Abstract
Background
Desmoplastic small round cell tumour (DSRCT) is a rare malignant neoplasm. Its radiological features have rarely been described.
Objective
To assess the CT parameters characteristic of DSRCT. We also report our experience with combined FDG PET/CT in staging and follow–up for DSRCT.
Materials and methods
The pretreatment diagnostic CT’s of 65 patients with DSRCT were evaluated. Pertinent imaging findings were catalogued, with histopathology or serial follow-up studies as reference standard. Combined FDG PET/CT examinations of 11 of these patients who underwent pretreatment imaging were also reviewed.
Results
Sixty-two patients presented with primary intra-abdominal disease; three had primary extra-abdominal tumours at presentation. The most common imaging finding of patients with intra-abdominal DSRCT was multiple peritoneal soft tissue masses, with a dominant mass in the retrovesical or rectouterine location in more than half of the cases. Forty percent had metastatic disease to the liver, lungs, spleen or bones at diagnosis. FDG PET/CT accurately detected 97.4% of all DSRCT lesions.
Conclusion
DSRCT typically presents as a large abdominopelvic mass with widespread peritoneal involvement predominantly in young males. Familiarity with its radiological features can help guide diagnosis and treatment. Functional imaging with PET/CT offers advantage over anatomical imaging for accurate disease staging.
Similar content being viewed by others
References
Bellah R, Suzuki-Bordalo L, Brecher E et al (2005) Desmoplastic small round cell tumor in the abdomen and pelvis: report of CT findings in 11 affected children and young adults. AJR 184:1910–1914
Pang B, Leong CC, Salto-Tellez M et al (2011) Desmoplastic small round cell tumor of major salivary glands: report of 1 case and a review of the literature. App Immunohistochem Mol Morphol 19:70–75
Rais H, Elmansouri F, Belaabidia B et al (2010) La tumeur desmoplastique à petites cellules rondes paratesticulaire: à propos d’une observation avec revue de la littérature. Cancer Radiothér 14:111–114
Roganovich J, Bisogno G, Cecchetto G et al (1999) Paratesticular desmoplastic small round cell tumor: case report and review of the literature. J Surg Oncol 71:269–272
Yoshida A, Edgar M, Garcia J et al (2008) Primary desmoplastic small round cell tumor of the femur. Skeletal Radiol 37:857–862
Venkateswaran L, Jenkins JJ, Kaste SC et al (1997) Disseminated intrathoracic desmoplastic small round-cell tumor: a case report. J Pediatr Hematol Oncol 19:172–175
Yin W-H, Guo S-P, Yang H-Y et al (2010) Desmoplastic small round cell tumor of the submandibular gland–a rare but distinctive primary salivary gland neoplasm. Hum Pathol 41:438–442
Gerald WL, Miller HK, Battifora H et al (1991) Intra-abdominal desmoplastic small round-cell tumor: report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol 15:499–513
Chouli M, Viala J, Dromain C et al (2005) Intra-abdominal desmoplastic small round cell tumors: CT findings and clinicopathological correlations in 13 cases. Eur J Radiol 54:438–442
Ota S, Ushijima K, Fujiyoshi N et al (2010) Desmoplastic small round cell tumor in the ovary: Report of two cases and literature review. J Obstet Gynaecol Res 36:430–434
Zhang WD, Li CX, Liu QY et al (2011) CT, MRI, and FDG-PET/CT imaging findings of abdominopelvic desmoplastic small round cell tumors: Correlation with histopathologic findings. Eur J Radiol 80:269–273
Pickhardt PJ, Fisher AJ, Balfe DM et al (1999) Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic correlation. Radiology 210:633–638
Levy AD, Arnáiz J, Shaw JC et al (2008) From the archives of the AFIP: primary peritoneal tumors: imaging features with pathologic correlation. Radiographics 28:583–607
Brodie SG, Stocker SJ, Wardlaw JC et al (1995) EWS and WT-1 gene fusion in desmoplastic small round cell tumor of the abdomen. Hum Pathol 26:1370–1374
Sawyer JR, Tryka AF, Lewis JM (1992) A novel reciprocal chromosome translocation t(11;22)(p13;q12) in an intraabdominal desmoplastic small round-cell tumor. Am J Surg Pathol 16:411–416
Zaloudek C, Miller TR, Stern JL (1995) Desmoplastic small cell tumor of the ovary: a unique polyphenotypic tumor with an unfavorable prognosis. Int J Gynecol Pathol 14:260–265
Agrons GA, Wagner BJ, Lonergan GJ et al (1997) From the archives of the AFIP. Genitourinary rhabdomyosarcoma in children: radiologic-pathologic correlation. Radiographics 17:919–937
Ueno T, Tanaka YO, Nagata M et al (2004) Spectrum of germ cell tumors: from head to toe. Radiographics 24:387–404
Goerres GW, Burger C, Kamel E et al (2003) Respiration-induced attenuation artifact at PET/CT: technical considerations. Radiology 226:906–910
Kushner BH, Laquaglia MP, Gerald WL et al (2008) Solitary relapse of desmoplastic small round cell tumor detected by positron emission tomography/computed tomography. J Clin Oncol 26:4995–4996
Ben-Sellem D, Liu K-L, Cimarelli S et al (2009) Desmoplastic small round cell tumor: impact of F-FDG PET induced treatment strategy in a patient with long-term outcome. Rare Tumors 1:e19
Amato RJ, Ellerhorst JA, Ayala AG (1996) Intraabdominal desmoplastic small cell tumor: report and discussion of five cases. Cancer 78:845–851
Kushner B, LaQuaglia M, Wollner N et al (1996) Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol 14:1526–1531
Lal DR, Su WT, Wolden SL et al (2005) Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 40:251–255
Conflict of interest
The authors declare no conflicts of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Arora, V.C., Price, A.P., Fleming, S. et al. Characteristic imaging features of desmoplastic small round cell tumour. Pediatr Radiol 43, 93–102 (2013). https://doi.org/10.1007/s00247-012-2485-0
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-012-2485-0