Context: Lithium-associated hyperparathyroidism (LAH) was first described in 1973 but many issues remain in question regarding the pathophysiology as well as the appropriate management of this condition. Objective: Report of four new cases and review of the literature. Results: We describe two males and two females, treated for more than 10 years with lithium due to bipolar disorder, who developed LAH. All underwent parathyroidectomy. In three cases (75%), pathology revealed multiglandular disease, with hyperplasia or two parathyroid adenomas. We observed a cure status in three (75%) of the operated patients. The fourth patient had a residual disease, but had controlled hypercalcemia under the calcimimetic drug cinacalcet. We also observed the association of LAH with incidental papillary thyroid carcinoma in two patients. Review of the literature identified a higher prevalence of LAH in women than men (four out of one) and a controversy in regard to the prevalence of multiglandular disease. As a result, there is no consensus regarding the preferred surgical procedure. The use of cinacalcet as an effective treatment of LAH was previously described in only five cases. Conclusion: In our view, there are apparently two different mechanisms leading to LAH: exacerbation of a pre-existing state of hyperparathyroidism and multiglandular disease. For uncontrolled hypercalcemia, parathyroidectomy is recommended. The issue of routine four-gland exploration and subtotal parathyroidectomy versus intraoperative PTH-determination-guided excision of enlarged glands is still unresolved. The use of the recently developed calcimimetics may offer an alternative to patients who are not candidates for surgery.
Coronary artery aneurysm is an uncommon occurrence, yet it is described more often today than in the past as coronary angiography is now routinely used for diagnosis and treatment of ischemic heart disease. However, there is no therapeutic consensus regarding this finding. We present a case of giant coronary artery aneurysm and review the literature on the use of polytetrafluoroethylene-covered stents as a therapeutic option for this condition. Combined antiaggregant therapy is needed after the procedure. Randomized controlled trials of surgery versus covered stents are necessary to define the best treatment for large coronary artery aneurysms.
Metastases appear in approximately 10% of patients with pheochromocytoma. There is no predictive marker of malignancy. The aim is to describe clinical course of patients with malignant pheochromocytoma and to identify predictive features of malignancy. The method involves retrospective analysis of patients files diagnosed with malignant pheochromocytoma at our institution between January 1, 1980 and December 31, 2008. We identified 16 patients with malignant pheochromocytoma. There were more men than women (10/6). Mean age of patients at time of diagnosis was 37.75-year-old. Time of occurrence of metastases ranged from 0 to 22 years after first diagnosis of pheochromocytoma. The mean size of the primary tumor was 12.1 cm. High levels of chromogranin A at the time of diagnosis were associated with the presence of metastases. The pheochromocytoma of the adrenal gland scoring scale (PASS) histological evaluation in adrenal primary tumors was above four in all cases but one. All patients had initial surgery, followed in most cases by palliative therapy: chemotherapy (streptozocin, cyclophosphamide-vincristine-dacarbazine, thalidomide, imatinib, everolimus) or (131)I-MIBG; only the latter had replicable encouraging response evaluation criteria in solid tumor response rates. We observed a 10-year survival rate of 50% after initial diagnosis of pheochromocytoma, and 25% after diagnosis of metastasis. Metastasis can occur very late after the initial diagnosis of pheochromocytoma. High chromogranin A levels may be associated with the presence of metastases and poor prognosis. Histological adrenal PASS higher than 4 appears to be suggestive of malignancy. The best therapeutic approach remains to be established.
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