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

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...

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Autores principales:	Arti Khatri, Nidhi Mahajan, Niyaz Ahmed Khan, Natasha Gupta
Formato:	article
Lenguaje:	EN
Publicado:	SpringerOpen 2021
Materias:	Adrenal Tumour Pheochromocytoma Synaptophysin Melan-A Case report Pediatrics RJ1-570 Surgery RD1-811
Acceso en línea:	https://doaj.org/article/a3a5887ccf224bd7bed8619cec68fa88
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spelling	oai:doaj.org-article:a3a5887ccf224bd7bed8619cec68fa882021-11-08T10:56:28ZMixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!10.1186/s43159-021-00134-32090-5394https://doaj.org/article/a3a5887ccf224bd7bed8619cec68fa882021-11-01T00:00:00Zhttps://doi.org/10.1186/s43159-021-00134-3https://doaj.org/toc/2090-5394Abstract 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.Arti KhatriNidhi MahajanNiyaz Ahmed KhanNatasha GuptaSpringerOpenarticleAdrenalTumourPheochromocytomaSynaptophysinMelan-ACase reportPediatricsRJ1-570SurgeryRD1-811ENAnnals of Pediatric Surgery, Vol 17, Iss 1, Pp 1-5 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Adrenal Tumour Pheochromocytoma Synaptophysin Melan-A Case report Pediatrics RJ1-570 Surgery RD1-811
spellingShingle	Adrenal Tumour Pheochromocytoma Synaptophysin Melan-A Case report Pediatrics RJ1-570 Surgery RD1-811 Arti Khatri Nidhi Mahajan Niyaz Ahmed Khan Natasha Gupta Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!
description	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.
format	article
author	Arti Khatri Nidhi Mahajan Niyaz Ahmed Khan Natasha Gupta
author_facet	Arti Khatri Nidhi Mahajan Niyaz Ahmed Khan Natasha Gupta
author_sort	Arti Khatri
title	Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!
title_short	Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!
title_full	Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!
title_fullStr	Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!
title_full_unstemmed	Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!
title_sort	mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!
publisher	SpringerOpen
publishDate	2021
url	https://doaj.org/article/a3a5887ccf224bd7bed8619cec68fa88
work_keys_str_mv	AT artikhatri mixedcorticomedullaryadrenalcarcinomainchildrenlooksaredeceptive AT nidhimahajan mixedcorticomedullaryadrenalcarcinomainchildrenlooksaredeceptive AT niyazahmedkhan mixedcorticomedullaryadrenalcarcinomainchildrenlooksaredeceptive AT natashagupta mixedcorticomedullaryadrenalcarcinomainchildrenlooksaredeceptive
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Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!

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