Primary diffuse large B-cell lymphoma of adrenal gland: A case report.

INTRODUCTION: Most adrenal tumors are benign and primary adrenal malignancies are relatively rare. Primary adrenal lymphoma (PAL) is a very rare and highly aggressive malignant tumor with unknown etiology, atypical clinical symptoms, nonspecific imaging manifestations, difficult disease diagnosis and poor prognosis. CASE REPORT: This case report details a 42-year-old woman who was admitted to the hospital with a 1-year-old bilateral adrenal mass and 1-month-old left upper abdominal pain. Enhanced CT of the abdomen showed a right adrenal nodule and a large occupying lesion in the left adrenal region, with a high probability of pheochromocytoma. Intraoperatively, a huge tumor measuring about 12*12*10 cm was found in the left adrenal region, infiltrating the left kidney, spleen and pancreatic tail. Postoperative pathology: lymphocytes were found in the renal capsule and subcapsule, lymphocytes were found in the pancreas; lymphocytes were found in the spleen. Consider a tumor of the lymphohematopoietic system, possibly lymphoma. CONCLUSION: This case demonstrates that primary adrenal diffuse large B-cell lymphoma (PADLBCL) is highly aggressive, has a poor prognosis, is prone to recurrence, has poor therapeutic outcomes, and is difficult to diagnose. Clinicians should consider the possibility of PADLBCL when encountering huge adrenal-occupying lesions and consider chemotherapy before surgery. Reducing the tumor size before surgery is a more favorable therapeutic approach, thus prolonging the patient life and improving the quality of survival.

Surgical versus medical management of patients with primary hyperaldosteronism and indeterminate adrenal vein sampling: A 10-year experience of the Cleveland Clinic.

In patients with primary hyperaldosteronism (PA), adrenal vein sampling (AVS) can identify patients suitable for unilateral adrenalectomy. However, in AVS with an indeterminate aldosterone-to-cortisol lateralization (ACL) ratio of 3.0-4.0, clinical guidance is unclear. The authors screened all patients undergoing AVS at the Cleveland Clinic from October 2010 to January 2021 and identified 18 patients with indeterminate ACL results. Ten underwent adrenalectomy and eight continued medical management. The surgical group was younger (58.5 vs. 68 years, p = .17), and more likely to have a unilateral imaging adrenal abnormality (90% vs. 38%, p = .043) and a lower contralateral suppression index (0.63 vs. 1.1, p = .14). Post-treatment, the surgical group had a significant reduction in diastolic blood pressure (-5.5 mmHg, p = .043) and aldosterone (4.40 vs. 35.80 ng/mL, p = .035) and required fewer anti-hypertensive medications (2 vs. 3, p = .015). These findings may support the benefit of adrenalectomy in a select group of patients with indeterminate ACL.

Brain tissue heterotopic in the adrenal gland in a child: a scarce case report.

Heterotopic brain tissue is rare and has not been reported. Our center made the first report. 4 years and 2 months old Girl presented with a cystic mass in the right adrenal gland 2 weeks after right upper abdominal pain. The operation was successful, and the diagnosis was confirmed by postoperative pathology. 6 months after the procedure, the incision healed well without recurrence. This case report has a detailed diagnosis and treatment process and satisfactory examination results. It can provide a reference for diagnosing and treating clinical HBT and reduce the risk of misdiagnosis and mistreatment.

True mesothelial cyst of the adrenal gland: its clinical profile and management.

The incidence of adrenal cysts is 0.06% and only 9% of these are true mesothelial cysts. Here, we present a case of a true mesothelial cyst together with a review of the literature. A female in her 30s presented to the surgical outpatient department complaining of right flank pain. Her contrast-enhanced CT scan revealed a 7.5×6.5×4.5 cm right adrenal gland cyst. The patient underwent a laparoscopic right adrenalectomy. Immunohistopathology revealed the cyst to be mesothelial in nature. The majority of true mesothelial adrenal cysts are benign, unilateral and more common in women. Any adrenal cyst diagnosed as a functional lesion or one that may be malignant or with a diameter of 5 cm or greater requires surgical care whereas smaller lesions can be managed conservatively. Laparoscopic adrenalectomy for an adrenal cyst of diameter greater than 6 cm is a safe and feasible procedure in expert hands if there is no invasion of surrounding tissue.

