Search

Introduction: Erdheim-Chester disease is a rare form of non-langerhans histiocytosis and its etiology is still not well established. The aims of the study were to assess the value of the bone scintigraphy and the 18 F-FDG PET/CT for the diagnostic and for the latter in the therapeutic evaluation., Methods: We retrospectively reviewed 49 patients suspected of Erdheim-Chester disease between 2004 and 2016. Bone scintigraphy was compared with histopathology and PET-CT to conventional morphological examinations and bone scintigraphy. For therapeutic evaluation, thresholds similar to PERCIST 1.0 were used., Results: Forty-nine bone scintigraphy were evaluated with a sensitivity of 100%, a specificity 97%, a positive predictive value 90% and a negative predictive value of 100%. Eight patients had at least an initial PET-CT. The sensitivity compared to conventional morphological examinations differed from the location but was excellent for orbital, bone and vascular involvements. Specificity was comparable between the different examinations. Six patients treated with interferon ® and three with vemurafenib ® were followed by PET-CT. PET-CT, in agreement to clinicobiological data, identified 4 partial responses and one complete response with interferon ® et two partial responses and one complete response with vemurafenib ® ., Conclusion: Our retrospective study suggests that bone scintigraphy and 18F-FDG PET/CT could be useful in the initial assessment of Erdheim-Chester disease but also for the latter in the therapeutic evaluation., (Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Purpose: Fluorodeoxyglucose positron emission tomography (FDG-PET) is a promising imaging technique that has already proven effective in modifying patient care in oncology. Fluorodeoxyglucose still remains the main radiopharmaceutical agent routinely used for PET imaging. A growing interest has recently lead to broaden PET research on benign disorders. The field of inflammatory or immune diseases and globally the field of internal medicine could also be impacted by FDG-PET., Main Points: Great vessels vasculitides and fever of unknown origin have both been studied by several teams and could become indications for PET. In addition, current indications now extend to paraneoplastic syndromes. It is thus possible to foresee that the clinical applications for PET will continue to expand in these patients., Perspectives and Projects: In the future, inflammatory arthritis, chronic inflammatory bowel diseases, systemic erythematous lupus, histiocytosis, or pulmonary and retroperitoneal fibrosis might benefit from PET even if, available data remains scarce to this day. Although PET will probably alter the landscape of patient management in internal medicine in the near future, additional clinical research is still needed to ascertain the exact role of PET.

Introduction: Takayasu arteritis (TA) is an inflammatory arteritis affecting large vessels, predominantly the aorta, its main branches, and the pulmonary arteries. Up to now, arteriography was considered as the "gold standard". But others exams are emerging in the management of TA: vascular ultrasound, angio-scanner, magnetic resonance imaging and 18FDG positron emission tomography (18FDG PET). Such investigations allow a study of the lumen but also of the arterial walls. However, at the time, no biological or radiological test is able to determine the activity of TA. 18FDG PET could be effective to estimate the disease activity., Exegesis: We report the case of a young woman for who 18FDG PET permit to assert a relapse of TA., Conclusion: 18FDG PET could be effective to estimate the disease activity.

Aim of the Study: To evaluate the efficiency of preoperative parathyroid ultrasonography and scintigraphy in the management of renal hyperparathyroidism., Patients and Methods: The charts of the last consecutive 200 patients who underwent surgery for renal hyperparathyroidism from 1998 to 2003 were retrospectively reviewed to collect data concerning parathyroid gland function, results of preoperative ultrasonography and scintigraphy, as well as modalities and results of surgical exploration., Results: Ultrasonography and scintigraphy sensibilities were 36.4% and 49.3%, respectively. Efficiency of both examinations was improved when they were combined (sensibility of 64.7%) and in those patients managed for recurrent hyperparathyroidism. Were more often detected by preoperative examinations glands with high weight and/or greatest diameter, orthotopic and inferior glands as well as glands exhibiting nodular hyperplasia content upon pathological examination., Conclusion: Parathyroid ultrasonography and scintigraphy are of poor interest in the management of renal hyperparathyroidism. In a preoperative setting, they should be performed only in patients with recurrent disease.

