Pheochromocytoma and paraganglioma in children: A review of medical and surgical management at a tertiary care center

Tuan H. Pham, Christopher Moir, Geoffrey B. Thompson, Abdalla E. Zarroug, Chad E. Hamner, David Farley, Jon Van Heerden, Aida N. Lteif, William Francis Young

Research output: Contribution to journalArticle

101 Citations (Scopus)

Abstract

OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children. METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients <18 years of age with histologically confirmed pheochromocytoma or paraganglioma. RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of >6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 ± 32 months. CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of >6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.

Original languageEnglish (US)
Pages (from-to)1109-1117
Number of pages9
JournalPediatrics
Volume118
Issue number3
DOIs
StatePublished - Sep 2006

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Paraganglioma
Pheochromocytoma
Tertiary Care Centers
Survival Rate
Mortality
Databases
Morbidity
Mutation
Incidence
Neoplasms

Keywords

  • Children
  • Paraganglioma
  • Pheochromocytoma
  • Surgery

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Pheochromocytoma and paraganglioma in children : A review of medical and surgical management at a tertiary care center. / Pham, Tuan H.; Moir, Christopher; Thompson, Geoffrey B.; Zarroug, Abdalla E.; Hamner, Chad E.; Farley, David; Van Heerden, Jon; Lteif, Aida N.; Young, William Francis.

In: Pediatrics, Vol. 118, No. 3, 09.2006, p. 1109-1117.

Research output: Contribution to journalArticle

Pham, TH, Moir, C, Thompson, GB, Zarroug, AE, Hamner, CE, Farley, D, Van Heerden, J, Lteif, AN & Young, WF 2006, 'Pheochromocytoma and paraganglioma in children: A review of medical and surgical management at a tertiary care center', Pediatrics, vol. 118, no. 3, pp. 1109-1117. https://doi.org/10.1542/peds.2005-2299
Pham, Tuan H. ; Moir, Christopher ; Thompson, Geoffrey B. ; Zarroug, Abdalla E. ; Hamner, Chad E. ; Farley, David ; Van Heerden, Jon ; Lteif, Aida N. ; Young, William Francis. / Pheochromocytoma and paraganglioma in children : A review of medical and surgical management at a tertiary care center. In: Pediatrics. 2006 ; Vol. 118, No. 3. pp. 1109-1117.
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abstract = "OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children. METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients <18 years of age with histologically confirmed pheochromocytoma or paraganglioma. RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64{\%}), palpitation (53{\%}), headache (47{\%}), and mass-related effects (30{\%}). Nine patients (30{\%}) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47{\%}) had malignant disease, whereas 16 (53{\%}) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of >6 cm. Surgical resection was performed for 28 patients (93{\%}), with perioperative mortality and major morbidity rates of 0{\%} and 10{\%}, respectively. Resection achieved symptomatic relief for 25 patients (83{\%}). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78{\%} and 31{\%}, respectively, and the mean survival time was 157 ± 32 months. CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47{\%}), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of >6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.",
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author = "Pham, {Tuan H.} and Christopher Moir and Thompson, {Geoffrey B.} and Zarroug, {Abdalla E.} and Hamner, {Chad E.} and David Farley and {Van Heerden}, Jon and Lteif, {Aida N.} and Young, {William Francis}",
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AU - Pham, Tuan H.

AU - Moir, Christopher

AU - Thompson, Geoffrey B.

AU - Zarroug, Abdalla E.

AU - Hamner, Chad E.

AU - Farley, David

AU - Van Heerden, Jon

AU - Lteif, Aida N.

AU - Young, William Francis

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N2 - OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children. METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients <18 years of age with histologically confirmed pheochromocytoma or paraganglioma. RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of >6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 ± 32 months. CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of >6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.

AB - OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children. METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients <18 years of age with histologically confirmed pheochromocytoma or paraganglioma. RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of >6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 ± 32 months. CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of >6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.

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KW - Pheochromocytoma

KW - Surgery

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