[Composite pheochromocytoma with ganglioneuroblastoma in an adult: a case report and literature review].

Authors

Y Y Song, J H Li, Y Q Yin, Y C Li, X Li, H L Liu, S C Zhang, G Q Yang, Z H Lyu, Y M Mu

Published in

Zhonghua yi xue za zhi. Volume 106. Issue 29. Pages 3061-3067. Aug 11, 2026.

Abstract

A rare case of composite pheochromocytoma (PCC) with ganglioneuroblastoma (GNB) was reported in this article, and the patient's diagnostic and treatment course-including biochemical evaluation, imaging findings, surgery, pathology, genetic testing, and postoperative follow-up-was retrospectively analyzed. Relevant literature in PubMed, Embase, CNKI, and Wanfang Data from database inception to October 1 2025 was systematically searched to identify comparable adult cases, and their clinicopathological features were summarized. The patient was a 48-year-old man who presented to the Department of Endocrinology, First Medical Center of Chinese PLA General Hospital on March 25, 2025, with hypertension for 3 months and a left adrenal mass detected 12 days earlier. Biochemical testing showed markedly elevated normetanephrine (NMN). MRI and ¹⁸F-NOTATATE somatostatin receptor positron emission tomography-computed tomography (¹⁸F-NOTATATE-PET-CT) revealed a cystic-solid mass in the left adrenal gland, with increased uptake in the solid component [The maximum standardized uptake value (SUVmax) was 9.4]. Robot-assisted laparoscopic tumor resection was performed on the patient. Composite PCC, consisting of a pheochromocytoma component and a GNB component, was confirmed by postoperative pathology. No clearly pathogenic variants were detected by germline whole-exome sequencing. After surgery, blood pressure was normalized without antihypertensive medication, and no evidence of recurrence or metastasis was observed at the 6-month follow-up. The literature review identified 14 adult cases of PCC with GNB; together with the present case, 15 cases were analyzed. The age [M(Q₁, Q₃)] was 49 (35, 55) years. Hypertension was documented in 11 patients, elevated catecholamines or their metabolites were observed in most cases, tumors>5 cm in maximum diameter were found in 8 patients, and hereditary syndromes were identified in 3 patients. Composite PCC with GNB in adults is exceedingly rare. Its clinical, biochemical, and imaging manifestations frequently overlap with those of conventional PCC, rendering definitive diagnosis dependent on histopathological examination. Prognosis appears to be correlated with tumor components, size, and genetic syndrome status. Therefore, comprehensive postoperative pathological evaluation, germline genetic testing, and long-term surveillance are strongly recommended.

PMID:
42571973
Bibliographic data and abstract were imported from PubMed on 10 Aug 2026.

Read full publication at:
Please sign in to see all details.

Stats

1-terrible, 9-excellent. How would you rate this publication? Sign in in to submit your rating.

Post a comment

You need to be signed in to post comments. You can sign in here.

Comments

There are no comments yet.