Endocrinol Metab.  2012 Mar;27(1):98-104. 10.3803/EnM.2012.27.1.98.

A Case of Malignant Pheochromocytoma Presenting as Inverted Takotsubo-Like Cardiomyopathy

• Jang JE
• Kwon HH
• Lee MJ
• Jung CH
• Bae SJ
• Kim HK
• Lee WJ

• KMID: 2134827
• DOI: http://doi.org/10.3803/EnM.2012.27.1.98

Abstract

Takotsubo cardiomyopathy or stress induced cardiomyopathy is characterized by acute transient left ventricular apical ballooning without significant coronary artery disease. The pathophysiology of Takotsubo cardiomyopathy remains unclear, but it has been suggested that the stress related neurohumoral factors, especially catecholamines, play an important role. Recently, several reports have described an inverted Takotsubo cardiomyopathy, which is characterized by the dysfunction of the basal and mid-ventricular segments sparing the apex of the heart. In this report, we present a case of a 50-year-old female with a transient left ventricular dysfunction in an inverted Takotsubo pattern, that later was diagnosed as a malignant pheochromocytoma.

Keyword

Pheochromocytoma; Takotsubo cardiomyopathy

MeSH Terms

Cardiomyopathies
Catecholamines
Coronary Artery Disease
Female
Heart
Humans
Middle Aged
Pheochromocytoma
Takotsubo Cardiomyopathy
Ventricular Dysfunction, Left
Catecholamines

Figure

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  Fig. 1 The chest X-ray before (A) and after the symptom developed (B). A. Normal chest X-ray without cardiomegaly and pulmonary congestion. B. Increased pulmonary vascular marking and pulmonary edema around perihilar area.

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  Fig. 2 Transthoracic echocardiography on admission. A. End systole. B. End diastole. It shows basal akinesia and thinning at mid to basal inferior wall with apical sparing.

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  Fig. 3 A. Enhanced abdomino-pelvic computed tomography (APCT) scan. B & C, I-123 metaiodobenzylguanethidine (MIBG) scan. A. APCT shows a 5.2 × 3 cm-sized round, lobulated mass with internal cystic change (arrow) adjacent to the right kidney. B & C. Post 4 hour and 1 day after I-123 MIBG injection. Increased I-123 MIBG uptake at right adrenal gland (circle) and focal increased I-123 MIBG uptake in L4 and left ilium (arrows), suggesting right adrenal gland pheochromocytoma with metastatic lesion.

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  Fig. 4 Histologic finding of right adrenal gland. A. Large and pinkish cells with pleomorphic nuclei and prominent nucleoli (H&E stain, × 400). B. Immunohistochemistry for chromogranin was positive in tumor cells (chromogranin, × 400). C. Synaptophysin staining was positive (synaptophysin, × 400). D. Immunohistochemical stain for sustentacular cell, S-100 was positive reaction (S-100, × 200). These findings confirmed the diagnosis of pheochromocytoma.

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