Composite Pheochromocytoma-Ganglioneuroma:A Rare Experiment of Nature

Khan, Amna; Solomon, Solomon S.; Childress, Richard Dale

doi:10.4158/ep09205.ra

Cited by 42 publications

(69 citation statements)

References 25 publications

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“…[1][2][3][4][5][6][7][8] The tumors appear to have a slight female preponderance (1.32:1), and were bilateral in approximately 5% of patients. The median age of patients at presentation was 54 years, although it ranged from 21 to 82 years.…”

Section: Discussionmentioning

confidence: 99%

“…Tumors may also include a third component and at least two cases in the literature have included a cortical adenoma along with a composite pheochromocytomaganglioneuroma. 1,8 There has been little long-term follow-up of patients with composite pheochromocytomas and the clinical course of these rare tumors have not been well established. Overall, the reported malignancy rate for composite tumors is about 25%, 1 and is typically associated with ganglioneuroblastomas, malignant peripheral nerve sheath components or neuroendocrine carcinomas.…”

Section: Discussionmentioning

confidence: 99%

“…1,8 There has been little long-term follow-up of patients with composite pheochromocytomas and the clinical course of these rare tumors have not been well established. Overall, the reported malignancy rate for composite tumors is about 25%, 1 and is typically associated with ganglioneuroblastomas, malignant peripheral nerve sheath components or neuroendocrine carcinomas. The reported risk of malignancy for pheochromocytoma-ganglioneuromas is much lower at about 3%, although follow-up is not always available ( Table 1).…”

Section: Discussionmentioning

confidence: 99%

“…1,3-5 Calcitonin production has also been reported. 1 Overall, 75% of patients have some preoperative evidence of functionality (Table 1). Although our patient's tumor was clearly functional based on her laboratory studies, these were not available at the time of her appendectomy.…”

Section: Discussionmentioning

confidence: 99%

“…Many patients with pheochromocytoma-paragangliomas have symptoms typical of classic pheochromocytomas as they produce catecholamines and their metabolites. 1 Patients may also rarely present with a watery diarrhea-hypokalemiaachlorhydria (WDHA) syndrome due to the secretion of vasoactive intestinal peptide (VIP). 1,3-5 Calcitonin production has also been reported.…”

Section: Discussionmentioning

confidence: 99%

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Incidental Finding of Composite Pheochromocytoma-Ganglioneuroma: Successful Management after Emergent Appendectomy and Review of the Literature

Royer¹,

Miron²,

Kirby³

et al. 2011

WJOES

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Composite pheochromocytoma-ganglioneuromas are rare adrenal tumors. They may be functional and while mostly sporadic, they may be associated with several genetic syndromes. Herein, we present the youngest reported patient with this tumor, which was incidentally discovered by CT scan performed prior to emergent appendectomy. The tumor was functional and she underwent a successful laparoscopic adrenalectomy with resolution of her biochemical abnormalities. Surgeons should be aware of this rare entity due to its uncertain long-term prognosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

Incidental Finding of Composite Pheochromocytoma-Ganglioneuroma: Successful Management after Emergent Appendectomy and Review of the Literature

Royer¹,

Miron²,

Kirby³

et al. 2011

WJOES

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Ganglioneuroma: An Overview

Erem

2012

Tumors of the Central Nervous System, Volume 9

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Composite phaeochromocytomas—a systematic review of published literature

Dhanasekar

Visakan

Tahir

et al. 2021

Langenbecks Arch Surg

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Introduction Composite phaeochromocytoma is a tumour containing a separate tumour of neuronal origin in addition to a chromaffin cell tumour. This study reports on two cases from a single centre’s records and presents a systematic literature review of composite phaeochromocytomas. Methods In addition to describing 2 case reports, a systematic search of the Medline database from inception up to April 2020 was done for human case reports on composite phaeochromocytomas. Relevant titles and/or abstracts were screened, and full texts were reviewed to identify appropriate studies. Data was extracted and a descriptive analysis of presentation, clinical features, management strategies and outcomes was performed. The quality of included studies was assessed using a critical appraisal checklist. Results There were 62 studies included, with a total of 94 patients. Of 91 patients where data was available, the median (range) age of patients was 48 (4–86) years. Of 90 patients where information was provided, 57% were female. In at least 28% of patients, a genetic cause was identified. Common presenting features include abdominal pain, palpable mass, cardiovascular and gastrointestinal symptoms. The most common tumour component with phaeochromocytoma is ganglioneuroma; other components include ganglioneuroblastoma, neuroblastoma and malignant peripheral nerve sheath tumours. In patients with follow-up data (n=48), 85% of patients were alive and well at a median (range) follow-up time of 18 (0.5–168) months. Conclusion Composite phaeochromocytoma is a rare tumour, with a significant genetic predisposition. This review summarises available epidemiological data, which will be useful for clinicians managing this rare condition.

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Composite Pheochromocytoma-Ganglioneuroma:A Rare Experiment of Nature

Cited by 42 publications

References 25 publications

Incidental Finding of Composite Pheochromocytoma-Ganglioneuroma: Successful Management after Emergent Appendectomy and Review of the Literature

Incidental Finding of Composite Pheochromocytoma-Ganglioneuroma: Successful Management after Emergent Appendectomy and Review of the Literature

Ganglioneuroma: An Overview

Composite phaeochromocytomas—a systematic review of published literature

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