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Sipple's syndrome associated with a large prolactinoma
J H Bertrand1, P Ritz, Y Reznik
1Department of Cardiology, Centre Hospitalo-Universitaire de Caen, France.
Clinical Endocrinology
|November 1, 1987
Summary
This case highlights a rare presentation of Sipple's syndrome, involving phaeochromocytoma and medullary thyroid carcinoma, complicated by catecholamine-induced cardiomyopathy. The co-occurrence with a macroprolactinoma is a novel finding in this multiple endocrine neoplasia spectrum.
Area of Science:
- Endocrinology
- Cardiology
- Oncology
Background:
- Multiple endocrine neoplasia (MEN) syndromes are rare genetic disorders.
- Sipple's syndrome (MEN2A) typically involves phaeochromocytoma, medullary thyroid carcinoma, and parathyroid disease.
- This case presents atypical features within the MEN spectrum.
Observation:
- A 26-year-old male presented with acute congestive heart failure and hypertension.
- Echocardiography revealed left ventricular thickening and reversible hypokinesia, suggestive of catecholamine-induced cardiomyopathy.
- Investigations identified phaeochromocytoma, bilateral medullary thyroid carcinoma (MTC), parathyroid adenoma, and a macroprolactinoma.
Findings:
- The patient had a family history of MTC.
- This is the first reported case of Sipple's syndrome associated with a macroprolactinoma.
- The combination of conditions did not fit classical MEN type I or II subsets.
Implications:
- This case expands the known clinical spectrum of MEN syndromes.
- Catecholamine-induced cardiomyopathy is a significant cardiac manifestation of phaeochromocytoma.
- Recognition of atypical MEN presentations is crucial for timely diagnosis and management.
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