An imaging and diagnostic conundrum-the adrenal haemangioma.

The adrenal haemangioma, a rare benign vascular tumour, is increasingly detected through abdominal imaging. Just over 70 surgical cases have been reported since 1955. Their potential large size and overlapping imaging features with adrenocortical carcinoma poses a diagnostic challenge. Adrenalectomy is often needed for a definitive diagnosis due to inconclusive imaging. We report the case of a 61-year-old female presenting with an incidental finding of a right-sided 9.5-cm adrenal mass on imaging. Due to the risk of adrenocortical carcinoma with inconclusive imaging findings, an open right adrenalectomy was performed. The patient was discharged after 6 days with no complications. Post-surgical histopathology confirmed a diagnosis of adrenal haemangioma with a secondary adrenal pseudocyst. The presence of an adrenal incidentaloma with discordant radiological features proves to be a diagnostic conundrum. Therefore, in the setting of contradictory radiology and concerning mass size, we recommend adrenalectomy for definitive diagnosis of an adrenal haemangioma.

Safety and surgical outcomes of robotic adrenalectomy from a 15-year experience at a single institution.

Robotic adrenalectomy (RA) has gained significant popularity in the management of adrenal gland diseases. We report our experience at a single tertiary institution and evaluate the safety and surgical outcomes of RA. The data of 122 consecutive patients who underwent RA from October 2009 to December 2022 at Korea University Anam Hospital (Seoul, Korea) were reviewed. There were no perioperative complications. Clinicopathological features and surgical outcomes were retrospectively analyzed through complete chart reviews. Noteworthy findings include the influence of sex, tumor size, and body mass index on operation time, with the female and small tumor groups exhibiting shorter operation times (P = 0.018 and P = 0.009, respectively). Pheochromocytoma was identified as a significant independent risk factor for a longer operation time in the multivariate analysis [odds ratio (OR), 3.709; 95% confidence interval (CI), 1.127-12.205; P = 0.031]. A temporal analysis revealed a decreasing trend in mean operation times across consecutive groups, reflecting a learning curve associated with RA adoption. RA is a safe and effective operative technique alternative to laparoscopic adrenalectomy that has favorable surgical outcomes and enhances the convenience of the operation.

Comparison of adrenalectomy with conservative treatment on mild autonomous cortisol secretion: a systematic review and meta-analysis.

Objective: This systematic review and meta-analysis was conducted to compare the benefits of adrenalectomy and conservative treatment for comorbidities associated with mild autonomous cortisol secretion (MACS) in patients diagnosed with MACS. Background: MACS is the most common benign hormone-secreting functional adrenal incidentaloma. Overproduction of cortisol is observed in MACS patients, resulting in a variety of long-term health issues, including arterial hypertension (HTN), diabetes mellitus (DM), dyslipidemia, obesity, and osteoporosis; however, the classic clinical manifestations of Cushing's syndrome (CS) are not present. Methods: A systematic search was conducted using MEDLINE, Embase, Web of Sciences, and Scopus databases on December, 2023. Two reviewers independently extracted data and assessed the quality of the included articles. A meta-analysis was performed to compare the beneficial effects of adrenalectomy versus conservative management for MACS-related comorbidities. Results: Fifteen articles were included in this study, which evaluated 933 MACS patients (384 Adrenalectomy and 501 Conservative treatment, and 48 excluded due to incomplete follow-up duration). MACS diagnosis criteria were different among the included articles. All studies, however, stated that there must be no overt CS symptoms. Meta-analysis demonstrates the overall advantage of adrenalectomy over conservative treatment for MACS-related comorbidities (Cohen's d = -0.49, 95% CI [-0.64, -0.34], p = 0.00). Subgroup analysis indicated that the systolic blood pressure (pooled effect size = -0.81, 95% CI [-1.19, -0.42], p = 0.03), diastolic blood pressure (pooled effect size = -0.63, 95% CI [-1.05, -0.21], p = 0.01), and BMD (pooled effect size = -0.40, 95% CI [-0.73, -0.07], p = 0.02) were significantly in favor of adrenalectomy group rather than conservative treatment but no significant differences between the two treatment groups in other MACS-related comorbidities were reported. Conclusion: Despite the limited and diverse data, this study demonstrates the advantage of adrenalectomy over conservative treatment for MACS-related comorbidities.

