Primary renal ganglioneuroblastoma in an adult

Jalleh, R. P.; Newman, P. L.; Jenkins, M. T.; Sturdy, D. E.

doi:10.1136/pgmj.66.780.869

Cited by 7 publications

(2 citation statements)

References 5 publications

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“…Primary renal GNB was described as a rare clinical entity nearly 30 years ago, and was considered to originate either in sequestered adrenal rests during the fetal life or the intrarenal sympathetic ganglia, rather than resulting from direct invasion from an adrenal neuroblastic tumor 1 . Moreover, a GNB is much less likely to originate in the kidney than a neuroblastoma, another subtype of PNT.…”

Section: Figmentioning

confidence: 99%

Primary renal ganglioneuroblastoma mimicking Wilms' tumor in a 3‐year‐old girl

Wang

2021

Pediatrics International

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Section: Figmentioning

confidence: 99%

Primary renal ganglioneuroblastoma mimicking Wilms' tumor in a 3‐year‐old girl

Wang

2021

Pediatrics International

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“…6,[25][26][27][28][29][30][31][32] In the neuroblastoma group, the median age was 32 (16.1-56). There were 9 males and 17 females.…”

Section: Discussionmentioning

confidence: 99%

Functional Retroperitoneal Neuroblastoma and Ganglioneuroblastoma in Adults

Mahkamova¹,

Harrison²

2014

World Journal of Endocrine Surgery

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Ganglioneuroblastoma and neuroblastoma are malignant catecholamine-secreting tumors arising from neural crest tissue. Thirty-eight cases of functional retroperitoneal extra-adrenal neuroblastoma and ganglioneuroblastoma in adults are reported in English literature. These tumors behave more aggressively in adults compared to children and have very poor prognosis. We report four adults who presented with retroperitoneal extraadrenal ganglioneuroblastoma and neuroblastoma between 2008 and 2012. Median age at presentation was 28.5 years (21-40 years). One patient was in late stages of pregnancy. Three patients presented with local pressure effects from the tumor and one patients manifested clinical features of catecholamine excess. Biochemical analysis showed raised urinary dopamine excretion in all our patients. Urinary noradrenaline and meta nephrines were elevated in three cases. Computed tomography/magnetic resonance imaging/meta-iodobenzylguanidine (CT/MRI/MIBG) confirmed stage IIB disease in one patient, stage III disease one patient and stage IV in two cases. Preoperative biopsy confirmed diagnosis in two cases. Complete surgical resection was performed in patient with stage II B disease and partial surgical resection was feasible in other cases. Histological diagnosis was ganglioneuroblastoma in two cases and neuroblastoma in two cases. Meta-iodobenzylglunidine/radiotherapy and chemotherapy were used for residual disease. Urinary catecholamines and metanephrines normalized after complete tumor resection but remained elevated in residual disease. Survival for neuroblastoma was 15 and 21 months after the surgery and 29 months for ganglioneuroblastoma. One patient remains free of disease at 6 months. Functioning ganglioneuroblastoma and neuroblastoma are rare in adults and only one other case ganglioneuroblastoma in pregnancy is reported in the world literature. Measurement of plasma/urinary excretion of catecholamines and their precursors and metabolites is helpful for diagnosis of neuroblastoma and ganglioneuroblastoma. Due to late presentation surgical resection is unlikely to be curative. Three of our patients developed local recurrence of disease. Urinary/plasma assay are used for monitoring disease relapse. Long-term survival is unusual.

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