Paraneoplastic Syndrome in Adrenocortical Carcinoma: The Unexpected Mime.

Adrenocortical carcinoma (ACC) is a malignancy of the adrenal cortex with a high morbidity and mortality. More than half of the cases are functional tumors. As different hormones can be co-secreted above physiologic levels, it causes a very broad variety of symptoms and makes differentiating from more common entities hard. Here we present a case of a patient with a newly diagnosed ACC who initially presented with acute pulmonary embolism and recurrent deep vein thromboses (DVT) in the setting of hypercortisolism. Imaging showed a left adrenal mass invading adjacent structures including a nonocclusive thrombus in the left renal vein. Intravenous anticoagulation and thrombectomy were initially performed, followed by removal of the tumor and adjacent metastatic disease. Pathology confirmed ACC. The patient underwent left adrenalectomy, left nephrectomy, splenectomy, distal pancreatectomy, and caval thrombectomy with inferior vena cava (IVC) filter placement. Intravenous anticoagulation and glucocorticoid replacement were also administered as part of the treatment plan. Unfortunately, the patient had multiple episodes of bleeding and thrombosis and was eventually discharged to hospice care. DVT in the setting of ACC can be caused by increased hypercoagulability from hypercortisolism, direct venous thrombosis, or vascular invasion. Thrombosis, especially in the inferior vena cava, has been associated with poor prognosis and survival rates. Clinicians should be aware of this rare complication given its immediate therapeutic repercussions and prognostic value.

The conundrum of differentiating Cushing's syndrome from non-neoplastic hypercortisolism: a systematic review and meta-analysis.

CONTEXT: Once hypercortisolemia is confirmed, differential diagnosis between Cushing's syndrome (CS) due to neoplastic endogenous hypercortisolism and non-neoplastic hypercortisolism (NNH, pseudo-Cushing's syndrome) is crucial. Due to worldwide corticotropin-releasing hormone (CRH) unavailability, accuracy of alternative tests to dexamethasone (Dex)-CRH, is clearly needed. OBJECTIVE: Assess the diagnostic accuracy of Dex-CRH test, desmopressin stimulation test, midnight serum cortisol (MSC), and late-night salivary cortisol (LNSC) levels to distinguish CS from NNH. METHODS: Articles through March 2022 were identified from Scopus, Web of Science, MEDLINE, EMBASE, and PubMed. All steps through the systematic review were performed independently and in duplicate and strictly adhered to the updated PRISMA-DTA checklist. DATA SYNTHESIS: A total of 24 articles (1900 patients) were included. Dex-CRH had a pooled sensitivity and specificity of 91% (95%CI 87-94%; I2 0%) and 82% (73-88%; I2 50%), desmopressin test 86% (81-90%; I2 28%) and 90% (84-94%; I2 15%), MSC 91% (85-94%; I2 66%) and 81% (70-89%; I2 71%), and LNSC 80% (67-89%; I2 57%) and 90% (84-93%; I2 21%), respectively. Summary receiver operating characteristics areas under the curve were Dex-CRH 0.949, desmopressin test 0.936, MSC 0.942, and LNSC 0.950 without visual or statistical significance. The overall risk of studies bias was moderate. CONCLUSION: Dex-CRH, the desmopressin stimulation test, and MSC have similar diagnostic accuracy, with Dex-CRH and MSC having slightly higher sensitivity, and the desmopressin test being more specific. LNSC was the least accurate, probably due to high heterogeneity, intrinsic variability, different assays, and lack of consistent reported cutoffs. When facing this challenging differential diagnosis, the results presented here should increase clinicians' confidence when deciding which test to perform.

Venous thromboembolism in Cushing syndrome: results from an EuRRECa and Endo-ERN survey.

