Thyrotropin variations may explain some positive radioiodine therapy scans in patients with negative diagnostic scans.

UNLABELLED: Thyroglobulin (Tg) is a specific marker of residual thyroid cancer or tumor recurrence. In patients with elevated Tg levels and negative diagnostic radioiodine (131I) whole-body scans (dWBS), administration of a therapy dose may reveal foci that were not initially apparent. The aim of this study was to identify factors, other than 131I activity, which might explain why a post-therapy 131I whole-body scan is sometimes positive despite a negative dWBS. PATIENTS AND METHODS: We reviewed data on all patients with elevated Tg levels and negative dWBS with 185 MBq 131I off-T4 at followup, who subsequently received an empiric therapy dose of 3700 MBq of 131I. During a 5-yr period, 22 patients met these criteria. 131I therapy could be given immediately after negative dWBS in 9 patients, with an average of 8 extra days of hypothyroidism. In the other 13 patients, therapy was given an average of 8 months later. RESULTS: The therapy scan was negative in 16 patients, while it showed uptake in the thyroid bed in 5 patients and distant metastases in two. In the latter two patients, the TSH level was suboptimal at the time of dWBS (9 and 25 microIU/ml), and had risen to 34 and 70 microIU/ml respectively at the time of therapy. Overall, a positive scan following therapy occurred in 7 patients (6/9 patients treated immediately and 1/13 patients treated in a separate setting; p<0.01). In patients with positive therapy scans, the mean TSH level was 73 microIU/ml at the time of dWBS and 103.5 microIU/ml at the time of therapy (41% increase; p<0.05). In patients with negative therapy scans the mean TSH level was 84 microIU/ml at dWBS and 86 microIU/ml at the time of the therapy scan (2% increase). CONCLUSIONS: Our study suggests that interval increase in TSH level with a longer period of stimulation may have contributed to making the whole-body scan positive at the time of therapy. Nowadays, patients with elevated Tg are directly given a therapy dose of 131I. Special care should be taken when preparing patients who have been on suppressive levothyroxine therapy for a long time, in order to avoid misclassifying the tumor as non-functioning.

Sick leave for follow-up control in thyroid cancer patients: comparison between stimulation with Thyrogen and thyroid hormone withdrawal.

INTRODUCTION: The clinical benefits of recombinant human thyroid-stimulating hormone (rhTSH; Thyrogen) are well established as an alternative stimulation procedure to thyroid hormone withdrawal in the diagnostic follow-up of thyroid cancer patients. By avoiding periods of hypothyroidism, patients do not suffer from a decreased quality of life and keep their ability to work. This study compared the frequency, the duration and the cost of sick leave for follow-up control between rhTSH and withdrawal. METHODS: The study population consisted of patients with thyroid carcinoma first treated by thyroidectomy and radioiodine ablation. Patients were recruited at their control visit between October 2004 and May 2006 in three hospitals, both prospectively and retrospectively. Collection data consisted of patient information, job characteristics and duration of sick leave during the month before and the month after control. The valuation of sick leave used the friction cost method. RESULTS: Among the 306 patients included, 292 (95%) completed the entire questionnaire. The mean age was 46.7 years. Among the 194 active patients, patients treated with rhTSH, when compared with patients treated by withdrawal, were less likely to require sick leave (11 vs 33%; P=0.001). The mean duration of sick leave was shorter (3.1 vs 11.2 days; P=0.002) and indirect costs due to absenteeism accounted for 454 Euro +/- 1673 vs 1537 Euro +/- 2899 for withdrawal stimulation. CONCLUSION: For active patients, rhTSH treatment reduced the length and the cost of sick leave by 8.1 days and 1083 Euro per control respectively, when compared with withdrawal treatment.

[Severe Graves' acropachy and dermopathy. Three case reports]. / Acropachye et dermopathie basedowiennes severes. Trois observations.

