Nadeema Rafiq, Tauseef Nabi, S. Dar, Shahnawaz Rasool
{"title":"嗜铬细胞瘤患者的特点及预后","authors":"Nadeema Rafiq, Tauseef Nabi, S. Dar, Shahnawaz Rasool","doi":"10.4103/ctm.ctm_5_20","DOIUrl":null,"url":null,"abstract":"Aim: Pheochromocytomas are rare neuroendocrine tumors with variable clinical presentation, including hypertension, and usually arise from the adrenal medulla. The aim of the current study was to evaluate the clinical characteristics and therapeutic outcomes of patients with pheochromocytoma. Methods: This was a single-center retrospective observational study designed to study the clinical, laboratory, radiological, and surgical outcomes of 14 patients diagnosed with pheochromocytoma. Results: Of 14 patients, 10 were females and 4 males, with a mean age of 38 ± 16 years at diagnosis. Headache (96.6%), palpitation (64.3%), abdominal pain (64.3%), and sweating (57.1%) were the most common presenting symptoms, while triad was present in 42.8%. Hypertension was the predominant clinical finding (92.8%) followed by orthostasis in 35.7% and hyperglycemia in 35.7%. Most pheochromocytomas were sporadic (85.7%), adrenal gland tumors (78.6%), and benign (92.8%); two were familial (each due to neurofibromatosis type and von Hippel–Lindau). Metanephrine and nor-metanephrine secretory pattern was seen in 58.3% of the patients, while metanephrine only was seen in 41.7% of the patients. More than half of the tumors (54.5%) were located on the left side, with 36.4% on the right side, and one patient had bilateral presentation of a tumor. Postsurgical remission of hypertension was found in 35.7% of the hypertensive patients. Biochemical cure was obtained in 85.7% and surgical cure rate in 78.5% of the patients. Patient survival ranged from 2 months to 9 years. Conclusions: The present study confirms that the clinical presentation of pheochromocytoma is variable and nonspecific. Although pheochromocytoma is a rare tumor, proper evaluation, preoperative preparation, and complete surgical excision are important for its management.","PeriodicalId":9428,"journal":{"name":"Cancer Translational Medicine","volume":"73 1","pages":"25 - 29"},"PeriodicalIF":0.0000,"publicationDate":"2020-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Characteristics and outcome of patients with pheochromocytoma\",\"authors\":\"Nadeema Rafiq, Tauseef Nabi, S. Dar, Shahnawaz Rasool\",\"doi\":\"10.4103/ctm.ctm_5_20\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Aim: Pheochromocytomas are rare neuroendocrine tumors with variable clinical presentation, including hypertension, and usually arise from the adrenal medulla. The aim of the current study was to evaluate the clinical characteristics and therapeutic outcomes of patients with pheochromocytoma. Methods: This was a single-center retrospective observational study designed to study the clinical, laboratory, radiological, and surgical outcomes of 14 patients diagnosed with pheochromocytoma. Results: Of 14 patients, 10 were females and 4 males, with a mean age of 38 ± 16 years at diagnosis. Headache (96.6%), palpitation (64.3%), abdominal pain (64.3%), and sweating (57.1%) were the most common presenting symptoms, while triad was present in 42.8%. Hypertension was the predominant clinical finding (92.8%) followed by orthostasis in 35.7% and hyperglycemia in 35.7%. Most pheochromocytomas were sporadic (85.7%), adrenal gland tumors (78.6%), and benign (92.8%); two were familial (each due to neurofibromatosis type and von Hippel–Lindau). Metanephrine and nor-metanephrine secretory pattern was seen in 58.3% of the patients, while metanephrine only was seen in 41.7% of the patients. More than half of the tumors (54.5%) were located on the left side, with 36.4% on the right side, and one patient had bilateral presentation of a tumor. Postsurgical remission of hypertension was found in 35.7% of the hypertensive patients. Biochemical cure was obtained in 85.7% and surgical cure rate in 78.5% of the patients. Patient survival ranged from 2 months to 9 years. Conclusions: The present study confirms that the clinical presentation of pheochromocytoma is variable and nonspecific. Although pheochromocytoma is a rare tumor, proper evaluation, preoperative preparation, and complete surgical excision are important for its management.\",\"PeriodicalId\":9428,\"journal\":{\"name\":\"Cancer Translational Medicine\",\"volume\":\"73 1\",\"pages\":\"25 - 29\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2020-04-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Cancer Translational Medicine\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/ctm.ctm_5_20\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Cancer Translational Medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/ctm.ctm_5_20","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Characteristics and outcome of patients with pheochromocytoma
Aim: Pheochromocytomas are rare neuroendocrine tumors with variable clinical presentation, including hypertension, and usually arise from the adrenal medulla. The aim of the current study was to evaluate the clinical characteristics and therapeutic outcomes of patients with pheochromocytoma. Methods: This was a single-center retrospective observational study designed to study the clinical, laboratory, radiological, and surgical outcomes of 14 patients diagnosed with pheochromocytoma. Results: Of 14 patients, 10 were females and 4 males, with a mean age of 38 ± 16 years at diagnosis. Headache (96.6%), palpitation (64.3%), abdominal pain (64.3%), and sweating (57.1%) were the most common presenting symptoms, while triad was present in 42.8%. Hypertension was the predominant clinical finding (92.8%) followed by orthostasis in 35.7% and hyperglycemia in 35.7%. Most pheochromocytomas were sporadic (85.7%), adrenal gland tumors (78.6%), and benign (92.8%); two were familial (each due to neurofibromatosis type and von Hippel–Lindau). Metanephrine and nor-metanephrine secretory pattern was seen in 58.3% of the patients, while metanephrine only was seen in 41.7% of the patients. More than half of the tumors (54.5%) were located on the left side, with 36.4% on the right side, and one patient had bilateral presentation of a tumor. Postsurgical remission of hypertension was found in 35.7% of the hypertensive patients. Biochemical cure was obtained in 85.7% and surgical cure rate in 78.5% of the patients. Patient survival ranged from 2 months to 9 years. Conclusions: The present study confirms that the clinical presentation of pheochromocytoma is variable and nonspecific. Although pheochromocytoma is a rare tumor, proper evaluation, preoperative preparation, and complete surgical excision are important for its management.