S. Silvera

18F-Fluorodeoxyglucose Positron Emission Tomography for the Diagnosis of Adrenocortical Tumors: A Prospective Study in 77 Operated Patients

CONTEXT Most adrenal incidentalomas are nonfunctioning adrenocortical adenomas (ACAs). Adrenocortical carcinomas (ACCs) are rare but should be recognized at an early stage. OBJECTIVE The objective of the study was to evaluate the usefulness of (18)F-fluorodeoxyglucose positron emission tomography ((18)F-FDG PET) to predict malignancy in patients without a previous history of cancer. DESIGN This was a prospective, multicenter study from 2001 to 2006. SETTING The study was conducted at a network of seven university hospitals in Paris. PATIENTS Seventy-seven patients were included. All underwent surgery because of hypersecretory and/or growing benign lesions (n = 18), obvious ACCs (n = 21), or radiologically indeterminate lesions (n = 38). MAIN OUTCOME MEASURE The degree of (18)F-FDG PET uptake [maximum standardized uptake value (maxSUV)] was related to the pathological findings serving as a reference, and its diagnostic value was compared with that of computerized tomography (CT) scan. RESULTS Pathology eventually diagnosed 43 ACAs, 22 ACCs, and 12 nonadrenocortical lesions. Using a cutoff value above 1.45 for adrenal to liver maxSUV ratio, the sensitivity and specificity to distinguish ACAs from ACCs were, respectively, 1.00 (95% confidence interval 0.85-1.00) and 0.88 (95% confidence interval 0.75-0.96). Among the 38 indeterminate lesions at CT scan, we could analyze a subgroup of 16 adrenocortical tumors with high unenhanced density (>10 HU) and an inappropriate washout: (18)F-FDG PET correctly predicted the benignity in 13 of 15 ACAs. CONCLUSIONS In a multidisciplinary team approach, (18)F-FDG PET helps to manage suspicious CT scan lesions. An adrenal to liver maxSUV ratio less than 1.45 is highly predictive of a benign lesion.

Efficiency and Tolerance of mitotane in Cushing's Disease in 76 patients from a single center

CONTEXT Alternatives to transsphenoidal pituitary surgery may be required in Cushings disease (CD) as a first- or second-line treatment. Mitotane is a potent anti-cortisolic drug but has been rarely investigated in the treatment of CD. OBJECTIVE Evaluation of the efficacy and tolerance of mitotane in CD patients. DESIGN AND SETTING Retrospective analysis of 76 patients treated with mitotane from 219 patients diagnosed with CD between 1993 and 2009 in a single center. MAIN OUTCOME MEASURE Remission was defined as normalization of 24-h urinary free cortisol (24-h-UFC). RESULTS Remission was achieved in 48 (72%) of the 67 long-term treated patients, after a median time of 6.7 (5.2-8.2) months. Mean plasma mitotane concentration at the time of remission was 10.5 ± 8.9 mg/l, with a mean daily dose of 2.6 ± 1.1 g. A negative linear relationship was observed between plasma mitotane concentration and 24-h-UFC (P<0.0001). Seventeen of 24 (71%) patients with durable remission subsequently experienced recurrence, after a median time of 13.2 (5.0-67.9) months. At the time of treatment discontinuation, ACTH concentration was statistically associated with a lower recurrence probability (hazard ratios 0.57 (0.32-1.00), P=0.05). Intolerance leading to treatment discontinuation occurred in 19 patients (29%). A pituitary adenoma became identifiable during mitotane treatment in 12 (25%) of the 48 patients with initial negative pituitary imaging allowing subsequent transsphenoidal surgery. CONCLUSION Mitotane is useful at different stages of CD. Mitotane dose adjustment based on plasma concentration monitoring and side effects could control hypercortisolism in the majority of CD patients.

Recurrent liver abscess secondary to ingested fish bone migration: report of a case.

