Lymphangiomatous endothelial cyst of the adrenal gland: A case report

Rekik, Issam; Amine, Mseddi Mohamed; Triki, Mouna; Chaabouni, Ahmed; Bouassida, Mounir

doi:10.1016/j.eucr.2021.101859

Cited by 4 publications

(7 citation statements)

References 3 publications

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“…Adrenal cysts are defined as benign, circumscribed, fluid-containing masses or nodules. They are rare with a reported incidence of 0.06% [ 61 ]. They are usually detected as incidental findings at the time of imaging studies, and account for up to 4% of adrenal masses [ 62 – 65 ].…”

Section: Question 3: What Are the Pathological Correlates Of Adrenal ...mentioning

confidence: 99%

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Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

et al. 2022

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The new WHO classification of adrenal cortical proliferations reflects translational advances in the fields of endocrine pathology, oncology and molecular biology. By adopting a question–answer framework, this review highlights advances in knowledge of histological features, ancillary studies, and associated genetic findings that increase the understanding of the adrenal cortex pathologies that are now reflected in the 2022 WHO classification. The pathological correlates of adrenal cortical proliferations include diffuse adrenal cortical hyperplasia, adrenal cortical nodular disease, adrenal cortical adenomas and adrenal cortical carcinomas. Understanding germline susceptibility and the clonal-neoplastic nature of individual adrenal cortical nodules in primary bilateral macronodular adrenal cortical disease, and recognition of the clonal-neoplastic nature of incidentally discovered non-functional subcentimeter benign adrenal cortical nodules has led to redefining the spectrum of adrenal cortical nodular disease. As a consequence, the most significant nomenclature change in the field of adrenal cortical pathology involves the refined classification of adrenal cortical nodular disease which now includes (a) sporadic nodular adrenocortical disease, (b) bilateral micronodular adrenal cortical disease, and (c) bilateral macronodular adrenal cortical disease (formerly known primary bilateral macronodular adrenal cortical hyperplasia). This group of clinicopathological entities are reflected in functional adrenal cortical pathologies. Aldosterone producing cortical lesions can be unifocal or multifocal, and may be bilateral with no imaging-detected nodule(s). Furthermore, not all grossly or radiologically identified adrenal cortical lesions may be the source of aldosterone excess. For this reason, the new WHO classification endorses the nomenclature of the HISTALDO classification which uses CYP11B2 immunohistochemistry to identify functional sites of aldosterone production to help predict the risk of bilateral disease in primary aldosteronism. Adrenal cortical carcinomas are subtyped based on their morphological features to include conventional, oncocytic, myxoid, and sarcomatoid subtypes. Although the classic histopathologic criteria for diagnosing adrenal cortical carcinomas have not changed, the 2022 WHO classification underscores the diagnostic and prognostic impact of angioinvasion (vascular invasion) in these tumors. Microscopic angioinvasion is defined as tumor cells invading through a vessel wall and forming a thrombus/fibrin-tumor complex or intravascular tumor cells admixed with platelet thrombus/fibrin. In addition to well-established Weiss and modified Weiss scoring systems, the new WHO classification also expands on the use of other multiparameter diagnostic algorithms (reticulin algorithm, Lin–Weiss–Bisceglia system, and Helsinki scoring system) to assist the workup of adrenal cortical neoplasms in adults. Accordingly, conventional carcinomas can be assessed using all multiparameter diagnostic scheme...

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Section: Question 3: What Are the Pathological Correlates Of Adrenal ...mentioning

confidence: 99%

“…7 ). The former is related to lymphatic rather than blood endothelium and occasional intraluminal protrusions can be observed [ 61 , 62 ]. The latter are flat or cuboidal mesothelial cells, devoid of atypia.…”

Section: Question 3: What Are the Pathological Correlates Of Adrenal ...mentioning

confidence: 99%

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

et al. 2022

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“… 6 They are usually unilateral, but in 8%–15% they are bilateral, with equal involvement of both adrenal glands. 6 , 8 , 9 Four main types are distinguished based on histological examination and incidence 1 : Endothelial cysts (45%) of lymphangiomatous or angiomatous origin, pseudocysts, often highly vascularized and hemorrhagic (39%), epithelial cysts (9%), and cysts of parasitic origin, often hydatid (7%). Endothelial cysts are postulated to be formed by dilated and thrombosed vessels with organization.…”

