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Related Experiment Videos

[Sipple's syndrome: a case report].

N Takaha1, Y Kobayashi, S Takahara

  • 1Department of Urology, Osaka University School of Medicine.

Hinyokika Kiyo. Acta Urologica Japonica
|November 1, 1990
PubMed
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This case report details a 41-year-old woman diagnosed with Sipple's syndrome, a rare genetic condition involving pheochromocytoma and medullary thyroid carcinoma. Surgical intervention included adrenalectomy and thyroidectomy, confirming the diagnosis.

Area of Science:

  • Endocrinology
  • Oncology
  • Genetics

Background:

  • Sipple's syndrome, also known as Multiple Endocrine Neoplasia type 2A (MEN2A), is a rare autosomal dominant disorder.
  • It is characterized by the development of medullary thyroid carcinoma, pheochromocytoma, and parathyroid hyperplasia.

Observation:

  • A 41-year-old woman presented with symptoms of diabetes mellitus and hypertension.
  • Hormonal and radiological evaluations confirmed bilateral adrenal pheochromocytoma and medullary thyroid carcinoma.
  • A familial history was negative, but two deceased sisters were suspected of having pheochromocytoma.

Findings:

  • The patient was diagnosed with Sipple's syndrome.
  • Surgical management included bilateral adrenalectomy followed by total thyroidectomy, parathyroid excision, and cervical lymph node dissection.

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  • Histopathology confirmed bilateral adrenal pheochromocytoma, medullary thyroid carcinoma, and parathyroid chief cell hyperplasia.

    • Implications:

    • This case highlights the importance of early diagnosis and surgical management of Sipple's syndrome.
    • The report contributes to the literature on MEN2A, likely representing the 88th reported case in Japan.
    • Understanding the clinical presentation and genetic basis of Sipple's syndrome is crucial for patient management and genetic counseling.