Background: Patients with Cushing syndrome (CS) are at increased risk of venous thromboembolism (VTE). Objective: The aim was to evaluate the current management of new cases of CS with a focus on VTE and thromboprophylaxis. Design and methods: A survey was conducted within those that report in the electronic reporting tool (e-REC) of the European Registries for Rare Endocrine Conditions (EuRRECa) and the involved main thematic groups (MTG's) of the European Reference Networks for Rare Endocrine Disorders (Endo-ERN) on new patients with CS from January 2021 to July 2022. Results: Of 222 patients (mean age 44 years, 165 females), 141 patients had Cushing disease (64%), 69 adrenal CS (31%), and 12 patients with ectopic CS (5.4%). The mean follow-up period post-CS diagnosis was 15 months (range 3-30). Cortisol-lowering medications were initiated in 38% of patients. One hundred fifty-four patients (69%) received thromboprophylaxis (including patients on chronic anticoagulant treatment), of which low-molecular-weight heparins were used in 96% of cases. VTE was reported in six patients (2.7%), of which one was fatal: two long before CS diagnosis, two between diagnosis and surgery, and two postoperatively. Three patients were using thromboprophylaxis at time of the VTE diagnosis. The incidence rate of VTE in patients after Cushing syndrome diagnosis in our study cohort was 14.6 (95% CI 5.5; 38.6) per 1000 person-years. Conclusion: Thirty percent of patients with CS did not receive preoperative thromboprophylaxis during their active disease stage, and half of the VTE cases even occurred during this stage despite thromboprophylaxis. Prospective trials to establish the optimal thromboprophylaxis strategy in CS patients are highly needed. Significance statement: The incidence rate of venous thromboembolism in our study cohort was 14.6 (95% CI 5.5; 38.6) per 1000 person-years. Notably, this survey showed that there is great heterogeneity regarding time of initiation and duration of thromboprophylaxis in expert centers throughout Europe.

[Dynamics of NT-proBNP and ST2 levels as markers of heart failure in patients with endogenous Cushing syndrome (hypercortisolism)].

AIM: To evaluate frequency of heart failure syndrome in patients with endogenous hypercortisolism and to establish relationship between effective treatment for hypercortisolism and regression of heart failure with particular emphasis on the observation of NT-proBNP and ST2 levels. MATERIALS AND METHODS: 56 patients with endogenous hypercortisolism (45 female, mean age 47 years [36; 55] hospitalized with endogenous hypercortisolism to National Medical Research Center for Endocrinology were enrolled in the study. All patients underwent comprehensive clinical investigation including expert echocardiography with speckle tracking and evaluation of NT-proBNP and ST2 cardiac biomarkers at baseline and 6 months after surgical treatment. RESULTS: According to clinical data and elevated biomarkers of cardiac stress 28 out of 56 patients (50%) at baseline met the criteria for heart failure. 20 patients were included in the final analysis. Follow-up investigation with focus on changes in NT-proBNP and ST2 levels demonstrated that surgical correction of endogenous hypercortisolism resulted in resolution of heart failure syndrome in 11 patients (55%). CONCLUSION: These preliminary data suggest that signs and symptoms of heart failure are observed in patients with endogenous hypercortisolism in about half the cases. Surgical correction results in resolution of heart failure in approximately two thirds of the cases. Prospective evaluation NT-proBNP and ST2 levels may provide important diagnostic and prognostic information in patients with endogenous hypercortisolism.

Outcome of CRH stimulation test and overnight 8 mg dexamethasone suppression test in 469 patients with ACTH-dependent Cushing's syndrome.

