HPT-JT has a frequent single-gland parathyroid involvement and a relatively increased risk of parathyroid carcinoma. The penetrance of the disease is high but incomplete. Regardless of the denomination of the syndrome, jaw tumors occur rarely, while uterine involvement is frequently present. Selective parathyroidectomy may be an effective strategy, but a prolonged follow-up is required because of the risk of recurrences and malignancies. A systematic investigation is also required because of associated malignancies.
Objective: To evaluate the usefulness of the combination of 99m Tc-sestamibi/ 99m Tc-pertechnetate subtraction scintigraphy (SS) and high-resolution neck ultrasonography (US) in patients with primary hyperparathyroidism (pHPT) undergoing parathyroidectomy. Design and Methods: Ninety-one patients with proved pHPT were studied, excluding patients with persistent or recurrent disease. There were 65 (71.4%) women and 26 (28.6%) men, with a median age of 59 years (range 18±78 years). All patients underwent both SS and US prior to surgery, and the results were compared with operative and histological findings. The intraoperative quick-parathyroid hormone assay was available for 52 (57.1%) patients. When multiglandular disease was found, both SS and US were considered truly positive only when at least two enlarged parathyroid (PT) glands had been localized. Results: Eighty-three (91.2%) solitary PT adenomas and three (3.3%) carcinomas were found. Moreover, two (2.2%) patients had a double adenoma and three (3.3%) patients had diffuse PT hyperplasia. The overall sensitivity of combined SS US was 94.5% (86.8% and 80.4% for SS and US respectively). There was a significant P , 0X05Y Student's t-test) difference in size between the PT glands correctly identified and undetected by SS, whereas the site of the removed PT tumors significantly P , 0X05Y Fisher exact test) influenced only the US sensitivity. Conclusions: When the preoperative localization of the PT glands is chosen, the combination of SS and US represents a reliable noninvasive localization technique and should be considered for use in each patient with pHPT undergoing surgery.
We undertook a retrospective study in 16 patients with parathyroid carcinoma, with the aim of conveying experience from management of this rare cause of hyperparathyroidism (HPT). From 1980 to 1996 we operated on 309 patients with HPT, 290 of whom had primary HPT, and parathyroid carcinoma was diagnosed in 16 (5.2%) cases. In none was the malignancy diagnosed preoperatively. The average serum calcium and parathyroid hormone levels were significantly higher (p < 0.05) than in patients with parathyroid adenoma, and the mean tumor size was 2.9 cm (median 2.5 cm, range 1.5-4.5 cm). Twenty-eight operations were performed with resulting normalization of serum calcium levels for more than 6 months in 11 patients. Six patients presented prolonged postoperative hypocalcemia (2-10 months), and five patients had persistent HPT. Ten patients experienced recurrent disease; the median disease-free period was 24 months (range 4-72 months). None of the patients was treated with chemotherapy, one patient underwent radiation therapy (50 Gy in 25 sessions) but required reoperation for local recurrence 4 months later. Three patients are still alive (two with recurrence). Average survival was 40 months (median 31 months, range 11-131 months).
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