INTRODUCTION: Traditionally described, severe Graves' acropachy and tibial myxoedema are now only encountered in certain severe forms of Graves' disease, where they can be difficult to diagnose and hence delay the initiation of treatment. OBSERVATIONS: Three patients presented with severe ophthalmopathy, pretibial myxoedema and acropachy of different clinical forms. DISCUSSION: In supplement to the usual biopsies and X-rays, bone scintigraphy provides early diagnosis of acropachy. The severity of the immune disease, the episodes of hypothyroidism and cigarette smoking are the 3 main factors contributing to these extra-thyroid manifestations of Graves' disease. There is currently no treatment that can permanently resolve the functional and aesthetic problems of dermopathy and acropachy.

[131 iodine for the treatment of benign goiters]. / L'iode 131 comme traitement des goitres bénins.

INTRODUCTION: In order to evaluate the efficacy of 131 Iodine on goitre volume and on thyroid function, we studied a cohort of patients exhibiting a multinodular and toxic or non toxic goitre. METHODS: This retrospective study was conducted at the Marc Linquette clinic in Lille, in collaboration with the department of nuclear medicine. Thirty-eight patients treated with 131 Iodine were included from 1995 to 2001. Clinical examination and serum analyses including TSH, free T4 and T3, anti-thyroid peroxidase and anti-thyroglobulin antibodies and TSH-receptor antibodies measurements were conducted on inclusion and then at 3, 6, 12 and 72 months. The activity of 131 Iodine corresponded to a standard dose or was calculated according to Marinelli's method. We excluded patients who had not undergone assessment at the above-mentioned time schedules. RESULTS: The treatment was indicated in 30 patients presenting with a non compressive but toxic goitre, in 5 patients with a toxic compressive goitre and in 3 patients with a compressive but non-toxic goitre. Surgery had been excluded for all these patients because of their age, their cardiac status or because they had refused surgery after failure with prior partial thyroidectomy or medical treatment. Among the toxic goitres, TSH levels were low and T3 and T4 increased in 17 patients. In the 18 others, hyperthyroidism was manifested by an isolated decrease of TSH. The thyroid volume before treatment, assessed in 20 patients, was of 18 to 135 cm3 (mean: 53 cm3). Treatment consisted in administration of radioactivity of 3 to 30 mCi in 30 patients and standard activity of 20 to 25 mCi in 8. Functional efficacy with reduction in hyperthyroidism was noted after 3 months, and corrected in nearly all patients after 1 year, and morphological efficacy, with a mean decrease of 33.5% in the size of the goitres. No supplementary surgery was required, notably for the initially compressed goitres. Immediate and long term tolerance was satisfactory. CONCLUSION: Metabolic 131Iodine radiotherapy is effective for the functional and morphological treatment of goitres with good tolerance and few side effects. 131 Iodine is a reasonable alternative in cases with absolute or relative contraindication for surgery.

Octreotide (long-acting release formulation) treatment in patients with graves' orbitopathy: clinical results of a four-month, randomized, placebo-controlled, double-blind study.

There are few effective, safe modalities for the management of Graves' ophthalmopathy (GO), a cell-mediated immune comorbidity of thyroid disease. Somatostatin analogs inhibit lymphocyte proliferation and activation, and accumulate in the orbital tissue of patients with GO. A double-blind, placebo-controlled study of a long-acting somatostatin analog [16 wk of long-acting release formulation of octreotide (octreotide-LAR)] was conducted in 51 patients with mild active GO with the aim of preventing deterioration and precluding the need for more aggressive therapeutic modalities, such as glucocorticoids or radiotherapy. No treatment effect was observed for the primary end point (a composite parameter defining the outcome as either success or failure on the basis of changes in class/grade of the severity index and Clinical Activity Scale of GO). The Clinical Activity Scale score was reduced for patients treated with octreotide-LAR, but without any significant difference with respect to patients receiving placebo. However, octreotide-LAR significantly reduced proptosis (as measured by exophthalmometry). This was associated with nonsignificant differences in favor of octreotide-LAR in a series of proptosis-related parameters: class III grade, opening of the upper eyelid, the difference in ocular pressure between primary position and upgaze, and extraocular muscle involvement. By magnetic resonance imaging evaluation the extraocular muscle volumes appeared reduced, but nonsignificantly. No significant correlation between the initial uptake of the somatostatin analog indium-labeled and the response to treatment was observed. One patient in the octreotide-LAR group developed gallstones. In this study, octreotide-LAR did not seem suitable to mitigate activity in mild GO. Surprisingly, it significantly reduced proptosis, one of the most debilitating symptoms of GO. Additional studies are warranted to define the benefit to risk ratio of the somatostatin analogs in this indication.