Multifocal, Asymmetric Bilateral Primary Aldosteronism Cannot be Excluded by Strong Adrenal Vein Sampling Lateralization: An International Retrospective Cohort Study.

BACKGROUND: Primary aldosteronism (PA) has been broadly dichotomized into unilateral and bilateral forms. Adrenal vein sampling (AVS) lateralization indices (LI) ≥2 to 4 are the standard-of-care to recommend unilateral adrenalectomy for presumed unilateral PA. We aimed to assess the rates and characteristics of residual PA after AVS-guided adrenalectomy. METHODS: We conducted an international, retrospective, cohort study of patients with PA from 7 referral centers who underwent unilateral adrenalectomy based on LI≥4 on baseline and/or cosyntropin-stimulated AVS. Aldosterone synthase (CYP11B2) immunohistochemistry and next generation sequencing were performed on available formalin-fixed paraffin-embedded adrenal tissue. RESULTS: The cohort included 283 patients who underwent AVS-guided adrenalectomy, followed for a median of 326 days postoperatively. Lack of PA cure was observed in 16% of consecutive patients, and in 22 patients with lateralized PA on both baseline and cosyntropin-stimulated AVS. Among patients with residual PA postoperatively, 73% had multiple CYP11B2 positive areas within the resected adrenal tissue (versus 23% in those cured), wherein CACNA1D mutations were most prevalent (63% versus 33% in those cured). In adjusted regression models, independent predictors of postoperative residual PA included Black versus White race (odds ratio, 5.10 [95% CI, 1.45-17.86]), AVS lateralization only at baseline (odds ratio, 8.93 [95% CI 3.00-26.32] versus both at baseline and after cosyntropin stimulation), and CT-AVS disagreement (odds ratio, 2.75 [95% CI, 1.20-6.31]). CONCLUSIONS: Multifocal, asymmetrical bilateral PA is relatively common, and it cannot be excluded by robust AVS lateralization. Long-term postoperative monitoring should be routinely pursued, to identify residual PA and afford timely initiation of targeted medical therapy.

Ectopic adrenal tissue in a cat that underwent an ovariohysterectomy.

OBJECTIVE: To describe the use of ultrasound and adrenal function testing to confirm that excised periovarian tissue is normal ectopic adrenal tissue (EAT). ANIMAL: A 6-month-old female domestic shorthair cat. CLINICAL PRESENTATION, PROGRESSION, AND PROCEDURES: The cat underwent an ovariohysterectomy procedure, during which abnormal tissue was discovered adjacent to both ovaries. The tissue was removed during the ovariohysterectomy and submitted for histopathology, which was consistent with adrenal gland tissue, initially raising concern for an inadvertent adrenalectomy. Abdominal ultrasound and an adrenal function test were performed that confirmed normal adrenal structure and function; thus, the removed structure was diagnosed as EAT. TREATMENT AND OUTCOME: The patient continued to recover from the procedure uneventfully at home. CLINICAL RELEVANCE: Parovarian nodules are an uncommon finding but when observed and biopsied, may be mistaken for physiologic adrenal tissue on the basis of histopathologic features alone without awareness of their existence and clinical context. The use of abdominal ultrasound and ACTH stimulation test offers an affirmative method of differentiating adrenal gland tissue from EAT.

Multiple radiologist review of adrenal CT still frequently misses lateralized surgical primary aldosteronism.