Biochemical Control in Cushing's syndrome: Outcomes of the treatment in a large single center cohort.

BACKGROUND: Normalization of hypercortisolism is essential to reduce morbidity and mortality in patients with Cushing's syndrome (CS). The aim of this analysis was to assess biochemical control rates in patients with Cushing's disease (CD), ectopic Cushing's syndrome (ECS) and adrenal Cushing's syndrome (ACS). METHODS: Patients with confirmed CS (n= 296) treated in a single tertiary care center were retrospectively analysed (185 CD, 27 ECS, 84 uni- and bilateral ACS). RESULTS: Firstline treatment led to biochemical control in 82% of the patients. Time to biochemical control (median, IQR) was longer in CD (11.0 weeks, 5.6-29.8; p< 0.05) than in ACS (7.7 weeks, 4.1-17.1) and ECS (5.6 weeks, 4.1-23.3). Disease persistence or recurrence after first-line therapy was observed more often in CD (24% and 18%; p< 0.05) than in ECS (15% and 15%) and ACS (6% and 4%). Total time in hypercortisolism since diagnosis was significantly shorter in patients with CD diagnosed since 2013, after specialized patient care was implemented, compared to patients diagnosed before 2013 (13.5 weeks, vs. 26.1 weeks; p< 0.0070). Control of hypercortisolism at last follow up (76 months, 38-163) was achieved in 94% of patients with ACS, 100% of patients with ECS and 92% of patients with CD. CONCLUSIONS: Biochemical control can be achieved in most patients with different subtypes of CS within a reasonable time frame. Control of hypercortisolism has improved over time.

The level of accordance between preoperative and postoperative diagnosis in patients undergoing adrenalectomy - a study of 230 consecutive cases.

Introduction: Patients are qualified for an adrenalectomy due to endocrine or oncologic reasons. Final histopathological diagnoses include a wide spectrum of more than a dozen entities. The aim of this study was to compare preoperative and postoperative diagnoses of patients undergoing adrenalectomy to determine the level of diagnostic accuracy, as well as sex and age of patients. Material and methods: A group of 214 patients (230 specimens in total) operated on in a single center was studied and their demographic and pathological data were investigated. Results: The majority of diagnoses were characterized by both high positive predictive value and sensitivity, excluding pheochromocytoma (60.0% and 67.7%, respectively) and adrenal cyst (100% and 37.5%, respectively). Patients operated on due to Cushing's syndrome were statistically significantly more often females (p = 0.009), while those with metastases (diagnosed both pre- and postoperatively) were more often males (both p = 0.001). Patients qualified due to non-functioning tumors were older than those with Cushing's or Conn's syndrome (p = 0.044 and p = 0.002, respectively). Conclusions: The lowest diagnostic accuracy is observed in cases of pheochromocytoma and adrenal cyst. Meticulous preparation of the patient for hormonal tests, including discontinuation of certain medications, is essential for obtaining accurate results. The diagnosis of Cushing's syndrome is more prevalent in females, while metastasis syndrome is more prevalent in males. Adrenocortical carcinoma may initially be diagnosed as a non-functioning tumor (1.6% of such cases) or a recurrence of a previously resected tumor, which should always raise a suspicion of a malignant neoplasm.

Abiraterone in Classic Congenital Adrenal Hyperplasia: Results of Medical Therapy Before Adrenalectomy.

We present the case of a 20-year-old woman with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency, with uncontrolled hyperandrogenemia despite supraphysiological glucocorticoid therapy. We used abiraterone acetate, an inhibitor of the 17-hydroxylase/17,20-lyase enzyme, to suppress adrenal androgen synthesis and allow physiological glucocorticoid and mineralocorticoid therapy, as a proof-of-concept, before proceeding to bilateral adrenalectomy. We report the patient's clinical course, the changes in adrenal steroids, and the immunohistochemistry of the adrenals.