Advances in diagnostic practices affect thyroid cancer incidence in France.

OBJECTIVE: To analyse trends in diagnostic practices of thyroid diseases and to relate them to the increase in thyroid cancer incidence in France over time. DESIGN: From 1980 to 2000, a French retrospective multicentric (three endocrinology and three nuclear medicine centres) study of thyroid diseases was conducted on 20 consecutive unselected patients' records, sampled every 5 years in each centre. METHODS: Characteristics of the population and diagnosis procedures (thyroid ultrasonography (US), radionuclide scan, cytology and hormonal measurements) were described over time. Changing trends in operated patients and in cancer prevalence were analysed as well as the impact of practices on cancer incidence. RESULTS: The study included 471 patients (82% female, mean age 46.7, range 9-84 years), referred for nodular thyroid diseases (66.7%) or thyroid dysfunctions (33.3%). A significant increase in US (3 to 84.8%) and cytological practices (4.5 to 23%), and a decrease (89.4 to 49.6%) in radionuclide scan procedures were observed over time. Although the proportion of patients undergoing surgery remained constant (24.8%), the prevalence of cancer increased among operated patients from 12.5 to 37% (P=0.006). In a Cox's proportional hazard model stratified on the clinical characteristics of patients, only the cytological practice, regardless of its results, was significantly associated with the occurrence of cancer: relative risk (RR)=4.4 (95% confidence interval (CI): 1.1-16; P=0.04). CONCLUSIONS: From 1980 to 2000, a major evolution in clinical practices has led to the increase in thyroid cancer reported in France. Such changes in medical, as well as in surgical and pathological, practices must be taken into account in incidence measurement.

Vitamin D deficiency and severe hyperparathyroidism.

The typical manifestations of severe hypercalcemia with osteitis fibrosa cystica have become exceedingly rare. We describe the case of a woman hospitalized for a tibial tumor with functional impotence, leading to a diagnosis of primary hyperparathyroidism (HPT I) associated with profound vitamin D deficiency. This 31-year-old woman was admitted, after two pregnancies complicated by the HELLP syndrome. Preoperative laboratory values were as follows: calcemia 4.05 mmol/l (2.2-2.6); urinary calcium 30 mmol/24 h (1.25-7.5); parathormone (PTH) 1 195 pg/ml (10-60); and 25 OH-vitamin D 13 nmol/l (22-120). Specific MIBI uptake by the tibial lesion oriented the diagnosis towards a brown tumor. After surgical excision of a parathyroid adenoma and the brown tumor (associated with tibial fracture), calcemia fell to 1.55 mmol/l and normalized after three months. Urinary calcium fell to 0.1 mmol/24 h and remained low during the 2 years following surgery. Vitamin D levels rapidly normalized on supplementation (87 nmol/l). PTH levels fell markedly after surgery but remained higher than normal till 2 years after surgery despite normalization of calcemia three months after. Bone repair, estimated by means of bone densitometry, improved from preoperative Z-score values of - 6.54, - 5.20 and - 3.50 in the left femoral neck, right femoral neck and lumbar spine, respectively, to - 0.20, - 1.55 and - 0.28, respectively, one year after surgery. In conclusion, this case illustrates: 1) the severe osseous expression of HPT probably related to vitamin D deficiency; 2) specific MIBI uptake by the bone lesion, orientating the diagnosis towards a brown tumor; 3) the consequences of vitamin D deficiency on postoperative outcome, with transient severe initial hypocalcemia related to bone calcium avidity; 4) a possible link between HPT and the HELLP syndrome.