Patients with primary aldosteronism (PA) have increased morbidity and mortality compared to those with essential hypertension. Accurate detection of lateralized PA is important so that affected patients can receive potentially curative adrenalectomy. However, around 40% of patients with lateralized PA have "normal" adrenal glands on computed tomography (CT). Additional independent review of imaging has been shown to improve diagnostic accuracy in many areas of imaging. Therefore, the authors sought to establish if multi-reader re-assessment of previously reported normal CT scans would result in increased detection of surgically remediable disease. The authors found that re-assessment of CT imaging by one, two, or three additional radiologists (or a combination thereof) slightly increased the detection of lateralized disease, but these differences were not statistically significant (p > .05). Readers had low inter-observer agreement (kappa = 0.17). If detection of a discrete nodule on CT was made a prerequisite for adrenal vein sampling (AVS), a second read by another reviewer would still result in an excess of missed cases (84.2%, 36.8%, and 65.8%, respectively, for each of the three independent reviewers). Therefore, a "normal" CT does not preclude the possibility of lateralized PA. Adrenal vein sampling should still be strongly considered wherever available and whenever surgery is considered for treatment of PA, irrespective of CT findings.

Diagnostic consistency between computed tomography and adrenal vein sampling of primary aldosteronism: leading to successful curative outcome after adrenalectomy; a retrospective study.

BACKGROUND: Adrenal computed tomography (CT) is a useful tool for locating adrenal lesion in primary aldosteronism (PA) patients. However, adrenal vein sampling (AVS) is considered as a gold standard for subtype diagnosis of PA. The aim of this study was to investigate the consistency of CT and AVS for the diagnosis of PA subtypes and evaluate the concordance of surgical outcomes. MATERIALS AND METHODS: This retrospective study included 264 PA patients having both CT and AVS. Diagnostic consistency between CT and AVS was accessed, and clinical and biochemical outcomes were evaluated at 6 months after adrenalectomy. RESULTS: Of all, 207 (78%) had a CT unilateral lesion, 31 (12%) CT bilateral lesion, and 26 (10%) CT bilateral normal findings. Among the CT unilateral lesion group, 138 (67%) had ipsilateral AVS lateralization. For CT bilateral lesion and bilateral normal, AVS unilateral lateralization was found in 17 (55%) and 2 (8%), respectively. The consistency between CT lesion and AVS lateralization including CT unilateral with AVS ipsilateral, and CT bilateral lesion with AVS bilateral patients was 63.8% (152/238). Of 77 patients with available data out of 138 patients who underwent adrenalectomy with consistency between CT and AVS, the clinical success rate was 96%, for 17 inconsistency patients out of 22 patients who underwent adrenalectomy, the clinical success rate was 94% after adrenalectomy following the lateralization result of AVS. CONCLUSION: CT is a useful tool to diagnose the adrenal lesion in PA patients. However, AVS is more sufficient to detect the unilateral PA subtype, which could provide curable treatment to surgical candidates of PA such that AVS can identify patients with contralateral PA in CT unilateral lesion and unilateral PA in CT bilateral lesion. The surgical outcome was successful when an adrenalectomy was performed according to the AVS lateralization result.

Success Rate of Adrenal Venous Sampling and its Determining Factors: Experience of a Single Center in Malaysia.

