Anesthesia management of a ganglioneuroma with seizures presenting as pheochromocytoma

Abstract

Ganglioneuromas (GN) are neural crest cell-derived tumors which may coexist with pheochromocytomas, secrete various neuropeptides or the symptoms may mimic that of a pheochromocytoma, producing hypertension or a hypotensive crisis during anesthesia for these tumors. We report here the case of a 7-year-old female child with an adrenal tumor suspected to be a pheochromocytoma, later confirmed by histology as a GN. This child presented with episodic headache, anxiety, palpitations and 3D helical (spiral) computed tomography of the abdomen revealed an adrenal tumor. In addition, the child was diagnosed to have a seizure disorder. She was managed as a pheochromocytoma. Although the child's preoperative catecholamine levels were normal, tumor manipulation caused a hypertensive crisis. We discuss the clinical characteristics of this unusual case, anesthesia management, and the postoperative course.

A not so incidental ‘incidentaloma’ − pediatric ganglioneuroma-associated cerebellar degeneration and super-refractory status epilepticus: case report and literature review

“…In general, reports of pediatric neurological paraneoplastic syndromes are rare ( 23 ). The most common are opsoclonus-myoclonus syndrome which is often antibody-negative and associated with neuroblastoma (50%), NMDAR AE or classic limbic encephalitis secondary to anti-NMDAR-IgG with an underlying ovarian teratoma (at much lower rates than adults), anti-Ma2 (di)encephalitis classically associated with testicular germ cell tumors, and anti-acetylcholine receptor positive myasthenia gravis associated with thymic hyperplasia or thymoma (more often in adults) ( 25 ).…”

Peri‐operative management of a child with ROHHAD‐NET syndrome undergoing neural crest tumour excision

“…Due to their close proximity to the neural tube, these tumours have the ability to secrete various vasoactive peptides. In rare cases, such tumours may co-exist with disorders such as neurofibromatosis, Beckwith-Wiedemann syndrome or Di-George syndrome [10]. Excision of neural crest tumours requires meticulous surgical dissection.…”

Drug-resistant epilepsy, early-onset hypertension and white matter lesions: a hidden paraganglioma

“…In this case, there was no evidence of excess of catecholamines, and although the authors postulated that intraoperative manipulation of the vagal nerve contributed to seizure remission, the origin of epilepsy remains unclear. In another case, a 7-year-old child was commenced on antiepileptic drugs following an acute presentation with headache, anxiety, palpitations and unilateral tonic–clonic seizures; 2 years later, she was found to have an adrenal ganglioneuroma, with intraoperative crisis suggestive of catecholamine excess 11. It is unclear what the course of the seizure disorder was prior to and following surgery.…”