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Prophylactic and therapeutic neck dissections are used to control or eliminate local nodal disease in patients with thyroid cancer. The purpose of this study was to evaluate the results and complications of neck dissection. From 1992 to 1999 a series of 115 consecutive neck dissections were performed in 74 patients (32 men, 42 women; mean age 48 years) with thyroid cancer and nodal metastases. Operations included central compartment, lateral modified, and suprahyoid dissection with and without total or completion thyroidectomy. Sixty-four percent of the patients had papillary, 4% follicular, and 32% medullary thyroid cancer. Complications included transient hypocalcemia (23%) defined by a postoperative serum calcium level of <2.0 mmol/L (8.0 mg/dl), one neck hematoma (0.9%), and one cardiac death (0.9%). There were no permanent recurrent nerve palsies. Hypocalcemia occurred more frequently when neck dissection was combined with total thyroidectomy than without it (p <0.005). In this group, the incidence of hypocalcemia was higher after central, than lateral, neck dissection. When neck dissection was performed without thyroidectomy, there was no difference in the rates of hypocalcemia between central, lateral, or central with lateral neck dissection (p = NS). Hypocalcemia did not increase with repeated neck dissections (p = NS). Permanent hypoparathyroidism occurred in 0.9%. There were no complications after suprahyoid dissection. The median duration of hospitalization was 1 day. Therapeutic neck dissection or repeated neck dissection can be performed relatively safely in patients with thyroid cancer. Hypocalcemia occurs most frequently when neck dissection is combined with total thyroidectomy.

Cardio-facio-cutaneous (CFC) and Costello syndrome (CS) are congenital disorders with a significant clinical overlap. The recent discovery of heterozygous mutations in genes encoding components of the RAS-RAF-MAPK pathway in both CFC and CS suggested a similar underlying pathogenesis of these two disorders. While CFC is heterogeneous with mutations in BRAF, MAP2K1, MAP2K2 and KRAS, HRAS alterations are almost exclusively associated with CS. We carried out a comprehensive mutation analysis in 51 CFC-affected patients and 31 individuals with CS. Twelve different BRAF alterations were found in twenty-four patients with CFC (47.0%), two MAP2K1 mutations in five (9.8%) and two MAP2K2 sequence variations in three CFC-affected individuals (5.9%), whereas three patients had a KRAS alteration (5.9%). We identified four different missense mutations of HRAS in twenty-eight cases with CS (90.3%), while KRAS mutations were detected in two infants with a phenotype meeting criteria for CS (6.5%). In 14 informative families, we traced the parental origin of HRAS alterations and demonstrated inheritance of the mutated allele exclusively from the father, further confirming a paternal bias in the parental origin of HRAS mutations in CS. Careful clinical evaluation of patients with BRAF and MAP2K1/2 alterations revealed the presence of slight phenotypic differences regarding craniofacial features in MAP2K1- and MAP2K2-mutation positive individuals, suggesting possible genotype-phenotype correlations.

Preoperative sestamibi (MIBI) and ultrasonography (US) are used to localize parathyroid tumors in patients with primary hyperparathyroidism (pHPT). The intraoperative quick PTH assay (qPTH) has been recommended to determine whether all hyperfunctioning parathyroid tissue has been removed. We questioned whether qPTH improves the results of parathyroidectomy in patients with pHPT. We analyzed 115 unselected patients with pHPT without a family history or multiple endocrine neoplasia but who had undergone parathyroidectomy. All 115 patients had successful operations without complications. Of these patients, 88 (77%) had solitary adenomas, 13 had double adenomas, 1 had triple adenomas, 12 had hyperplasia, and 1 had carcinoma. Overall, MIBI was correct in 72% (76/106), US in 49% (49/99), and qPTH in 80% (92/115). For preoperative studies showing a single tumor, MIBI was correct in 83% (73/88), US was correct in 71% (45/63), and combined MIBI and US were correct in 95% (37/39). Adding qPTH in this subgroup did not improve the successful focused approach: 70% for MIBI, 65% for US, and 87% for combined MIBI and US. However, adding qPTH improved the overall success of parathyroidectomy (MIBI 92%, US 86%, combined MIBI and US 97%), but at the cost of unnecessary further exploration (MIBI 13%, US 6%, combined MIBI and US 8%). We conclude that when the same solitary tumor is identified by both MIBI and US, a focused exploration can be done with a 95% success rate. Adding qPTH to MIBI or US can improve the success rate but at a significant cost. General exploration of all parathyroid glands, however, has the highest success rate (100%).