Chromogranin A assay and (131)I-MIBG scintigraphy for diagnosis and follow-up of pheochromocytoma.

UNLABELLED: We assessed the performance of a new serum chromogranin A (CgA) assay in combination with the results of (131)I-metaiodobenzylguanidine (MIBG) scintigraphy for diagnosis and follow-up in 89 patients with clinical findings suggestive of pheochromocytoma. METHODS: The study population consisted of 41 patients with proven pheochromocytoma and 48 patients with refuted pheochromocytoma. Eighty-seven scintigraphy examinations were performed, 52 in patients with proven pheochromocytoma (39 before surgery and 13 after surgery) and 35 in patients with refuted pheochromocytoma. RESULTS: The sensitivity of the CgA level was 90.2%, and the specificity was 99.0% and 92.3% in the control and refuted pheochromocytoma groups, respectively. A significant relationship was seen between serum levels of CgA and tumor mass (r = 0.70; P < 10(-5)). The postoperative CgA level was an early and accurate predictor of curative surgery or relapse. The concordance between CgA levels and scintigraphic data was 90.8%. CONCLUSION: Serum CgA level is an effective marker of pheochromocytoma. Increased levels strongly correlate with tumor mass; therefore, small tumors may go undetected. The concordance between CgA level and the results of (131)I-MIBG scintigraphy is high. A CgA level in the reference range is highly predictive of normal scintigraphy findings.

Lingual thyroid. A case report.

We describe an unusual clinical case in which a non functional cervical thyroid coexisted with a functional ectopic lingual thyroid. A twenty-year-old woman was referred for hypothyroidism treated with L-thyroxin related to a basilingual tumor diagnosed 8 years previously. Oropharyngeal examination showed a spherical basilingual tumor 2.5 cm in diameter. Laboratory findings during treatment with 50 microgram of L-thyroxin daily showed: TSH 6,280 microIU/ml (N: 4,4 to 3.6), FT3 4,2 pmol/l (N: 3.3 to 5.1), and FT4 15.4 pmol/l (N: 10,5 to 25,5). Antithyroid antibodies were absent. Cervical ultrasonography showed a small hypoechogenic, heterogeneous orthotopic thyroid gland confirmed by cervical computed tomography thyroid. An I(123) scan revealed uptake above the chin on the profile, and no significant uptake is the area of the normal thyroid. The lack of iodine uptake by the cervical thyroid remains unexplained. In conclusion, this report of an ectopic thyroid location in unusual because of the coexistence of non functional cervical thyroid and of a partially functional lingual thyroid tissue. The pathogenesis of this association remains unclear.

Somatostatin receptor scintigraphy to predict the clinical evolution and therapeutic response of thyroid-associated ophthalmopathy.

