Role of Different Variants of Leptin Receptor in Human Adrenal Tumor Types.

The aim of the study was to evaluate the diagnostic and prognostic significance of leptin receptor isoforms in adrenal tumors. In a single-center study, 96 patients (19 with adrenal cortical carcinoma and 77 with benign tumors) underwent an adrenalectomy. A total of 14 unaffected adrenal gland tissues from kidney donors were used as controls. Fasting blood samples were collected for laboratory tests, and mRNA expressions of leptin receptor isoforms were assessed by RT-qPCR. The study analyzed correlations between mRNA expressions and clinical data and measured NCI-H295R cell proliferation via a real-time cell analyzer. All adrenal lesions expressed leptin receptor isoforms. Significantly lower LepR1 expression was observed in carcinoma tissues than in adenomas and controls (p = 0.016). Expressions of LepR3&LepR6 were correlated with overall survival (p = 0.036), while LepR2&LepR4 and LepR5 expressions were inversely related to morning serum cortisol levels (p = 0.041). Leptin reduced NCI-H295R cell proliferation (p < 0.0001). The study highlights the diagnostic and prognostic significance of leptin receptor isoforms in adrenal tumors. Specifically, LepR1 may serve as a diagnostic marker for carcinomas, while LepR3&LepR6 have potential use as prognostic markers.

Extended Right Hepatectomy to Inferior Vena Cava Under Total Vascular Exclusion, Veno-Venous Bypass and In Situ Hypothermic Perfusion of the Future Liver Remnant.

BACKGROUND: Venous obstruction at the hepatic veins-inferior vena cava confluence can be particularly challenging to manage if an associated liver resection is needed. Total vascular exclusion (TVE) with veno-venous bypass (VVB) and hypothermic in situ perfusion (HP) of the future liver remnant can be used in these conditions.1,2 METHODS: The patient was a 58-year-old with a voluminous adrenal cancer invading the kidney, the right liver and the retrohepatic inferior vena cava with intraluminal thrombus extending up to the hepatic veins confluence. A right hepatectomy, extended to segment 1, the right kidney, and the retrohepatic inferior vena cava was planned. RESULTS: The parenchymal liver transection was performed under a TVE, VVB, and HP of the left liver to decrease blood losses and risk of postoperative liver failure. Vena cava reconstruction was achieved by a ringed Gore-Tex prosthesis with reimplantation of the left renal vein. Total duration of veno-venous bypass and liver vascular exclusion were 2 h 40 min and 2 h 10 min, respectively. The patient was discharged on postoperative day 17. CONCLUSIONS: Total vascular exclusion with veno-venous bypass and in-situ liver hypothermic perfusion increases the safety of major liver resection requiring complex vascular reconstruction.1,2 TVE under VVB and HP of the future liver remnant is used at our institution when: (1) TVE will last more than 30 min; (2) vascular reconstruction is needed; (3) in the presence of venous obstruction; (4) in the presence of injured liver parenchyma; and (5) in the presence of cardiovascular comorbidities.

[Myxoid variant of adrenocortical carcinoma]. / Carcinome corticosurrénalien variante myxoïde : aspects clinico-pathologiques et immunohistochimiques d'une variante rare et agressive.

We report two cases of patients presenting myxoid variant of adrenocortical carcinoma (ACC). This very rare variant is characterized by a tumoral proliferation organized in trabeculae, cords or even pseudo-glands within an acellular myxoid materiel stained by Alcian Blue and negative for PAS. Tumor cells have a small to medium size and have little atypia. Their immunohistochemical profil (positivity of Synaptophysin, SF1, Melan A, Vimentin and Inhibin, with a weak or negative pancytokeratin expression) eliminate the main differential diagnoses (metastasis of a myxoid adenocarcinoma and soft tissue myxoid tumor). Many scoring systems have been proposed in order to evaluate the risk of malignancy of these lesions: the Weiss score seems less efficient to evaluate malignancy in this variant than the reticulinic algorithm or the Helsinki score. Prognosis of myxoid variant of ACC seems worse than classical ACC.

