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335350

Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!

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Last updated: 05 Jan 2025

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Abstract

Background
Mixed cortico-medullary adrenal carcinoma (MCMAC) is an extremely rare entity with scarce literature on its cytomorphology.
Case presentation
A 2-year-old girl presented with abdominal pain for 3 days and a past history of fever with significant weight loss. On examination, a non-tender left hypochondrial firm mass and an enlarged left supraclavicular node were found. Twenty-four-hour urinary levels of VMA were marginally high. Contrast-enhanced computed tomography of the abdomen showed a suprarenal heterogeneous mass encasing major vessels. Aspiration cytology of both mass and node showed similar features comprising a predominant population of singly scattered large cells with moderate cytoplasm, eccentric nucleus and prominent nucleolus in a necrotic background. Tumour cells expressed Synaptophysin and Melan-A. In view of increasing respiratory distress, debulking surgery was performed, and histopathology of the specimen revealed the presence of both malignant medullary and cortical components supported by immunohistochemistry making a final diagnosis of MCMAC. The patient succumbed to death in the postoperative period. The cytology slides were reviewed and were seen to show a dual cell population.
Conclusion
Coexistent malignant cortical and medullary tumour of the adrenal gland is the first case reported in the paediatric age group in the literature with only three previous case reports in adults.

DOI

10.1186/s43159-021-00134-3

Keywords

Adrenal, tumour, Pheochromocytoma, Synaptophysin, Melan-A, Case Report

Authors

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Arti

Last Name

Khatri

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Orcid

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First Name

Nidhi

Last Name

Mahajan

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Email

nidhi0615@gmail.com

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First Name

Niyaz Ahmed

Last Name

Khan

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Orcid

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First Name

Natasha

Last Name

Gupta

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Volume

17

Article Issue

1

Related Issue

45423

Issue Date

2021-02-01

Receive Date

2021-09-14

Publish Date

2021-11-01

Print ISSN

1687-4137

Online ISSN

2090-5394

Link

https://apsj.journals.ekb.eg/article_335350.html

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https://apsj.journals.ekb.eg/service?article_code=335350

Order

335,350

Publication Type

Journal

Publication Title

Annals of Pediatric Surgery

Publication Link

https://apsj.journals.ekb.eg/

MainTitle

Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!

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Type

Article

Created At

20 Dec 2024