Pediatric Radiology

, Volume 47, Issue 12, pp 1615–1621 | Cite as

Imaging of renal medullary carcinoma in children and young adults: a report from the Children’s Oncology Group

  • Jesse K. Sandberg
  • Elizabeth A. Mullen
  • Mariana M. Cajaiba
  • Ethan A. Smith
  • Sabah Servaes
  • Elizabeth J. Perlman
  • James I. Geller
  • Peter F. Ehrlich
  • Nicholas G. Cost
  • Jeffrey S. Dome
  • Conrad V. Fernandez
  • Geetika Khanna
Original Article

Abstract

Background

Renal medullary carcinoma is a rare renal malignancy of childhood. There are no large series describing the imaging appearance of renal medullary carcinoma in children.

Objective

To characterize the clinical and imaging features of pediatric renal medullary carcinoma at initial presentation.

Materials and methods

We retrospectively analyzed images of 25 pediatric patients with renal medullary carcinoma enrolled in the Children’s Oncology Group renal tumors classification, biology and banking study (AREN03B2) from March 2006 to August 2016. Imaging findings of the primary mass, and patterns of locoregional and distant spread were evaluated in correlation with pathological and surgical findings.

Results

Median age at presentation was 13 years (range: 6-21 years), with a male predominance (3.2:1). The overall stage of disease at initial presentation was stage 1 in 1, stage 2 in 2 and stage 4 in 22. Maximum diameter of the primary renal mass ranged from 1.6 to 10.3 cm (mean: 6.6 cm) with a slight right side predilection (1.5:1). Enlarged (>1 cm short axis) retroperitoneal lymph nodes were identified at initial staging in 20/25 (80%) cases, 10 of which were histologically confirmed while the others did not undergo surgical sampling. Enlarged lymph nodes were also identified in the mediastinum (14/25; 56%) and supraclavicular regions (4/25; 16%). Metastatic disease was present in the lungs in 19/25 (76%) and liver in 6/25 (24%). The pattern of lung metastases was pulmonary lymphangitic carcinomatosis: 10 cases (9 bilateral, 1 unilateral), pulmonary nodules with indistinct margins: 6 cases, pulmonary nodules with distinct margins: 2 cases, while 1 case had pulmonary nodules with both indistinct and distinct margins. Pulmonary lymphangitic carcinomatosis was pathologically confirmed in 4/10 cases. All cases with pulmonary lymphangitic carcinomatosis had associated enlarged mediastinal lymph nodes.

Conclusion

Renal medullary carcinoma in children and young adults presents at an advanced local and distant stage in the majority of patients. The diagnosis of renal medullary carcinoma should be considered when a child or young adult presents with a poorly defined/infiltrative, centrally located renal mass, especially in the setting of known sickle cell hemoglobinopathy. Distant metastases are common at initial presentation in the lungs, distant lymph nodes and liver and often involve multiple sites simultaneously. Pulmonary lymphangitic carcinomatosis, a distinctive and uncommon form of lung metastasis in children, is common in this patient population.

Keywords

Children Computed tomography Lymphangitic carcinomatosis Medullary carcinoma Renal tumor Sickle cell 

Notes

Compliance with ethical standards

Conflicts of interest

None

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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  • Jesse K. Sandberg
    • 1
  • Elizabeth A. Mullen
    • 2
  • Mariana M. Cajaiba
    • 3
  • Ethan A. Smith
    • 4
  • Sabah Servaes
    • 5
  • Elizabeth J. Perlman
    • 3
  • James I. Geller
    • 6
  • Peter F. Ehrlich
    • 7
  • Nicholas G. Cost
    • 8
  • Jeffrey S. Dome
    • 9
  • Conrad V. Fernandez
    • 10
  • Geetika Khanna
    • 1
  1. 1.Mallinckrodt Institute of RadiologyWashington University School of MedicineSt. LouisUSA
  2. 2.Department of Pediatric OncologyChildren’s Hospital Boston/Dana-Farber Cancer InstituteBostonUSA
  3. 3.Department of Pathology and Laboratory Medicine, Ann & Robert H. Lurie Children’s Hospital of ChicagoNorthwestern University Feinberg School of MedicineChicagoUSA
  4. 4.Section of Pediatric Radiology, C. S. Mott Children’s Hospital, Department of RadiologyUniversity of Michigan Health SystemAnn ArborUSA
  5. 5.Department of RadiologyChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  6. 6.Division of Pediatric Oncology, Cincinnati Children’s Hospital Medical CenterUniversity of CincinnatiCincinnatiUSA
  7. 7.Section of Pediatric Surgery, C. S. Mott Children’s Hospital, Department of SurgeryUniversity of Michigan Health SystemAnn ArborUSA
  8. 8.Division of Urology, Department of SurgeryUniversity of Colorado School of MedicineAuroraUSA
  9. 9.Division of Pediatric OncologyChildren’s National Medical CenterWashingtonUSA
  10. 10.Department of PediatricsDalhousie University & IWK Health CentreHalifaxCanada

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