Retroperitoneal composite pheochromocytoma-ganglioneuroma: a case report and review of literature

Hu, Jinchen; Wu, Jitao; Cai, Li; Jiang, Lei; Lang, Zhiqiang; Qu, Guimei; Liu, Houcai; Yang, Wei‐Dong; Yu, Guohua

doi:10.1186/1746-1596-8-63

Cited by 29 publications

(29 citation statements)

References 15 publications

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“…These tumours are quite rare and show features of PHEO/PGL combined with neurogenic features, such as Schwann cells embedded in ganglion cells as seen in ganglioneuromas. These tumours typically occur in the adrenal gland (50‐70 reported cases) but can rarely be found in the retroperitoneum (5 case reports), as in our case . There is a known association between composite PHEO–ganglioneuroma tumours and NF1, but no case reports have shown an extra‐adrenal composite tumour in the setting of NF1 …”

Section: Discussionmentioning

confidence: 60%

Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1

et al. 2016

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Section: Discussionmentioning

confidence: 60%

Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1

et al. 2016

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“…Slightly more than 20 cases have been reported. Majority of the cases were noted in the retroperitoneum and urinary bladder [104][105][106][107][108][109][110][111][112]. Cases have been described in cauda equine and posterior mediastinum [113,114].…”

Section: Composite Paragangliomamentioning

confidence: 99%

“…Some of the cases was symptomatic with catecholamine-related signs [104]. Majority of the reported cases consisted of paraganglioma and ganglioneuroma [104,[106][107][108][109][110][111][112][113][114]. Also, there are a few examples of composite paraganglioma and neuroblastoma [105,115,116].…”

Section: Composite Paragangliomamentioning

confidence: 99%

Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours

2017

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The fourth edition of the World Health Organization (WHO) classification of endocrine tumours contains substantial new findings for the adrenal tumours. The tumours are presented in two chapters labelled as "Tumours of the adrenal cortex" and "Tumours of the adrenal medulla and extra-adrenal paraganglia." Tumours of the adrenal cortex are classified as cortical carcinoma, cortical adenoma, sex cord stromal tumours, adenomatoid tumour, mesenchymal and stromal tumours (myelolipoma and schwannoma), haematological tumours, and secondary tumours. Amongst them, schwannoma and haematological tumours are newly documented. The major updates in adrenal cortical lesions are noted in the genetics of the cortical carcinoma and cortical adenoma based on the data from The Cancer Genome Atlas (TCGA). Also, a system for differentiation of oncocytoma from oncocytic cortical carcinoma is adopted. Tumours of the adrenal medulla and extra-adrenal paraganglia comprise pheochromocytoma, paraganglioma (head and neck paraganglioma and sympathetic paraganglioma), neuroblastic tumours (neuroblastoma, nodular ganglioneuroblastoma, intermixed ganglioneuroblastoma, and ganglioneuroma), composite pheochromocytoma, and composite paraganglioma. In this group, neuroblastic tumours are newly included in the classification. The clinical features, histology, associated pathologies, genetics, and predictive factors of pheochromocytoma and paraganglioma are the main changes introduced in this chapter of WHO classification of endocrine tumours. The term "metastatic pheochromocytoma/paraganglioma" is used to replace "malignant pheochromocytoma/paraganglioma." Also, composite pheochromocytoma and composite paraganglioma are now documented in separate sections instead of one. Overall, the new classification incorporated new data on pathology, clinical behaviour, and genetics of the adrenal tumours that are important for current management of patients with these tumours.

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“…Watery diarrhoea has been described in some cases attributable to increased secretion of vasoactive intestinal peptides. 10 and non-pheochromocytoma components were capable of secreting VIP. This further supported its common origin.…”

Section: Discussionmentioning

confidence: 97%

Retroperitoneal composite pheochromocytoma/paraganglioma complicating pregnancy: a rare case report

et al. 2015

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Pheochromocytoma is a tumor of adrenal medulla. Pheochromocytoma arising outside the adrenal medulla is commonly called paraganglioma. These tumours arise from paraganglia of the autonomic nervous system, most commonly occurring in head and neck region, and much less frequently in the retroperitoneum. But when they occur below the diaphragm in the organ of zuckerkandl or retroperotoneum, they are also called as extra adrenal pheochromocytoma. Composite pheochromocytoma/paraganglioma is a tumor composed of pheochromocytoma/ paraganglioma along with another tumor of neural crest origin composed of either a ganglio-neuroma/ ganglioneuroblastoma /neuroblastoma or schwannoma. They are mostly located in the adrenal gland, while extra adrenal composite pheochromocytoma is extremely rare. Only 4 cases in the retroperitoneum have been described in the literature. Composite tumors of the adrenal medulla are rare tumors accounting for less than 3% of all sympathoadrenal tumors. We report a rare case of retroperitoneal extra adrenal composite pheochromocytoma in a 19 year old female with a history of 3 months amenorrhoea.

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Retroperitoneal composite pheochromocytoma-ganglioneuroma: a case report and review of literature

Cited by 29 publications

References 15 publications

Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1

Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1

Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours

Retroperitoneal composite pheochromocytoma/paraganglioma complicating pregnancy: a rare case report

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