Splenectomy for splenic metastases from malignant adrenal pheochromocytoma: a case report

Splenic metastasis is generally not a common clinical event. However, metastasis to the spleen from adrenal pheochromocytoma is extremely rare and has not been reported in literature. This report presents a case of a 58 year-old male patient who developed spleen-only metastases in July 2007. The patient had a previous history of left epinephroectomy for adrenal pheochromocytoma in January 2003. Abdominal computed tomography demonstrated multiple enhancing lesions suggestive of metastases; thus splenectomy was performed. Pathological examinations confirmed the diagnosis of splenic metastases from pheochromocytoma. The patient was alive without recurrence 48 months after splenectomy. qttis study is the first report on splenic metastasis from previous adrenal pheochromocytoma, and long-term survival was achieved by splenectomy. A history of malignancy indicates a high index of suspicion for splenic metastasis, and long-term survival can be achieved by splenectomy for spleen-only metastasis.

Giant adrenal tumor presenting as Cushing’s syndrome and pheochromocytoma:A case report

We report a case of a 35-year-old lady who presented with Cushingoid features and associated raised urinary metanephrine.The patient underwent open adrenelectomy.Histopathological examination revealed adreno-cortical carcinoma with microscopic lymphovascular invasion.Postoperative period was uneventful and is on follow-up for the last one year and is doing well.

Removal of Dominant Adrenal Lateralized by Glucagon-Stimulated Adrenal Venous Sampling Alleviates Hypertension in Bilateral Pheochromocytoma

In bilateral pbeochromocytoma, localization of the dominant adrenal is challenging but highly important since the removal of dominant side can markedly improve cardiovascular outcomes. To demonstrate the usefulness of glucagon-stimulated BAVS （bilateral adrenal venous sampling） in determining the dominant adrenal to be removed, the authors reviewed records of patients who underwent BAVS with glucagon stimulation from 1997-2010. Nineteen out of 44 patients were diagnosed with bilateral pheochromocytoma. Mean age at diagnosis was 33 ± 14 years. Duration of hypertension was 5 ± 6 years with highest systolic BP （blood pressure） of 186 ±30 mmHg and diastolic BP of 113 ±18 mmHg. Headache （68%） is the most common symptom followed by paroxysmal hypertension （58%）. Majority were taking 〉 3 anti-hypertensive drugs. On glucagon-stimulated BAVS, 63% had right adrenal dominance. Three patients, who were hypertensive for 1, 6 and 12 years, underwent removal of the dominant adrenal. On follow-up （mean period = 36 months）, there was marked improvement in BP control [pre-op vs. post-op： （systolic） 160-240 mmHg vs. 120-150 mmHg; （diastolic） 90-110 mmHg vs. 70-90 mmHg] and reduction in number of anti-hypertensive medications （from 3-5 to 2 classes of drugs）. BAVS with glucagon stimulation is a valuable tool in the identification of the dominant adrenal to be removed in patients with bilateral pheochromocytoma to alleviate chronic hypertension.

Ultrasonographic Changes of Adrenal Glands Size under Trilostane Therapy, Their Correlation with Clinical Signs and Endocrine Tests and Possibility of Introducing Ultrasound as Additional Monitoring Modality of Cushing’s Syndrome

A prospective study of 15 dogs with diagnosed Cushing’s syndrome was made to find a correlation between adrenal size enlargement, clinical signs and results of an ACTH stimulation test. To our knowledge, no study was made trying to correlate changes in adrenal size, response to therapy and ACTH stimulation test results. Ultrasonographic evaluation and an endocrine test (ACTH stimulation test/“pre pill”) were performed in each dog two weeks after initiating the therapy, two to four weeks after changing the dosage of trilostane and ideally 10 weeks after establishing the right dosage of trilostane (two owners showed up later on date due pandemic of COVID-19). The results indicate that there is correlation between adrenal size enlargement and response to trilostane therapy. In this study, all dogs that had increase in adrenal gland size of more than 7% showed as well clinical improvement of HAC (hyperadrenocorticism) signs under trilostane therapy. Further, possible multicentric, studies should be performed to verify assumed correlation between adrenal size enlargement and positive response to trilostane therapy.

