A Case of Malignant Pheochromocytoma with Holt-Oram Syndrome

• YOSHIHARA Ai

  Department of Medicine, Institute of Clinical Endocrinology, Tokyo Women's Medical University

• TANABE Akiyo

  Department of Medicine, Institute of Clinical Endocrinology, Tokyo Women's Medical University

• SAITO Hiroshi

  Department of Medicine, Institute of Clinical Endocrinology, Tokyo Women's Medical University

• HIZUKA Naomi

  Department of Medicine, Institute of Clinical Endocrinology, Tokyo Women's Medical University

• ISHIZAWA Akira

  National Center of Child Health and Development

• HORIKAWA Reiko

  National Center of Child Health and Development

• TAKANO Kazue

  Department of Medicine, Institute of Clinical Endocrinology, Tokyo Women's Medical University

Abstract

A 23-year-old female patient with malignant pheochromocytoma was admitted to the Tokyo Women's Medical University. The patient had been clinically diagnosed with Holt-Oram syndrome at birth. Since she had complex congenital heart disease, chronic heart failure, and severe hypoxia, the risk surrounding surgery to remove the primary tumor was predicted to be very high, and subsequently, chemotherapy was performed. The patient was not able to continue chemotherapy due to adverse effects. However, for one year, both her hypertension and catecholamine-dependent symptoms were well controlled by an alpha-adrenergic and beta-adrenergic receptor blockade, although the patient did experience high plasma norepinephrine levels. To our knowledge, this is the first report of a patient with the combination of malignant pheochromocytoma and Holt-Oram syndrome. A correlation between chronic hypoxia and pheochromocytoma has been reported. This instructive case reminds us to consider the possibility of pheochromocytoma with congenital heart disease when these types of unexpected or unusual symptoms are encountered.<br>

Journal

• Endocrine Journal

  Endocrine Journal 55 (1), 153-159, 2008

  The Japan Endocrine Society

Keywords

• Malignant pheochromocytoma
• Holt-Oram syndrome

Details

• CRID: 1390282681275713536
• NII Article ID: 10021264881
• NII Article ID: 130004443362
• NII Book ID: AA10901436
• DOI: 10.1507/endocrj.k07e-011
• ISSN: 13484540
• ISSN: 09188959
• Web Site: http://www.jstage.jst.go.jp/article/endocrj/55/1/55_K07E-011/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2008175209
• Text Lang: en
• Data Source:
  • JaLC
  • Crossref
  • CiNii Articles
• Abstract License Flag: Disallowed