Molaei Akbar, Abarzadeh-Bairami Vahideh, Sadat-Ebrahimi Seyyed-Reza
Cardiovascular Research Center, Shahid Madani Heart Center, Tabriz University of Medical Sciences, Tabriz, Iran.
Pediatric Health Research Center, Tabriz Children Hospital, Tabriz University of Medical Sciences, Tabriz, Iran.
BMC Pediatr. 2020 Jun 17;20(1):299. doi: 10.1186/s12887-020-02197-4.
Pheochromocytomas are rare tumors originating in chromaffin cells which predominantly are located in adrenal glands. Sustained or paroxysmal hypertension (HT) is the most frequent sign of pheochromocytoma. In some cases, it is associated with the classic triad including episodic headaches, sudoresis, and tachycardia; however, we present a case of pheochromocytoma with first presentation of cardiomyopathy.
The authors describe a rare case of a pheochromocytoma which was first presented with cardiomyopathy in a 7-year-old patient. The patient was admitted with malaise, abdominal pain, polydipsia, and myalgia. Further evaluations revealed hyperglycemia, mild dehydration and sinus tachycardia but no HT. Echocardiography demonstrated some of the signs of cardiomyopathy which was incorrectly diagnosed as viral myocarditis. The patient was discharged with this diagnosis but he presented again with HT crisis a few months later. A diagnosis of pheochromocytoma was assigned after the evaluation of the HT secondary causes. The diagnosis was confirmed by metanephrine assay and the tumor was localized in the adrenal gland using the abdominal MRI.
Pheochromocytoma can present itself with normotensive cardiomyopathy. Therefore, the possibility of pheochromocytoma should be considered in patients with cardiomyopathy especially in those with positive familial history.
嗜铬细胞瘤是起源于嗜铬细胞的罕见肿瘤，主要位于肾上腺。持续性或阵发性高血压是嗜铬细胞瘤最常见的症状。在某些情况下，它与包括发作性头痛、多汗和心动过速在内的经典三联征有关；然而，我们报告一例以心肌病为首发表现的嗜铬细胞瘤病例。
作者描述了一例罕见的嗜铬细胞瘤病例，该病例首次出现在一名7岁患者身上，以心肌病为首发表现。患者因不适、腹痛、多饮和肌痛入院。进一步检查发现高血糖、轻度脱水和窦性心动过速，但无高血压。超声心动图显示了一些心肌病的体征，被误诊为病毒性心肌炎。患者以此诊断出院，但几个月后再次出现高血压危象。在评估继发性高血压病因后确诊为嗜铬细胞瘤。通过甲氧基肾上腺素测定确诊，并用腹部MRI将肿瘤定位在肾上腺。
嗜铬细胞瘤可表现为血压正常的心肌病。因此，对于患有心肌病的患者，尤其是有阳性家族史的患者，应考虑嗜铬细胞瘤的可能性。