To report hounsfeld units or not: There is no question.

INTRODUCTION: Most adrenal incidentalomas are not appropriately evaluated. Reporting the mass in the radiology report summary and providing recommendations in the report can positively impact evaluation. This study evaluated the effect of reporting Hounsfield units(HU) on adrenal incidentaloma follow-up. METHODS: Patients with adrenal incidentalomas identified on noncontrast CT scan from 2015 to 2020 â€‹at a tertiary care institution were studied. Chart review was conducted. Patient and imaging characteristics were compared between patients who did and did not have HU reported. Outcomes of interest were 1)outpatient referral, 2)biochemical evaluation, and 3)dedicated imaging if appropriate. Multivariate analysis determined the impact of HU, reporting in the summary and provision of recommendations on the outcomes. RESULTS: 363 patients were studied, 36(9.9 â€‹%) had HU reported. When HU were used in addition to recommendations and reporting in the summary, the likelihood of outpatient referral increased from 10.1 to 32.6-fold (95%CI 7.7-138.1, p â€‹< â€‹0.001). Similarly, the likelihood of biochemical workup increased from 2.5 to 7.8-fold (95%CI 2.5-24.1, p â€‹< â€‹0.001). CONCLUSION: Recording adrenal incidentaloma HU on non-contrast CT scans was associated with increased rates of outpatient referral and biochemical workup.

[Adrenal carcinoma induced hypoglycemia]. / Hipoglucemia inducida por carcinoma adrenal.

Adrenal carcinoma is a rare malignancy of poor prognosis. The most common clinical presentation is secondary to hormone production, while the development of symptomatic hypoglycemia is exceptional. We report the case of a 37 year old-woman admitted to hospital with severe hypoglycemia, hypertension, hypokalemia and amenorrhea. In the laboratory we found hypoglycemia, with low insulin levels, and androgen levels in tumor range. CT of abdomen and pelvis showed a heterogeneous lesion of solid appearance without a cleavage plane relative to liver parenchyma, and intense contrast enhancement. Retroperitoneal mass was removed, and the patient evolved without complications, blood glucose and potassium were normalized, blood pressure stabilized and menstrual cycles recovered.

[Adrenocortical carcinoma in women of reproductive age]. / Carcinoma adrenocortical en mujeres en edad fértil.

Adrenocortical carcinomas are rare tumors, usually hyperfunctioning, with poor overall survival. Frequent age of presentation is described in adults between 40 and 60 years of age, predominantly female. Two unusual cases of adrenal carcinoma diagnosed in young women are presented. The first one was discovered in the second trimester of gestation, with signs and symptoms of hypercortisolism and localized adrenal lesion, which was resolved with complete resection by week 20 of pregnancy. In the second case, the patient begined with clinical manifestations of rapidly progressive virilization, the biochemical pattern being pure hyperandrogenism. In both cases, despite complete resection, the high Ki67 as the main prognostic factor leaded to categorization as "high risk of recurrence". In addition, pregnancy and glucocorticoid secretory pattern have been associated as additional risk factors of recurrence. This is particularly high within the first two years after diagnosis. There is controversy about the use of adjuvant mitotane in these patients, and the general recommendation is to be started no longer than 3 months after surgery. However, the available evidence does not suggest that its use is ineffective beyond that period. Limitations, such as the course of pregnancy and the immediate puerperium, as well as the difficulty of accessing this medication in our environment, prevented the early initiation of adjuvant treatment with mitotane in both cases, although there is still concern whether its administration would still be appropriate.