Objective: To evaluate diagnostic accuracy of the corticotropin-releasing hormone (CRH) stimulation test and the overnight 8 mg dexamethasone suppression test (DST) for the differentiation of Cushing's disease (CD) and ectopic Cushing's syndrome (ECS). Methods: Retrospective study in 6 European centers. Inclusion criteria: patients with a) overt adrenocorticotropin (ACTH)-dependent Cushing's syndrome at the time of dynamic testing, b) histopathological confirmed tumors and/or c) postoperative biochemical remission and/or adrenal insufficiency. Optimal cut-offs were calculated via receiver operating characteristic (ROC) analysis using CD as reference. Results: 469 patients were analyzed [78% females; median age 43 years (IQR 19)]. CRH test and overnight 8 mg DST were performed in 420 [CD, n=394 (94%); ECS, n=26 (6%)] and 237 patients [228 CD (96%), 9 ECS (4%)]. Both tests were performed in 205 patients (44%). The post-CRH %-increase at 30 minutes of both ACTH (cut-off ≥31%, sensitivity 83%, specificity 85%, AUC 0.81) and cortisol (cut-off ≥12%, sensitivity 82%, specificity 89%, AUC 0.86) discriminated best between CD and ECS. A test duration of >60 minutes did not improve diagnostic performance of the CRH test. The optimal cortisol cut-off for the %-suppression during the 8 mg DST was ≥55% (sensitivity 80%, specificity 78%, AUC 0.75). Conclusion: The CRH test has equivalent sensitivity but higher specificity than the 8 mg DST and is therefore the test of first choice. The diagnostic outcome of ACTH and cortisol is well comparable, however, sampling beyond 60 minutes post-CRH does not provide diagnostic benefits.

Manifestaciones clínicas predictivas del diagnóstico del hipercortisolismo endógeno / Clinical Manifestations Predicting Endogenous Hypercortisolism

Introducción: El diagnóstico clínico del hipercortisolismo endógeno puede ser complejo si las manifestaciones clínicas no son patognomónicas. Es importante conocer cuáles son las distintivas en nuestros pacientes, que permitan diagnosticar la enfermedad de forma precoz. Objetivo: Determinar sensibilidad, especificidad y valor predictivo positivo de las manifestaciones clínicas para diagnosticar el hipercortisolismo endógeno. Métodos: Estudio descriptivo transversal. Grupo I: 65 pacientes con hipercortisolismo endógeno y grupo II (comparación): 75 con sospecha clínica de hipercortisolismo endógeno a los que se les descartó la enfermedad. Se estudiaron pacientes con diagnóstico de hipercortisolismo endógeno (2004-2017), atendidos en el Instituto de Endocrinología. Se determinaron los valores de sensibilidad, especificidad y valor predictivo positivo para el diagnóstico de hipercortisolismo endógeno de cada síntoma y signo por separado, así como para las combinaciones de tres manifestaciones clínicas; además se realizó una regresión logística binaria para identificar las combinaciones de sintomatologías que mejor predicen la presencia de hipercortisolismo endógeno. Se consideró diferencia estadística significativa con p ≤ 0,05. Resultados: La mayoría de los síntomas presentó una sensibilidad inferior al 45 por ciento. La circunferencia de la cintura fue el único signo que mostró alta sensibilidad (76,9 por ciento), baja especificidad (28,6 por ciento) con valor predictivo positivo de 42,0 por ciento. Cuando se presentan dos o tres de estos signos: rubicundez, cara de "luna llena" e hirsutismo la posibilidad de tener hipercortisolismo endógeno es 75,4 por ciento, con valor predictivo positivo de 71 por ciento y, cuando no están presentes la probabilidad de no tenerlo es de 77,5 por ciento. Conclusiones: La combinación de sensibilidad, especificidad y valor predictivo positivo de los síntomas y signos de forma aislada no predice el diagnóstico de la enfermedad; sin embargo, la presencia de dos o tres de los síntomas y/o signos tiene una elevada sensibilidad y valor predictivo positivo para el diagnóstico del hipercortisolismo endógeno. Las combinaciones que mejor predicen la posibilidad de tener un hipercortisolismo endógeno son: piel fina, edema y acné; cara de "luna llena", hirsutismo y rubicundez, así como "giba de búfalo", hematomas y estrías(AU)

