Retroperitoneal paraganglioma causing proximal ureteric obstruction in a child: diagnostic conundrum and immunohistochemistry characteristics (case report).
Hypertension
case report
extra-adrenal
resected
tumor
Journal
The Pan African medical journal
ISSN: 1937-8688
Titre abrégé: Pan Afr Med J
Pays: Uganda
ID NLM: 101517926
Informations de publication
Date de publication:
2022
2022
Historique:
received:
03
04
2021
accepted:
01
09
2022
entrez:
2
1
2023
pubmed:
3
1
2023
medline:
4
1
2023
Statut:
epublish
Résumé
Paragangliomas are extremely rare neuroendocrine tumors with an incidence of 0.3 cases per million in children. They arise from several sites in the body including the orbit, ear, nose, larynx, carotid area, mediastinum, duodenum, and the genitourinary tract. Germline mutations have been identified in paragangliomas involving the proto-oncogene RET, tumor suppressor genes VHL, and NF1 The commonest clinical presentation is hypertension but incidentally detected forms have been reported during imaging and on a few occasions the diagnosis was missed. The authors report on a case of a 12-year-old male with a history of fever, left flank pain, vomiting, and headaches, finally diagnosed by pathological examination of a resected retroperitoneal mass invading the proximal left ureter. Immunohistochemistry was positive for neuron-specific enolase, NSE (chief cells), and S-100 protein (sustentacular cells) all consistent with paragangliomas. The challenges faced by the team in the preoperative evaluation and surgical treatment are reported.
Identifiants
pubmed: 36591002
doi: 10.11604/pamj.2022.43.79.29159
pii: PAMJ-43-79
pmc: PMC9789786
doi:
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
79Informations de copyright
Copyright: Terkaa Atim et al.
Déclaration de conflit d'intérêts
The authors declare no competing interests.
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