Cystic adrenal mass revealing a pheochromocytoma in the setting of multiple endocrine neoplasia: A case report.

Pheochromocytoma, a neuroendocrine tumor, represents a rare medical condition characterized by the excessive secretion of catecholamines. These tumors often exhibit distinctive features on imaging studies, notably appearing hypervascular. Furthermore, they may present as cystic masses with thin walls, a characteristic that becomes more evident following the administration of contrast medium. The cystic form of adrenal pheochromocytoma, as exemplified in our case, is particularly uncommon, thus underscoring the importance of recognizing its atypical presentation. Accurate diagnosis hinges on a thorough understanding of both the clinical manifestations and radiological findings suggestive of pheochromocytoma. However, definitive confirmation typically necessitates histological examination of the surgical specimen post-adrenalectomy. By shedding light on this rare variant, our case emphasizes the critical role of comprehensive diagnostic approaches in managing such complex medical conditions. Additionally, it underscores the significance of multidisciplinary collaboration among clinicians, radiologists, and pathologists to ensure timely and accurate diagnosis, ultimately guiding appropriate treatment strategies and optimizing patient outcomes.

Efficacy and safety of radiofrequency ablation and laparoscopic adrenalectomy for primary aldosteronism: a meta­analysis.

OBJECTIVE: To compare the efficacy (including blood pressure, medication reduction, serum potassium, and clinical success) and safety parameters (including operative time, length of hospital stay, blood loss, hypertension crisis rate, and complication rate) of radiofrequency ablation (RFA) and laparoscopic adrenalectomy (LA) in the treatment of primary aldosteronism (PA). METHODS: Literature search was performed on PubMed, EMBASE, The Cochrane Library (Issue 8, 2023), Web of Science, China National Knowledge Infrastructure, and Wanfang from inception to August 2023. Study selection, data extraction, and risk of bias assessment were performed by two independent reviewers. Quality assessment was conducted using the Newcastle-Ottawa scale. The Stata 12.0 software was used for statistical analyses. Pooled odds ratios (OR) with corresponding 95% confidence interval (CI) were calculated for categorical outcomes, while mean difference (MD) with corresponding 95% CI were calculated for continuous outcomes. RESULTS: A total of 5 studies involving 204 patients (LA, n = 127; and RAF, n = 77) were included. LA had better diastolic blood pressure control than RFA (WMD = 5.19; 95% CI 0.96-9.43); however, the RFA demonstrated better shorter operative time (WMD = - 57.99; 95% CI - 116.54 to 0.57), and shorter length of hospital stay (OR - 1.6; 95% CI - 2.37 to - 0.83) compared to LA. All remaining parameters were comparable between the interventions. CONCLUSION: While grossly comparable in efficacy as treatment options for PA, RFA may allow for shorter operative time and hospital stay, less intraoperative blood loss, and lower hospitalization costs. However, LA has better diastolic blood pressure control. Even so, we still need larger prospective studies, specifically with comparative hypertension response (short and long term) and number of post-procedural antihypertensive medication requirement.

A novel method of tunneling retroperitoneoscopic adrenalectomy: a prospective study.

