Endothelial Cysts Research Articles

FRI230 Cystic Pheochromocytoma

Abstract Disclosure: S. Saberi: None. A 67-year-old woman presented with right abdominal tenderness, abdominal distention, gas and belching. Her blood pressure was 134/70, pulse 84 with no palpable abdominal mass. Her medical history includes depression and anxiety which developed 17 years prior and later resolved. She was diagnosed with hypertension approximately 20 years earlier initially well-controlled with candesartan-hydrochlorothiazide 16-12.5 mg daily and metoprolol succinate ER 50 mg daily. Two years prior to presentation her blood pressure rose to the 180s/90s requiring doubling of her candesartan-hydrocholorothiazide. An abdominal ultrasound was obtained which showed an abdominal mass. Subsequently, a CT of the abdomen and MRI of the abdomen with and without contrast were obtained revealing a 10 cm predominantly cystic adrenal mass with partial irregular wall thickening and nodularity causing mass effect on the inferior vena cava, liver and right kidney. Results of lab testing revealed an elevated plasma free metanephrines 1187 pg/ml (normal < 57 pg/ml), plasma free normetanephrines 1911 pg/ml (normal < 148 pg/ml), total free metanephrines and normetanephrines 3098 pg/ml (normal < 205 pg/ml). Her other adrenal biochemical evaluation was normal. She underwent open right adrenalectomy after adequate pre-operative blockade. Post-operatively her plasma free normetanephrines decreased to 0.47 nmol/L (normal < 0.90 nmol/L) and plasma free metanephrines were less than 0.20 nmol/L (normal < 0.50 nmol/L). Cystic Pheochromoctyoma: Spontaneous rupture of cystic pheochromocytoma can cause an adrenergic crisis. Cystic pheochromocytoma may develop due to hemorrhage within the lesion, necrosis and subsequent cyst formation (1). Patients may present with abdominal pain, GI symptoms or palpable masses rather than classic symptoms of pheochromocytoma (2).The differential diagnosis of cystic adrenal lesions includes lymphangiomas, pseudocysts of other surrounding structures (such as liver, pancreas, kidney, spleen, retroperitoneal and urachal and mesenteric cysts), dermoids, adrenal carcinoma, hydatid cysts of the adrenal glands (3).Small case series report the incidence of adrenal cysts in adrenal disease as 5.1% (4). A female predominance has been reported in prior studies (4). More commonly cystic adrenal lesions are unilateral and present in persons aged 30s-50s (2). Adrenal cysts are classified based on their origin as endothelial cysts derived from malformation of the lymphatic or arteriovenous systems, pseudocysts derived from prior hemorrhage, epithelial cysts with thin wall and serous fluid, or parasitic cysts. Necrosis and liquefaction in the adrenal lesion may lead to fluid accumulation and a cystic appearance. Large cysts are at risk for hemorrhage and rupture. All adrenal cysts require biochemical evaluation. Particular attention should be paid to predominantly cystic masses with any solid component as up to 7% of these may be malignant (5). Uniform guidelines for surgical management of these lesions have not been well established. Patients with symptoms such as abdominal pain, biochemical abnormalities, complications such as pressure on adjacent organs or rupture with cyst content leakage, suspicion of malignancy, cysts larger than 5 cm or increasing size of the cyst should be considered for surgical intervention (5, 6). In patients presenting with abdominal pain and hypertension who are found to have a cystic adrenal lesion pheochromocytoma should be considered. Presentation: Friday, June 16, 2023

Adrenal Hemangiomatous Endothelial Cyst That was Difficult to Differentiate from Adrenal Malignant Tumor: A Case Report

A 65-year-old woman was referred to our hospital for fever and diagnosed with pyelonephritis. Abdominal computed tomography showed a right adrenal tumor incidentally, that was 6.5 cm in diameter. We could not rule out malignant disease by magnetic resonance imaging examination and performed resection of the right adrenal tumor. The histopathological examination revealed an adrenal hemangiomatous endothelial cyst, and there was no evidence of malignancy. It was difficult to differentiate between adrenal cyst and adrenal cancer in preoperative diagnostic imaging because the tumor contained hemorrhage and necrotic tissue.

