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Abstract：
BACKGROUND: Malignant pheochromocytomas are rare endocrine tumors that develop within chromaffin tissue. The diagnosis of malignancy is based on neoplastic recurrence or the presence of metastasis in organs that lack chromaffin tissue. We report a series of four cases because of their diagnostic and therapeutic particularities.
METHODS: we describe four clinical cases of patients with malignant pheochromocytoma whose Menard triad \"headache-palpitations-sweating\" was present in three out of four patients, the methoxylated derivatives were measured in 4 patients, 3 of which had high values, all of our patients carried out a CT scan which objectified signs of malignancy, MRI was performed on a single patient; presenting with a neoplastic recurrence; looking for a locoregional invasion.
CONCLUSIONS: Pheochromocytoma (PC) is a rare neuroendocrine tumor derived from the chromaffin cells of the adrenal medulla. Its annual incidence is 2 to 8 per million adults. A peak frequency is observed between 30 and 40 years of age. Approximately 10% of pheochromocytomas are malignant and in 10% of cases, bilateral localization is observed. Criteria for malignancy include the invasion of neighboring organs, a large tumor, the presence of lymphadenopathy on imaging, or fixation on scintigraphy. Surgery for MAP is not always curative. In the case of multiple liver metastases, treatment is based on adrenalectomy, which can be effectively combined with chemoembolization, cryoablation, or radiofrequency techniques.
CONCLUSIONS: The main prognostic factors of the malignant pheochromocytomas are a large tumor volume, the existence or number of visceral metastases, and the presence of a mutation in the SDHB (Succinate dehydrogenase B) gene.
METHODS: we describe four clinical cases of patients with malignant pheochromocytoma whose Menard triad \"headache-palpitations-sweating\" was present in three out of four patients, the methoxylated derivatives were measured in 4 patients, 3 of which had high values, all of our patients carried out a CT scan which objectified signs of malignancy, MRI was performed on a single patient; presenting with a neoplastic recurrence; looking for a locoregional invasion.
CONCLUSIONS: Pheochromocytoma (PC) is a rare neuroendocrine tumor derived from the chromaffin cells of the adrenal medulla. Its annual incidence is 2 to 8 per million adults. A peak frequency is observed between 30 and 40 years of age. Approximately 10% of pheochromocytomas are malignant and in 10% of cases, bilateral localization is observed. Criteria for malignancy include the invasion of neighboring organs, a large tumor, the presence of lymphadenopathy on imaging, or fixation on scintigraphy. Surgery for MAP is not always curative. In the case of multiple liver metastases, treatment is based on adrenalectomy, which can be effectively combined with chemoembolization, cryoablation, or radiofrequency techniques.
CONCLUSIONS: The main prognostic factors of the malignant pheochromocytomas are a large tumor volume, the existence or number of visceral metastases, and the presence of a mutation in the SDHB (Succinate dehydrogenase B) gene.
摘要：
背景：恶性嗜铬细胞瘤是在嗜铬细胞组织内发展的罕见内分泌肿瘤。恶性肿瘤的诊断基于肿瘤复发或缺乏嗜铬细胞组织的器官中转移的存在。由于其诊断和治疗的特殊性，我们报告了四例病例。
方法：我们描述了4例恶性嗜铬细胞瘤患者的临床病例，其中四分之三的患者存在Menard三联症“头痛-心悸-出汗”，在4名患者中测量了甲氧基化衍生物，其中3个具有很高的价值，我们所有的病人都进行了CT扫描，客观地显示了恶性肿瘤的迹象，对一名患者进行了MRI检查；出现肿瘤复发；寻找局部侵袭。
结论：嗜铬细胞瘤（PC）是一种罕见的神经内分泌肿瘤，来源于肾上腺髓质的嗜铬细胞。其年发病率为每百万成年人2至8人。在30至40岁之间观察到峰值频率。大约10％的嗜铬细胞瘤是恶性的，在10％的病例中，观察到双边定位。恶性肿瘤的标准包括侵犯邻近器官，一个大肿瘤,影像学上淋巴结病的存在，或固定在闪烁显像上。MAP的手术并不总是治愈的。在多发性肝转移的情况下，治疗是基于肾上腺切除术，可以有效地与化疗栓塞相结合，冷冻消融,或者射频技术.
结论：恶性嗜铬细胞瘤的主要预后因素是肿瘤体积大，内脏转移的存在或数量，以及SDHB（琥珀酸脱氢酶B）基因中存在突变。
方法：我们描述了4例恶性嗜铬细胞瘤患者的临床病例，其中四分之三的患者存在Menard三联症“头痛-心悸-出汗”，在4名患者中测量了甲氧基化衍生物，其中3个具有很高的价值，我们所有的病人都进行了CT扫描，客观地显示了恶性肿瘤的迹象，对一名患者进行了MRI检查；出现肿瘤复发；寻找局部侵袭。
结论：嗜铬细胞瘤（PC）是一种罕见的神经内分泌肿瘤，来源于肾上腺髓质的嗜铬细胞。其年发病率为每百万成年人2至8人。在30至40岁之间观察到峰值频率。大约10％的嗜铬细胞瘤是恶性的，在10％的病例中，观察到双边定位。恶性肿瘤的标准包括侵犯邻近器官，一个大肿瘤,影像学上淋巴结病的存在，或固定在闪烁显像上。MAP的手术并不总是治愈的。在多发性肝转移的情况下，治疗是基于肾上腺切除术，可以有效地与化疗栓塞相结合，冷冻消融,或者射频技术.
结论：恶性嗜铬细胞瘤的主要预后因素是肿瘤体积大，内脏转移的存在或数量，以及SDHB（琥珀酸脱氢酶B）基因中存在突变。