Composite pheochromocytoma masquerading as solidpseudopapillary neoplasm of pancreas

Pheochromocytoma and ganglioneuroma form rare composite tumours of the adrenal medulla comprising less than 3% of all sympathoadrenal tumours. We present a case of intraoperatively detected adrenal medullary tumour of composite pheochromocytoma and ganglioneuroma diagnosed on histopathology, in a normotensive patient. A 50-year-old male with a past history of chronic obstructive pulmonary disease presented with abdominal pain and significant weight loss since one month. Ultrasound and contrast-enhanced computed tomography abdomen revealed a large lobulated lesion in the distal body and tail of pancreas suggestive of solid and papillary neoplasm of body and tail of pancreas. Intra-operatively, a 15 cm × 10 cm solid lesion with cystic areas was seen arising from the left lower pole of the adrenal gland pushing the pancreas which appeared unremarkable. In our case, exploratory laparotomy with tumour excision was done. Extensive sectioning and microscopic examination of this adrenal tumour confirmed a diagnosis of composite Pheochromocytoma with Ganglioneuroma on histopathology. Immunophenotyping with S-100 further supported the diagnosis. The goal of this report is to increase the awareness of this rare disease and to further identify its variable presentation.

Spontaneous intraperitoneal bleeding caused by adrenal pheochromocytoma

Ddrenal pheochromocytomas is a rare catecholaminesecreting tumor that originates from chromaffin cellsin the adrenal medulla. The classic triad of pheochromocytoma presentation is episodic headache, sweating, and palpitations. Persistent hypertension is frequently considered part of the presentation. Very rarely, they may present as spontaneous intrapeiritoneal bleeding One case of spontaneous intraperitoneal bleeding caused by adrenal pheochromocytoma is presented.

Overview of Adrenals Tumors in Dakar Hospitals

Introduction: Adrenal tumours (AT) are commonly encountered in clinical practice. For any patient presenting an adrenal mass, there are two crucial points to consider: is the adrenal mass malignant and is it hormonally active? The objective of our study was to evaluate the epidemiological, diagnostic, therapeutic, and evolutionary aspects of AT in a developing country. Methodology: We conducted a retrospective study spanning 17 years (from January 2005 to October 2022) in four Dakar hospital services. We included all patient medical records with explored AT. Results: AT was diagnosed in 35 patients who had a mean age of 36.62 years (range: 12-79), and a female predominance (26 women to 9 men). Among these patients, 17.2 % had incidental AT. Hypertension was the commonest presenting symptom in 27 patients (77%), which was associated with Mnards triad in 14 patients (40%). Abdominal pain was reported by 20 patients (57%), and 14 presented with an abdominal mass. Among the patients, 29 had functional AT: 22 with pheochromocytoma, 5 with primary hyperaldosteronism, and 2 with hypercortisolism. Imaging evaluations revealed that 33 patients had unilateral AT and one had bilateral AT. Tumour sizes varied, 5 patients had tumours 10 cm. Two patients presented with metastases. The therapeutic approach involved adrenalectomy in 32 patients. Perioperative complications were observed in five patients, including haemorrhage (two patients), hypotension (two cases), and hypertensive crisis (one case). One patient with bilateral pheochromocytoma developed acute adrenal insufficiency postoperatively, followed by abdominal herniation. Histopathological examination confirmed pheochromocytoma in 21 patients, Conns adenoma in 5, adrenocortical carcinoma in 3, cortisol adenoma in 2, and adrenal cyst in 1 patient. Non-functional AT included adrenocortical carcinoma (three patients), adrenal cyst (one patient), and pheochromocytoma (two patients). After a one-year follow-up, 29 patients with benign tumours had favourable outcomes, while death occurred within six months of diagnosis in five cases. A patient who had bilateral pheochromocytomas, with complications, died before surgery. Conclusion: In our practice, AT are predominantly pheochromocytomas, being typically diagnosed when they become symptomatic large tumours and, due to this large size, laparotomy is the preferred surgical approach. Unfortunately, the prognosis for malignant tumours is poor.

Human-like adrenal features in Chinese tree shrews revealed by multi-omics analysis of adrenal cell populations and steroid synthesis

The Chinese tree shrew(Tupaia belangeri chinensis)has emerged as a promising model for investigating adrenal steroid synthesis,but it is unclear whether the same cells produce steroid hormones and whether their production is regulated in the same way as in humans.Here,we comprehensively mapped the cell types and pathways of steroid metabolism in the adrenal gland of Chinese tree shrews using single-cell RNA sequencing,spatial transcriptome analysis,mass spectrometry,and immunohistochemistry.We compared the transcriptomes of various adrenal cell types across tree shrews,humans,macaques,and mice.Results showed that tree shrew adrenal glands expressed many of the same key enzymes for steroid synthesis as humans,including CYP11B2,CYP11B1,CYB5A,and CHGA.Biochemical analysis confirmed the production of aldosterone,cortisol,and dehydroepiandrosterone but not dehydroepiandrosterone sulfate in the tree shrew adrenal glands.Furthermore,genes in adrenal cell types in tree shrews were correlated with genetic risk factors for polycystic ovary syndrome,primary aldosteronism,hypertension,and related disorders in humans based on genome-wide association studies.Overall,this study suggests that the adrenal glands of Chinese tree shrews may consist of closely related cell populations with functional similarity to those of the human adrenal gland.Our comprehensive results(publicly available at http://gxmujyzmolab.cn:16245/scAGMap/)should facilitate the advancement of this animal model for the investigation of adrenal gland disorders.

