Primary malignant small round cell tumor of the abdomen: CT findings in five cases.

Varma, Datla G; McDaniel, Kevin; Ordóñez, Nelson G.; Granfield, C. A. J.; Charnsangavej, Chusilp; Wallace, Sidney

doi:10.2214/ajr.158.5.1566662

Cited by 14 publications

(1 citation statement)

References 5 publications

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“…Intra-abdominal small round-cell tumours are very rare malignancies and only a few authors have reported series of more than five patients (Gerald et al, 1991;Varma et al, 1992;Ordofiez et al, 1993;Frappaz et al, 1994;Farhat et al, 1995). Here, we report our experience of multiple tumour marker screening in seven patients.…”

Section: Discussionmentioning

confidence: 99%

Ca125 and neuron-specific enolase (NSE) as tumour markers for intra-abdominal desmoplastic small round-cell tumours

Fizazi

Farhat²,

Théodore³

et al. 1997

Br J Cancer

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Summary Seven consecutive patients with intra-abdominal desmoplastic small round-cell tumours were screened at presentation for carcinoembryonic antigen (CEA), Ca19-9, Cal5-3, Ca125, alpha-fetoprotein (AFP), human chorionic gonadotrophin (hCG) and neuronspecific enolase (NSE). Initially elevated tumour markers were used to monitor therapy and follow-up. Tumour marker assays were all in the normal range, with the exception of Cal 25 and NSE. The Cal 25 level was initially high in six of the seven patients (86%) with a median value of 200 U ml-' and a range of 22-735 U ml-'. The NSE value was elevated before therapy in three of the five patients (60%) for whom assay results were available, with a median of 19 ng ml-' and a range of 6.8-37.5 ng ml'. Cal 25 normalized in five out of six cases and NSE always normalized during chemotherapy, but neither of these two tumour markers correlated specifically with response, as only one patient experienced a partial response, five tumour stabilization and the remaining patient tumour progression. At progression, Cal 25 was again elevated in two out of four cases several weeks before clinical relapse and NSE in only one out of three cases. Cal 25 and NSE are frequently raised in the serum of patients with intra-abdominal desmoplastic small round-cell tumours before therapy, but are not reliable monitors of the course of the disease. However, normalization is frequently associated with an improvement of symptoms or a moderate clinical response.

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Section: Discussionmentioning

confidence: 99%

Ca125 and neuron-specific enolase (NSE) as tumour markers for intra-abdominal desmoplastic small round-cell tumours

Fizazi

Farhat²,

Théodore³

et al. 1997

Br J Cancer

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Primary Tumors

Rossi

Rossi²

2001

CT of the Peritoneum

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Desmoplastic small round cell tumour: the radiological, pathological and clinical features

et al. 2013

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ObjectivesDesmoplastic small round cell tumours (DSRCTs) are rare aggressive tumours of young adults that present late and have poor prognosis. This review discusses distinctive radiological features, histopathology and clinical course of this soft-tissue sarcoma.MethodsFrom 1991 to 2012, the radiology of 20 patients with pathologically proven DSRCT was independently reviewed by two experienced radiologists. The clinical presentation, treatment and outcome were recorded.ResultsPatients: 16 men, four women; mean age 28.3 years. Computed tomography (CT) demonstrated peritoneal/omental masses without an organ of origin (94 %), with the majority of cases demonstrating large (>5 cm) dominant soft-tissue deposit (80 %) with multiple smaller foci. CT and magnetic resonance imaging (MRI) typically demonstrated heterogeneous soft-tissue enhancement with cystic degeneration. A minority (20 %) demonstrated calcification. Lymph node enlargement occurred in 50 % of cases. Distant metastatic disease occurred in 25 %. Painful abdominal masses were clinically predominant. Treatment strategies include combination chemotherapy with debulking surgery and/or radiotherapy. Median survival from diagnosis was 22.8 months.ConclusionFeatures of multifocal peritoneal/omental masses, usually in combination with a dominant soft tissue deposit, are distinctive in this rare sarcoma. CT/MRI defines the extent of disease and characterises supporting imaging findings. Prolific desmoplastic reaction histologically separates DSRCT from similar subtypes. Combination treatment strategies can infer a survival benefit but prognosis remains poor.Teaching Points• DSRCTs are rare tumours of young adults (mean age 28.3 years) with a male predominance (4:1).• Painful abdominal masses clinically predominate. Non-specific features of malignancy can be present.• Multifocal peritoneal masses with a dominant soft tissue lesion is a distinctive imaging finding.• A large desmoplastic reaction differentiates DSRCTs from histologically similar round cell subtypes.• Despite debulking surgery with adjuvant chemotherapy, median survival from diagnosis is 22.3 months.

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Primary malignant small round cell tumor of the abdomen: CT findings in five cases.

Abstract: Malignantsmall round cell tumor of the abdomen is a newly described clinicopathologic entity.

Cited by 14 publications

References 5 publications

Ca125 and neuron-specific enolase (NSE) as tumour markers for intra-abdominal desmoplastic small round-cell tumours

Ca125 and neuron-specific enolase (NSE) as tumour markers for intra-abdominal desmoplastic small round-cell tumours

Primary Tumors

Desmoplastic small round cell tumour: the radiological, pathological and clinical features

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