A case of Adrenal Endothelial Cyst in a young female

Adrenal cysts are rare, often asymptomatic and non-functional. Here, we discuss the case of a 24-year-old young female who had been experiencing unexplained abdominal heaviness and pain for 6 months’ duration. An almost 5 cm anechoic lesion without a septum was detected by abdominal sonography across the left abdominal region. A 4.8x4.2x4.8cm suprarenal cyst was discovered on abdominal computed tomography (CT) scan displacing the left kidney inferiorly and anteriorly. There was no evidence of hormonal hypersecretion clinically or biochemically. She underwent successful laparoscopic left adrenalectomy, revealing a histopathological diagnosis of an endothelial cyst without any features of malignancy. The postoperative course was uneventful without recurrence during the period of follow-up. Current literature on the management of adrenal cysts is sparse although, personalized management with multidisciplinary team involvement is paramount importan

Adrenal cysts: an emerging condition.

Adrenal cysts are rare lesions representing approximately 1-2% of adrenal incidentalomas. The majority of these rare lesions are benign. Rarely, phaeochromocytomas and adrenal malignant masses can present as cystic lesions and can occasionally be difficult to distinguish from benign cysts. Histologically, adrenal cysts are subdivided into pseudocysts, endothelial cysts, epithelial cysts and parasitic cysts. The radiological appearance of an adrenal cyst is generally similar to that of cysts in the kidney. They are thus well demarcated, usually rounded, with a thin wall and homogenous internal structure, low attenuating (<20 Hounsfield Units) on CT, low signalling on T1-weighted MRI sequences and high signalling on T2-weighted MRI sequences, and anechoic or hypoechoic on ultrasonography. Benign adrenal cysts have a slight female predominance and are usually diagnosed between the ages of 40 and 60. Most adrenal cysts are asymptomatic and are detected incidentally, although very large adrenal cysts can lead to mass effect symptoms, with surgery required to alleviate the symptoms. Thus, conservative management is usually recommended for asymptomatic cysts. However, when uncertainty exists regarding the benign nature of the cyst, additional work-up or follow-up is needed. The management of an adrenal cyst should preferably be discussed at an adrenal multidisciplinary team meeting.

A Case Report of Giant Adrenal Cyst with Spontaneous Rupture and Hemorrhage

A case of huge adrenal cyst with spontaneous rupture and hemorrhage was reported. A 27-year-old female patient came to our hospital in emergency department due to "right upper abdomen and right low back pain and discomfort for 2 days". Whole abdominal enhanced CT revealed a right adrenal cyst with rupture and hemorrhage. An emergency exploratory laparotomy revealed a huge right retroperitoneal hematoma with a size of 25.0cmx20.0cmx18.0cm. Combined with pathology, adrenal endothelial cyst with bleeding was considered. To investigate the follow-up, diagnosis and treatment of giant adrenal cyst based on the literature.

Malignant psammomatous melanotic schwannoma mimicking adrenal cyst: case report

BackgroundMelanotic schwannoma is a melanin producing nerve sheath tumors. Rarely, it can be associated with psammoma bodies, called psammomatous melanotic schwannoma. Psammomatous melanotic schwannomas are associated in up to 10% of the cases with Carney’s syndrome. The rarity of the lesion, which may present at different localizations create difficulty in placing a correct initial diagnosis. Definitive diagnosis is made after complete tumor excision and pathomorphological evaluation. The prognosis depends on the anatomical localization, local invasion and presence of a high mitotic index. The main pathomorphological differential diagnosis includes schwannomas and other melanin producing tumors as melanoma.Case presentationWe present a case of an 11-year-old female with cystic lesion adjacent to right adrenal gland, mimicking adrenal cyst. Ultrasound guided biopsy was undertaken due to the cystic appearance of the formation and the lack of certain diagnosis from the non-invasive diagnostic tests. No signs of cellular and nuclear atypism were observed. The diagnosis of benign endothelial cyst with spontaneous hemorrhage was suggested. The patient underwent transabdominal laparoscopic adrenalectomy en-bloc with the cyst to prevent spillage of the cyst content due to the intimate adhesion of the lesion to the adrenal gland and vena cava inferior. Pathomorphological examination revealed malignant psammomatous melanotic schwannoma. The adrenal gland was intact with no tumor infiltration. The patient was followed up on the 1st and 2nd month afterwards the surgery by MRI with no signs of local recurrence and postoperative complications.ConclusionPsammomatous melanotic schwannoma near adrenal gland are rare and present difficulty with exact preoperative diagnosis. Complete resection should always be provided. Laparoscopic surgery is feasible if radical excision is not compromised. Long-term follow-up and Carney’s syndrome surveillance after complete excision are recommended especially in young patients.