The Diagnosis and Treatment of Virilizing and Fem- inizing Adrenal Syndrome

Objective: To inquire into diagnosis, and treatment of virilizing andfeminizing a-drenal syndrome, differential diagnosis between benign and malignant sex hormoneproducing adrenal neo-plasma and, treatment principles of congenital adrenal hyperplasia (CAH).Methods: Eight cases of CAH and 5 cases of sex hormone producing adrenal neoplasma were admitted tohospital during 1986-1996. The former included 3 rare cases of 17 a hydroxylase deficiency. Thelatter included 3 cases of feminizing adrenal tumor and 2 cases of virilizing adrenal tumor.Results: Weight, size and CT of the tumor, DHEA, 17 -ks, sex hormone levels, infiltration, andmetastasis were closely related to the degree of differentiation of the tumors. Conclusion:Virilizing and feminizing adrenal neoplasm were removed surgically by different incisions. Modifiedsubcostal incision was recommended as the best choice for huge adrenal mass. Corticoadrenal hormonetreatment fa CAH should be individualized according to the different types of the disease. Sexhormones were not suitable for children suffering from 17 hydroxylase deficiency before puberty.

Histological Study on the Adrenal Gland of Zebra

In order to explore the histological structure of zebra and to protect en- dangered species diversity, it is especially important to study the zebra adrenal his- tological structure. 2 zebra adrenals were studied by means of histological methods. The adrenal gland tissues were dissected into serial paraffin sections at the thick- ness of 3 μm. After HE and Masson staining, they were observed and photographed by the light microscope and Image-Pro Plus 6.0. The results show that zebra adrenal capsule is covered with thick connective tissue, which is about 230 μm. A large number of nerves and blood vessels are distributed in the capsule. The area of cortex is about 5 times larger than medullae. Zona glomerulosa is thin, about 300？m. The cells were arranged as column and ball. Zona fasciculata with the thickness of 1 750 μm is 6 times as thick as zona glomerulosa. The cells were ar- ranged in cord. Zona reticular with the thickness of 250 wm was closely adjacent to medulla and formed a jagged boundary. The medulla with the area of 4 mm2 was deeply dyed. It was concluded that some sympathetic ganglion cells were found in the medulla arranged in groups and surrounded with zona reticular cells but lots of the cells were found in zona reticular. Some connective tissue with chromaffin cells was found in the medulla center vein. The purpose of the present study provides reliable evidences for further study on morphology, physiology and pathology of the zebra.

Primary Adrenal Tumors-Five Years Single Centre Experience

Primary adrenal tumors are less commonly encountered in clinic. They may be functional/non-functional and noted incidentally when investigating for other problems. However there is no study available in Asian countries, and especially India regarding the incidence and natural history of these tumors. We carried out a study on adrenal tumors noted in our set-up for last five years to document the prevalence of adrenal tumors, their presentation along with the most common tumor followed by incidence of other tumors and their clinical and pathological presentation. This was a retrospective study in our hospital from June 2006 to 2011. 29 adrenalectomies performed over 5 years. Their clinical presentation and investigations were recorded and analyzed. Adrenal tumors accounted for 0.6% of total surgical specimens, 18 out of 29 belonged to males. Majority (85.2%) of the patients were adults with mean age, 39.8 years. Majority (79.3%) had presented with symptoms, incidentalomas were found in 20.7% patients out of which 2 belonged to potential kidney donors. Nonfunctioning tumors were noted in 58.6% patients. Pheochromocytoma was the commonest tumor noted in 68.9% followed by adrenal cortical adenoma in 13.7%, carcinoma in 6.9% and lipoma, myelolipoma and ganglioneuroma, 3.45% each. All the patients are doing well over a mean follow-up of 2.5 years. To our knowledge this is the first study on incidence and natural history of primary adrenal tumors which are less commonly encountered and usually have good prognosis.

Coexistence of the Hypersecretion of Catecholamine,Adrenal Cortical Nodular Hyperplasia,and Nephrotic-Range Proteinuria of Focal Segmental Scleronephrosis:Is It Fat-Induced Hypertension?

