Paraganglioma: Not just an extra-adrenal pheochromocytoma

Amanda M. Laird, Paul G. Gauger, Gerard M. Doherty, Barbra S. Miller

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Purpose: Pheochromocytoma (PCC) and paraganglioma (PG) are evaluated and treated similarly. This study evaluates the hypothesis that tumor characteristics and outcome of patients with PCC and PG are equivalent. Methods: Records of patients from a single institution undergoing resection of PCC or PG from 1999 to 2010 were reviewed. Data were collected for demographics, operative records, laboratory and pathologic results, adjuvant and palliative therapy given, recurrence, and length of survival. Descriptive statistics were used to describe differences between patients with benign and malignant PCC and PG. Analysis was performed using the Wilcoxon-Mann- Whitney test with p=0.05 considered as significant. Results: One hundred fifteen patients were identified (106 PCC and nine PG). Of the tumors, 5.2% were bilateral and 10.4% were malignant. Forty-three of the 115 patients underwent genetic testing; 21out of 37 (56.8%) PCC and five out of six (83.3%) PG had a genetic mutation. Twelve patients (seven PCC and five PG) had malignant tumors. Malignant PG (mPG) exhibited more invasive pathologic characteristics. The median sizes of benign and malignant PCC (mPCC) were 4.0 (0.7-14 cm) and 5.5 cm (3.7- 11.2 cm), respectively, p=0.03. The median sizes of benign and mPG were 4.1 (2.7-5.4 cm) and 5.8 cm (4-6.2 cm), respectively, p=0.11. Sites of recurrence were similar between the groups. Patients with mPG received chemotherapy more often than those with mPCC. With a median follow-up of 54.7 months (2.0-185.3), two out of five mPG and zero out of seven mPCC had died of the disease. Conclusion: Tumor size does not appear to correlate with malignancy in a clinically significant manner. Malignant paraganglioma may be more aggressive than malignant pheochromocytoma and is frequently offered more adjuvant therapy. PCC and PG should be evaluated separately in future analyses of these diseases.

Original languageEnglish (US)
Pages (from-to)247-253
Number of pages7
JournalLangenbeck's Archives of Surgery
Volume397
Issue number2
DOIs
StatePublished - Feb 2012
Externally publishedYes

Fingerprint

Paraganglioma
Pheochromocytoma
Neoplasms
Recurrence
Genetic Testing
Palliative Care
Demography
Drug Therapy

Keywords

  • Adrenal
  • Adrenal cancer
  • Malignant paraganglioma
  • Malignant pheochromocytoma
  • Paraganglioma
  • Pheochromocytoma

ASJC Scopus subject areas

  • Surgery

Cite this

Laird, A. M., Gauger, P. G., Doherty, G. M., & Miller, B. S. (2012). Paraganglioma: Not just an extra-adrenal pheochromocytoma. Langenbeck's Archives of Surgery, 397(2), 247-253. https://doi.org/10.1007/s00423-011-0871-y

Paraganglioma : Not just an extra-adrenal pheochromocytoma. / Laird, Amanda M.; Gauger, Paul G.; Doherty, Gerard M.; Miller, Barbra S.

In: Langenbeck's Archives of Surgery, Vol. 397, No. 2, 02.2012, p. 247-253.

Research output: Contribution to journalArticle

Laird, AM, Gauger, PG, Doherty, GM & Miller, BS 2012, 'Paraganglioma: Not just an extra-adrenal pheochromocytoma', Langenbeck's Archives of Surgery, vol. 397, no. 2, pp. 247-253. https://doi.org/10.1007/s00423-011-0871-y
Laird, Amanda M. ; Gauger, Paul G. ; Doherty, Gerard M. ; Miller, Barbra S. / Paraganglioma : Not just an extra-adrenal pheochromocytoma. In: Langenbeck's Archives of Surgery. 2012 ; Vol. 397, No. 2. pp. 247-253.
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abstract = "Purpose: Pheochromocytoma (PCC) and paraganglioma (PG) are evaluated and treated similarly. This study evaluates the hypothesis that tumor characteristics and outcome of patients with PCC and PG are equivalent. Methods: Records of patients from a single institution undergoing resection of PCC or PG from 1999 to 2010 were reviewed. Data were collected for demographics, operative records, laboratory and pathologic results, adjuvant and palliative therapy given, recurrence, and length of survival. Descriptive statistics were used to describe differences between patients with benign and malignant PCC and PG. Analysis was performed using the Wilcoxon-Mann- Whitney test with p=0.05 considered as significant. Results: One hundred fifteen patients were identified (106 PCC and nine PG). Of the tumors, 5.2{\%} were bilateral and 10.4{\%} were malignant. Forty-three of the 115 patients underwent genetic testing; 21out of 37 (56.8{\%}) PCC and five out of six (83.3{\%}) PG had a genetic mutation. Twelve patients (seven PCC and five PG) had malignant tumors. Malignant PG (mPG) exhibited more invasive pathologic characteristics. The median sizes of benign and malignant PCC (mPCC) were 4.0 (0.7-14 cm) and 5.5 cm (3.7- 11.2 cm), respectively, p=0.03. The median sizes of benign and mPG were 4.1 (2.7-5.4 cm) and 5.8 cm (4-6.2 cm), respectively, p=0.11. Sites of recurrence were similar between the groups. Patients with mPG received chemotherapy more often than those with mPCC. With a median follow-up of 54.7 months (2.0-185.3), two out of five mPG and zero out of seven mPCC had died of the disease. Conclusion: Tumor size does not appear to correlate with malignancy in a clinically significant manner. Malignant paraganglioma may be more aggressive than malignant pheochromocytoma and is frequently offered more adjuvant therapy. PCC and PG should be evaluated separately in future analyses of these diseases.",
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