Related Experiment Videos
Pheochromocytoma with multiple islet cell carcinoma
N Tamasawa1, A Terada, T Kodama
1Third Department of Internal Medicine, Hirosaki University School of Medicine, Aomori, Japan.
Presse Medicale (Paris, France : 1983)
|January 8, 1994
Summary
A rare case of a 17-year-old female presented with pheochromocytoma and multiple malignant islet cell tumors. This unique combination suggests a mixed form of multiple endocrine neoplasia (MEN) 1 and MEN 2.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- Multiple Endocrine Neoplasia (MEN) syndromes are rare genetic disorders characterized by tumors in endocrine glands.
- MEN 1 and MEN 2 are distinct syndromes with specific tumor associations.
Observation:
- A 17-year-old female presented with a right adrenal pheochromocytoma.
- Diagnostic imaging unexpectedly revealed multiple pancreatic tumors.
- No other endocrine neoplasms were found in the pituitary, thyroid, or parathyroid glands.
Findings:
- The patient underwent adrenalectomy and total pancreatectomy.
- Pheochromocytoma was benign; pancreatic tumors were malignant, non-functioning islet cell tumors.
- This presentation is hypothesized to be a mixed form of MEN 1 and MEN 2.
Implications:
- This case expands the known spectrum of MEN syndromes.
- Highlights the importance of thorough investigation in rare tumor presentations.
- Suggests potential genetic overlap or novel mechanisms in MEN syndromes.