Pheochromocytoma Support Foundation

  Shining the light on pheochromocytoma awareness through education, early detection, and support. 

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Treatment of a Pheochromocytoma

Information coming soon. 

Treatment of a Malignant Pheochromocytoma

Pheochromocytoma (PCC) is a rare disease, mainly sporadic, but also associated with some familial disorders, with a malignancy frequency of approximately 10%. Only the presence of distant metastases, derived from large pleomorphic chromaffin cells, is widely accepted as a criterion of malignancy. 

Variable symptoms may be caused by production and release of catecholamines. Since there is no curative treatment for malignant PCC and due to its unfavorable prognosis, assuring quality of life is one of the main therapeutic objectives. Besides a long-term medical treatment of symptoms using selective alpha-1 blockers and nonselective, noncompetitive alpha- and/or beta-blockers, debulking surgery is the first treatment step. 

In case of a sufficient uptake of (123)I-MIBG treatment with targeted radiation therapy, use of (131)I-MIBG is an option as an adjuvant therapy, following debulking surgery. 

Chemotherapy should be applied to patients without positive MIBG-scan, with no response to (131)I-MIBG or progression after radionuclide treatment, and especially in cases with high proliferation index. The most effective chemotherapy regimen appears to be the CVD-scheme, including cyclophosphamide, vincristine, and dacarbazine. The so-called targeted molecular therapies with treatment combinations of temozolomide and thalidomide, or sunitinib monotherapy, and novel therapeutic somatostatin analogues have shown promising results to improve the prognosis of metastatic PCC.

Adjallé, R., Plouin, P. F., Pacak, K., & Lehnert, H. (2009). Treatment of malignant pheochromocytoma. Hormone and Metabolic Research = Hormon- Und Stoffwechselforschung = Hormones Et Métabolisme, 41(9), 687.

Current Progress and Future Challenges in the Biochemical Diagnosis and Treatment of Pheochromocytomas and Paragangliomas

Findings from five independent studies - with close to 350 patients with pheochromocytoma and more than 2,500 in whom the tumor was excluded - indicate that measurements of plasma free metanephrines provide an overall diagnostic sensitivity of 98% and specificity of 92%. The recommendation that initial testing for the tumor should always include measurements of either plasma or urinary fractionated metanephrines results from recognition of the high diagnostic sensitivity of measurements of plasma metanephrines. The few patients with pheochromocytoma in whom the test may not yield a positive result include those with very small tumors or microscopic disease and others with tumors that do not produce norepinephrine and epinephrine. Such patients are typically normotensive and do not exhibit symptoms of catecholamine excess. Additional measurements of methoxytyramine can be useful for detecting those tumors that produce only dopamine. Suboptimal diagnostic specificity and difficulties in distinguishing true- from false-positive elevations of plasma metanephrines remain challenges for diagnosis. Improvements in analytical technology (e.g., liquid chromatography with tandem mass spectrometry) and new strategies for follow-up testing provide possible solutions to these problems. The single most important remaining clinical care challenge is the development of effective cures for patients with malignant disease. Current treatments, none of which are truly satisfactory, include chemotherapy and radiopharmaceutical therapy with (131)I-labelled M-iodobenzylguanidine or radioactive somatostatin analogues.
Eisenhofer, G, Siegert, G, Kotzerke, J, Bornstein, S R, & Pacak, K. (2008). Current progress and future challenges in the biochemical diagnosis and treatment of pheochromocytomas and paragangliomas. Hormone and Metabolic Research = Hormon- Und Stoffwechselforschung = Hormones Et Métabolisme, 40(5), 329.

Diagnosis and Treatment of a Pheochromocytoma During Pregnancy

The data of four cases of pheochromocytoma was analyzed retrospectively. Their ages were 41, 28, 32 and 30 years old, and the four patients were at 32nd week, 12th week, 14th week and 13th week of gestation. 

All patients had hypertension during pregnancy, accompanied with headache, dizziness, palpitation and sweating. 

The 24-h urinary catecholamines (24 h UCA) increased significantly. Ultrasound and MRI confirmed the diagnosis of pheochromocytoma. 

Results: One case had Cesarean section at 32 weeks of gestation, and a healthy baby girl was delivered smoothly. Laparoscopic resection of the right adrenal pheochromocytoma was performed at the same time, and an adrenal tumor of 7.0 cm was resected successfully. 

Two cases chose abortion and laparoscopic resection of pheochromocytoma was performed. 

One case chose abortion and refused further treatment. Histopathology confirmed the diagnosis of pheochromocytoma. 

Conclusions: For hypertension in pregnant women during pregnancy, typical paroxysmal hypertension accompanied by triad of headache, palpitation and sweating, pheochromocytoma should be considered. 

Early diagnosis can reduce the maternal and fetal mortality significantly. Second trimester of pregnancy is the ideal time for surgical treatment. Laparoscopic resection of pheochromocytoma during pregnancy is safe and effective. 

Dong, D., & Li, H. (2014). Diagnosis and treatment of pheochromocytoma during pregnancy. Journal Of Maternal-Fetal & Neonatal Medicine, 27(18), 1930-1934. doi:10.3109/14767058.2014.880883