Management of thyroid-associated ophthalmopathy remains a topic of controversy. Immunosuppressive treatments have to be applied at peak disease activity and before criteria of severity develop. Expression of somatostatin receptors on activated lymphocytes allows scintigraphic imaging with indium-111 pentetreotide. We conducted a prospective study with 17 patients who presented severe ophthalmopathy (11 Graves' disease, four Hashimoto's thyroiditis, two isolated in appearance: Means' syndrome). Each patient underwent hormonal (free T3 and TSH) and immunological (TBII) assessment, an orbital computed tomography scan or magnetic resonance imaging, a visual functional examination and 111In-pentetreotide orbital scintigraphy before undergoing treatment by steroids and/or radiotherapy, independently of scintigraphic results. At 4 and 24 h after the intravenous injection of 111 MBq of 111In-pentetreotide, planar imaging centred on the head and neck (anterior and both lateral views) was carried out. Retrobulbar uptake was assessed by visual semi-quantitative analysis (score given by two independent trained observers) and by quantitative analyses (regions of interest, orbit/brain uptake indices). Patients were ophthalmologically followed up for 6 months and then classified as improved or not. Visual semi-quantitative analysis of 4-h/24-h planar images was correlated with the ophthalmological evolution (chi 2 test, P < 0.01). All ten patients in whom scintigraphy was considered positive were clinically improved at 6 months, and of the seven patients in whom scintigraphy was negative, six were not improved. Nevertheless, objective quantitative analysis did not succeed in confirming these results. We conclude that 111In-pentetreotide scintigraphy requires further developments, including quantitative single-photon emission tomographic acquisition, if its role as a guide to therapeutic strategy in thyroid-associated ophthalmopathy is to be confirmed.

[Usefulness and limits of intraoperative hormone measurements in surgery of endocrine duodeno-pancreatic tumors: experience of 72 cases]. / [Utilité et limites des dosages hormonaux peropératoires dans la chirurgie des tumeurs endocrines duodéno-pancréatiques: expérience de 72 cas.

Intraoperative hormonal measurement has been successfully used to guide the surgical treatment of various endocrine diseases. In this study, we report the results of intraoperative insulin measurement in patients with organic hypoglycemia (n = 52 operations in 51 patients) and the results of intraoperative gastrin measurement in patients with gastrinoma (n = 20). Measurements were done in the systemic and portal blood at the beginning of the operation, and 20 min after removal of the lesion(s), with intra-operative secretin stimulation test for gastrinoma in some cases. Results accurately predicted cure (insulinomas) or non-cure (half of gastrinomas) of the disease. Limitations of the method are the possibility of normal hormone base line levels at the time of surgery and the importance of secretion of pro-insulin products by insulinomas not taken in account by the assay with monoclonal antibodies.

Factor analysis of dynamic series (FADS) in somatostatin receptor imaging.

UNLABELLED: The aim of this article was to study the physiopathology of tumoral uptake of 111In-pentetreotide using factorial analysis of dynamic series (FADS) and to assess the usefulness of this analysis in somatostatin receptor scintigraphy. METHODS: Forty-one patients were included, 24 women and 17 men. After intravenous injection of 111 MBq 111In-pentetreotide, dynamic image acquisition (68 images of 30 s) began in front of the suspected tumoral site: thoracic in 10 patients with medullary carcinoma of the thyroid and 2 patients with bronchogenic carcinoid, and abdominal in 12 cases of midgut carcinoid and 17 cases of other gastroenteropancreatic neuroendocrine tumors. FADS was performed with FAMIS software. Static images were obtained 4 h and 24 h later. For every patient, surgery and/or clinical follow-up (4 y) was used to classify results as true (T) or false (F) positive (P) or negative (N) and to evaluate both the sensitivity of static images and the usefulness of FADS. RESULTS: Of the 14 cases of carcinoid tumor, 5 patients were TN; 9 patients were TP with static images but only 8 were TP with FADS (a bronchogenic carcinoid of 6 mm was missed). Of the 17 cases of gastroenteropancreatic neuroendocrine tumor, static images were TP in 9 patients, and FADS were TP in 5 of these patients (and 4 FN). Static images and FADS were FN in 4 patients and TN in 3 patients, and in the 2 last patients static images were FP, but FADS were TN. Of the 10 cases of medullary carcinoma of the thyroid, static images and FADS were TN in 1 patient, static images were TP in 3 patients and FADS were TP in 2 of these patients (and 1 FN). In the six last cases, static images were FN, but FADS were FN in 3 patients and TP in 3 patients, showing an infiltrate. CONCLUSION: FADS demonstrates that tumoral kinetics are similar to those of the spleen. FADS can show a diffuse tumoral uptake corresponding to tumoral infiltrate in medullary carcinoma of the thyroid or in hepatic miliaria, whereas static images were normal or doubtful.

