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Ovarian Adrenal Rest Tumors Undetected by Imaging Studies and Identified at Surgery in Three Females with Congenital Adrenal Hyperplasia Unresponsive to Increased Hormone Therapy Dosage

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Abstract

Patients with congenital adrenal hyperplasia have a predisposition for developing adrenal rest tumors. In contrast to testicular adrenal rest tumors, ovarian adrenal rest tumors are less common, and only a few cases have been reported in the literature. This report presents three Chinese female congenital adrenal hyperplasia patients (9 to 15 years of age) with small ectopic adrenal cortical nodules that were not detected by imaging but were diagnosed at surgery. All three patients developed virilization with elevation of 17- hydroxyprogesterone, androstenedione, and androgen levels despite receiving maximum adrenal hormone replacement therapy. Ultrasound and magnetic resonance imaging of the abdomen and pelvis suggested bilateral expansion of the adrenal glands, but no lesions of the ovaries were observed. Laparoscopy and/or laparotomy revealed small nodular lesions surrounding the pelvic gonad in all three cases. Histopathological examination of the resected tissue in all cases revealed hyperplasic nodules of cells surrounded by fibrous tissue. The cells were arranged as nests with abundant cytoplasm, which were partially lightly stained with a small centered nucleus. Immunohistochemistry staining revealed the cells to be synaptophysin positive, melan-A positive, and chromogranin A negative, indicating the cells were adrenocortical tissue and not adrenal medullary cells. Thus, the findings of the histopathological examination were consistent with ovarian adrenal rest tumors. Female congenital adrenal hyperplasia patients with virilization who showed an inadequate response to hormone therapy and had negative imaging results may benefit from laparoscopic examination or laparotomy in order to confirm the diagnosis of ovarian adrenal rest tumors while receiving unilateral subtotal adrenalectomy or subtotal bilateral adrenalectomy.

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Acknowledgements

None declared.

Authors’ Contributions

We declare that all the listed authors have participated actively in the study and all meet the requirements of authorship. Dr. JCL designed the study and wrote the protocol and performed the research/study. Dr. HDC wrote the first draft of the manuscript and performed the research/study. Dr. LH performed the research/study. Dr. ZHZ and Dr. ZS managed the literature searches and analyses. Dr. HJ undertook the statistical analysis. Dr. JZ was responsible for the immunohistochemical examinations. All authors read and approved the final manuscript.

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Authors and Affiliations

  1. Pediatric Surgery Department of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China

    Hua-dong Chen, Li-e Huang, Zhi-hai Zhong, Hong Jiang & Jun-cheng Liu

  2. Pediatric Department of the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China

    Zhe Su

  3. Department of Pathology, Sun Yat-sen University Cancer Center, Guangzhou, China

    Jing Zeng

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  1. Hua-dong Chen
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  2. Li-e Huang
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  3. Zhi-hai Zhong
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  4. Zhe Su
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  5. Hong Jiang
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  6. Jing Zeng
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  7. Jun-cheng Liu
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Corresponding author

Correspondence to Jun-cheng Liu.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Chen, Hd., Huang, Le., Zhong, Zh. et al. Ovarian Adrenal Rest Tumors Undetected by Imaging Studies and Identified at Surgery in Three Females with Congenital Adrenal Hyperplasia Unresponsive to Increased Hormone Therapy Dosage. Endocr Pathol 28, 146–151 (2017). https://doi.org/10.1007/s12022-016-9461-4

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  • DOI: https://doi.org/10.1007/s12022-016-9461-4

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