Composite Pheochromocytoma—A Rare Clinical Entity: A Case Series

JOURNAL TITLE: Indian Journal of Endocrine Surgery and Research

Author
1. Pushkar Sharma
2. Santosh Raj
3. Paul M Jacob
4. Marie T Manipadam
5. Elanthenral Sigamani
ISSN
DOI
10.5005/jp-journals-10088-11151
Volume
16
Issue
1
Publishing Year
2021
Pages
4
Author Affiliations
    1. Department of Endocrine Surgery, Christian Medical College, Vellore, Tamil Nadu, India
    1. Department of Endocrine Surgery, Christian Medical College, Vellore, Tamil Nadu, India
    1. Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
    1. Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
    2. Christian Medical College and Hospital, Vellore, Tamil Nadu, India
    1. Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
  • Article keywords
    Adrenal, Composite Pheochromocytoma, Ganglioneuroma, Multiple endocrine neoplasia

    Abstract

    Background: Composite pheochromocytoma is a rare entity with tumor comprising both pheochromocytoma and neuroblastic elements. The clinical outcome of such a composite tumor is not fully understood. In this case series, we report three cases of composite pheochromocytoma managed at our institute from 2016 to 2021. Results: We had three patients with MEN2A syndrome with composite tumor who underwent adrenalectomy, all of which were ganglioneuroma with pheochromocytoma. None of the three cases had recurrence or metastasis associated with composite adrenal tumor on 13-month mean follow-up. Conclusion: We noticed a higher incidence of syndromic association in composite adrenal tumors. The clinical and radiological presentation was dominated by the pheochromocytoma component.

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