A 15-Year Change of an Adrenal Endothelial Cyst

Patient: Male, 61-year-old Final Diagnosis: Adrenal endothelial cyst Symptoms: No obvious self-conscious symptoms Medication:— Clinical Procedure: Laparoscopic adenectomy Specialty: Urology Objective: Rare disease Background:Adrenal gland cysts are rare and often occur without any symptoms. Even with advanced imaging modalities, it is still difficult to differentiate a benign adrenal neoplasm from a malignant one. Therefore, it is difficult to arrive at a definitive diagnosis and provide treatment.Case Report:We describe a patient with asymptomatic adrenal incidentaloma. The patient was lost to follow-up until 7 years later. On resuming follow-up, an enlarged suprarenal tumor was noted on ultrasound imaging. Magnetic resonance imaging revealed a 6×4 cm tumor mass, and the peripheral part expressed progressive enhancement on dynamic contrast-enhanced images. Laboratory data showed slight hypokalemia, and a complete endocrine assessment was performed, which showed no abnormality. Because malignancy of the adrenal gland remained suspected, a laparoscopic adenectomy was performed. The pathological result showed an adrenal endothelial (vascular) cyst with the formation of thrombi and calcification, without any evidence of malignancy.Conclusions:Adrenal cystic lesions can change with time. Routine imaging studies during follow-up are recommended, and endocrine evaluations should be performed as an initial adrenal tumor work-up. Surgery is the treatment of choice when the cyst is >6 cm in size, malignancy is suspected, or abnormal endocrine activity is present.

Adrenal cysts of lymphatic origin: A clinical and pathological study of six cases and systematic literature review

Adrenal cysts are rare, benign, and usually asymptomatic, being detected as an incidental finding on imaging methods. Adrenal Cysts of Lymphatic Origin (ACLO) and Adrenal Lymphangiomas (AL) are types of endothelial cyst and are the most prevalent subtype in this series. This study aims to present a single institutional experience of these rare cysts and compare their features with those found in the review of existing literature on ACLO and AL. Overall, thirteen cases of adrenal cysts were diagnosed and surgically excised during the study period, onto which we performed immunohistochemistry using a panel of antibodies (CD31, CD34, Pan Cytokeratin AE-1/AE-3, Factor VII, D2–40, and ERG). Four cases of ACLO and two AL were found. The lesions predominantly affected right adrenal, and the majority of patients were middle-age females, of Caucasian ethnicity, and asymptomatic. In our literature review, we found 108 cases of ACLO/AL from 57 articles with similar sex and age distribution. The diagnosis and subclassification of adrenal cysts are challenging, and there is a significant overlapping between the definition of ACLO and AL.

A Cyst-ematic Analysis of the Adrenal Gland: A Compilation of Primary Cystic Lesions From Our Institution and Review of the Literature.

Although primary adrenal lesions and metastases are most commonly encountered, de novo cysts of the adrenal gland can occasionally occur. We analyzed the pathology archives at the Hospital of the University of Pennsylvania for diagnoses of cystic lesions of the adrenal gland between 1998 and 2020. Patient demographics and clinical information were extracted, and pathology slides and stains, as appropriate, were analyzed. The literature between 1998 and 2020 was also searched for large case series involving cystic adrenal lesions. We found 18 cystic lesions of the adrenal gland in our archives, categorized as pseudocysts (7), endothelial cysts (5), mesothelial cysts (2), mature teratoma (1), cystic adrenal cortical adenoma (1), cystic pheochromocytoma (1), and cystic metastasis (1). The mean age at time of diagnosis was 52.3 years, and the mean size of the cysts was 6.0 cm. There was a slight female predilection, and cysts were more likely to be found on the left side. All lesions were incidental findings. The cystic metastasis was clear cell renal cell carcinoma. Our findings were also compared with those found in the literature published within the same timeframe. Adrenal cysts are rare entities. Our findings are concordant with other large studies and provide additional cases to the existing literature. In addition, our literature search expands the possible differential diagnoses for cystic lesions arising in the adrenal gland.

