Hormone-secreting ganglioneuroma: A rare entity

Ganglioneuromas are slow-growing benign counterparts of neuroblastoma groups along with ganglioneuroblastomas. Herein, we report a rare case of hormone-secreting benign ganglioneuroma in a 4-year-old kid. A 4-year-old male child was referred to our outdoor patient department from the primary health...

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Main Authors: Aastha Shah, Darshan Thummar, Maitrik Mehta, Dishu Shah, U Suryanarayan, Dinesh Anand
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2021-01-01
Series:Journal of Radiation and Cancer Research
Subjects:
Online Access:http://www.journalrcr.org/article.asp?issn=2588-9273;year=2021;volume=12;issue=3;spage=131;epage=133;aulast=Shah
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spelling doaj-37cf88a251d34e19bdba324a46832bc32021-10-07T05:41:55ZengWolters Kluwer Medknow PublicationsJournal of Radiation and Cancer Research2588-92732468-92032021-01-0112313113310.4103/jrcr.jrcr_73_20Hormone-secreting ganglioneuroma: A rare entityAastha ShahDarshan ThummarMaitrik MehtaDishu ShahU SuryanarayanDinesh AnandGanglioneuromas are slow-growing benign counterparts of neuroblastoma groups along with ganglioneuroblastomas. Herein, we report a rare case of hormone-secreting benign ganglioneuroma in a 4-year-old kid. A 4-year-old male child was referred to our outdoor patient department from the primary health center to evaluate for the persistent complaint of increase in weight and raised blood pressure. On computed tomography (CT) of the abdomen and pelvis, an 6.4 cm × 6.5 cm mass lesion was seen over the right adrenal gland which was compressing segments VI and VII of the liver, and the right hilar region showed a 1.6 cm × 1.2 cm × 0.9 cm enlarged lymph node. The rest of the scan was clear. The patient was therefore started on cyclophosphamide and vincristine, received two cycles, and 4 weeks following it, a diagnostic CT scan of the abdomen, pelvis, and thorax was undertaken which showed a 5.1 cm × 4.5 cm × 6 cm lesion over the right suprarenal region abutting inferior vena cava, inferior surface of the liver, and superior renal surface. Surgical laparotomy followed by right adrenalectomy was done which on pathological evaluation to a surprise showed features suggestive of maturing ganglioneuroma with no evidence of any blastemal components. However, the posterior resection margin turned out to be positive. Radiation was omitted despite the margins being positive. Very few cases of hormone-secreting pediatric ganglioneuroma have been reported in literature and their management requires a meticulous approach.http://www.journalrcr.org/article.asp?issn=2588-9273;year=2021;volume=12;issue=3;spage=131;epage=133;aulast=Shahbenignganglioneuromahormoneradiationsurgery
collection DOAJ
language English
format Article
sources DOAJ
author Aastha Shah
Darshan Thummar
Maitrik Mehta
Dishu Shah
U Suryanarayan
Dinesh Anand
spellingShingle Aastha Shah
Darshan Thummar
Maitrik Mehta
Dishu Shah
U Suryanarayan
Dinesh Anand
Hormone-secreting ganglioneuroma: A rare entity
Journal of Radiation and Cancer Research
benign
ganglioneuroma
hormone
radiation
surgery
author_facet Aastha Shah
Darshan Thummar
Maitrik Mehta
Dishu Shah
U Suryanarayan
Dinesh Anand
author_sort Aastha Shah
title Hormone-secreting ganglioneuroma: A rare entity
title_short Hormone-secreting ganglioneuroma: A rare entity
title_full Hormone-secreting ganglioneuroma: A rare entity
title_fullStr Hormone-secreting ganglioneuroma: A rare entity
title_full_unstemmed Hormone-secreting ganglioneuroma: A rare entity
title_sort hormone-secreting ganglioneuroma: a rare entity
publisher Wolters Kluwer Medknow Publications
series Journal of Radiation and Cancer Research
issn 2588-9273
2468-9203
publishDate 2021-01-01
description Ganglioneuromas are slow-growing benign counterparts of neuroblastoma groups along with ganglioneuroblastomas. Herein, we report a rare case of hormone-secreting benign ganglioneuroma in a 4-year-old kid. A 4-year-old male child was referred to our outdoor patient department from the primary health center to evaluate for the persistent complaint of increase in weight and raised blood pressure. On computed tomography (CT) of the abdomen and pelvis, an 6.4 cm × 6.5 cm mass lesion was seen over the right adrenal gland which was compressing segments VI and VII of the liver, and the right hilar region showed a 1.6 cm × 1.2 cm × 0.9 cm enlarged lymph node. The rest of the scan was clear. The patient was therefore started on cyclophosphamide and vincristine, received two cycles, and 4 weeks following it, a diagnostic CT scan of the abdomen, pelvis, and thorax was undertaken which showed a 5.1 cm × 4.5 cm × 6 cm lesion over the right suprarenal region abutting inferior vena cava, inferior surface of the liver, and superior renal surface. Surgical laparotomy followed by right adrenalectomy was done which on pathological evaluation to a surprise showed features suggestive of maturing ganglioneuroma with no evidence of any blastemal components. However, the posterior resection margin turned out to be positive. Radiation was omitted despite the margins being positive. Very few cases of hormone-secreting pediatric ganglioneuroma have been reported in literature and their management requires a meticulous approach.
topic benign
ganglioneuroma
hormone
radiation
surgery
url http://www.journalrcr.org/article.asp?issn=2588-9273;year=2021;volume=12;issue=3;spage=131;epage=133;aulast=Shah
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