BACKGROUND: Primary hyperaldosteronism has been regarded as the commonest and potentially curable cause of secondary hypertension in up to 80% of cases if this condition is detected early. Laparoscopic adrenalectomy proved to be a promising curable method for primary hyperaldosteronism secondary to aldosterone producing adenoma, while primary hyperaldosteronism secondary to bilateral adrenal hyperplasia requires optimization of medical treatment. Adrenal venous sampling (AVS) has been recommended by the Endocrine Society's guideline in addressing the subtypes of primary hyperaldosteronism. Therefore, determining success rate of AVS in our center is crucial in the management of primary hyperaldosteronism and to prevent redundant procedures. The objectives of this study were to evaluate the success rate of AVS in our center and the associated factors that correlate with the success rate. METHODS: A retrospective study in a single center, all adult patients who were diagnosed with primary hyperaldosteronism and underwent AVS in our center between 2014 until 2022 were included. Successful samples defined by a selectivity index of ≥2, that is, the ratio of adrenal vein cortisol level to the peripheral vein cortisol level, were evaluated. The baseline demographic characteristics of each patient, technical parameters of AVS, and anatomical parameters of right adrenal vein from the venographic images were evaluated and analyzed to correlate with the outcome of AVS. A P-value of <0.05 was considered statistically significant. RESULTS: The overall success rate of AVS in our center was 61.3%. The success rate significantly increased to 80.6% in the trained interventional radiologist (IR) group, which represents a single IR who had completed specific training in AVS, compared to non-trained IR group which consisted of a total of 10 IRs without specific AVS training (P = 0.046). The right AVS had lower success rate than the left AVS (64% vs. 94.6%). The type of right adrenal vein pattern showed significant association with the outcome of AVS (P = 0.014). There were 6 types of right adrenal vein patterns observed in our study; Type 1 - gland-like pattern with numerous branches, Type 2 - delta pattern, Type 3 - triangular pattern with central "blush", Type 4 - no discernible pattern and Type 5 - spidery or stellate pattern. The sixth pattern was the hepatic radicles blush with hepatic drainage, for which blood sampling were also collected due to its appearance was frequently indistinguishable from the Type 1 pattern. The results showed Type 2 pattern had the highest incidence (30.5%; 32/105 samples) and Type 5 had the highest success rate (100%; 11/11 samples), while the hepatic radicles blush pattern had the highest incidence in fail samples resulting in the highest failure rate (94.7%; 18/19 samples). The visualization of the inferior emissary vein (IEV) from the venographic images during right AVS had a significant association with the successful AVS (97.5% success rate; 39/40 samples; P = 0.003). Contrariwise, blood sampling withdrawn from the right adrenal vein in the presence of hepatic drainage communication associated with the failure AVS (86.7% failure rate; 26/30 samples; P = 0.001). CONCLUSIONS: The overall success rate of both AVS in our center was 61.3%, and the success rate was higher in the trained IR group than the non-trained IR group. The visualization of IEV and the type of right adrenal vein patterns were the key determining factors with significant association to the successful right AVS. Conversely, blood sampling withdrawn from the hepatic radicles and hepatic drainage had significant association with the failure right AVS.

Giant left pheochromocytoma with vascular anomalies and pelvic horseshoe kidney: a case report.

BACKGROUND: Pheochromocytoma is a neuroendocrine tumor, and its treatment is dependent on surgical resection. Due to the wide availability of cross-sectional imaging, pheochromocytomas are commonly seen as small tumors less than 10 cm in size and are mostly treated with minimally invasive surgery. Their concomitant presence with horseshoe kidney or other anatomical and vascular anomalies is rare. Herein, we present a surgically complex giant pheochromocytoma case who underwent an open left radical adrenalectomy. CASE PRESENTATION: A 41-year-old Hispanic female presented with a 12 × 8 cm left hypervascular adrenal mass, pelvic horseshoe kidney, and severely dilated large left retro-aortic renal vein which branched into a left adrenal vein, congested left ovarian vein, and left uterine plexus. She was managed with insulin and metformin for uncontrolled diabetes with an A1c level of 9% and doxazosin for persistent hypertension. Clinical diagnosis of pheochromocytoma was confirmed with elevated urine and serum metanephrine and normetanephrine. The pre-operative ACTH was within normal range with a normal dexamethasone suppression test and 24-hour urine free cortisol. The adrenalectomy of the highly aggressive adrenal mass was performed via open approach to obtain adequate surgical exposure. Due to the large size of the tumor and its significant involvement with multiple adjacent structures, coordination with multiple surgical teams and close hemodynamic monitoring by anesthesiology was required for successful patient outcomes including preservation of blood supply to the pelvic horseshoe kidney. The histopathological diagnosis was pheochromocytoma with negative surgical margins. The patient was followed at 1, 4, 12, and 24 weeks postoperatively. She had a normal postoperative eGFR and was able to discontinue antihypertensive and antidiabetic medications at four weeks. She had transient adrenal insufficiency, which resolved at five months. The horseshoe kidney was intact except for a minimal area of hypo-enhancement in the left superior renal moiety due to infarction, which was significantly improved at six months. CONCLUSION: Our patient had a giant pheochromocytoma with anatomical variations complicating an already surgically challenging procedure. Nonetheless, with multiple provider collaboration, detailed pre-operative surgical planning, and meticulous perioperative monitoring, radical resection of the giant pheochromocytoma was safe and feasible with successful postoperative outcomes.