Section: Discussionmentioning

confidence: 99%

“… 9 More rarely, they may be the cause of an acute abdominal pain syndrome due to an intracystic hemorrhage or a state of shock due to retroperitoneal hemorrhage. 1 …”

Section: Discussionmentioning

confidence: 99%

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Endothelial cyst of the adrenal gland: A rare case report

Laasri,

EL Harras,

Izi

et al. 2024

SAGE Open Medical Case Reports

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Adrenal gland cysts are rare and uncommon manifestations. Mostly asymptomatic, discovered incidentally during radiological studies or at autopsy, or without characteristic symptoms. The spectrum of these entities may include benign cysts or malignant cystic neoplasms. They are classified into four types: pseudocysts, endothelial cysts, epithelial cysts, and parasitic cysts. Though pseudocysts are reported to be the most frequently clinically recognized adrenal cysts in surgical series, endothelial cysts are more frequent in autopsy series. Even with advanced imaging modalities, it is still difficult to differentiate a benign adrenal neoplasm from a malignant one. As a result, getting a definitive diagnosis and starting treatment is challenging. In both symptomatic and asymptomatic cases with a large diameter or increasing sizes during follow-up, or with any abnormality of adrenal hormones, surgery is the treatment of choice in symptomatic. Herein, we present a 47-year-old female with a nonfunctional left adrenal endothelial cyst, who was incidentally found during a computerized tomography scan. The patient presented with left-sided nephritic colic due to renal lithiasis. The objective of this paper is to recall the clinical characteristics and to specify the diagnostic contribution of imaging as well as the therapeutic modalities of this entity.

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The diagnostic dilemma of adrenal endothelial tumors: analysis of 21 cases and systematic review of the literature

Coscia,

Ravaioli,

Tucci

et al. 2024

Preprint

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Purpose Adrenal endothelial tumors are mainly represented by adrenal cavernous hemangiomas (ACHs) and adrenal cystic lymphangiomas (ACLs). Their radiological features often overlap with malignant tumors, therefore ruling out malignancy becomes mandatory. We analyzed clinical, radiological, and histopathological data to identify specific characteristics of these tumors. Methods We reviewed 21 patients with ACHs (n = 12), ACLs (n = 8) or adrenal cysts (n = 1) confirmed by histopathology. We selected 82 papers from PubMed to provide a systematic review of the literature. Results In our cohort, median age at diagnosis was 58 years, with sex evenly distributed. All tumors were unilateral (median size = 44 mm), with 8 cases of increasing tumor size. All tumors exhibited non-contrast CT density > 10 Hounsfield Unit (HU). Calcifications were found in 5 cases. Hormonal studies revealed 11 non-functioning tumors and 2 cortisol-secreting tumors. Elevated urinary metanephrines were found in 2 cases. Immunostaining showed CD31/CD34/factor VIII expression in ACHs (n = 5, 24%) and podoplanin expression in ACLs (n = 6, 29%). Literature review revealed 71 reported cases of ACHs and 104 reported cases of ACLs. Median age at diagnosis was 46 years, with a slightly female prevalence (63%). Median tumor size was 48 mm. 84 cases were symptomatic, with life-threatening hemorrhage reported in only 3 patients. Calcifications were found in 23% of cases. Surgical approaches varied, with open and laparoscopic adrenalectomy performed in 55 and 42 patients respectively. Conclusions ACHs and ACLs represent a diagnostic dilemma in clinical practice due to their rarity and their misleading imaging features.

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Lymphangiomatous endothelial cyst of the adrenal gland: A case report

Cited by 4 publications

References 3 publications

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

Endothelial cyst of the adrenal gland: A rare case report

The diagnostic dilemma of adrenal endothelial tumors: analysis of 21 cases and systematic review of the literature

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