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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
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