Los carcinomas adrenocorticales son tumores infrecuentes, habitualmente hiperfuncionantes y con una supervivencia global pobre. La edad frecuente de presentación se describe en adultos entre 40 a 60 años, con predominio en sexo femenino. Se presentan dos casos inusuales de carcinoma adrenal diagnosticados en mujeres en edad fértil. El primero de ellos se descubrió en el segundo trimestre de gestación, con un cuadro de hipercortisolismo y lesión adrenal localizada, que resolvió con resección completa hacia la semana 20. En el segundo, la paciente debutó con manifestaciones clínicas de virilización rápidamente progresiva, siendo el hiperandrogenismo puro el patrón bioquímico hallado. En ambos casos, a pesar de haberse realizado la resección completa, el Ki67 elevado como principal factor pronóstico condujo a categorizarlas como de "alto riesgo de recurrencia". Asimismo, se ha asociado a la gestación y al patrón secretor de glucocorticoides como factores adicionales de mayor riesgo de recurrencia. Este es particularmente elevado dentro de los dos primeros años posteriores al diagnóstico. Existe aún controversia sobre el uso de mitotane adyuvante en estos pacientes, y su inicio está recomendado hasta los tres meses del postquirúrgico. Sin embargo, la evidencia disponible no permite suponer la falta de eficacia si se utiliza fuera de ese período. Los limitantes, como fueron el curso de la gestación y el puerperio inmediato, así como la dificultad para el acceso a la medicación en nuestro medio, impidieron el inicio precoz del tratamiento adyuvante en ambos casos, aunque surge la inquietud de si aún sería oportuna su instauración.

Adrenal carcinoma as the first manifestation of a Li­Fraumeni syndrome in three paediatric patients.

Not required for clinical vignette.

Adrenal carcinoma: a case report.

BACKGROUND: Adrenocortical carcinoma is a rare malignancy (0.5-2 cases/million/year) with a poor prognosis. Hypercortisolism, virilization, and compressive features are among the common presentations of adrenocortical carcinoma. Hematuria is one of the rare initial presentations of adrenocortical carcinoma reported in the literature. We report a case of adrenal carcinoma presenting with microscopic hematuria. CASE PRESENTATION: A 67-year-old Sri Lankan patient with diabetes, hypertension, and ischemic heart disease presented with an acute coronary event. During the routine evaluation, microscopic hematuria was detected without proteinuria or active sediments. She denied any painful micturition, previous similar episodes, or abdominal pain. Further evaluation revealed a hypokalemia with biochemical evidence of hypercortisolism and high testosterone levels with suppressed adrenocorticotropic hormone levels. On imaging, there was evidence of a right suprarenal mass 7 cm × 3 cm × 6 cm in size that was hypoechoic and lobulated and suggestive of a lipid-poor tumor. She underwent adrenalectomy. By the time of surgery 3 weeks later, significant weight gain with features of Cushing syndrome, including hirsutism, skin atrophy, easy bruising without virilization, and proximal myopathy, were noted. Histology identified a right-sided adrenal tumor with capsular and vascular invasion. Hypercortisolism and hematuria disappeared after surgery. The patient was referred for further oncological management. CONCLUSION: This case illustrates a rare presentation of adrenal carcinoma. Awareness of this presentation may facilitate early evaluation and management.

PET-CT in Clinical Adult Oncology: III. Gastrointestinal Malignancies.

PET-CT is an advanced imaging modality with many oncologic applications, including staging, assessment of response to therapy, restaging and longitudinal surveillance for recurrence. The goal of this series of six review articles is to provide practical information to providers and imaging professionals regarding the best use of PET-CT for specific oncologic indications, and the potential pitfalls and nuances that characterize these applications. In the third of these review articles, key tumor-specific clinical information and representative PET-CT images are provided to outline the role that PET-CT plays in the management of patients with gastrointestinal malignancies. The focus is on the use of 18F fluorodeoxyglucose (FDG), rather than on research radiopharmaceuticals under development. Many different types of gastrointestinal tumors exist, both pediatric and adult. A discussion of the role of FDG PET-CT for all of these is beyond the scope of this review. Rather, this article focuses on the most common adult gastrointestinal malignancies that may be encountered in clinical practice. The information provided here will provide information outlining the appropriate role of PET-CT in the clinical management of patients with gastrointestinal malignancies for healthcare professionals caring for adult cancer patients. It also addresses the nuances and provides interpretive guidance related to PET-CT for imaging providers, including radiologists, nuclear medicine physicians and their trainees.