ABSTRACT Introduction: The clinical diagnosis of endogenous hypercortisolism can be complex if clinical manifestations are not pathognomonic. It is important to know what are distinctive in our patients, which allows to diagnose the disease early. Objective: To determine the sensitivity, specificity and positive predictive value of clinical manifestations for diagnosis. of endogenous hypercortisolism. Methods: Descriptive and cross-sectional study. Group I was made up of 65 patients with endogenous hypercortisolism; group II (comparison) was made up of 75 patients with clinical suspicion of endogenous hypercortisolism, finally ruled out. We studied patients diagnosed of endogenous hypercortisolism from 2004 to 2017, treated at the Endocrinology Institute. Sensitivity, specificity and positive predictive values were determined for the diagnosis of endogenous hypercortisolism for each symptom and sign separately, as well as for the combinations of three clinical manifestations; in addition, binary logistic regression was performed to identify the combinations of symptoms that best predict the presence of endogenous hypercortisolism. A statistically significant difference was considered with p ≤ 0.05. Results: Most of the symptoms presented a sensitivity lower than 45 percent. Waist circumference was the only sign that showed high sensitivity (76.9 percent), low specificity (28.6 percent), and positive predictive value of 42.0 percent. When two or three of these signs (redness, "full-moon" face, and hirsutism) are present, the possibility of having endogenous hypercortisolism is 75.4 percent, with positive predictive value of 71 percent; and, when they are not present, the probability of not having it is 77,5 percent. Conclusions: The combination of sensitivity, specificity and positive predictive value of the symptoms and signs in isolation does not predict the diagnosis of the disease; however, the presence of two or three of the symptoms and/or signs has a high sensitivity and positive predictive value for the diagnosis of endogenous hypercortisolism. The combinations that best predict the possibility of having an endogenous hypercortisolism are thin skin, edema and acne; "full-moon" face, hirsutism and redness; as well as "buffalo hump," bruises and stretch marks(AU)

Case Report-Secondary Antibody Deficiency Due to Endogenous Hypercortisolism.

Therapeutic corticosteroids have an immunosuppressive function involving several pathways, including lymphocytopenia and hypogammaglobulinemia. While these effects have been well-described in patients that received corticosteroids for therapeutic reasons, the effects of endogenous corticosteroids on the immune system are less well-understood. Here, we describe a 21-year old patient with hypercortisolism due to an ACTH producing thymic tumor. In this patient, we observed a decrease in some of the immunoglobulin classes, and in specific B and T cell populations that resembled effects caused by corticosteroid treatment. IgG levels were restored following treatment and normalization of the hypercortisolism.

Endogenous hypercortisolism inducing reversible ocular hypertension.

PURPOSE: To describe the clinical findings of two patients with reversible ocular hypertension secondary to endogenous hypercortisolism. DESIGN: Retrospective, observational case series. SUBJECTS: A 65-year-old man (patient 1) and a 21-year-old woman (patient 2) were both found to have Cushing's syndrome after presentation to our clinic with elevated intraocular pressures (IOP). METHODS: Clinical histories, ophthalmic examinations including IOP measurements, optical coherence tomography of the retinal nerve fiber layer, visual field testing, magnetic resonance imaging and computerized tomography of two patients were reviewed between 2007 and 2019. OBSERVATIONS: Patient 1 demonstrated elevated IOP (maximum 26 mmHg OD and 22 mmHg OS) and bilateral disc edema. Following diagnosis of Cushing's syndrome, the patient underwent two pituitary resections and bilateral adrenalectomy, with subsequent resolution of his hypercortisolism and ocular hypertension (OHT). Patient 2 presented with blurred vision and found to have OHT (maximum 32 mmHg OU). Following diagnosis of Cushing's disease and two resections of her adrenocorticotropic hormone (ACTH) producing pituitary adenoma, her IOPs normalized. Both patients maintained normal IOPs after resolution of their endogenous hypercortisolism and discontinuation of topical IOP-lowering medication. CONCLUSIONS AND IMPORTANCE: Ocular hypertension induced by endogenous hypercortisolism is, in some cases, fully reversible following normalization of cortisol levels. These findings suggest that the physiologic changes to the trabecular meshwork induced by endogenous hypercortisolism may be fully reversible.