Two patients had a long history of hypertension and one of them also had a nephrotic-range proteinuria secondary to focal segmental scleronephrosis which had been diagnosed by kidney biopsy. They presented with uncontrolled hypertension. Laboratory examination suggested hypersecretion of catecholamines by the left adrenal glands in both patients and primary aldosteronism in one of them. A computed tomography scan revealed small nodules on left adrenal gland in both patients. Patients underwent laparoscopic left adrenalectomy. After surgery, blood pressure was normalized and proteinuria was resolved. Most interestingly, prominent adipocytes infiltrated were detected in the adrenal cortex and were associated with the presence of lymphocytes, which suggested that adipocytes might have a role in the pathogenesis of these diseases in our patients.

Adrenal myelolipoma within myxoid cortical adenoma associated with Conn's syndrome

The coexistence of myelolipoma within adrenal cortical adenoma is extremely rare,for both tumors present usually as separate entities.There are only 16 such cases reported worldwide.To the best of our knowledge,the case we reported here is the first one of myxoid adrenal cortical adenoma associated with myelolipoma reported.A 32-year-old Chinese woman with 4-year history of hypertension was presented in our study.Computed tomography(CT)of the abdomen showed a large heterogene- ously-enhancing mass(4.5 cm in diameter)in the left suprarenal region.Clinical history and laboratory results suggest a metabolic disorder as Conn's syndrome.The patient underwent a left adrenalectomy,and a histopathological study confirmed the mass to be a myxoid adrenal cortical adenoma containing myelolipoma.The patient was postoperatively well and discharged uneventfully.In the present case report,we also discuss the etiology of simultaneous myelolipoma and adrenal adenoma associated with Conn's syndrome,and the methods of the diagnosis and differential diagnosis.

Case of metachronous bilateral isolated adrenal metastasis from colorectal adenocarcinoma and review of the literature

rarely has a solitary, metachronous bilateral adrenal metastasis of colorectal cancer been reported. We depict a 41-year-old man who underwent sigmoid colon cancer radical surgery followed by adjuvant chemotherapy for alocally ulcerative sigmoid adenocarcinoma with metachronous bilateral adrenal metastasis revealed by a computed tomography scan. histopathological examination showed adenocarcinoma, compatible with metastasis from the rectal cancer. the level of serum carcinoembryonic antigen had indicative significance for the presence of adrenal metastasis in the reported series. We performed a literature analysis related to this pathological characteristic and attach importance to consistent, vigilant radiological surveillance of the adrenal glands in the patients' follow up for colorectal cancer with or without subsequent adrenal metastasis.

Expression of Angiotensin Ⅱ Receptors in Aldosterone-producing Adenoma of the Adrenal Gland and Their Clinical Significance

The expression of angiotensin Ⅱ type 1 receptor (AT1R) and angiotensin Ⅱ type 2 receptor (AT2R) in aldosterone-producing adenoma (APA) of the adrenal gland was detected, and their relationship with clinical indexes of APA was analyzed. The mRNA expression of AT1R and AT2R in 50 cases of APA and tissues adjacent to tumors and 12 cases of normal adrenal tissues was detected by using reverse transcriptase polymerase chain reaction (RT-PCR). The expression of AT1R and AT2R proteins in paraffin-embedded slices of tissue was detected by immunohistochemistry. The expression of AT1R in adenoma, tissues adjacent to tumor, and normal tissues of the adrenal gland showed no significant differences. The expression of AT2R in APA tissue was lower than that in normal adrenal gland tissues (P<0.05). Correlation analysis of the mRNA expression level of AT2R and clinical data from patients demonstrated that AT2R expression was negatively related to plasma aldosterone concentration (PAC) (r=-0.467, P<0.05), but positively related with plasma renin activity (PRA) (r=0.604, P<0.05). It is concluded that down-regulation of the AT2R expression is possibly related with the tumorigenesis of APA.

Laparoscopic resection of an adrenal pseudocyst mimicking a retroperitoneal mucinous cystic neoplasm

Adrenal pseudocysts are rare cystic masses that arise within the adrenal gland and are usually non-functional and asymptomatic. Adrenal pseudocysts consist of a fibrous wall without a cellular lining. We report a patient with a 9 cm, left-sided suprarenal cystic mass who presented with abdominal discomfort of 2 years＇ duration. A 38-year-old woman was referred to our service for evaluation of abdominal discomfort and gastrointestinal symptoms. Routine laboratory tests were within normal limits. An abdominal computed tomography scan showed a 9 cm × 8 cm × 8 cm well-defined cystic lesion displacing the left kidney. Magnetic resonance imaging showed a cystic lesion with low signal intensity on the Tl-weighted image and high signal intensity on the T2-weighted image. A laparoscopic left adrenalectomy was performed to diagnose the lesion. The final pathology showed an adrenal pseudocyst without a cellular lining. The patient had no postoperative complications and she was discharged four days after surgery.