The challenge to differentiate between sarcoma or adrenal carcinoma-an observational study.

BACKGROUND: Adrenal sarcomas are rare malignant tumors with structural and clinical similarities to sarcomatoid adrenocortical carcinoma. Preoperative diagnosis of tumors of the adrenal gland can be challenging and often misleading thus detaining patients from appropriate oncological strategies. OBJECTIVE: This analysis of a case series evaluated the predictive capability of the primary clinical diagnosis in case of malignancies of the adrenal gland. METHODS: Thirty two patients were treated from 2009 to 2015 at our clinic and analyzed retrospectively. All patients had computed tomography and/or magnet resonance imaging and a primary histopathological examination at our institution after surgery. Ten questionable cases were surveyed by a reference pathologist. RESULTS: Twelve out of 32 diagnoses had to be revised (37.5%). Only 15 out of 24 tumors primarily classified as adrenocortical carcinoma were finally described as primary adrenal cancer. We found two leiomyosarcomas, one liposarcoma, one sarcomatoid adrenocortical carcinoma, and one epitheloid angiosarcoma among 12 misleading diagnoses. Other tumors turned out to be metastases of lung, hepatocellular, and neuroendocrine tumors. Larger tumors were significantly more often correctly diagnosed compared to smaller tumors. Four patients of the group of revised diagnoses died whereas all patients with confirmed diagnoses survived during the follow-up. CONCLUSION: Preoperative assessment of tumors of the adrenal gland is still challenging. In case of wrong primary diagnosis, the prognosis could be impaired due to inadequate surgical procedures or insufficient preoperative oncological treatment.

An Adrenocortical Carcinoma Evolving After Nine Years of Latency From a Small Adrenal Incidentaloma.

Adrenal incidentalomas (AIs) are common incidental findings in medical practice with clinical significance. Although most AIs are nonsecretory and nonmalignant, they require a short course of follow-up over one to two years to rule out malignancy or hormonal secretion according to clinical practice guidelines. However, this can result in some adrenocortical carcinomas (ACCs) being missed if they transform at a later stage or evolve slowly. Here, we report one such case of an AI, which although remained indolent, eventually transformed into an ACC many years after the initial detection.

Primary Adrenal Leiomyosarcoma: Clinical, Radiological, and Histopathological Characteristics.

Primary adrenal leiomyosarcoma (PAL) is a rare, high-grade proliferating mesenchymal tumor with a considerable risk of metastasis, deriving from the smooth muscle wall of a central adrenal vein, or its tributaries. Roughly 40 patients with PAL have been reported in the literature. Herein, we present 3 patients with incidentally discovered PAL, along with an overview of the current knowledge on the clinical, radiological, and histopathological characteristics of PAL.

[An Uncommon Cause of Arterial Hypertension]. / Seltene Ursache einer arteriellen Hypertonie.

An Uncommon Cause of Arterial Hypertension Abstract. A 54-year-old patient was suffering from arterial hypertension, which was not treated sufficiently despite an antihypertensive therapy with three different types of drugs. In addition, the patient complained an increase in weight of ten kilos during the last year and a new onset of diabetes mellitus type 2. Investigations as to secondary forms of hypertension by MRI revealed an adrenal tumor with a diameter of approx. 6 cm suspect for an adrenal carcinoma. An ACTH-independent cushing syndrome was diagnosed by 24-hour urines collection, 1-mg dexamethasone supression test, and midnight-cortisol measurement. After a laparoscopic tumor excision, histopathological analyses confirmed an adrenal carcinoma.

Resection of Large Urological Tumors With or Without Inferior Vena Cava Extension in Jehova's Witnesses.