Primary adenomatoid tumor of the adrenal gland: A case report and literature review.

RATIONALE: Adenomatoid tumors are rare benign tumors, mainly involving the reproductive tract, such as the epididymis in men and the uterus and fallopian tubes in women. However, a few cases can occur outside the reproductive tract. Herein, we report a rare case of a primary adenomatoid tumor of the adrenal gland. PATIENT CONCERNS: A 50-year-old man underwent ultrasound examination and was found to have a right adrenal mass without elevated blood pressure, weakness after fatigue, frequent nocturnal urination urgency, pain, or a history of hematuria. The patient's general health was normal. Computed tomography revealed a polycystic mixed-density lesion in the right adrenal region, approximately 7.3 × 4.5 cm in size. DIAGNOSES: Based on the clinical information, morphological features, and immunohistochemistry results, a pathological diagnosis of primary adenomatoid tumor of the adrenal gland was made. INTERVENTION: Excision of the right adrenal gland and tumor through the 11 ribs. OUTCOMES: The patient's postoperative course was uneventful. LESSONS: Preventing misdiagnosis adenomatoid tumors with other types of adrenal gland tumors or metastatic tumors is imperative. Morphological and immunohistochemical features can help diagnose primary adenomatoid tumors of the adrenal gland.

Transvenous Radiofrequency Catheter Ablation for an Aldosterone-Producing Tumor of the Left Adrenal Gland: A First in Human Case Report.

PURPOSE: To describe a novel technique of transvenous radiofrequency catheter ablation of an aldosterone-producing adenoma (APA) of the left adrenal gland using the GOS System (Japan Lifeline, Tokyo, Japan). Using the GOS system, a flexible radiofrequency tip catheter can be inserted into the adrenal central and tributary veins, the drainers for functional tumors. MATERIALS AND METHODS: An APA at the left adrenal gland, which was diagnosed by segmental adrenal venous sampling following administration of 0.25 mg cosyntropin, was ablated using the GOS catheter inserted into adrenal tributary veins via a right femoral vein 7-Fr sheath. The effect of radiofrequency ablation on APA was assessed using the international consensus on surgical outcomes for unilateral primary aldosteronism (PA). RESULTS: No device-related complications were observed. The patient was deeply sedated under blood pressure and heart rate control with continuous administration of ß-blockers. Then, the tumor and surrounding adrenal gland were cauterized at 7000 J two times each in sequence. The output time was 7-11 min for each ablation and 80 min in total. For blood pressure and pulse rate control, esmolol hydrochloride and phentolamine mesylate were used. The contrast enhancement of APA disappeared on dynamic CT immediately after the procedure. PA was biochemically cured until 12 months after the procedure. CONCLUSION: Using the radiofrequency device with the GOS catheter and system is a method for cauterizing adrenal tumors from blood vessels. This approach resulted in a marked reduction in aldosterone concentrations and a complete biochemical cure of PA over the observation period.

Retroperitoneal yolk sac tumour encroaching the liver and adrenal gland with tumour thrombus in cavo-atrial region and hepatic veins.