Pyogenic liver abscess is an unusual cause of fever and abdominal pain, but it is potentially fatal. It is rarely caused by a local event, but rather by hematogenous dissemination or biliary tract disease. We report an uncommon case of liver abscess caused by the migration of a fish bone through the gastrointestinal wall.

Pregnancy Does Not Accelerate Corticotroph Tumor Progression in Nelson's Syndrome

CONTEXT Pituitary surgery is the first line of treatment for Cushings disease; when surgery fails, bilateral adrenalectomy may be proposed, particularly for women with a desire for pregnancy. Little is known about the impact of pregnancy on corticotroph tumor progression after bilateral adrenalectomy. OBJECTIVE The aim was to evaluate the impact of pregnancy on corticotroph tumor progression after bilateral adrenalectomy in Cushings disease and to assess maternal and pregnancy outcomes. DESIGN We conducted a retrospective cohort study. SETTING Patients who became pregnant after bilateral adrenalectomy were followed in a single center. PATIENTS Twenty pregnancies from 11 patients with Cushings disease were treated by bilateral adrenalectomy and no pituitary irradiation. MEASUREMENTS Corticotroph tumor progression was assessed by serial pituitary magnetic resonance imaging and plasma ACTH measurements before, during, and after pregnancy. Comparisons were performed using paired Wilcoxon rank tests. Data on maternal and neonatal outcomes were recorded by correspondence from patients and obstetricians. RESULTS Corticotroph tumor progression occurred in eight of 17 pregnancies, and ACTH increased in eight of 10 pregnancies. However, rates of increase during or after pregnancy were not faster than those observed before pregnancy. Maternal complications occurred in four pregnancies from two patients, including gestational hypertension in three and gestational diabetes mellitus in three. Among these four pregnancies, three had a favorable outcome, and one led to an in utero death after eclampsia, due to loss to follow-up. No other maternal or fetal complications were reported. CONCLUSION Pregnancy does not accelerate corticotroph tumor progression after bilateral adrenalectomy. Pregnancy is manageable, provided the patients can be followed closely.

Value of 18-F-FDG PET/CT and CT in the Diagnosis of Indeterminate Adrenal Masses

The purpose of this paper was to study the value of 18-FDG PET/CT and reassess the value of CT for the characterization of indeterminate adrenal masses. 66 patients with 67 indeterminate adrenal masses were included in our study. CT/MRI images and 18F-FDG PET/CT data were evaluated blindly for tumor morphology, enhancement features, apparent diffusion coefficient values, maximum standardized uptake values, and adrenal-to-liver maxSUV ratio. The study population comprised pathologically confirmed 16 adenomas, 19 metastases, and 32 adrenocortical carcinomas. Macroscopic fat was observed in 62.5% of the atypical adenomas at CT but not in malignant masses. On 18F-FDG PET/CT, SUVmax and adrenal-to-liver maxSUV ratio were significantly lower in adenomas than in malignant tumors. An SUVmax value of less than 3.7 or an adrenal-to-liver maxSUV ratio of less than 1.29 is highly predictive of benignity.

When adrenal Cushing's and phaeochromocytoma meet

Department of Endocrinology (C Ghander MD, D Lalej MD, Prof L Groussin PhD), Department of Nuclear Medicine (F Tenenbaum MD), Department of Pathology (F Tissier PhD), Department of Radiology (S Silvera MD), and Department of Endocrine Surgery (Prof B Dousset MD), Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Paris, France; and Institut National de la Santé et de la Recherche Médicale Unité, Institut Cochin, Paris, France (F Tissier, Prof L Groussin)

A Concomitant False-Negative 18F-FDG PET Imaging in an Adrenocortical Carcinoma and a High Uptake in a Corresponding Liver Metastasis