Background: Renal and adrenal tumors with/without tumor thrombus in the inferior vena cava (IVC) pose a challenge to the surgeon due to the potential for massive hemorrhage and tumor thromboemboli. The situation would be more critical for Jehovah's Witness (JW) patients which refuse blood transfusion. A transplant-based (TB) approach to these tumors in JWs would result a safe surgical method, providing limited blood loss and perioperative complications. We report our experience using a TB surgical approach in JW harboring large adrenal/renal tumors with/without tumor thrombus trying to determine its usefulness in this setting. Patients and Methods: From 2003 to 2011, 7 patients underwent resection of renal/adrenal tumors with/without tumor thrombus in the IVC by means of a TB approach. Thrombus level was renal (n = 2), retrohepatic (n = 1), and suprahepatic (n = 1). The remaining 3 patients did not present thrombus. No pre-operative optimization or cell-saver were used. Estimated blood loss, perioperative complications (Clavien-Dindo and cause), hemoglobin/hematocrit loss, and length of stay were considered main outcomes. Results: The intervention was successfully completed without transfusion in all cases. Operative time and blood loss were 2.5 h (range: 1.83-5.75) and 150 cc (range: 100-750), respectively. No major post-operative complications were registered. However, minor complications were detected in 57% of the patients included. Median hemoglobin loss was 1.13 mg/dL, which translated a median hematocrit loss of 2.3%. Patients were discharged in a median of 7 days (range 5-20). Conclusions: A TB-surgical approach provides enhanced retroperitoneal exposure and optimal vascular control, thus limiting operative blood loss or major complication development, thus resulting useful in JWs.

CME-Sonografie 94: Nebennieren.

Adrenal glands are often not examined in abdominal ultrasound because it is thought that the adrenal glands cannot be visualized. The normal right adrenal gland can be represented in most cases and the left one very often. Sonography makes an important contribution to the diagnosis of adrenal diseases. In addition to bleeding and tuberculosis, there are benign tumors (adrenal adenomas), pheochromocytomas, and adrenal carcinomas. There are also adrenal metastases and lymphomas.

[CME Sonography 94/Answers: Adrenal Glands]. / CME-Sonografie 94/Antworten: Nebennieren.

CME Sonography 94/Answers: Adrenal Glands Abstract. Adrenal glands are often not examined in abdominal ultrasound because it is thought that the adrenal glands cannot be visualized. The normal right adrenal gland can be represented in most cases and the left one very often. Sonography makes an important contribution to the diagnosis of adrenal diseases. In addition to bleeding and tuberculosis, there are benign tumors (adrenal adenomas), pheochromocytomas, and adrenal carcinomas. There are also adrenal metastases and lymphomas.

Hypertensive choroidopathy and adrenal carcinoma: A new association. / Coroidopatía hipertensiva y carcinoma adrenal: una nueva asociación.

A 17 year-old Caucasian female presented with decreased vision, with no other symptoms reported at that time. Ophthalmological examination revealed typical signs of hypertensive choroidopathy, Elschnig spots, and Siegrist streaks. Further medical examination found high blood pressure (220/155mmHg), with a metastatic hormone-secreting adrenal carcinoma being the underlying cause of these alterations. To our knowledge, this is the first reported case of hypertensive choroidopathy associated with adrenal carcinoma.

Current Standards and Recent Advances in Biomarkers of Major Endocrine Tumors.

The complexity of endocrine tumor diagnosis stems from its variable symptoms and presentation that may mimic many other disease states, or display asymptomatic properties for a prolonged amount of time. Early and accurate disease identification is needed for better patient prognosis. The key to this may be in using validated biomarkers with enhanced sensitivity and specificity. Several biomarkers are consistently used across various endocrine tumor types, possibly indicating a deeper pathophysiological mechanism behind endocrine cancer genesis and development. For example, carbohydrate antigen (CA) is measured in both pancreatic adenocarcinoma as well as ovarian cancer for diagnosis, surveillance, and risk stratification. The discovery of measuring miRNAs that are highly expressed in malignant tumors is also a novel strategy across multiple endocrine tumor types, and is propelling the future advancement of biomarker development. This review introduces currently utilized biomarkers in some of the commonly known endocrine tumors, including thyroid, adrenal, pituitary, pancreatic, and gonadal carcinoma, as well as future research directions.