BACKGROUND: To introduce the surgical technique and our team's extensive experience with tunnel method in laparoscopic adrenalectomy. METHODS: From July 2019 to June 2022, we independently designed and conducted 83 cases of " Tunnel Method Laparoscopic Adrenalectomy," a prospective study. There were 45 male and 38 female patients, ages ranged from 25 to 73 years(mean: 44.6 years).The cases included 59 adrenal cortical adenomas, 9 pheochromocytomas, 6 cysts, 4 myelolipomas, 1 ganglioneuroma, and 4 cases of adrenal cortical hyperplasia. In terms of anatomical location, there were 39 cases on the left side, 42 on the right side, and 2 bilateral cases. Tumor diameters ranged from 0.6 to 5.9 cm(mean: 2.9 cm). Utilizing ultrasound monitoring, percutaneous puncture was made either directly to the target organ or its vicinity, and the puncture path was manually marked. Then, under the direct view of a single-port single-channel laparoscope, the path to the target organ in the retroperitoneum or its vicinity was further delineated and separated. This approach allowed for the insertion of the laparoscope and surgical instruments through the affected adrenal gland, thereby separating the surface of the target organ to create sufficient operational space for the adrenalectomy. RESULTS: All 83 surgeries were successfully completed. A breakdown of the surgical approach reveals that 51 surgeries were done using one puncture hole, 25 with two puncture holes, and 7 with three puncture holes. The operation time ranged from 31 to 105 min (mean: 47 min), with a blood loss of 10 to 220mL (mean: 40 mL). Notably, there were no conversions to open surgery and no intraoperative complications. Postoperative follow-up ranged from 6 to 28 months, during which after re-examination using ultrasound, CT, and other imaging methods, there were no recurrences or other complications detected. CONCLUSIONS: The completion of the tunnel method laparoscopic adrenalectomy represents a breakthrough, transitioning from the traditional step-by-step separation of retroperitoneal tissues to reach the target organ in conventional retroperitoneoscopic surgery. This method directly accesses the target organ, substantially reducing the damage and complications associated with tissue separation in retroperitoneoscopic surgery, As a result, it provides a new option for minimally invasive surgery of retroperitoneal organs and introduces innovative concepts to retroperitoneoscopic surgery.

A prospective case series to evaluate subcostal nerve injury with high-resolution ultrasound in posterior retroperitoneoscopic adrenalectomy.

BACKGROUND: Posterior retroperitoneoscopic adrenalectomy has several advantages over transabdominal laparoscopic adrenalectomy regarding operating time, blood loss, postoperative pain, and recovery. However, postoperatively several patients report chronic pain or hypoesthesia. We hypothesized that these symptoms may be the result of damage to the subcostal nerve, because it passes the surgical area. METHODS: A prospective single-center case series was performed in adult patients without preoperative pain or numbness of the abdominal wall who underwent unilateral posterior retroperitoneoscopic adrenalectomy. Patients received pre- and postoperative questionnaires and a high-resolution ultrasound scan of the subcostal nerve and abdominal wall muscles was performed before and directly after surgery. Clinical evaluation at 6 weeks was performed with repeat questionnaires, physical examination, and high-resolution ultrasound. Long-term recovery was evaluated with questionnaires, and photographs from the patients were examined for abdominal wall asymmetry. RESULTS: A total of 25 patients were included in the study. There were no surgical complications. Preoperative visualization of the subcostal nerve was possible in all patients. At 6 weeks, ultrasound showed nerve damage in 15 patients, with no significant association between nerve damage and postsurgical pain. However, there was a significant association between nerve damage and hypoesthesia (p = 0.01), sensory (p < 0.001), and motor (p < 0.001) dysfunction on physical examination. After a median follow-up of 18 months, 5 patients still experienced either numbness or muscle weakness, and one patient experienced chronic postsurgical pain. CONCLUSION: In this exporatory case series the incidence of postoperative damage to the subcostal nerve, both clinically and radiologically, was 60% after posterior retroperitoneoscopic adrenalectomy. There was no association with pain, and the spontaneous recovery rate was high.

Risk factors for postoperative fever after laparoscopic adrenalectomy focusing on hormones produced: a case control study.

