The Effect of Surgeon Expertise on the Outcome of Patients with Adrenocortical Carcinoma

Complete surgical removal of adrenocortical carcinoma (ACC) represents the only chance of long-term cure. In this study, we compared the long-term outcomes of ACC patients depending on whether they had adrenal surgery performed in a high-volume (HVC) or in a low-volume (LVC) center. This retrospective study included 49 patients from the Croatian ACC Registry with the European Network for the Study of Adrenal Tumors (ENSAT) stage I–III ACC, of which 35 underwent surgery in a HVC whereas 14 of them were operated in one of the LVCs. Patients operated in the LVCs had a significantly higher rate of ACC recurrence (57.1% vs. 22.9%; p = 0.02). Accordingly, RFS was significantly longer in patients operated on in HVC (p = 0.04). The difference in RFS remained significant after controlling for age, gender, tumor size, Ki-67 index, Weiss score, and type of surgery (HR 4.55; 95% CI 1.16–17.88; p = 0.03). In addition, there is a tendency towards longer DSS in patients in the HVC group compared to those in the LVC group (p = 0.05). These results point to the centralization of adrenal surgery as a key prerequisite for improving the outcomes of ACC patients.

Innovative multidimensional models in a high-throughput-format for different cell types of endocrine origin

The adrenal gland provides an important function by integrating neuronal, immune, vascular, metabolic and endocrine signals under a common organ capsule. It is the central organ of the stress response system and has been implicated in numerous stress-related disorders. While for other diseases, regeneration of healthy organ tissue has been aimed at such approaches are lacking for endocrine diseases - with the exception of type-I-diabetes. Moreover, tumor formation is very common, however, appropriate high-throughput applications reflecting the high heterogeneity and furthermore relevant 3D-structures in vitro are still widely lacking. Recently, we have initiated the development of standardized multidimensional models of a variety of endocrine cell/tissue sources in a new multiwell-format. Firstly, we confirmed common applicability for pancreatic pseudo-islets. Next, we translated applicability for spheroid establishment to adrenocortical cell lines as well as patient material to establish spheroids from malignant, but also benign adrenal tumors. We aimed furthermore at the development of bovine derived adrenal organoids and were able to establish steroidogenic active organoids containing both, cells of cortical and medullary origin. Overall, we hope to open new avenues for basic research, endocrine cancer and adrenal tissue-replacement therapies as we demonstrate potential for innovative mechanistic insights and personalized medicine in endocrine (tumor)-biology.

Genitourinary Tract Tumors in Children: An Update

Background: Genitourinary tract tumors in children are less common than in adults. Most of these tumors have different genetic backgrounds, clinical presentation, and oncologic behavior than their adult counterpart. As a result of low prevalence in children, some of the treatment approaches and recommendations are based on treatment experience in adult patients. However, thanks to scientific and technological development, survival rates have risen considerably. Objective: This paper presents a review of the principal features of the tumors involving the genitourinary tract in children and an update in genetic background, diagnosis, and treatment. Methods: A narrative review was performed on published literature about genitourinary tract tumors in pediatric patients. Papers presented in English and Spanish literature were reviewed. PubMed, Science Direct, and SciELO databases were used to collect information and present this article. Results: Kidney tumors are the most common type of genitourinary tumors in children. Among those, Wilms tumor represents the majority of cases and shows the successful work of clinical trial groups studying this tumor type. Other tumors involving the genitourinary tract in children include Rhabdomyosarcoma, Transitional cell carcinoma, Testicular, and Adrenal tumors. Conclusion: Genitourinary tract tumors in children represent significant morbidity and economic burden, so awareness in early diagnosis represents improvement in treatment, clinical and oncological outcomes.

The F-18 FDG PET/CT evaluation of the metastatic adrenal lesions of the non-lung cancer tumors compared with pathology results

Background The aim of this study was to evaluate the role of F-18 FDG PET/CT imaging in the diagnosis of primary and metastatic adrenal tumors that originate from non-lung cancer primary tumors. Results F-18 FDG PET/CT images of patients (8 male and 6 female; mean: 55.36 ± 16.2 years old) who attended with the diagnosis of primary or adrenal metastatic lesions other than lung cancer metastasis were evaluated in a retrospective manner. The diameter of the adrenal lesions was mean: 23.93 ± 36.6 mm with SUVmax levels of mean: 9.98 ± 7.8. The primary site of 2/3 of the patients with unknown primary was the adrenal gland, and in one of the patients primary tumor remained unknown during follow-up. Conclusions According to the results of this study, F-18 FDG PET/CT has high diagnostic performance in the diagnosis of the adrenal gland primary and metastatic lesions, which originate from non-lung cancer tumors. Further studies in the larger series are warranted.

