Results suggest a lifestyle program focusing on cognitive-behavioral skills training alone is insufficient for promoting routine physical activity in these patients.

The records of 110 patients (58 females and 52 males) with histologically proven adrenal cortical carcinoma were studied. Fifty-six of 110 (50.9%) patients had abdominal symptoms and 33 (30.0%) had a palpable abdominal mass. Surgery for localized and regional disease was associated with a disease-free survival time of at least 2 years in 28 of the 50 (56%) patients. Abdominal radiotherapy, systemic chemotherapy, and 1,1 dichloro-2 (O-chlorophenyl)-2 (P-chlorophenyl) ethane (OPDDD) were effective in three of 19 (15.8%) patients, three of 31 (9.7%) patients, and 21 of 72 (29.2%) patients, respectively. Combined radiotherapy and OPDDD did not confer any additional benefit over OPDDD alone. Six of seven patients who received OPDDD as adjuvant treatment are still alive at 1 to 4 years. Distant metastases occurred most commonly in the lungs, liver, peritoneum, lymph nodes, and bones. Only 23% of the patients survived 5 years or more. We conclude that early diagnosis and complete surgical excision offer the best prospects for long-term survival and that the efficacy of adjuvant OPDDD needs to be evaluated further.

ObjectiveTo evaluate surgical complications, patterns of lymph node metastases, and calcitonin response to compartment-oriented lymphadenectomy in patients with primary or recurrent medullary thyroid carcinoma (MTC). Summary Background DataThe majority of patients with invasive MTC have metastasis to regional lymph nodes at the time of diagnosis, as evidenced by the frequent finding of persistently elevated calcitonin levels after thyroidectomy and the high rates of recurrence in the cervical lymph nodes reported in retrospective studies. These data have provided the rationale for surgeons to perform a more extensive lymphadenectomy at the time of initial thyroidectomy and to consider reoperative cervical lymphadenectomy in patients with persistently elevated calcitonin levels after thyroidectomy. MethodsForty patients underwent surgery for MTC from 1991 to 1997 (23 sporadic cases, 17 familial cases). Patients were divided into three groups based on whether they had undergone previous thyroidectomy and on the results of standardized staging studies performed after referral to the authors' institution. Group 1 (11 patients) had received no previous surgery; group 2 (13) underwent thyroidectomy before referral and had an elevated calcitonin level without radiologic evidence of local-regional or distant metastases; and group 3 (16) underwent thyroidectomy before referral and had an elevated calcitonin level with radiologic evidence of local-regional recurrence. The central neck compartment was dissected in all patients; preoperative staging and the extent of previous surgery dictated the need for lateral (modified radical) neck dissection. After primary or reoperative surgery, calcitonin levels were assessed.

Seventy‐seven patients with histologically proven adrenal cortical carcinoma seen at the University of Texas M. D. Anderson Hospital (1950–1981) were studied. Thirty‐nine were women (mean age at diagnosis, 36.6 years), and 38 were men (mean age at diagnosis, 48.3 years). On presentation, 41 of 74 had abdominal symptoms (55.4%) and 25 of 74 had an abdominal mass (33.8%). Twenty‐six patients (33.8%) were found to have clinically functional disease (18 women, 8 men). At diagnosis, 26 (33.8%) had clinical or radiologic signs of distant metastases. Preoperative radiologic studies yielded an abnormal intravenous pyelogram in 42 of 51 (82%), an abnormal abdominal ultrasound in eight of nine (88.9%), abnormal computerized tomography in 10 of 10 (100%), and abnormal arteriogram in 18 of 19 (94.7%). Surgery for localized or regional disease was associated with a disease‐free interval of at least 2 years in 16 of 34 patients (47%). The use of op'DDD, abdominal radiotherapy, and systemic chemotherapy produced demonstrable effects in nine of 47 (19.1%), two of nine (22.2%), and three of 26 (11.5%), respectively. Distant metastases occurred in 60 patients, commonly in lung, liver, peritoneal and pleural surfaces, lymph nodes, and bone. Analysis of survival data showed a 5‐year survival of approximately 30%. The authors concluded that early diagnosis and radical surgery offer the only prospects of long‐term survival and the possibility of cure.

Hypothesis: Multiple endocrine neoplasia type 1 (MEN 1) syndrome is an autosomal dominant disorder caused by germline mutations in the MEN1 gene and characterized by multiple endocrine tumors, most notably in the parathyroid glands, pituitary, and pancreas. The syndrome demonstrates variable expressivity and considerable genetic heterogeneity. Patient data were examined for possible associations between genotype and phenotype. Design: We reviewed recorded medical data from 1975 to 2001 on patients with MEN 1 and compared specific types and locations of MEN1 gene mutations with manifestations of the syndrome. Patients and Results: We identified 109 affected patients from 24 MEN 1 kindreds. The phenotypic expression of MEN 1 in affected individuals included hyper-parathyroidism in 74%, pancreatic endocrine tumors in 51%, and pituitary tumors in 35%. Twelve of 14 insulinomas occurred in patients with pituitary tumors. Mutation analysis was completed in 14 of 24 kindreds (80 of the 109 patients). Mutations were most common in exons 2 (31%), 9 (15%), and 10 (23%). All 21 patients with frameshift mutations (and known pancreatic endocrine tumor status) had such tumors. Pituitary tumors were associated with frameshift mutations in exon 2. Conclusions: The type and location of MEN1 mutations may be associated with the phenotypic expression of specific tumors. Such information may assist in the genetic counseling and surveillance of at-risk patients. A specific genotype-phenotype correlation is unlikely because of the heterogeneity of the mutations in the MEN1 gene.