This Retrospective Study Aims: To define a clinical and secretory profile of paragangliomas extra-adrenal chromaffin tumors., Methods: From 1971 throughout 2002, 39 paragangliomas have been observed in 38 patients (22 male, 16 female, average age 41,2 years)., Results: Four were located above the diaphragm, 35 were sub-phrenic (6 of the organ of Zuckerkandl), 32 secreted catecholamines, 23 were hypertensive (with only one without hypersecretion of catecholamines). Among 29 (131)I-metaiodobenzylguanidine scans (MIBG) reviewed, 20 tumors took up the radiopharmaceutical. The treatment was surgical in 35 cases with addition of external radiotherapy and MIBG in one case each; two patients died before any treatment. Two patients with persistent disease after surgery were successfully treated by surgery or MIBG. Histologically, 20 were malignant and 17 were seemingly benign. All exclusive dopamine secreting paragangliomas were malignant. Six patients relapsed two of which for a tumor initially classified as benign. The treatment of recurrences was surgical, by MIBG or by external radiotherapy. Nine patients had a family history of chromaffin tumor(s). The genetic survey made in five of these nine patients was positive in all cases.

Aims: To analyse the results of re-operations for persistent (p) or recurrent (r) primary hyperparathyroidism (PHPT)., Patients and Methods: From 1965 throughout 2001, 1888 patients were operated on for PHPT. The cure rate after initial surgery was 97.6%. Seventy-seven (4.1%) were reoperated for p PHPT (n = 54) or r PHPT (n = 23). Thirty-two out of 77 (41%) had been primarily operated elsewhere. In 15 cases (20%) PHPT was genetically determined. The re-operation was undertaken on average 40.7 months after initial surgery (1 day-190 months)., Results: Two out of 77 were cases of familial hypocalciuric hypercalcaemia. Among the 75 patients reoperated for true PHPT, 23 (31%) had uniglandular disease (UGD) and 52 (69%) had multiglandular disease (MGD). There were two cases of recurrent parathyroid carcinoma. Overall 97 pathological glands were resected, 37% being orthotopic and 63% heterotopic. The re-operation was performed by a cervical approach in 80%, by a mediastinal approach in 15%, whereas 5% involved excision of antebrachial implants. In 96% of cases the parathyroid glands were in the cervical position. Among the preoperative localisations studies the sensitivity of scintigraphy utilising 2-methoxyisobutyl-isonitril (MIBI) was 61%. Utilising both MIBI and cervical ultrasound the sensitivity was 64%. Sixty-eight out of 75 (91%) were cured of their hypercalcaemia, but at the cost of permanent hypoparathyroidism in 9% of cases. No sporadic adenoma appears to have been missed. The seven failures after re-operation (9%) involved five cases of MGD, of which four were sporadic, two cases of carcinoma and one case of parathyreomatosis. 39 patients (51%) had more than four parathyroid glands and in 22/39 cases at least one supernumerary gland was pathological., Conclusion: The re-operations for PHPT were essentially due to MGD that was either sporadic or genetically determined. Often the offending supernumerary gland was not detected by imaging studies. Avoiding failures entails an initial bilateral cervicotomy with thymic exploration after MIBI scintigraphy to exclude a mediastinal focus.

The use of fluorine-18 fluorodeoxyglucose (FDG) in positron emission tomography (PET) is a major advance in imaging in the recent past. Its use in breast and gynaecologic cancers is not well established and needs to be discussed for each patient. This discussion must rely on a better knowledge of potential interests as well as of the limits of metabolic or logistic limits of PET.

One hundred patients with primary hyperparathyroidism underwent radioguided surgery within 90 to 180 minutes following IV injection of a diagnostic dose of MIBI and after parathyroid scanning with planar and oblique views. Exclusion criteria were thyroid pathology requiring surgery and suspicion of multiple endocrine neoplasia. A>20% step-up between the background noise and tissue uptake was the diagnostic threshold for parathyroid hyperfunctionning tissue, and this was observed in only 15% of cases overall all cured, but two. This method of detection is technically demanding and various angles of application of the probe can result in significant discrepancies of recorded uptake for the same spot. Our results demonstrate a physiological step-up between the ipsilateral unaffected upper and lower quadrants of the neck (range: - 34% to - 5%), seemingly because of the proximity of supra-aortic vessels (upper neck-upper mediastinal gradient: - 57% to - 21%). Therefore, significant ratios are meaningful only between either the symmetrical left and right controlateral quadrants, 8% of pre-operative scannings were non-contributory, and probe detection was contributory in 3/8 cases only. Benefit of the technique is limited in routine, but it can be helpful in redo cases if the offending gland is not located in close surroundings of tissues physiologically taking up the radio pharmaceutical (salivary glands, great vessels and heart).

