Clinical features and outcomes of 20 patients with abdominopelvic desmoplastic small round cell tumor

F A Angarita; S Hassan; A J Cannell; B C Dickson; R A Gladdy; C J Swallow; A Gupta; M E Blackstein; J A McCart

doi:10.1016/j.ejso.2016.08.017

Clinical features and outcomes of 20 patients with abdominopelvic desmoplastic small round cell tumor

Eur J Surg Oncol. 2017 Feb;43(2):423-431. doi: 10.1016/j.ejso.2016.08.017. Epub 2016 Oct 4.

Authors

F A Angarita¹, S Hassan², A J Cannell³, B C Dickson⁴, R A Gladdy⁵, C J Swallow⁵, A Gupta⁶, M E Blackstein⁷, J A McCart⁸

Affiliations

¹ Department of Surgery, Mount Sinai Hospital, Toronto, ON, M5G 1X5, Canada; Department of Surgery, University of Toronto, Toronto, ON, M5S 1A1, Canada.
² Department of Surgery, Mount Sinai Hospital, Toronto, ON, M5G 1X5, Canada; Department of Biomedical Engineering, Oregon Health and Science University, Portland, OR, 97239, USA.
³ Department of Surgery, Mount Sinai Hospital, Toronto, ON, M5G 1X5, Canada.
⁴ Department of Laboratory Medicine and Pathology, Mount Sinai Hospital, Toronto, ON, M5G 1X5, Canada; Department of Laboratory Medicine and Pathology, University of Toronto, Toronto, ON, M5S 1A1, Canada.
⁵ Department of Surgery, Mount Sinai Hospital, Toronto, ON, M5G 1X5, Canada; Department of Surgery, University of Toronto, Toronto, ON, M5S 1A1, Canada; Department of Surgical Oncology, Princess Margaret Cancer Centre, Toronto, ON, M5G 1X8, Canada.
⁶ Department of Medical Oncology, Princess Margaret Cancer Centre, Toronto, ON, M5G 1X8, Canada; Division of Haematology/Oncology, Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada.
⁷ Department of Medical Oncology, Princess Margaret Cancer Centre, Toronto, ON, M5G 1X8, Canada; Department of Medicine, University of Toronto, Toronto, ON, M5S 1A1, Canada; Department of Medicine, Mount Sinai Hospital, Toronto, ON, M5G 1X5, Canada.
⁸ Department of Surgery, Mount Sinai Hospital, Toronto, ON, M5G 1X5, Canada; Department of Surgery, University of Toronto, Toronto, ON, M5S 1A1, Canada; Department of Surgical Oncology, Princess Margaret Cancer Centre, Toronto, ON, M5G 1X8, Canada. Electronic address: andrea.mccart@sinaihealthsystem.ca.

PMID: 27890349
DOI: 10.1016/j.ejso.2016.08.017

Abstract

Introduction: Desmoplastic small round cell tumor (DSRCT) is a rare mesenchymal malignancy. We describe our experience with treating DSRCT at a large sarcoma referral center.

Methods: A retrospective chart review was performed on DSRCT patients referred to our institution (1998-2014). Pathology specimens were reviewed to confirm the diagnosis. Clinical and imaging were extracted and summarized with descriptive statistics. Univariate analysis was performed to evaluate the association between patient, tumor, and treatment variables and overall survival (OS).

Results: In this study cohort of 20 patients, median age at presentation was 29 y (range 18-43) and 90% were male. Fifty-five percent presented with metastasis. Patients underwent chemotherapy (n = 20), radiation therapy (n = 3), and cytoreductive surgery (CRS) (n = 5). Median OS was 22 m (interquartile range: 12-28 m). Five-year OS rate was 20%. Extra-abdominal metastasis was associated with a higher hazard ratio (HR) of mortality (HR: 3.1, 95% C.I. 1.0-9.4, p = 0.04), while CRS improved OS (HR: 0.1, 95% C.I. 0.03-0.7, p = 0.02).

Conclusions: Despite aggressive treatment, less than half of the patients were dead of DSRCT within 2 years of presentation. Although a select group of patients who underwent CRS had improved OS, novel treatments are urgently needed.

Keywords: Chemotherapy; Desmoplastic small round cell tumor; Multimodal management; Small round blue cell tumors; Surgery.

MeSH terms

Abdominal Neoplasms / mortality
Abdominal Neoplasms / pathology
Abdominal Neoplasms / therapy*
Adolescent
Adult
Combined Modality Therapy
Cytoreduction Surgical Procedures
Desmoplastic Small Round Cell Tumor / mortality
Desmoplastic Small Round Cell Tumor / pathology
Desmoplastic Small Round Cell Tumor / therapy*
Female
Humans
Male
Retrospective Studies
Survival Rate
Treatment Outcome