Coincidence of Large Adrenal Cyst and Prominent Hyporeninemic Hyperaldosteronism

A 67-year-old Japanese woman who had end-stage renal disease was referred to our hospital for kidney transplantation. Abdominal CT revealed a large adrenal mass with inhomogeneity. She had a history of hospitalization for stroke and heart failure and exhibited prominent hyporeninemic hyperaldosteronism. Histological examination of the resected tumor with anti-CYP11B2 antibody indicated that she had a vascular endothelial cyst with primary aldosteronism (PA) due to multiple adrenocortical micronodules. This report implicates the pathological interaction between adrenal vascular cysts and PA-mediated vascular damage of the adrenal vein.

Endothelial Cyst of the Left Adrenal Gland in Female Patient Presented as a Cystic Tumor of the Pancreas: A Case Report

Large adrenal gland cysts represent a rare clinical entity than can be misdiagnosed as pancreatic cystic tumor and eventually change the preoperative plan of the surgeon. Their sex predomination is with female to male ratio of 3:1, occurring mostly in middle aged women and are mostly always unilateral and left-sided in 55% of cases. The treatment for cysts larger than 5 cm in diameter is surgical regardless of the symptoms, due to their potential for malignancy. We present a case of left adrenal gland endothelial cyst primarily presented on CT scan as a cystic tumor of the pancreatic tale. Therefore, even when the proper preoperative imaging tools are used, the diagnosis is not certain and the preoperative plan is changed during the surgery. Keywords: adrenal gland, endothelial cyst. DOI: 10.7176/JHMN/85-06 Publication date: January 31 st 2021

A Case of an Adrenal Hemangiomatous Endothelial Cyst Presented with Anemia

A Case of an Adrenal Hemangiomatous Endothelial Cyst Presented with Anemia

Adrenal endothelial cyst associated with adrenocortical adenoma: a case report and literature review

Abstract We present a case of a middle-aged male Chinese patient who was asymptomatic with a large (6 × 7 cm) right adrenal mass was found in this patient upon routine health examination. He underwent laparoscopic right adrenalectomy after comprehensive evaluation, and the mass was finally diagnosed as right adrenal endothelial (vasculature) cyst associated with adrenocortical adenoma according to pathological and immunohistochemical studies. The puzzling image resemblance of a variation of adrenal cyst to carcinoma necessitated histological examination for confirmative diagnosis. The development of endothelial cyst is extremely rare, and its association with other adrenal neoplasms is even rarer. Herein, we report a new case of adrenal endothelial cyst associated with adrenocortical adenoma, which was almost indistinguishable from adrenocortical carcinoma, and hope that it would be helpful in the diagnosis of other similar cases.

Adrenokortikal adenomda myelolipomatöz değişiklikler

Myelolipoma is a rare benign tumor which is generally detected incidentally. The tumor consists of mature fat tissue and hematopoietic cells that resemble bone marrow. The coexistence of myelolipoma and non-functional adrenocortical adenoma in the same gland is exceedingly rare. We herein present two cases of adrenal myelolipoma in association with non-functional adrenocortical adenoma in left adrenal gland of a 62-year-old woman and non-functional adrenocortical adenoma combined with myelolipoma and endothelial cyst, in right adrenal gland of a 73-years-old man. Microscopically, in the central of the tumor, there was a myelolipomatous area composed of adipocytes and hematopoietic cells surrounded by sheets of adenoma cells. The histopathological diagnosis was “myelolipomatous changes within a non-functional adrenocortical adenoma”. The cases are presented here with their rarity and pathological differential diagnosis.

Inside of an adrenal cyst

This is a 36-year old menstruating female who accused abdominal pain of non-specific pattern during the last days. Abdominal ultrasound and computer tomography confirmed a left adrenal mass of 6 centimetres with cystic appearance without solid elements. No other causes of abdominal pain were identified. Adrenalectomy was performed. The pre- and post-operatory panel of endocrine assessments was normal. Pathological report confirmed an endothelial cyst at the level of a normal adrenal gland. The endothelial cells are introduced in hematoxylin eosin stain at histological report (Figure I: 4X, Figure II: 40X). The immunochemistry reaction is positive for factor VIII in cyst cells of adrenal origin. (Figure III: FVIII positive immunostain in fused and cubic cells, 20X, Figure IV: FVIII in fused cells, 20X). Further on the clinical evolution was good. Adrenal cysts may be endothelial related, epithelial derived and pseudocysts. They are found in both adults and children but the level of evidence varies from cases series to cases reports. (1,2,3) The risk of malignancy or rupture are the major complications. (1) Endothelial cysts seem more frequent in women. (2) The scenario of detection may be of a typical adrenal incidentaloma or starting from non-specific local as seen here.

