PDF(Pubmed)
Abstract：
BACKGROUND: 3P association (3PA) is a rare condition with co-occurrence of pituitary adenoma and pheochromocytoma/paraganglioma. There have been less than a hundred documented cases of 3PA, which can be sporadic or related to genetic mutations. The present case report describes the first Iranian patient with 3PA and a 90th case of 3PA in the available literature.
UNASSIGNED: A 36-year-old Caucasian male was admitted with headache and sudden increase in blood pressure. An abdominal CT scan revealed a retroperitoneal mass posterior to the inferior vena cava, later removed and diagnosed as a pheochromocytoma. Four years later, he noticed occasional mild headaches and a painless mass on the right side of his neck. The ultrasonography evaluations suggested a carotid body tumor, which was surgically removed. About a month after his second surgery, the severity of the patient\'s headaches worsened, and he developed right homonymous hemianopia. A brain MRI showed a mass in favor of macroadenoma, craniopharyngioma, or meningioma, and elevated prolactin level led to the diagnosis of macroprolactinoma.
METHODS: Based on the provided history, this patient was diagnosed with 3PA, and a genetic study identified a positive succinate-dehydrogenase-complex subunit b mutation, possibly linked to his family history of carotid body tumor.
RESULTS: He has remained symptom-free during his visits every 3 months.
CONCLUSIONS: The number of cases diagnosed with 3PA worldwide is increasing. Using clinical and genetic assessments, we can timely diagnose and adequately monitor individuals with or at risk of 3PA.
UNASSIGNED: A 36-year-old Caucasian male was admitted with headache and sudden increase in blood pressure. An abdominal CT scan revealed a retroperitoneal mass posterior to the inferior vena cava, later removed and diagnosed as a pheochromocytoma. Four years later, he noticed occasional mild headaches and a painless mass on the right side of his neck. The ultrasonography evaluations suggested a carotid body tumor, which was surgically removed. About a month after his second surgery, the severity of the patient\'s headaches worsened, and he developed right homonymous hemianopia. A brain MRI showed a mass in favor of macroadenoma, craniopharyngioma, or meningioma, and elevated prolactin level led to the diagnosis of macroprolactinoma.
METHODS: Based on the provided history, this patient was diagnosed with 3PA, and a genetic study identified a positive succinate-dehydrogenase-complex subunit b mutation, possibly linked to his family history of carotid body tumor.
RESULTS: He has remained symptom-free during his visits every 3 months.
CONCLUSIONS: The number of cases diagnosed with 3PA worldwide is increasing. Using clinical and genetic assessments, we can timely diagnose and adequately monitor individuals with or at risk of 3PA.
摘要：
背景：3P关联（3PA）是垂体腺瘤和嗜铬细胞瘤/副神经节瘤同时发生的罕见疾病。记录的3PA病例不到100例，可能是零星的或与基因突变有关。本病例报告描述了现有文献中的首例3PA伊朗患者和第90例3PA患者。
一名36岁的白人男性因头痛和血压突然升高入院。腹部CT扫描显示下腔静脉后方有腹膜后肿块，后来切除并诊断为嗜铬细胞瘤。四年后,他注意到偶尔有轻微的头痛和颈部右侧的无痛肿块。超声检查显示颈动脉体瘤,手术切除了.在他第二次手术一个月后,患者头痛的严重程度加重，他发展出了正确的同音偏盲。脑部MRI显示肿块有利于大腺瘤，颅咽管瘤,或者脑膜瘤，泌乳素水平升高导致大泌乳素瘤的诊断。
方法：根据提供的历史记录，这个病人被诊断为3PA,一项基因研究发现琥珀酸脱氢酶复合物亚基b突变阳性，可能与他的颈动脉体瘤家族史有关.
结果：患者在每3个月就诊期间保持无症状。
结论：全球诊断为3PA的病例数正在增加。使用临床和遗传评估，我们可以及时诊断和充分监测患有3PA或处于3PA风险的个体。
一名36岁的白人男性因头痛和血压突然升高入院。腹部CT扫描显示下腔静脉后方有腹膜后肿块，后来切除并诊断为嗜铬细胞瘤。四年后,他注意到偶尔有轻微的头痛和颈部右侧的无痛肿块。超声检查显示颈动脉体瘤,手术切除了.在他第二次手术一个月后,患者头痛的严重程度加重，他发展出了正确的同音偏盲。脑部MRI显示肿块有利于大腺瘤，颅咽管瘤,或者脑膜瘤，泌乳素水平升高导致大泌乳素瘤的诊断。
方法：根据提供的历史记录，这个病人被诊断为3PA,一项基因研究发现琥珀酸脱氢酶复合物亚基b突变阳性，可能与他的颈动脉体瘤家族史有关.
结果：患者在每3个月就诊期间保持无症状。
结论：全球诊断为3PA的病例数正在增加。使用临床和遗传评估，我们可以及时诊断和充分监测患有3PA或处于3PA风险的个体。
