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Journal of Case Reports and Images in Pathology - Journal of Case Reports and Images in Pathology - Case Reports, Case Series, Case in Images, Clinical Images

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Case Report
 
Nephroblastic elements in a retroperitoneal immature teratoma with elevated serum alpha-fetoprotein
Chiu-Hsuan Cheng1, Shang-Hsien Yang2, Borcherng Su3
1MBBS, Resident pathologist, Department of Pathology, Buddhist Tzu Chi General Hospital, Hualien city, Hualien county, Taiwan.
2MD, Attending pediatrician and pediatric hemato-oncologist, Department of Pediatric Hematology and Oncology, Buddhist Tzu Chi General Hospital, Hualien city, Hualien county, Taiwan, School of Medicine, Tzu Chi University, Hualien city, Hualien county, Taiwan.
3DDS, PhD, Attending pathologist, Department of Pathology, Buddhist Tzu Chi General Hospital, Hualien city, Hualien county, Taiwan, School of Medicine, Tzu Chi University, Hualien city, Hualien county, Taiwan.

Article ID: 100008Z11CC2016
doi:10.5348/Z11-2016-8-CR-4

Address correspondence to:
Borcherng Su
Complete Mailing Address - No. 701, Section 3, Zhongyang road Hualien City
Hualien County
Taiwan, 970

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How to cite this article:
Cheng CH, Yang SH, Su B. Nephroblastic elements in a retroperitoneal immature teratoma with elevated serum alpha-fetoprotein. J Case Rep Images Pathol 2016;2:15–19.


Abstract
Introduction: Teratoma and Wilms' tumor are among the most frequent tumors of childhood. The occurrence of nephroblastic elements in a teratoma is extremely rare. Only eight cases that occurred in the retroperitoneum have been reported in English literature. Such occurrence complicates the differential diagnosis because of the overlapping histopathological findings between teratoma with nephroblastic elements and teratoid Wilms' tumor. Alpha-fetoprotein (AFP) is a recognized marker of immature teratomas. The source of AFP is believed to be from gastrointestinal epithelium and neuroepithelium. To our knowledge, this is the first case of elevated serum AFP level observed in a retroperitoneal immature teratoma with nephroblastic elements.
Case Report: A three and a half-month-old Taiwanese female infant presented with an abdominal distension. A retroperitoneal neoplasm was detected by the abdominal computed tomography scans. Histologically, nephroblastic elements in lobular contour were noted comprising about 15% per slide along with mixed elements of grade 3 teratoma in organoid arrangement. Her preoperative serum AFP level (1182 ng/ml) was above the physiologic level for her age. It decreased to 312.5 ng/ml at one week after tumor excision. She was well with no evidence of tumor recurrence at the age of one and a half years with unremarkable AFP blood level.
Conclusion: We made the diagnosis of retroperitoneal immature teratoma with nephroblastic elements due to their preservation of lobular shape and organoid arrangement of mixed elements from different germ layers. A significant AFP level elevation may be noted in immature teratomas regardless of the presence of nephroblastic elements.

Keywords: Alpha-fetoprotein, Nephroblastoma, Retroperitoneal neoplasm, Teratoma, Wilms' tumor


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Author Contributions
Chiu-Hsuan Cheng – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Shang-Hsien Yang – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Borcherng Su – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Chiu-Hsuan Cheng et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.