Benign vascular tumors, cysts, and pseudocysts of the adrenal gland: a contemporary multi-institutional clinicopathological analysis of 55 cases

Benign adrenal vascular tumors, cysts, and pseudocysts are a heterogeneous group of relatively uncommon entities that may pose diagnostic challenges radiologically and pathologically. However, there are only a few small cases series systematically characterizing the clinicopathological features of these lesions. We identified 55 cases of benign adrenal vascular tumors, cysts, and pseudocysts (23 pseudocysts, 17 hemangiomas, 8 lymphangiomas, 6 angiomatous endothelial cysts, and 1 arteriovenous malformation) from a multi-institutional urologic pathology database between 2000 and 2017 and retrospectively analyzed their clinicopathological features. We found that these lesions have a female predominance and most are right sided. These lesions may occur simultaneously with other adrenal tumors associated with hormonal hypersecretion. A substantial portion of pseudocysts were semisolid or solid with no fluid collection, mimicking a solid adrenal tumor and resulting in adrenalectomy. In addition, a small proportion of benign vascular lesions may have coexisting epithelial tumors, requiring extensive specimen sampling and thorough microscopic examination.

Spinal Dermal Sinus and Sacral Endothelial Cyst with Recurrent Bacterial Meningitis: A Case Report and Systematic Review of the Literature

Spinal Dermal Sinus and Sacral Endothelial Cyst with Recurrent Bacterial Meningitis: A Case Report and Systematic Review of the Literature

Adrenal cysts-a report of four cases including one with bilateral involvement

Adrenal cystic lesions are rare entities. Four types of cystic lesions of the adrenal gland have been described in literature namely, epithelial cysts, endothelial cysts, pseudocysts and parasitic cysts. Tumors of the adrenal gland, both benign and malignant may also show cystic change and have to be considered in the differential diagnosis of cystic lesions of the adrenal gland. Lymphangioendotheliomas, though common at other sites, are unusual in the adrenal glands and bilateral involvement is extremely rare. We present four cases of adrenal cysts, two cases of lymphangioendothelial cysts and two cases of pseudocysts from 108 adrenalectomy specimens received over 5 years. Two of these had hypertension which resolved after surgery. All of our cases did well postoperatively. Prognosis of these benign cysts is excellent and the patients are disease-free after surgery.

Vascular Cyst of Adrenal: A Report of Two Cases

Vascular cysts of the adrenal gland are rare heterogeneous group of lesions with an autopsy incidence of 0.06%. They have female preponderance with most common presentation being pain abdomen. Cysts of the adrenal glands are classified as (a) parasitic cysts (b) epithelial cyst (c) pseudocyst and (d) endothelial cysts. Out of these endothelial cysts constitute about 84% of the cases. As the clinical features and radiological findings of these lesions are nonspecific, a proper histological examination along with immunohistochemistry are necessary for a definitive diagnosis. Here, we report two cases of vascular endothelial cyst of the adrenal gland to emphasize the histomorphological and immunohistochemical features of these lesions.

Non-Tumor Cystic Lesions of the Adrenal Gland.

Adrenal cystic lesions are uncommon but due to the improved radiologic imaging techniques their appearance seems to increase. We analyzed the clinical and radiological findings of 10 patients with adrenal cysts and the pathological features of the operative material. Standard dissection procedure and paraffin embedded tissue sections were made, stained by HE and immunohistochemically with CD34, CD 31, Factor 8, Podoplanin, CKWS and AE1/AE3 RESULTS: The mean age of the patients was 40.6 years; female to male ratio was 2.3:1. All the cysts were diagnosed as cystic lesions radiologically except one. The most present clinical symptom was abdominal pain. The diameter of the cysts measured from 2 to 7 cm. Four of the cysts were diagnosed as pseudocysts and six as endothelial. Six cysts were lined by CD34(+) and CD31(+) cells, four were lined by Factor 8(+) and podoplanin(+) cells and four had no lining. Endothelial cysts were more common cysts in our study and the immunohistochemical results suggested common vascular origin to all endothelial cysts and supported additional separation of angiomatous and lymphangiomathous adrenal vascular cysts.