Primary hyperparathyroidism (PHP) due to adenoma or hyperplasia is common and is being increasingly diagnosed. Overall 80% are due to single adenoma, 15% to multiglandular hyperplasia, 3% to multiple adenomata (usually 2) and 2% to carcinoma. In 20-30% the glands will be ectopically placed, usually within the neck, though can be anywhere from the skull base to the diaphragm.
Surgery is the treatment and a longterm debate rages regarding whether to localise the glands first using ultrasound, scintigraphy, CT or MRI before a primary operation. Blind surgical exploration is 95% successful and the best localization procedure. However it is not perfect and second operations with higher morbidity, following non-invasive or invasive localisation tests are then required.
We present a unique case due to 4 discrete mediastinal adenomata.
A 48 year old woman with PHP had an initial negative neck exploration which localised 3 normal parathyroids. Subsequent Technetium scanning failed to localise any ectopic adenomata though an anterior mediastinal mass visualised on CT proved to be a 'nest' of 4 adenomata at second operation. Full cure was achieved.
Such pathology has not been reported previously though extranumery normal glands are a relatively common post-mortem finding. Also it is unusual for scintography to miss such a large mass of active tissue. Up to 38% of abnormal glands in reoperative cases are mediastinal compared to 2% at first operation. This suggests initial pre-operative screening including the mediastinum would be useful especially for patients at risk from second operations which have higher morbidity. Performance of this operation under local anaesthetia could change opinions once again however.
Using this illustrated case we review current screening and treatment options and the debate over pre-operative screening in this condition
03 - 04 Dec 2001
Society for Endocrinology