Concomitant composite adrenal pheochromocytoma, multiple gastric stromal tumours and pseudohermaphrodism in a patient with von Recklinghausen's disease
2006

Rare Case of Adrenal Tumor and Gastric Tumors in a Patient with Neurofibromatosis

Sample size: 1 publication Evidence: low

Author Information

Author(s): Lisewski Dean, Ryan Simon, Lim Ee Mun, Frost Felicity, Nguyen Hieu

Primary Institution: Sir Charles Gairdner Hospital, Nedlands, Western Australia

Hypothesis

This case report aims to explore the occurrence of composite adrenal pheochromocytoma and multiple gastric stromal tumors in a patient with von Recklinghausen's disease.

Conclusion

The study presents a unique case of an 82-year-old woman with neurofibromatosis type 1 who had both adrenal pheochromocytoma and multiple gastric stromal tumors.

Supporting Evidence

  • Pheochromocytoma occurs in 1% of patients with neurofibromatosis type 1.
  • Composite tumors in this syndrome are much rarer, with isolated case reports in the literature.
  • The patient had elevated urinary catecholamine excretion, confirming the functional nature of the adrenal tumor.
  • Pathological findings confirmed the presence of both pheochromocytoma and ganglioneuroma in the adrenal tumor.

Takeaway

This study is about an 82-year-old woman who had a rare combination of tumors related to a genetic condition called neurofibromatosis.

Methodology

The patient underwent imaging studies, biochemical investigations, and surgical procedures including laparoscopic left adrenalectomy.

Limitations

The study is based on a single case report, limiting the generalizability of the findings.

Participant Demographics

An 82-year-old woman with a history of neurofibromatosis type 1.

Digital Object Identifier (DOI)

10.1186/1477-7800-3-11

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