Management of occult adrenocorticotropin-secreting bronchial carcinoids: limits of endocrine testing and imaging techniques.

The differential diagnosis and the identification of the source of ACTH in occult ectopic Cushing's syndrome due to a bronchial carcinoid still represents a challenge for the endocrinologist. We report our experience in six patients with occult bronchial carcinoid in whom extensive hormonal, imaging, and scintigraphic evaluation was performed. All patients presented with hypercortisolism associated with high plasma ACTH values. The CRH test and high dose dexamethasone suppression test suggested an ectopic source of ACTH in three of six patients. During bilateral inferior petrosal sinus sampling, none of the patients showed a central to peripheral ACTH gradient. At the time of diagnosis, none of the patients had radiological evidence of the ectopic source of ACTH, whereas pentetreotide scintigraphy identified the lesion in two of four patients. Finally, a chest computed tomography scan revealed the presence of a bronchial lesion in all patients, and pentetreotide scintigraphy identified four of six lesions. In all patients a bronchial carcinoid was found and removed. In one patient with scintigraphic evidence of residual disease after two operations, radioguided surgery, using a hand-held gamma probe after iv administration of radiolabeled pentetreotide, was performed; this allowed detection and removal of residual multiple mediastinal lymph node metastases. In conclusion, our data show that there is not a single endocrine test or imaging procedure accurate enough to diagnose and localize occult ectopic ACTH-secreting bronchial carcinoids. Radioguided surgery appears to be promising in the presence of multiple tumor foci and previous incomplete removal of the tumor.

Left thoracic approach for cancer of cardia: early and late results.

BACKGROUND: The surgical treatment of cancer of the cardia is controversial and results are often disappointing. Concern exists not only with regards to the surgical approach but also to the extent of the resection. The authors analyze their experience over a 20-year period adopting almost exclusively a "limited" esophagogastrectomy with a wide regional lymphadenectomy through a left thoracotomy. The aim of the study is to determine if this approach actually plays a role in the treatment of this tumor. METHODS: 148 patients were evaluated for cardial cancer. Of these 22 (14.8%) were not resectable and 6 (4%) received other types of resections for technical reasons. 120 patients are the basis of the present analysis. More than 75% of patients were in stage III or IV. Follow-up was completed in 92.5% of cases; all surviving patients had at least 5 years of follow-up. RESULTS: Four (3.3%) patients died in the postoperative period. In 6 cases (5%) an anastomotic leakage occurred and this caused the death of 2 patients. Nine (7.5%) patients had severe pulmonary complications. Dysphagia was relieved in all non complicated patients. 13 (10.8%) patients had anastomotic recurrence. Overall survival rate after 5 years was 25.62 +/- 6.1%. A significant difference in survival was noted in patients at stages II and III after 5 years (61.3% vs 18.6, p < 0.02). CONCLUSIONS: This operation has proved to be a good option providing satisfying long-term results and a lower incidence of complications if compared with more extended procedures. It can be performed in the majority of patients with carcinoma of the cardia with a low mortality and morbidity and with excellent palliation of dysphagia. In our opinion it remains an optimum treatment for cardial cancer.

Use of radioguided surgery with [111In]-pentetreotide in the management of an ACTH-secreting bronchial carcinoid causing ectopic Cushing's syndrome.

Intraoperative [111In]-pentetreotide scintigraphy with a hand-held gamma detector probe has recently been proposed to increase the intraoperative detection rate of small neuroendocrine tumors and their metastases. We report a case of a 28-yr-old woman with ectopic Cushing's syndrome due to an ACTH-secreting bronchial carcinoid, in whom the use of radioguided surgery improved disease management. At presentation, radiolabeled pentetreotide scintigraphy was the only procedure able to detect the ectopic source of ACTH. After radiologic confirmation, the patient underwent removal of a bronchial carcinoid, with disease persistence. After surgery, pentetreotide scintigraphy showed pathologic uptake in the mediastinum not previously detected at surgery and only subsequently confirmed by radiologic studies. Despite a second thoracic exploration, hormonal, scintigraphic, and radiological evidence of residual disease persisted. Radioguided surgery was then performed using a hand-held gamma probe 48 h after iv administration of a tracer dose of radiolabeled [111In-DTPA-D-Phe1]-pentetreotide, which permitted detection and removal of multiple residual mediastinal lymph node metastases. Clinical and radiologic cure, with no evidence of tracer uptake at pentetreotide scintigraphy, was subsequently observed. The use of an intraoperative gamma counter appears a promising procedure in the management of metastatic ACTH-secreting bronchial carcinoids.

Preliminary report of a kindred affected from MEN IIa.

Preliminary data of a kindred with multiple endocrine neoplasia type IIa (MEN IIa) are presented. The study concerns 20 subjects belonging to 4 generations. The first is a man, previously adrenalectomized for pheochromocytoma, suffering from thyroid node with high serum calcitonin (CT) and normal tests for parathyroid function, who underwent total thyroidectomy, removal of a grossly enlarged parathyroid and subsequent autotransplantation, because the other glands seemed to be macroscopically uninvolved. In 4 further patients total thyroidectomy and removal of the parathyroid glands with autotransplantation were performed. In all patients, despite biochemical tests had disclosed parathyroid hyperfunction in only one, one or more enlarged parathyroid glands were found, subsequently diagnosed as adenomatous or with focal areas of "chief-cell" hyperplasia. In 3 out of 4 patients, a single test among those performed to detect the presence of a pheochromocytoma was positive. However, because of the lack of a clear correlation among data from biochemistry, stimulation tests and morphological investigations, adrenalectomy has not been performed. Nevertheless these patients will be closely followed up, in order to promptly state when andrenalectomy has to be performed.

Seventeen-year-long follow-up of a family affected by type 2A multiple endocrine neoplasia (MEN 2A).

This paper reports the results of a 17-year-long follow-up covering 17 members of a family affected by multiple endocrine neoplasia (MEN) type 2A, first diagnosed in 1980. This family is enrolled in our screening program. The thyroid, parathyroid and adrenal glands of the family members were investigated using the most sophisticated and sensitive techniques which have become available during this period, and their DNA was genetically tested for detecting RET mutations. Thanks to the combination of these two approaches it was possible to confirm the diagnosis in the members concerned from the genetic point of view, and to achieve an early diagnosis in the young members of the last generation before the clinical onset of the disease. The detection of a RET mutation also prompted a prophylactic thyroidectomy in a four year-old boy, in a pre-tumoral stage of the disease. Lastly, evidence is provided that genetic analysis of the DNA of the chorionic villi can be carried out as a prenatal test during routine amniocentesis.