Aim: The classic morphological techniques for the localization of insulinomas and gastrinomas are of limited value. Endoscopic ultrasonography and somatostatin receptor scintigraphy have shown high sensitivity for the detection of gastroenteropancreatic endocrine tumors. The aim of the study was to evaluate the sensitivity of endoscopic ultrasonography and that of somatostatin receptor scintigraphy in the localization of insulinomas and gastrinomas., Patients and Methods: This retrospective study concerned 54 patients with insulinoma (n=29) or gastrinoma (n=26) operated on between March 1991 and March 2000 and who had at least one among the two tested examinations. Forty-two patients had scintigraphy (17 with insulinoma, 25 with gastrinoma), 47 had endoscopic ultrasonography (28 with insulinoma, 17 with gastrinoma). One of the ten patients with MEN 1 had both tumors. All diagnosis were confirmed by histologic examination., Results: The sensitivity of scintigraphy for the localization of insulinomas was 47%. There was one false positive. Sensitivity of endoscopic ultrasonography for insulinomas was 85%. The sensitivity of scintigraphy in the detection of gastrinomas was 65% for the tumors in the duodenopancreatic area, 20% for the tumors in the pancreatic tail and 71% for metastasis. The sensitivity of endoscopic ultrasonography was 46% for duodenal tumors, 75% for pancreatic tumors and 57% for lymph node metastasis. The combination of both localization studies increased sensitivity to 94%., Conclusion: Endoscopic ultrasonography and somatostatin receptor scintigraphy are the gold standard for localization of gastrinomas. Association of both examinations increases the sensitivity. Scintigraphy for the detection of insulinomas should be performed when endoscopic ultrasonography is negative.

Study Aim: To report feasibility and efficacy of radioguided mini invasive hyperparathyroidism surgery., Patients and Method: From November 1998 to August 2000, 75 patients with primary hyperparathyroidism have been operated on by radioguided surgery within 90 to 180 minutes following i.v. injection of a diagnostic dose of MIBI and after parathyroid scanning with planar and oblique views. Exclusion criteria were thyroid pathology requiring surgery and suspicion of multiple endocrine neoplasia. A 20% step-up between the background noise and tissue uptake was the diagnostic threshold for parathyroid hyperfunctioning tissue., Results: A 20% step-up was observed in only 17% of cases overall; all cured, but two. Various angles of application of the probe can result in significant discrepancies of recorded uptake for the same spot. These results demonstrate a physiological step-up between the ipsilateral unaffected upper and lower quadrants of the neck (range: -17 to -8%), because of the proximity of supraortic vessels (upper neck-upper mediastinal gradient: -44 to -30%). Therefore, significant ratios are meaningful only between either the symmetrical left and right controlateral quadrants respectively, and not between the upper and the lower ipsilateral quadrants. Eleven per cent (8/75) of preoperative scannings were non-contributory, and probe detection was contributory in 3/8 cases only., Conclusion: Benefit of the technique is limited in routine, but it can be helpful in redo cases if the offending gland is not located in close surroundings of tissues physiologically taking up the radiopharmaceutical (salivary glands, great vessels and heart).

Study Aim: CT scan performed for non-adrenal related symptoms detects an adrenal mass or 'incidentaloma' in 0.4 to 4.3% of cases, and most authors advocate a non-operative policy, after minimal but careful work-up aimed at excluding pheochromocytoma and aldosteronoma. The breakthrough of laparoscopic adrenalectomy has led some to challenge this attitude. This retrospective study focused on the outcome of non-operated adrenal masses., Patients and Method: From 1986 through 1999, 126 patients (64 men and 62 women) presented with an incidental mass of the adrenal fossa, and a non-surgical attitude was elicited. Mean size was 36.5 mm in diameter. All patients underwent an in-depth clinical, biochemical and imaging work-up. They have been stratified into two groups: group I: no contraindication to surgery (n = 95); and group II: contraindication to surgery (frail patients, invasive adrenal or metastatic extra-adrenal cancer) (n = 31)., Results: With a mean follow-up of 4.3 years, 17 patients were lost to follow-up (13.5%), including 11/95 in group I; 36 were dead (28.5%), including 12/95 in group I (no adrenal-related death) and 24/31 in group II; 72 were alive and well without operation, including only one in group II; one patient was operated for a benign adrenal adenoma removed at the time of surgery for aortic aneurysm., Conclusion: Careful clinical, biochemical, imaging and nor-iodo-cholesterol scintigraphy with definite uptake by the adrenal mass, a strong indicator of benignancy, allows surgical indication to be postponed, and is likely to cancel it if, at one-year follow-up, imaging studies show no change in the mass.