BACKGROUND: Laparoscopic adrenalectomy is widely performed for a number of hormone-producing tumors and postoperative management depends on the hormones produced. In the present study, we conducted a retrospective analysis to clarify the risk factors for postoperative complications, particularly postoperative fever after laparoscopic adrenalectomy. METHODS: We analyzed 406 patients who underwent laparoscopic adrenalectomy at our hospital between 2003 and 2019. Postoperative fever was defined as a fever of 38 °C or higher within 72 h after surgery. We investigated the risk factors for postoperative fever after laparoscopic adrenalectomy. RESULTS: There were 188 males (46%) and 218 females (54%) with a median age of 52 years. Among these patients, tumor pathologies included 188 primary aldosteronism (46%), 75 Cushing syndrome (18%), and 80 pheochromocytoma (20%). Postoperative fever developed in 124 of all patients (31%), 30% of those with primary aldosteronism, 53% of those with pheochromocytoma, and 8% of those with Cushing syndrome. A multivariate logistic regression analysis identified pheochromocytoma and non-Cushing syndrome as independent predictors of postoperative fever. Postoperative fever was observed in 42 out of 80 cases of pheochromocytoma (53%), which was significantly higher than in cases of non-pheochromocytoma (82/326, 25%, p < 0.01). In contrast, postoperative fever developed in 6 out of 75 cases of Cushing syndrome (8%), which was significantly lower than in cases of non-Cushing syndrome (118/331, 35.6%, p < 0.01). CONCLUSION: Since postoperative fever after laparoscopic adrenalectomy is markedly affected by the hormone produced by pheochromocytoma and Cushing syndrome, it is important to carefully consider the need for treatment.

Case report: 65-year-old man with metachronous left sided adrenal metastasis of hepatocellular carcinoma.

INTRODUCTION AND IMPORTANCE: Due to therapeutic advances and improvements in follow-up care, the diagnosis and treatment of extrahepatic metastases of hepatocellular carcinoma [HCC] have gained clinical significance. However, adrenal gland metastases of HCC remain a rare clinical encounter. Several systemic and local treatment options are discussed in current literature. Adrenalectomy in cases of isolated adrenal metastases with well-controlled intrahepatic lesions has been shown to benefit patients in case series. PRESENTATION OF THE CASE: This 65-year-old patient presented with suspected metachronous left sided adrenal metastasis seven years after bisegmentectomy for HCC and after undergoing trans-arterial chemoembolization [TACE] for multifocal intrahepatic recurrences while being listed for liver transplantation "beyond Milan criteria". Adrenalectomy was suggested for histopathological confirmation of the suspected metastasis and re-consideration for liver transplant. The resection was performed laparoscopically and metastasis of HCC was confirmed in histopathological analysis. Postoperatively, the patient recovered quickly. However, the patient decided against re-listing for liver transplantation. CLINICAL DISCUSSION: Current literature suggests, that minimally-invasive adrenalectomy should be considered in patients with no more than two extrahepatic lesions, a Child-Pugh-Score of less than A5, low alpha-fetoprotein [AFP] levels <100 ng/ml and size <3 cm. The oncological goal should be to achieve a tumor free extrahepatic situation with a potential oncological benefit. CONCLUSION: Our patient presented as an ideal candidate for resection of the adrenal gland metastasis and could have been re-assessed postoperatively for liver transplantation. Still, more research is needed to improve patient-selection for metastasectomy in HCC.

Long-term outcome of retroperitoneoscopic partial versus total adrenalectomy in patients with Cushing's syndrome.

BACKGROUND: We analyze the long-term outcome of surgery for Cushing's syndrome (CS) and the influence of the extent of surgical resection on the duration of postoperative cortisone substitution. METHODS: One-hundred forty-one patients (129 female, 12 males; mean age: 45.7 ± 12.8 years) operated between January 2000 to June 2020 were included in the analysis. Patients suffered from manifest (124) or subclinical (17) CS due to benign unilateral adrenal neoplasia. All tumors were removed by the posterior retroperitoneoscopic approach. 105 patients had total (TA) and 36 partial (PA) adrenalectomies. All patients were discharged with ongoing corticosteroid supplementation therapy. RESULTS: Follow-up data could be obtained for 83 patients. Twenty-four (1 male, 23 females; mean age 42.3 years) underwent PA and 59 TA (6 males, 53 females; mean age 44.6 years). Mean follow-up time was 107 ± 68 months (range: 6-243 months). The median duration of postoperative corticosteroid therapy was 9.5 months after PA and 11 months after TA (p = 0.1). Significantly, more patients after total adrenalectomy required corticosteroid therapy for more than 24 months (25% vs. 4%; p = 0.03). Recurrent ipsilateral disease occurred in one case after partial adrenalectomy and was treated by completion adrenalectomy. A case of contralateral recurrence associated with subclinical Cushing's syndrome was observed after total adrenalectomy. CONCLUSIONS: The risk of local recurrence after partial adrenalectomy in CS is low. Cortical-sparing surgery may shorten corticosteroid supplementation therapy after surgery.

