Treatment of malignant pheochromocytoma.
Treatment of malignant pheochromocytoma.
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DOI：
10.1055/s-0029-1231025
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发表时间：
2009-09
期刊：
影响因子：
--
通讯作者：
Lehnert H
中科院分区：
文献类型：
--
作者：
Adjallé R;Plouin PF;Pacak K;Lehnert H
嗜铬细胞瘤（PCC）是一种罕见的疾病，主要是散发的，但也与一些家族性疾病有关，恶性肿瘤的频率约为10%。只有来自大多形性染色质细胞的远处转移被广泛接受为恶性肿瘤的标准。各种症状可能由儿茶酚胺的产生和释放引起。由于恶性PCC无法治愈，且预后不良，因此保证生活质量是主要的治疗目标之一。除了使用选择性α-1阻滞剂和非选择性、非竞争性α-和/或β-阻滞剂长期治疗症状外，减脂手术是治疗的第一步。如果123I-MIBG在靶向放射治疗中有足够的吸收，在减容手术后，使用131I-MIBG作为辅助治疗是一种选择。对于mibg扫描未呈阳性、131I-MIBG无反应或放射性核素治疗后进展的患者，特别是增殖指数高的患者，应应用化疗。最有效的化疗方案似乎是cvd方案，包括环磷酰胺、长春新碱和达卡巴嗪。所谓的靶向分子疗法，即替莫唑胺和沙利度胺联合治疗，或舒尼替尼单药治疗，以及新型治疗性生长抑素类似物，已经显示出有希望的结果，因此应该鼓励临床试验，以改善转移性PCC的预后。在这篇综述中，目前的治疗方式和新的分子策略在这种疾病的管理进行了讨论，并提出了一种治疗算法。
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 α-1 blockers and nonselective, noncompetitive α- and / or β-blockers, debulking surgery is the first treatment step. In case of a sufficient uptake of 123I-MIBG treatment with targeted radiation therapy, use of 131I-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 131I-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 and should thus encourage clinical trials to improve the prognosis of metastatic PCC. Within this review the current treatment modalities and novel molecular strategies in the management of this disease are discussed and a treatment algorithm is suggested.
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影响因子：
3.8
作者：
Frilling, Andrea;Weber, Frank;Broelsch, Christoph E.
通讯作者：
Broelsch, Christoph E.
DOI：
10.1007/s00259-007-0652-6
发表时间：
2008-04-01
影响因子：
9.1
作者：
Gedik, Gonca Kara;Hoefnagel, Cornelis A.;Olmos, Renato A. Valdes
通讯作者：
Olmos, Renato A. Valdes
影响因子：
3.2
作者：
Grossrubatscher, Erika;Dalino, Paolo;Loli, Paola
通讯作者：
Loli, Paola
影响因子：
9
作者：
Goldstein, RE;O'Neill, JA;Scott, HW
通讯作者：
Scott, HW
影响因子：
19.7
作者：
Hoegerle, S;Nitzsche, E;Neumann, HPH
通讯作者：
Neumann, HPH