Paediatric germ cell tumours (GCT) are rare tumours and are unique because of varied clinical presentation and locations. Yolk sac tumour is the predominant malignant histology and a serum marker; alpha fetoprotein is used to see treatment response and recurrent disease. It is extremely rare to find a retroperitoneal GCT with tumour thrombus extending up to the cavo-atrial region with involvement of the hepatic veins. We report a case of retroperitoneal yolk sac tumour (RPYST) with extension to the liver and right adrenal gland along with tumour thrombus in the inferior vena cava and in the right and middle hepatic veins. The child was operated after satisfactory response to chemotherapy. Excision of the tumour along with the right adrenal gland and around 5 cm of retro-hepatic caval resection was done. Inferior vena cava resection was tolerated without reconstruction. Currently child is disease-free and symptom-free at 22 months of follow-up with normal serum marker.

Surgery based on computed tomography images might be feasible for primary aldosteronism patients with visible unilateral adenoma.

Primary aldosteronism (PA) with unilateral adrenal disease can be cured or improved by adrenalectomy. Adrenal venous sampling (AVS) is recommended to identify patients for surgical management. However, surgeries based on computed tomography (CT) images are only advocated for PA patients aged <35 with visible unilateral adenoma. Herein, we aimed to compare CT-based and AVS-based surgery outcomes for PA patients with visible unilateral adenomas for different age groups. A total of 178 PA patients who underwent unilateral adrenalectomy between June 2018 and January 2021 were included in the study based on CT (n = 54) or AVS (n = 124). Demographics, diagnostics, and follow-up data were retrospectively collected. Clinical and biochemical outcomes were analyzed according to Primary Aldosteronism Surgical Outcome (PASO) criteria at 1-year follow-up. Our results showed that complete clinical success (46.3% vs. 47.6%, p = 0.875) and complete biochemical success (88.8% vs. 91.9%, p = 0.515) were similar between the two groups. Age stratification revealed that patients >55 years old were likely to have worse biochemical outcomes; however, these were still not significantly different (21.4% vs. 8.6%, p = 0.220). Of the 114 AVS-based patients who achieved complete biochemical success, 37 (32.4%) with bilateral normal or bilateral abnormal CT images changed treatment options according to AVS results, 1 (0.9%) avoided adrenalectomy on the wrong side. Our results indicated that surgery based on CT images might be feasible for highly selected PA patients with visible unilateral adenomas and less limited by age, while for those with normal adrenal or bilateral adrenal lesions, treatment strategy must be decided by AVS.

It is easy and effective to locate adrenal gland during retroperitoneal laparoscopic left adrenalectomy by the landmark of left PFSV.

To evaluate the feasibility and clinical significance of the left perinephric fat sac vein (PFSV) as an anatomical landmark in locating left adrenal gland (LAD) during retroperitoneal laparoscopic left adrenalectomy (RLLA). In this study, a total of 36 patients who underwent RLLA were enrolled from February 2019 and March 2021. By following a vein vessel on the internal surface of perinephric fat sac (PFS), known as PFSV, LAD was searched finally along the upper edge of this vein. The demographic and clinical characteristics of these patients were acquired, including tumor features and perioperative outcomes (operating time, estimated blood loss, complications). The operations were successfully completed in all the 36 patients without conversion to open surgery. In addition, the LAD was successfully found along the upper edge of PFSV in 34 patients. For all operations, the mean operative time was 75 min (range 60-95) and the estimated blood loss was 20 ml (range 10-50). The median oral intake was 20.7 h (range 6-39). The median hospital stay was 6.3 days (range 4-9), and the median follow-up was 12.3 months (range 9-17). Moreover, no intraoperative complications were observed and no residual tumors were detected after 9 to 15 months follow-up. It may be a safe and efficient procedure to use PFSV as a landmark for searching LAD during RLLA, especially for beginners. However, more studies with larger sample size are need to be conducted to further evaluate the outcomes of this method and the significance of PFSV in searching LAD during RLLA.