Institut National de la Sante et de la Recherche Mediale Unite 1016, Centre National de la Recherche Scientifique Unite Mixte de Recherche 8104, Institut Cochin, Department of Endocrinology, Metabolism and Cancer (F.Ti., B.Ra., J.B., X.B., B.D., L.G.), 75014 Paris, France; Universite Paris Descartes, Sorbonne Paris Cite, Faculte de Medecine (C.G., F.Ti., J.B., X.B., B.D., B.Ri., P.L., L.G.), 75006 Paris, France; and Assistance Publique des Hopitaux de Paris, Hopital Cochin, Department of Endocrinology, Center for Rare Adrenal Diseases (C.G., J.B., X.B, L.G.), INCa Comete Network (F.Ti., J.B., B.D., X.B.), and Departments of Pathology (F.Ti.), Radiology (S.S., P.L.), Digestive and Endocrine Surgery (B.D.), and Nuclear Medicine (F.Te., B.Ri.), 75014 Paris, France

Unexpected diagnosis for an adrenal tumor: synovial sarcoma

A 39-year-old woman presented with an incidentally discovered mass of the left adrenal fossa. Computed tomography and magnetic resonance imaging did not show any other lesion. Histologically, this mass was composed of a dense proliferation of spindle cells with a fibrosarcomatous-like pattern. Immunohistochemistry using anticytokeratin showed some epithelial cells within the tumor. The diagnosis of primitive synovial sarcoma of the left adrenal fossa was confirmed by the presence of the characteristic t(X;18) translocation. Despite radiotherapy, several chemotherapies, and 2 other surgical resections, the patient died 30 months after the initial diagnosis. To our knowledge, this report constitutes the first described case of synovial sarcoma arising in the adrenal gland.

IRM de diffusion en imagerie musculo-tendineuse et ligamentaire : interet pour les lesions traumatiques du LCA

Objectifs Connaitre les avancees recentes sur l’application des sequences en diffusion aux structures musculo-tendineuses. Decrire l’imagerie des muscles et des tendons obtenue en diffusion. Connaitre une application pratique : les lesions traumatiques du LCA. Points cles Visualisation indirecte de la structure musculaire et tendineuse. Detection precoce de certains phenomenes pathologiques intramusculaires. L’IRM de diffusion differencie de maniere fiable les ruptures partielles du LCA des ruptures completes. Resume Utilisees en neuro-radiologie, les sequences IRM en diffusion sont maintenant etudiees dans d’autres domaines de la radiologie. Les sequences en diffusion et tenseur de diffusion permettent d’observer indirectement l’anatomie cellulaire des elements musculaires et tendineux ainsi que les modifications structurales induites par differents stimuli. Elles montrent egalement les plages de souffrance musculaire des myopathies mais sans caractere de specificite. Concernant les ligaments, notre etude sur les lesions traumatiques du LCA a montre que l’association des sequences Tl-T2FatSat-Diffusion presente une meilleure sensibilite et specificite pour le diagnostic des ruptures partielles ou completes du LCA par rapport aux sequences T1-T2FatSat utilisees seules.

From benign adrenal incidentaloma to adrenocortical carcinoma: an exceptional random event

New European guidelines for the management of adrenal incidentalomas were recently released. One of the most novel recommendations is to stop following patients when they present a typical, small and non-secreting adenoma. We report here the case of a 71-year-old man with such an adenoma, who developed an adrenocortical carcinoma (ACC) fourteen years later, with subsequent metastases and death. Clinically, he had a normal blood pressure and no sign of hormonal hypersecretion. The hormonal work-up showed no hormone excess: urinary free cortisol level was normal, the diurnal cortisol rhythm was respected and urinary catecholamine metabolites levels were normal. Computed tomography (CT) scan showed a homogeneous lesion, with a low density. The lesion remained unchanged during the five years of follow-up. Eight years after the last CT, a large right heterogeneous adrenal mass was incidentally discovered during an ultrasound examination. On CT scan, it was a 6 cm heterogeneous tumor. On hormonal work-up, there was no secretion. The patient was operated of an adrenalectomy, and the histology described an ACC with a Weiss score at 8, with no benign contingent. To our knowledge, this is the first case of an ACC occurring in a patient with prior adrenal imaging showing a typical benign adenoma.