Gastroscopy was unremarkable, whereas diagnostic laparoscopy confirmed imaging results with a peritoneal cancer index of 39. Extensive immunohistochemical and consecutive molecular investigations led to the diagnosis of an intraperitoneal desmoplastic small round cell tumor. Conclusion Our case report demonstrates a very rare cause of recurrent abdominal pain. Desmoplastic small round cell tumor is a rare and highly aggressive undifferentiated sarcoma, which mainly affects young men. Treatment options include chemotherapy, radiotherapy, cytoreductive surgery, and/or hyperthermic intraperitoneal chemotherapy. Standardized treatment protocols are still lacking because only a few cases have been described so far. Differential diagnoses include all malignancies with peritoneal masses.

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Desmoplastic Small Round Cell Tumor: Imaging Pattern of Disease at Presentation

Cited by 39 publications

References 59 publications

Primary desmoplastic small round cell tumor of the tibia: PET/CT and MRI presentation of a rare case and review of the literature

Primary desmoplastic small round cell tumor of the tibia: PET/CT and MRI presentation of a rare case and review of the literature

Tumor abdominal desmoplásico de células pequeñas y redondas

Presentation of a rare, highly aggressive peritoneal disease: desmoplastic small round cell tumor and its therapeutic options

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