Somatostatin receptor imaging in somatotroph and non-functioning pituitary adenomas: correlation with hormonal and visual responses to octreotide.

OBJECTIVE: A multicentre study was undertaken to determine the value of somatostatin receptor (sst) scintigraphy in predicting hormonal and visual responses to octreotide treatment in GH-secreting and non-functioning pituitary adenomas. SUBJECTS AND METHODS: Somatostatin receptor scintigraphy was performed in 48 patients (19 acromegaly, 29 non-functioning pituitary adenomas with ophthalmological defects). Results were expressed as an uptake index of the pituitary area. A threshold for positivity was determined in 23 subjects considered as controls. Thirty-five patients were treated for 1 month with octreotide (300 micrograms daily). The therapeutic response was assessed on GH and IGF-I suppression or evolution of the ophthalmological defects. The relationships between the somatostatin receptor scintigraphy result, the therapeutic effect of octreotide and in vitro studies performed in 12 tumours were studied. RESULTS: From the results of control subjects the uptake index threshold for positivity was 2. In patients, somatostatin receptor scintigraphy was positive in 64% and there was no relationship between uptake index and tumour size. In GH tumours, somatostatin receptor scintigraphy was positive in 68%; uptake index was related to octreotide-induced GH and IGF I suppression. The positive predictive value was 100% and the negative predictive value was 50%. In vitro studies showed detectable binding sites for somatostatin with sst2 and sst5 expression in the 4 GH tumours studied although somatostatin receptor scintigraphy was negative in 2 cases. In non-functioning pituitary adenomas somatostatin receptor scintigraphy was positive in 62%. Based on visual effects, the positive predictive value was 61% and the negative predictive value was 100%. A wide distribution of somatostatin binding sites was found in 8 non-functioning pituitary adenomas with expression of sst2 only. CONCLUSION: In the conditions of the study, in patients with acromegaly, positive somatostatin receptor scintigraphy predicts a hormonal response but the value of somatostatin receptor scintigraphy is limited by its low negative predictive value. In patients with non-functioning pituitary adenomas, negative somatostatin receptor scintigraphy predicts that there will be no visual improvement during octreotide treatment.

[Preoperative localization of parathyroid lesions with Tc-99m-MIBI/iodine 123]. / Localisation préopératoire des lésions parathyroïdiennes par la scintigraphie 99mTc MIBI/iode 123.

OBJECTIVE: Assess the sensitivity of MIBI-Tc for the detection of parathyroid lesions and define the threshold of detection by evaluating contrast uptake at 20 minutes and 2 hours. METHODS: Fifty-five operated and cured patients were studied. Image acquisitions were made 20 minutes and 2 hours after injection of 555 MBq MIBI-Tc then 2 hours after injection of 5.55 MBq iodine 123. RESULTS: A total of 87 pathological glands were found including 47 adenomas (80 to 8820 mg) and 40 hyperplasias (44 to 2175 mg). Detection sensitivity was 81% for adenomas and 30% for hyperplasia, but the cell type did not appear to be a determining factor. CONCLUSION: Scintigraphic visualization of parathyroid lesions is more frequent when there is a single lesion. At least one lesion was seen in 80% of the patients. The threshold of detection was 225 mg. Enhancement of parathyroid uptake with MIBI-Tc compared with the thyroid between 20 minutes and 2 hours was not a constant and of unknown origin.

Comparison of technetium-99m methoxyisobutylisonitrile and gallium-67 citrate scanning in the assessment of lymphomas.