Hypercoagulability based on thromboelastography is common in dogs undergoing adrenalectomy.

OBJECTIVE: Thromboelastography (TEG) is a whole blood assay that yields global assessment of hemostasis, as it evaluates clot time, strength, and kinematics of clot formation and lysis. The main objective was to describe preoperative TEG findings in dogs that had an adrenalectomy performed and, secondarily, to describe TEG findings in the dogs with or without hyperadrenocorticism (HAC). ANIMALS: 30 dogs that had preoperative TEG and adrenalectomy performed. METHODS: Medical records between 2018 and 2022 were reviewed. Signalment, diagnostic data, and perioperative treatment were abstracted. RESULTS: 53% (16/30) of the dogs were hypercoagulable, and none were hypocoagulable. Based on histopathology, 6 of 9 dogs with adenocarcinoma were hypercoagulable, 4 of 8 with pheochromocytoma were hypercoagulable, and 6 of 10 with adenoma were hypercoagulable. None of the 3 dogs with other histopathologic diagnoses or combinations of diagnoses (adrenocortical hyperplasia, poorly differentiated sarcoma, and both adrenocortical adenocarcinoma and pheochromocytoma) were hypercoagulable. Of the 14 dogs tested preoperatively for HAC, 4 of 8 HAC dogs were hypercoagulable and 2 of 6 non-HAC dogs were hypercoagulable. CLINICAL RELEVANCE: The present report describes for the first time TEG findings for dogs undergoing adrenalectomy and suggests that the majority of dogs with adrenal neoplasia are hypercoagulable based on TEG results.

Outcomes of Minimally Invasive Adrenalectomy for Large Adrenal Masses: A Multi-Centre Experience in Saudi Arabia.

Introduction Advancements in radiological imaging technology have increased the discovery of adrenal incidentalomas. Large adrenal tumors (LATs) are not common, and the likelihood of malignancy increases with tumor size. LATs were defined as tumors larger than four centimeters (cm) with various pathologic diagnoses. Traditionally, open adrenalectomy was considered the gold standard for LATs, but with recent advancements in minimally invasive surgery (MIS), optimum perioperative and long-term outcomes are achievable by the MIS approach. The findings presented in this paper show that even large adrenal masses measuring up to 21 centimeters can be safely removed using a minimally invasive approach. Methodology After Institutional Review Board (IRB) approval, we reviewed medical records of adult patients who had adrenalectomies at two Saudi Arabian centers from January 2013 to February 2023. Inclusion criteria were laparoscopic or robotic adrenalectomy and adrenal lesions ≥5cm. Pediatric patients and those with open adrenalectomies were excluded. Pre-surgery, patients had imaging studies to assess mass characteristics. Pheochromocytoma patients received a 2-week adrenergic blocker treatment. Perioperative data including demographics, comorbidities, mass characteristics, surgery details, and follow-up were analyzed using SPSS-23. Patients provided informed consent and had follow-up appointments and imaging. Results Our experience involved 35 patients, 29 of whom received laparoscopic treatment and six of whom underwent robotic surgery. Of the 35 patients, more than half were females (57.1%), with a mean age of 41.7±14.9 years, the youngest and oldest participants being 16 and 73 years of age, respectively. The mean body mass index (BMI) of the participants was in the overweight range (26±6.0 kg/m2). The most common mode of presentation was incidental (42.9%), followed by hypertension (17.1%). Most patients had right-sided adrenal gland involvement (48.6%), with only four patients showing bilateral involvement. Most of the patients were classified as American Society of Anesthesiology score (ASA) 2 (40.0%) or ASA 3 (40.0%). Most of the patients were diagnosed with myelolipoma or adenoma (22.9% each) followed by pheochromocytoma (17.1%). The average estimated blood loss (EBL) was 189.3±354.6 ml for patients who underwent laparoscopic surgery and 80.0 ±34.6 ml for patients who underwent robotic surgery. The average operative room time (ORT) was 220.1±98.7 minutes (min) for laparoscopic surgery and 188.3±10.3 min for robotic surgery. One patient had to be converted from laparoscopic to open surgery due to aortic injury. The average length of stay (LOS) was 9.5±6.7 days for laparoscopic treatment and 5.5±1.9 days for robotic surgery. The mean tumor size in the greatest dimension was 8.0±4.4 cm. Only one patient who underwent unilateral laparoscopy experienced perioperative complications and converted to open surgery; nine patients who underwent unilateral laparoscopy required blood transfusion, and none of the patients who underwent robotic surgery required transfusion. None of the 35 patients experienced a recurrence of their adrenal disease during the mean follow-up period which lasted around 58 months. Conclusion MIS in Saudi Arabia is growing and is a safe method for LATs, with satisfactory surgical results compared to the traditional open surgery approach. It offers advantages in terms of EBL, complications, and disease recurrence.

