19808858

Adrenocortical Tumor with Two Distinct Elements Revealed by Combined 18F-Fluorodeoxyglucose Positron Emission Tomography and 131I Nor-Cholesterol Scintigraphy

Rossella Libé, Frédérique Tissier, Marie Bienvenu, Lionel Groussin, Sylvie Joannidis, Chantal Hignette, Bertrand Dousset, Paul Legmann, Marc Faraggi, Bruno Richard, Xavier Bertagna, and Florence Tenenbaum

Institut National de la Santé et de la Recherche Médiale Unité 567 (R.L., F.Ti., L.G., X.B.), Centre National de la Recherche Scientifique Unité Mixte de Recherche 8104, Institut Cochin, Department of Endocrinology, Metabolism and Cancer, 75014 Paris, France; Université Paris Descartes (F.Ti., L.G., B.D., P.L., M.F., B.R., X.B.), 75006 Paris, France; Assistance Publique des Hôpitaux de Paris, Hôpital Cochin, Department of Endocrinology, Center for Rare Adrenal Diseases (R.L., L.G., X.B.), INCa Comete Network (R.L., L.G., X.B.), Departments of Pathology (F.Ti.), Radiology (M.B., P.L.), Digestive and Endocrine Surgery (B.D.), and Nuclear Medicine (M.B., B.R., F.Te.), 75014 Paris, France; Nuclear Medicine Hospital European George Pompidou (C.H., M.F.), 75015 Paris, France; and Department of Internal Medicine (S.J.), Sud Essonne Hospital, 91150 Etampes, France

A 67-yr-old woman with an adrenal incidentaloma was diagnosed with ACTH-independent Cushing’s syn- drome (urinary free cortisol = 286 µg/24 h; normal range, 25-90). She also had high plasma deoxycorticosterone, 11- deoxycortisol, and testosterone levels-findings suggestive of an adrenocortical carcinoma.

Computed tomography imaging showed a heterogeneous right adrenal mass (38 × 44 mm) with two components that had high and low attenuation densities for the upper and lower aspects, respectively (Fig. 1A).

Positron emission tomography (PET) scanning with 18F-fluorodeoxyglucose (FDG) and a [60-131]]iodom- ethyl-19 nor-cholesterol (NP-59) scintigraphy showed a mirror image of the two components, suggesting a malig- nant upper tumor and a benign lower tumor, both of ad- renocortical origin (Fig. 1, B and C).

Pathology confirmed the anticipated natures of the two different components: the upper one was an adrenocorti- cal carcinoma (Weiss score = 7), and the lower one was an adrenocortical adenoma (Weiss score = 2) (Fig. 2).

Previous cases have documented the presence of a dou- ble component in adrenocortical tumors (1,2), suggesting

FIG. 1. A, Computed tomography scan. The upper component of the mass showed an unenhanced density of 34 Hounsfield units (HU) (enhanced density at 1 min of 75 HU, delayed density at 10 min of 56 HU, relative washout < 50%). The lower component had an unenhanced density of 3.6 HU. B, PET scanning with 18F-FDG showing an uptake localized to the upper component with a maximum standardized uptake value (maxSUV) of 7.5 and an adrenal to liver maxSUV ratio of 3.26. C, [60-131|]lodomethyl-19 nor-cholesterol scintigraphy was performed with single photon emission computed tomographic (SPECT) images and allowed three-dimensional reconstruction. Only the lower component showed tracer uptake. The green arrow shows the malignant component of the tumor.

A

CT scan

B 18F-FDG PET

C 1311 nor-cholesterol

adrenal/liver max SUV= 3.26

a multistep adrenal tumorigenesis. Recently, we showed that 18F-FDG PET helped distinguish between benign and malignant adrenal lesions (3).

In addition metastatic disease to the adrenal gland may be detected by 18F-FDG PET (4).

Abbreviations: FDG, Fluorodeoxyglucose; PET, positron emission tomography.

FIG. 2. A, Gross appearance of the adrenocortical tumor. * , Carcinomatous area; ** , adenomatous area. B, Histological appearance of the adrenocortical tumor, in the black square enlarged from panel A. * , Carcinomatous area; ** , adenomatous area (×200). C, Carcinomatous area showing compact cells, nuclear atypia, and abnormal mitoses (arrow) (HES, ×200). D, Adenomatous area showing characteristic alveolar architecture and lipid rich cells (HES, ×200).

A

B

C

*

*

*

D

**

**

**

This is the first report of an adrenocortical neoplasm in which a combined scintigraphic approach could pre- dict the nature of the two distinct components. The hormonal profile (high androgens and steroid precur- sors concentrations) was indicative of a malignant ad- renocortical neoplasm that the 18F-FDG PET localized in the upper component, whereas the sole NP-59 uptake in the lower component predicted an adrenocortical ad- enoma (5).

Acknowledgments

Address all correspondence and requests for reprints to: Dr. Florence Tenenbaum, Service de Médicine Nucléaire, Hôpital Cochin, 27, rue du Fbg St Jacques, 75014 Paris, France. E- mail: florence.tenenbaum@ cch.aphp.fr.

Disclosure Summary: The authors have nothing to disclose.

References

1. Bernard MH, Sidhu S, Berger N, Peix JL, Marsh DJ, Robinson BG, Gaston V, Le Bouc Y, Gicquel C 2003 A case report in favour of a multistep adrenocortical tumorigenesis. J Clin Endocrinol Metab 88:998-1001

2. Gaujoux S, Tissier F, Groussin L, Libé R, Ragazzon B, Launay P, Audebourg A, Dousset B, Bertagna X, Bertherat J 2008 Wnt/ß- catenin and 3’,5’-cyclic adenosine 5’-monophosphate/protein ki- nase A signaling pathways alterations and somatic ß-catenin gene mutations in the progression of adrenocortical tumors. J Clin En- docrinol Metab 93:4135-4140

3. Groussin L, Bonardel G, Silvéra S, Tissier F, Coste J, Abiven G, Libé R, Bienvenu M, Alberini JL, Salenave S, Bouchard P, Bertherat J, Dousset B, Legmann P, Richard B, Foehrenbach H, Bertagna X, Tenenbaum F 2009 18F-FDG PET for the diagnosis of adrenocortical tumors: a pro- spective study in 77 operated patients. J Clin Endocrinol Metab 94: 1713-1722

4. Boland GW, Blake MA, Hahn PF, Mayo-Smith WW 2008 Inciden- tal adrenal lesions: principles, techniques, and algorithms for imag- ing characterization. Radiology 249:756-775

5. Gross MD, Shapiro B 1993 Clinical review: clinically silent adrenal masses. J Clin Endocrinol Metab 77:885-888