Calidad de vida en pacientes operados de hipercortisolismo endógeno / Quality of life in patients operated for endogenous hypercortisolism

RESUMEN Introducción: El hipercortisolismo endógeno provoca cambios en diferentes áreas de la vida en el sentido percibido de bienestar, lo que afecta la calidad de vida; esta debe tenerse en cuenta en la atención integral al paciente. Objetivos: Describir la calidad de vida percibida en los pacientes con hipercortisolismo endógeno e identificar si existe relación entre calidad de vida y algunas características clínicas y hormonales. Métodos: Estudio transversal, 60 pacientes: grupo I (con hipercortisolismo endógeno operados con menos de cinco años) y grupo II (personas con sospecha clínica de hipercortisolismo endógeno), edad entre 20 y 60 años. Se aplicaron cuestionarios de calidad de vida relacionados con el hipercortisolismo endógeno: CushingQoL y Tuebingen CD-25 y planilla de recolección de datos generales, clínicos y hormonales. Se determinó mediana (variables cuantitativas) y frecuencia en las cualitativas. La asociación del puntaje de los cuestionarios y variables cuantitativas, mediante el coeficiente de correlación de Spearman. Se compararon los valores promedio del puntaje entre categorías de las variables cualitativas (clínicas y hormonales categorizadas), mediante la prueba U-Mann Whitney; se consideró diferencia estadística significativa con p ≤ 0,05. Resultados: La mediana del puntaje de los dominios psicoemocional, sexualidad, área social, área corporal, y la suma de todos los dominios fueron mayores en el grupo I. La sexualidad (4,5), el área corporal (6,0) y la suma de todos los dominios (36,5) del I vs. 1,0, 4,0 y 28,0 respectivamente (grupo II), del Tuebingen CD-25 mostraron diferencias estadísticamente significativas, demostrando peor calidad de vida en el grupo I. Con el CushingQoL la mediana del puntaje del grupo I fue menor que la del II (54,1 vs. 74,0), con diferencias estadísticamente significativas (p = 0,00), lo que evidencia peor calidad de vida para el grupo I. Conclusiones: El hipercortisolismo endógeno incide negativamente en la calidad de vida percibida en el grupo de pacientes operados, especialmente en las áreas social, corporal, cognitiva, sexual, y de la conducta alimentaria(AU)

ABSTRACT Introduction: Endogenous hypercortisolism causes changes in different areas of life and the perceived sense of well-being, which affects the quality of life. This should be taken into account in the patient comprehensive care. Objectives: To describe the quality of life perceived in patients with endogenous hypercortisolism and to identify if there is a relationship between quality of life and some clinical and hormonal characteristics. Methods: Cross-sectional study, 60 patients: group I (with endogenous hypercortisolism operated less than five years) and group II (people with clinical suspicion of endogenous hypercortisolism), age between 20 and 60 years. Quality of life questionnaires related to endogenous hypercortisolism were applied: CushingQoL and Tuebingen CD-25 and general, clinical and hormonal data collection form. Median (quantitative variables) and frequency in the qualitative ones were determined. The association of the score of questionnaires and quantitative variables were obtained by means of the Spearman correlation coefficient. The average score values were compared between categories of qualitative variables (clinical and hormonal categorized), using the U-Mann Whitney test; significant statistical difference was considered with p ≤ 0.05. Results: The median score of the psycho-emotional domains, sexuality, social area, body area, and the sum of all domains were higher in group I. Sexuality (4.5), body area (6.0) and the sum of all domains (36.5) of group I vs. 1.0, 4.0 and 28.0 respectively (group II), of the Tuebingen CD-25 showed statistically significant differences, which is worse quality of life in group I. Using the CushingQoL, the median score of group I showed to be lower than in group II (54.1 vs. 74.0) there are statistically significant differences (p = 0.00), which shows worse quality of life in group I. Conclusions: Endogenous hypercortisolism negatively affects the quality of life perceived in the group of operated patients, especially in the social, bodily, cognitive, sexual areas, and eating behaviour(AU)

Avascular necrosis of the femoral head: An exceptional complication of cushing's disease.