Carcinoma suprarrenal: presentación de caso clínico / Adrenal carcinoma: clinical case presentation / Carcinoma adrenal: apresentação de caso clínico

El carcinoma suprarrenal es una enfermedad muy poco frecuente de presentación clínica poco específica, donde los síntomas por la propia neoplasia son los predominantes. El objetivo de esta publicación es presentar un caso clínico jerarquizando los estudios de imagen para el diagnóstico y la planificación de la táctica para la cirugía.

Adrenal carcinoma is a very rare disease with nonspecific clinical presentation, where symptoms from the neoplasia itself are predominant. The aim of this publication is to present an uncommon clinical case, emphasizing the role of imaging studies in diagnosis and surgical strategy planning.

O carcinoma adrenal é uma doença muito rara, com apresentação clínica inespecífica onde predominam os sintomas decorrentes da própria neoplasia. O objetivo desta publicação é apresentar um caso clínico infrequente priorizando exames de imagem para diagnóstico e planejamento de táticas cirúrgicas.

Carcinoma adrenocortical en mujeres en edad fértil / Adrenocortical carcinoma in women of reproductive age

Resumen Los carcinomas adrenocorticales son tumores infre cuentes, habitualmente hiperfuncionantes y con una supervivencia global pobre. La edad frecuente de pre sentación se describe en adultos entre 40 a 60 años, con predominio en sexo femenino. Se presentan dos casos inusuales de carcinoma adrenal diagnosticados en mujeres en edad fértil. El primero de ellos se descubrió en el segundo trimestre de gestación, con un cuadro de hipercortisolismo y lesión adrenal localizada, que resolvió con resección completa hacia la semana 20. En el segundo, la paciente debutó con manifestaciones clínicas de virilización rápidamente progresiva, sien do el hiperandrogenismo puro el patrón bioquímico hallado. En ambos casos, a pesar de haberse realizado la resección completa, el Ki67 elevado como principal factor pronóstico condujo a categorizarlas como de "alto riesgo de recurrencia". Asimismo, se ha asociado a la gestación y al patrón secretor de glucocorticoides como factores adicionales de mayor riesgo de recurrencia. Este es particularmente elevado dentro de los dos primeros años posteriores al diagnóstico. Existe aún controversia sobre el uso de mitotane adyuvante en estos pacientes, y su inicio está recomendado hasta los tres meses del postquirúrgico. Sin embargo, la evidencia disponible no permite suponer la falta de eficacia si se utiliza fuera de ese período. Los limitantes, como fueron el curso de la gestación y el puerperio inmediato, así como la difi cultad para el acceso a la medicación en nuestro medio, impidieron el inicio precoz del tratamiento adyuvante en ambos casos, aunque surge la inquietud de si aún sería oportuna su instauración.

Abstract Adrenocortical carcinomas are rare tumors, usually hyperfunctioning, with poor overall survival. Frequent age of presentation is described in adults between 40 and 60 years of age, predominantly female. Two unusual cases of adrenal carcinoma diagnosed in young women are presented. The first one was discovered in the sec ond trimester of gestation, with signs and symptoms of hypercortisolism and localized adrenal lesion, which was resolved with complete resection by week 20 of pregnancy. In the second case, the patient begined with clinical manifestations of rapidly progressive virilization, the biochemical pattern being pure hyperandrogenism. In both cases, despite complete resection, the high Ki67 as the main prognostic factor leaded to categorization as "high risk of recurrence". In addition, pregnancy and glucocorticoid secretory pattern have been associated as additional risk factors of recurrence. This is particularly high within the first two years after diagnosis. There is controversy about the use of adjuvant mitotane in these patients, and the general recommendation is to be started no longer than 3 months after surgery. However, the available evidence does not suggest that its use is ineffective beyond that period. Limitations, such as the course of pregnancy and the immediate puerperium, as well as the difficulty of accessing this medication in our environment, prevented the early initiation of adjuvant treatment with mitotane in both cases, although there is still concern whether its administration would still be appropriate.