Adrenal lymphangioma masquerading as a pancreatic tail cyst

Cystic lymphangiomas of the adrenal gland are rare. A 79-year-old female presented in the emergency room with epigastric discomfort, and an immovable mass was palpated in her abdomen upon physical examination. Imaging studies revealed a large cystic lesion in the pancreatic tail. The radiologic impression ruled out the possibility of a mucinous cystic neoplasm, or a pseudocyst in the pancreas. The operative findings demonstrated that the cystic mass originated in the left adrenal gland. A laparoscopic excision of the cystic mass was performed, and immunohistochemistry confirmed that this mass was a lymphangioma of the adrenal gland. Several prior reports have suggested that lymphangioma can mimic renal or splenic cysts. However, lymphangioma cases mimicking pancreatic cysts are very rare.

Evaluation of Normal Adrenal Gland Volume by 64-slice CT

Objective To measure volume and other parameters of normal adrenal glands in Chinese adults with 64-slice multidetector CT, to evaluate the relationship of volume result with age, sex and body size, and to explore the correlations between adrenal volume and other measurements. Methods This study was based on 125 acquired contrast-enhanced upper abdominal CT scans performed with a 64-slice CT. The final study group consisted of 81 patients (49 males, 32 females). Portal venous phase images were studied for the measurements. Both the reconstruction interval and thickness were 1.5 mm. Each adrenal gland was outlined manually with computer-assistant technology to calculate its volume. The maximal sectional area, length, width and thickness of each adrenal gland were also measured. Results The mean age of total population was 47.9±13.0 (range: 20-76) years. The left, right, and total adrenal gland volumes were 4.23±0.74 (range: 2.85-5.83) cm 3 , 4.26±0.86 (2.59-6.56) cm 3 , and 8.50±1.40 (5.80-11.39) cm 3 , respectively. These volumes increased with weight (r=0.381, 0.389, and 0.437 respectively, all P<0.001), height (r=0.386, P<0.001; r=0.297, P=0.007; r=0.384, P<0.001) and body surface area (r=0.406, 0.392, and 0.452, all P<0.001). There was no significant difference in left, right or total adrenal volume with regard to sex after applying General Linear Model procedure to reduce the impact of weight (F=1.304, 0.064, and 0.597, all P>0.05), nor did volume change significantly with age (r=-0.033, -0.014, and -0.026, all P>0.05). Nearly all descriptors of bilateral adrenal glands correlated with ipsilateral volume except thickness (r=-0.027, P=0.814) and width (r=0.166, P=0.138) in the left side. Among these parameters, length had a stronger correlation with volume than others in the both left (r=0.412, P<0.001) and right (r=0.516, P<0.001) adrenal glands. Conclusion Our study has defined the volume distribution and other parameters of normal adrenal glands in Chinese adults, which provide a baseline for future studies.

Diffuse large B cell lymphoma with bilateral adrenal and hypothalamic involvement: A case report and literature review

BACKGROUND Non-Hodgkin’s lymphoma(NHL)can involve extralymphatic organs,resulting in diverse clinical manifestations,especially if the endocrine organs are affected.This type of involvement can often be difficult to detect accurately.Until now,no patients with NHL and concomitant bilateral adrenal and hypothalamic involvement have been reported.The purpose of this article is to discuss the diagnosis and treatment of lymphoma with bilateral adrenal gland and hypothalamic involvement so as to help physicians avoid misdiagnosis and missed diagnosis.CASE SUMMARY We describe a case of a 52-years-old male patient with bilateral adrenal masses,who presented with a fever of unknown origin on admission.Subsequently,hypopituitarism of the anterior pituitary followed by posterior pituitary developed.18fluorine-fluorodeoxyglucose positron emission tomography/computed tomography(PET/CT)showed lesions with a high metabolism in both adrenal glands,hypothalamus,left supraclavicular lymph nodes,and other organs.The etiological diagnosis was determined based on a left supraclavicular lymph node biopsy.The patient,who eventually present with panhypopituitarism,was finally diagnosed with diffuse large B cell lymphoma with bilateral adrenal gland and hypothalamic involvement.After immunochemotherapy,glucocorticoids administration and desmopressin acetate replacement therapy,the symptoms of fever and panhypopituitarism improved,and all the lesions reduced in size.CONCLUSION This report demonstrates that,although synchronous involvement of two endocrine organs is rare in NHL,extra caution should be taken when dysfunction occurs in multiple endocrine organs.