Avascular necrosis (AVN) of the femoral head is a well-recognized complication of steroid treatment. Exogenous glucocorticoids' administration and alcoholism are the most common nontraumatic etiologies. AVN secondary to endogenous hypercortisolism (HC) is rare; very few case reports are available on this complication. We describe a 28-year-old female that has presented an AVN with Cushing's syndrome. Biochemical investigations confirmed HC. Magnetic resonance imaging (MRI) showed a pituitary adenoma that was resected transsphenoidally. The postoperative failure directed to radiosurgery treatment. Then, the patient then expressed a significant clinical improvement while developing adrenocorticotropic deficiency; hence, steroids were indicated. During the discovery of Cushing disease, the patient presented also limping and progressive pain in right hip. The pelvis MRI showed an AVN of the right femoral head. The patient underwent a total replacement of the right hip sine presenting an extensive AVN. This case elucidates that AVN could be an early manifestation of Cushing's disease.

Résumé L'osteonécrose avasculaire (ONAV) de la tête fémorale est une complication bien reconnue des traitements par des corticostéroïdes. L'administration de glucocorticoid exogène et l'alcoolisme sont les étiologies non-traumatiques les plus communes. L'ONVA secondaire à hypercortisolism endogène (HC) est rare; très peu de rapports de cas sont disponibles sur cette complication. Nous décrivons une femme de 28 ans qui a présenté un ONAV avec le syndrome de Cushing. Les études biochimiques ont confirmé HC. L'imagerie par résonance magnétique (IRM) a montré un adénome pituitaire qui a été réséqué de façon trans-sphénoïdal. L'échec post-opératoire a orienté au traitement par radiochirurgie. Ce dernier traitement a permis une amélioration clinique significative tout en développant une déficience adrenocorticotropique; d'où, l'intérêt d'avoir indiqué les corticostéroïdes. Pendant la découverte de maladie Cushing, le patient avait présenté aussi un boitement avec douleur progressive dans la hanche droite. L'IRM du bassin a montré un ONAV de la tête fémorale droite. La patiente a subi un remplacement total du sinus droit de hanche présentant un ONAV étendue. Ainsi ce cas élucide bien que l'ONAV pourrait être une manifestation précoce de la maladie de Cushing Mots-clés: Avasculaire, nécroses, Maladie de Cushing, hypercortisolism endogène.

Tratamiento farmacológico del síndrome de Cushing / Drug treatment of Cushing's syndrome

El tratamiento farmacológico, aunque no constituye la primera línea de tratamiento en el hipercortisolimo endógeno, desempeña un importante rol en determinados pacientes con síndrome de Cushing. Diversos fármacos pueden ser utilizados, de forma aislada o combinada. El ketoconazol es una opción útil, económica, con determinada seguridad y eficacia a largo plazo. Se realiza una revisión de los medicamentos con mejores resultados, dosis, eficacia, efectos adversos y perspectivas, teniendo en cuenta ensayos clínicos en los que han sido empleados(AU)

Although the drug treatment is not a first line therapy for endogenous hypercortisolism, it plays a key role in certain patients suffering Cushing's syndrome. Several drugs may be used in isolation or in combination. Ketoconazol is a useful, economic, safe and efficacious option at long term. A review was made of drugs with best results, doses, efficacy, adverse effects and perspectives, taking into account clinical assays in which they have been used(AU)

Occurrence of De Quervain's Thyroiditis after Resolution of Hypercortisolism following Pasireotide Treatment for Cushing's Disease and Surgery for an Adrenocortical Adenoma: Report of Two Cases.