Aim of the Study: To evaluate the results of parathyroid scinti scans (sestamibi or tetrofosmin) for detection of hyperplastic parathyroid glands responsible for renal hyperparathyroidism., Methods: Injection of 15 mCi sestamibi or tetrofosmin and gammacamera acquisition of images focused on neck and mediastinum, 20 minutes and 2 hours thereafter. Injection of 150 mCi Iodine 123, acquisition of images 2 hours afterwards and visual subtraction., Patients: 51 patients with renal insufficency or renal transplant were referred for surgical treatment of hyperparathyroidism. 52 scintiscans (sestamibi n = 19, tetrofosmin n = 33) were performed before operation (subtotal parathyroidectomy, bilateral thymectomy and parathyroid tissue cryopreservation)., Results: 180 hyperplastic parathyroid glands were resected, 71 of which had been detected by scintiscan. The factors modifying the results were the weight of the resected lesion and reoperation. All hyperplastic glands were detected in only 1 out of 41 scintiscans performed before first hand operations, whereas all missed glands were imaged in 8 out of the 10 explorations performed before reoperation for persistent renal hyperparathyroidism. The radionuclide, the type of hyperparathyroidism, the parathyroid location, patient's age and gender did not influence the results. No false-positive result was observed., Conclusion: Parathyroid scintiscan should not be routinely performed before the first neck exploration for renal hyperparathyroidism. It is mandatory in those cases needing reoperation for recurrent disease.

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.

TSH-secreting adenoma is a rare entity; a series of 69 cases has been collected by Faglia in 1989 and 78 new cases were published from 1987 to 1994. We report two new cases which have been explored by octreotide scintigraphy before treatment with the SRIH analogue. An in vitro SRIH receptor study was also performed in the first patient. This patient, a young man, suffered from hyperthyroidism with enhanced FT3 and FT4 concentrations without decreased TSH values. Plasma alpha subunit level was slightly increased. He had a pituitary tumor, positive in Octreoscan but responded partially to treatment by SRIH analogue. The tumor was in part surgically removed and the SRIH receptors revealed a homogeneous density; their number was equal to those of GH-secreting tumor but their affinity was lower. The second patient, an elderly woman was not surgically treated because the octreotide treatment dramatically improved both tumor volume and thyroid hormone or alpha subunit levels. The tumor, associated to a probable meningioma, was also positive in Octreoscan and was characterized by a highly increased alpha subunit plasma level. These results are discussed by comparison with those of the literature.

Somatotropin- and thyrotropin-secreting adenomas are well known for positive uptake of radio-labeled octreotide in vivo, this fact is not so well assessed for gonadotropin-secreting adenomas (GSA). We report one case of positive somatostatin receptor scintigraphy in a woman suffering from histologically proven GSA. This 63 year old patient has been suffering for two years of akinetic syndrome when the outcome of diplopia led to the discovery of a large hypophyseal tumor spreading till V3 floor and in left cavernous sinus by resonance magnetic imaging (RMI). Clinical examination showed anterior hypopituitarism and bitemporal hemianopsia. Biologically, blood gonadotropins were decreased more on LH (0.6 UI/l, N > 15) than on FSH (10 UI/l; N > 20). A lack of response of gonadotropins to LHRH with low blood estradiol concentration (< 10 pg/ml) was noticed. Basal blood measurement of alpha subunit was at 0.17 microgram/l (N = 0.10-1.6) and increased at 0.39 microgram/l after stimulation by LHRH. Although in low range of normal values, other hypophyseal hormones were normal except prolactinemia (45 mg/L; N < 20), however stimulated by TRH and related to dopaminergic deconnection; Indium 111 labeled octreotide scintigraphy showed an over uptake of the tumor. Three month treatment by octreotide (100 micrograms x 3/day subcutaneously) did not allow to decrease significantly FSH concentration or to reduce the tumoral mass. Incomplete removal of the tumor was performed by transphenoidal route. Immunohistochemical analysis revealed positive immunostaining for alpha subunit and FSH beta on numerous cells while the labeling was slightly less strong for LH beta. These data evoked a GSA. This case record depicts the possibility of detection of GSA by somatostatin receptor imaging. However a positive result does not preclude of somatostatin analog therapeutic efficiency.

Intraoperative radio-isotope detection is an old idea which has been recently revived following the development of new detectors. The authors describe two points of view concerning this technique: external detection or surface detection. Based on the experience of more than 250 cases, they review the current status of the indications for ttris technique in endocrine surgery. They discuss the prospects of this technique in the light of the development of nuclear medicine and the basic requirements of surgery: new tracers and intraoperative scintigraphy.