Adrenal tumors in patients with neuroendocrine neoplasms.

PURPOSE: To study the prevalence of primary adrenal tumors and adrenal metastases in patients with neuroendocrine neoplasms (NENs) and describe these in detail. NENs can be further divided into neuroendocrine tumor (NET) and neuroendocrine carcinoma (NEC). METHODS: A review of medical files was conducted for all patients who underwent a 68Gallium-DOTATOC-PET/CT during 2010-2023 or adrenalectomy during 1999-2023 at the Karolinska University Hospital. RESULTS: In total, 68Gallium-DOTATOC-PET/CT was performed on 1750 individuals with NEN, among whom 12 (0.69%) had adrenal tumors. Of these, 9 (0.51%) were NEN metastases. Out of 1072 adrenalectomies, 4 (0.37%) showed evidence of NEN metastases. Thus, 16 patients with NEN exhibited adrenal tumors. The adrenal tumors were found on average 5 years after the NEN diagnosis and 19% of the adrenal tumors with simultaneous NEN were benign. Few had all adrenal hormones measured. None had an adrenal insufficiency nor an adrenal biopsy. Another synchronous metastasis was found in 69% at the time of the adrenal tumor discovery. During the median 2-year follow-up, 38% of the subjects had deceased (with the exclusion of individuals presenting supposedly benign adrenal tumors 31%) all due to tumor complications. A comparison between individuals identified through 68Gallium-DOTATOC-PET/CT and those who underwent adrenalectomy revealed a higher prevalence of NETs in the former group and NECs in the latter group. CONCLUSION: Adrenal primary tumors and adrenal metastases are infrequent occurrences in patients with NEN. Most cases involved the presence of NEN metastasis upon the initial discovery of adrenal tumors. The overall prognosis was found to be favorable.

A fatal misdiagnosis of page kidney - case report.

Page kidney is a condition where external compression of the renal artery and renal parenchyma leads to subsequent ischaemia and activation of renin-angiotensin-aldosterone axis. A 42-year-old female with hirsutism, hypertension and diabetes was diagnosed with a right adrenal mass and underwent laparoscopic adrenalectomy. Her hypertension worsened postoperatively and was managed medically. Subsequently she developed a right flank pain on the fifth postoperative day and died suddenly the next day. Autopsy revealed a pale body with cushingoid appearance. Surgical scars were healthy. Internal examination of the abdomen revealed a haemoperitoneum of 500 ml together with a large subcapsular haematoma measuring 1000 ml surrounding the right kidney, compressing the right renal artery. Kidneys were pale and the right kidney was soft and friable. Cortical surface of the right kidney demonstrated a possible surgical puncture site with an overlying thrombus together with a contused inferior vena cava. Other organs were pale but appeared otherwise normal. Histology revealed diffuse cortical necrosis of right kidney and features of adult respiratory distress syndrome in the lungs. Haemorrhagic shock following laparoscopic adrenalectomy for right adrenal tumor was declared as the cause of death, contributed by the development of the Page kidney. Trauma of several aetiologies including laparoscopic abdominal surgery may contribute to Page kidney. It presents with flank pain, hypertension and renal mass. Since postoperative blood loss usually manifests as hypotension, resulting hypertension may mislead the attending clinicians. Once diagnosed, it can be managed with surgical drainage and antihypertensives.