The aim of this study was to compare the value of scintigraphy using technetium-99m methoxyisobutylisonitrile (MIBI) with that of scintigraphy using gallium-67 citrate in the assessment of Hodgkin's disease and non-Hodgkin's lymphoma and to relate these results with those of CT scan and MRI. Fifty-eight patients were included either for a follow-up examination or for monitoring of their treatment. Twenty-three residual masses were studied. A whole-body scan was performed, followed by single-photon emission computed tomography (SPET) 20 min after injection of 740 MBq of 99mTc-MIBI and 72 h after injection of 185 MBq of 67Ga citrate. The overall sensitivity of 99mTc-MIBI and 67Ga citrate was 71% and 68%, respectively, and the overall specificity was 76% and 44%, respectively. For residual masses, the sensitivity was 44% with both tracers and the specificity was 80% with 99mTc-MIBI and 53% with 67Ga citrate. The positive predictive values were 85% and 68% and the negative predictive values were 59% and 44%, respectively. The signal-to-background ratio was 1.5 for 99mTc-MIBI and 2 for 67Ga citrate. At present, 99mTc-MIBI cannot replace 67Ga citrate in the assessment of lymphomas.

Scintiscans and carcinoid tumors.

BACKGROUND: The presence of somatostatin receptors on carcinoid tumors mediates imaging of tumor extent and inhibition of tumor growth and marker secretion. This prospective study aimed to evaluate radiolabeled somatostatin analog scans in the therapeutic workup of carcinoids. METHODS: Twenty-one patients with carcinoids underwent 26 scans with iodine octreotide or indium pentetreotide. The results for tumor and metastases imaging were analyzed and compared with those of a short inhibition test of marker secretion and with those of metaiodobenzylguanidine scan. RESULTS: The sensitivity for obtaining images of the overall 43 tumor sites was 72%. We had no false-positive results. Unknown tumor sites were discovered in three patients. The results were slightly better with indium pentetreotide and metastases imaging. A positive scan did not always preclude responsiveness to the functional effect of octreotide. Results of somatostatin analog scans were better than those with metaidobenzylguanidine. The two techniques were complementary in one patient. CONCLUSIONS: The choice of treatment for patients with carcinoid tumors should benefit from functional inhibition test with octreotide and from indium pentetreotide and metaidobenzylguanidine scans.

Somatostatin analogs for the localization and preoperative treatment of an adrenocorticotropin-secreting bronchial carcinoid tumor.

The diagnosis of the ectopic ACTH syndrome often remains difficult. Although bilateral inferior petrosal sinus sampling has recently offered a new approach, it does not help to localize an occult nonpituitary tumor. We report the case of a 45-yr-old woman whose hypercortisolism highly suggested the ectopic ACTH syndrome: elevated urinary free cortisol (3234 nmol/day, normal 28-143) was not suppressed by the high-dose dexamethasone test (2789 nmol/day); increased plasma ACTH (21.8 pmol/L, normal 2-11.4) did not respond to the ovine CRH test (23.8 pmol/L); and pituitary magnetic resonance imaging was negative. The thorax computed tomographic scan showed a questionable 7-mm nodular lesion in the upper part of the left lung. Because a 3-day trial of octreotide administration (200 micrograms sc every 8 h) induced a dramatic clinical and biological response with a drop in urinary free cortisol from 1738 to 441 nmol/day we performed a scintigraphy with [111In]pentetreotide; it revealed a single-well limited area of abnormal uptake at the exact location of the suspected thoracic lesion. This nodule was removed surgically after preparation of the patient by a 1-month treatment with octreotide: the tumor proved to be a typical bronchial carcinoid, containing extremely high concentrations of immunoreactive ACTH (198 pmol/mg wet wt tissue) and POMC messenger RNA by Northern blot. The presence of somatostatin receptors in the tumor was confirmed by in vitro radioautography. After surgery plasma cortisol and ACTH were undetectable. Somatostatin radioanalog scintigraphy should be considered as a new investigative tool in patients with suspected ectopic ACTH syndrome.