18F-FDG PET/CT image findings of a dog with adrenocortical carcinoma

Background In human medicine, 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) has been used to differentiate between benign and malignant adrenal tumors and to identify metastases. However, canine adrenocortical carcinomas identified by 18F-FDG PET/computed tomography (CT) have not been reported. Case presentation A 13-year-old, castrated male, Cocker Spaniel dog with severe systolic hypertension exhibited an adrenal mass approximately 3.6 cm in diameter on ultrasonography. There was no evidence of pulmonary metastasis or vascular invasion on thoracic radiography and abdominal ultrasonography, respectively. 18F-FDG PET/CT was performed to identify the characteristics of the adrenal mass and the state of metastasis. One hour after injection of 5.46 MBq/kg 18F-FDG intravenously, the peripheral region of the adrenal mass visually revealed an increased 18F-FDG uptake, which was higher than that of the liver, and the central region of the mass exhibited necrosis. The maximal standardized uptake value (SUV) of the adrenal mass was 3.24; and relative SUV, calculated by dividing the maximal SUV of the adrenal tumor by the mean SUV of the normal liver, was 5.23. Adrenocortical carcinoma was tentatively diagnosed and surgical adrenalectomy was performed. Histopathologic examination of the resected adrenal mass revealed the characteristics of an adrenocortical carcinoma. After adrenalectomy, systolic blood pressure reduced to below 150 mmHg without any medication. Conclusion This is the first case report of 18F-FDG PET/CT findings in a dog with suspected adrenocortical carcinoma and may provide valuable diagnostic information for adrenocortical carcinoma in dogs.

Initial experience with laparoscopic posterior retroperitoneal adrenalectomy in single tertiary center

Purpose: Laparoscopic posterior retroperitoneal adrenalectomy (LPRA) is a surgical method that accesses the adrenal gland through the back. The aim of this study was to report initial experience of LPRA and evaluate possibilities for surgical application.Methods: From March 2018 to December 2019, a total of 30 consecutive patients diagnosed with adrenal tumor underwent surgical treatment at Pusan National University Hospital were enrolled. Clinicopathologic features and various peri- and postoperative parameters were analyzed by retrospective medical record review. The mean age of the patients was 48.20 ± 13.66 years.Results: The mean body mass index (BMI) was 25.50 ± 4.30 kg/m2 . Primary hyperaldosteronism was the most frequently preoperative diagnosed disease (n = 13, 43.4%), followed by adrenal incidentaloma (n = 8, 26.6%), Cushing syndrome (n = 5, 16.6%) and pheochromocytoma (n = 4, 13.3%). The mean size of postoperative adrenal tumor was 2.72 ± 1.76 cm. The mean operating time was 162 ± 58.14 minutes. Among the 30 patients, 28 patients underwent total adrenalectomy (93.3%) and two patients underwent cortical sparing adrenalectomy (6.7%). When LPRA was performed for patients with BMI > 23.16 kg/m2 , the operating time was longer than the average (P = 0.016).Conclusion: LPRA was suitable and safe for patients with benign adrenal tumors. BMI, retroperitoneal fat density and postoperative adrenal weight may be related to the operating time, so they should be considered when deciding on a surgical method for adrenalectomy.

Familial Hyperaldosteronism Type 3 with a Rapidly Growing Adrenal Tumor: An In Situ Aldosterone Imaging Study

Primary aldosteronism is most often caused by aldosterone-producing adenoma (APA) and bi-lateral adrenal hyperplasia. Most APAs are caused by somatic mutations of various ion channels and pumps, the most common being the inward-rectifying potassium channel KCNJ5. Germ line mutations of KCNJ5 cause familial hyperaldosteronism type 3 (FH3), which is associated with severe hyperaldosteronism and hypertension. We present an unusual case of FH3 in a young woman, first diagnosed with primary aldosteronism at the age of 6 years, with bilateral adrenal hyperplasia, who underwent unilateral adrenalectomy (left adrenal) to alleviate hyperaldosteronism. However, her hyperaldosteronism persisted. At the age of 26 years, tomography of the remaining adrenal revealed two different adrenal tumors, one of which grew substantially in 4 months; therefore, the adrenal gland was removed. A comprehensive histological, immunohistochemical, and molecular evaluation of various sections of the adrenal gland and in situ visualization of aldosterone, using matrix-assisted laser desorption/ionization imaging mass spectrometry, was performed. Aldosterone synthase (CYP11B2) immunoreactivity was observed in the tumors and adrenal gland. The larger tumor also harbored a somatic β-catenin activating mutation. Aldosterone visualized in situ was only found in the subcapsular regions of the adrenal and not in the tumors. Collectively, this case of FH3 presented unusual tumor development and histological/molecular findings.

Giant Adrenal Lipoma with Hemorrhage Requiring Extended Surgical Resection

Adrenal lipoma is a rare, benign tumor, reported to account for 0.7% of primary adrenal tumors. A 69-year-old man presented with left lateral abdominal pain. Computed tomography (CT) was performed, and a huge, irregularly shaped retroperitoneal tumor of uneven internal density was identified, with the border between the tumor and the pancreas and kidney being unclear. Active hemorrhage was also depicted. The tumor consisted mainly of fat, with the exception of the hematoma; it measured 200 mm; and the boundary between it and nearby organs, such as the pancreas, was unclear. Despite angiography being performed twice, the responsible vessel was not identified. Thus, for the purpose of both diagnosis and treatment, we resected the tumor, and considering the possibility of a malignancy, such as liposarcoma, we also resected the pancreatic body and tail and the spleen. The final histopathologic diagnosis was benign adrenal lipoma with hemorrhage, with no invasion to surrounding tissue. Hemorrhage within an adrenal tumor is rare. Most adrenal lipomas are small “incidentalomas” and asymptomatic. With development of a large adrenal lipoma comes the possibility of hemorrhage along with the possibility of features suggestive of malignancy. We encountered a giant adrenal lipoma with hemorrhage and, because of the aforementioned features, performed extended surgical resection, seen in retrospect as oversurgery. The widespread use of CT has led to an increased number of reported cases of adrenal lipoma. We anticipate an accumulation of case reports, which will allow for development of an appropriate treatment algorithm.