Outcome in dogs with invasive adrenal gland tumors that did not pursue adrenalectomy.

OBJECTIVE: To report the survival times in dogs diagnosed with adrenal tumors with vascular or soft tissue invasion that did not undergo adrenalectomy. ANIMALS: Retrospective case series of 32 client-owned dogs. METHODS: The medical records of a referral veterinary hospital were reviewed to identify dogs that were diagnosed with an invasive adrenal mass and did not undergo adrenalectomy between January 2013 and December 2022. Data collected included signalment, examination findings, and diagnostic results from the initial presentation. Descriptive statistics were calculated to summarize dog signalment information, and Kaplan-Meier survival analysis was performed for calculation of median survival time. RESULTS: Most dogs (n = 28) had vascular invasion, primarily into the caudal vena cava. Surgery was offered but not pursued due to perceived risk of sudden death (n = 5), risk of hemorrhage (4), or concurrent diagnosis of disseminated intravascular coagulation (1). Only 1 dog pursued stereotactic body radiation therapy, and 1 was prescribed toceranib phosphate (Palladia). Of these 32 dogs, 30 (93.8%) died or were euthanized and 2 (6.2%) dogs survived. The median follow-up time was 49 days (range, 0 to 1,910 days). The median survival time was 50 days (95% CI, 4 to 194 days). The most common cause of death or euthanasia was hemoabdomen (n = 8). CLINICAL RELEVANCE: Nonsurgical management of invasive adrenal tumors was associated with short survival times in this case series.

Prognostic significance of lymph node count in surgically treated patients with T2-4 stage nonmetastatic adrenocortical carcinoma.

PURPOSE: The role of lymphadenectomy and the optimal lymph node count (LNC) cut-off in nonmetastatic adrenocortical carcinoma (nmACC) are unclear. METHODS: Within the Surveillance, Epidemiology, and End Results (SEER) database, surgically treated nmACC patients with T2-4 stages were identified between 2004 and 2020. We tested for cancer-specific mortality (CSM) differences according to pathological N-stage (pN0 vs. pN1) and two previously recommended LNC cut-offs (≥4 vs. ≥5) were tested in pN0 and subsequently in pN1 subgroups in Kaplan-Meier plots and multivariable Cox regression models. RESULTS: Of 710 surgically treated nmACC patients, 185 (26%) underwent lymphadenectomy and were assessable for further analyses based on available LNC data. Of 185 assessable patients, 152 (82%) were pN0 and 33 (18%) were pN1. In Kaplan-Meier analyses, CSM-free survival was 74 vs. 14 months (Δ 60 months, P ≤ 0.001) in pN0 vs. pN1 patients, respectively. In multivariable analyses, pN1 was an independent predictor of higher CSM (HR:3.13, P < 0.001). In sensitivity analyses addressing pN0, LNC cut-off of ≥4 was associated with lower CSM (multivariable hazard ratio [HR]: 0.52; P = 0.002). In sensitivity analyses addressing pN0, no difference was recorded when a LNC cut-off of ≥5 was used (HR:0.60, P = 0.09). In pN1 patients, neither of the cut-offs (≥4 and ≥5) resulted in a statistically significant stratification of CSM rate, and neither reached independent predictor status (all P > 0.05). CONCLUSIONS: Lymphadenectomy provides a prognostic benefit in nmACC patients and identifies pN1 patients with dismal prognosis. Conversely, in pN0 patients, a LNC cut-off ≥4 identifies those with particularly favorable prognosis.