OBJECTIVE: An increased prevalence of thyroid autoimmunity has been observed after successful treatment of Cushing's syndrome. On the other hand, De Quervain's thyroiditis (DQT), in which autoimmunity is not a pathogenetic contributor, has not been reported during recovery from Cushing's syndrome. We describe 2 female patients with DQT coinciding with the resolution of hypercortisolism after treatment of Cushing's syndrome/disease. METHODS: The first patient had been diagnosed with Cushing's disease due to a corticotroph pituitary microadenoma, declined neurosurgery, and was receiving pharmacological treatment with pasireotide. Her hypercortisolism was optimally controlled with a minimum dose. The second patient had undergone unilateral adrenalectomy due to a cortisol-secreting adenoma and was on tapering doses of hydrocortisone due to a suppressed corticotroph axis. Both patients presented with clinical, functional, and imaging features of DQT at a time when their endogenous glucocorticoid levels were very low. RESULTS: Oral glucocorticoid treatment was administered in both cases, resulting in prompt recovery. CONCLUSIONS: The incidence of DQT following the resolution of hypercortisolism, either medical or surgical, has not been previously described. The exact pathogenetic mechanism can only be speculated on. Perhaps the relative or absolute glucocorticoid deficiency after effective treatment of hypercortisolism alters immunologic responses and renders patients more vulnerable to thyrolytic processes.

Carcinoma adrenal de rápida evolución / Rapidly evolving adrenal carcinoma

Introducción: el carcinoma suprarrenal primario es un tumor poco frecuente, altamente agresivo, de crecimiento rápido, con mayor incidencia entre los 40 y 60 años de edad. Los carcinomas funcionantes representan hasta un 79 por ciento de los tumores corticales, más frecuentes en el sexo femenino, y de estos el 50 por ciento se manifiestan clínicamente como un síndrome de Cushing. La extensión del tumor a estructuras vecinas es común y empeora el pronóstico. La supervivencia media es de 2 años desde el diagnóstico, en particular, cuando existen metástasis en hígado y pulmón. Objetivo: describir las características clínicas, los procederes diagnósticos y terapéuticos de una paciente con carcinoma adrenal de rápida evolución. Presentación del caso: paciente LRS, femenina, blanca, de 49 años de edad, con antecedentes de hipertensión arterial y diabetes mellitus tipo 2, que asiste a consulta por descontrol metabólico y de la hipertensión arterial. Al examen físico, se constatan signos sugestivos de hipercortisolismo, sintomatología que a los 2 meses se acentuó notablemente. Se realizaron estudios basales, dinámicos, imagenológicos y anatomopatológicos, que corroboraron el diagnóstico presuntivo. Se realiza adrenalectomía izquierda con adenectomía regional, y se confirma por anatomía patológica el diagnóstico de carcinoma suprarrenal izquierdo. A los pocos meses de la intervención la paciente fallece con metástasis óseas en columna vertebral. Conclusiones: el reconocimiento temprano de los síntomas y signos de hiperfunción adrenal es muy importante para el diagnóstico y tratamiento oportuno del carcinoma adrenal(AU)

Introduction: primary suprarrenal carcinoma is a highly aggressive rare carcinoma of rapid growth, with greater incidence in 40-60 years age group. The functioning carcinomas represent up to 79 percent of cortical tumors that are more frequent in females, and 50 percent of them clinically manifests as Cushing syndrome. The extension of the tumor to neighboring structures is common and worsens the prognosis. Mean survival rate is 2 years from the time of diagnosis, particularly when there are liver and lung metastases. Objective: to describe the clinical characteristics, the diagnostic and therapeutic procedures in a female patient with rapidly evolving adrenal carcinoma. Case presentation: a patient LRS, female, Caucasian, 49 years-old, with a history of blood hypertension and type 2 diabetes mellitus, who went to the hospital because of lack of metabolic control and blood hypertension. On physical examination, there were observed some signs suggestive of hypercortisolism, symptomatology that became notably acute two months later. Basal, dynamic, imaging and anatomopathological studies were made to corroborate the presumptive diagnosis. Left adrenalectomy with regional adenectomy was performed, and the diagnosis of left suprarenal carcinoma was confirmed through pathological anatomy. Few months later, the patient died from osseous metastasis in her spinal cord. Conclusions: early recognition of symptoms and signs of adrenal hyperfunction is very important for diagnosis and timely treatment of adrenal carcinoma(AU)