[Sipple syndrome (bilateral phaeochromocytoma medullary C-cell carcinoma of the thyroid gland) with exceptionally prolonged clinical course].
The sporadic occurrence of the Sipple syndrome with bilateral phaeochromocytoma and medullary thyroid carcinoma is a well-known pathological entity. The present report refers to a patient with medullary thyroid carcinoma, initially misdiagnosed as Hurthle-cell adenoma after partial resection of the thyroid gland. 5 and 8 years later the patient underwent bilateral adrenalectomy for phaeochromocytoma. 4 years after the second phaeochromocytoma a palpable thyroid nodule developed, thyroidectomy was performed and the tumour diagnosed as a medullary thyroid carcinoma. This diagnosis was confirmed by reexamination of the histological specimens obtained during the first surgical intervention. We were prompted to report the current case history, because of the protracted course of the medullary thyroid carcinoma in this patient and to point out the value of the determination of pentagastrin stimulated calcitonin values in patients with medullary thyroid carcinoma for diagnosis, postoperative follow-up and in family screening studies.
D000310:Adrenal Gland Neoplasms / Q000473:pathology*; D000315:Adrenalectomy; D000328:Adult; D002116:Calcitonin / Q000032:analysis; D002277:Carcinoma / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000473:pathology* / Q000601:surgery; D009364:Neoplasm Recurrence, Local; D010673:Pheochromocytoma / Q000473:pathology*; D013004:Somatostatin / Q000097:blood; D013964:Thyroid Neoplasms / Q000175:diagnosis / Q000473:pathology*; D013965:Thyroidectomy
D013004:Somatostatin; D002116:Calcitonin
Sipple-Syndrom (bilaterale Phäochromozytome und medulläres C-Zell-Karzinom der Schilddrüse) mit besonders protrahiertem Verlauf.