Ganglioneuromas across age groups: Systematic review of individual patient data

Abstract

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Presentation and outcomes of adrenal ganglioneuromas: A cohort study and a systematic review of literature

“…Interestingly, only one other study in our systematic review reported 3 out of 27 patients with AGN having composite AGN‐PHEO 10 . A recent systematic review of all‐site ganglioneuromas that included both case series and case reports described a total of 43 non‐neuroblastic composite ganglioneuromas out of 364 patients 30 . This difference seen between the large number of composite tumours in our cohort compared with those included in our systematic review, as well as the systematic review by Fliedner et al, is noteworthy.…”

“…Patients in our single‐centre and systematic review experienced no recurrence or AGN‐specific mortality. A recent systematic review of patients with ganglioneuromas at any site also demonstrated that patients with these tumours have an excellent prognosis 30 . This suggests that patients with pathologically proven AGN do not need a long‐term follow‐up.…”

“…Possible explanations for this difference include potential under-reporting, exclusion of composite tumours from previous studies, under-recognition of this association or referral bias.Patients in our single-centre and systematic review experienced no recurrence or AGN-specific mortality. A recent systematic review of patients with ganglioneuromas at any site also demonstrated that patients with these tumours have an excellent prognosis 30. This suggests that patients with pathologically proven AGN do not need a long-term follow-up.…”

Gegenüberstellung zweier Schwann-Zell-haltiger orbitaler Tumoren: Schwannom und Ganglioneurom

“…Das Ganglioneurom hingegen geht von Sympathogonien der Neuralleiste entlang der sympathischen Achse aus und tritt vor allem im Abdomen und Becken inkl. der Nebennierenrinde auf, ge-folgt von einer Lokalisation im Hals-Nacken-Bereich sowie dem Thorax [8]. Eine primäre orbitale Lokalisation ist extrem selten und wurde bisher erst in 3 Fällen berichtet [5,9].…”

“…Hierbei wird angenommen, dass möglicherweise Reste der Neuroblastomzellen zu reifen Ganglioneuromen differenzierten. Zudem wurde eine Assoziation von Ganglioneuromen mit Neurofibromatose 1 und der multiplen endokrinen Neoplasie Typ 2 (MEN 2) beschrieben [8].…”