Spinal adrenal cortical adenoma associated with Beckwith-Wiedemann syndrome: case report and review of the literature

Giner, Javier Micó; Esteban, I.; Carceller, Fernando; Saceda, Javier; Regojo, R.Ma

doi:10.1007/s00381-017-3397-y

Cited by 5 publications

(2 citation statements)

References 15 publications

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“…This photomicrograph illustrates a sporadic nodular adrenocortical disease The green circle outlines the lesion presentation in para-ovarian tissues [22]. Rare locations of adrenal rests include the placenta [36], broad ligament [22,37,38], retroperitoneum [39,40], urinary bladder and prostate [41], groin [42,43], choroid plexus [44], spine [45][46][47], and lung [48,49]. Among various locations, adrenal cortical choristomas have also been described within TPIT-lineage pituitary neuroendocrine tumors (corticotroph PitNETs) [50,51].…”

Section: Question 2: Why Do Pathologists Need To Know Ectopic or Hete...mentioning

confidence: 99%

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 2: Why Do Pathologists Need To Know Ectopic or Hete...mentioning

confidence: 99%

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

et al. 2022

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“…Although rare, various adrenal gland tumorous conditions (including cytomegaly of the fetal adrenal cortex, benign cyst, hyperplasia, adenoma, adenocarcinoma of the cortex, neuroblastoma, and pheochromocytoma) can develop in BWS patients. There were reports of neoplasms from the heterotopic adrenal cortex located in the liver [7], hilum of the kidney [11] and the spinal cord [12] of BWS patients (Table 2).…”

Section: Discussionmentioning

confidence: 99%

Adrenal Cortical Neoplasm with Uncertain Malignant Potential Arising in the Heterotopic Adrenal Cortex in the Liver of a Patient with Beckwith-Wiedemann Syndrome

Kim

Song

Yoon

et al. 2019

J Pathol Transl Med

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Patients with Beckwith-Wiedemann syndrome (BWS) are predisposed to developing embryonal tumors, with hepatoblastoma being the most common type. Our patient showed hemihypertrophy, macroglossia, and paternal uniparental disomy in chromosome 11 and was diagnosed with BWS. When the patient was 9 months old, a 2.5×1.5 cm oval hypoechoic exophytic mass was detected in the inferior tip of his right liver. Preoperative imaging identified it as hepatoblastoma; however, histologic, immunohistochemistry, and electron microscopic findings were compatible with adrenal cortical neoplasm with uncertain malignant potential. The origin of the adrenal tissue seemed to be heterotopic. Here, we describe for the first time an adrenal cortical neoplasm with uncertain malignant potential arising in the heterotopic adrenal cortex located in the liver of a patient with BWS.

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Ectopia of the adrenal

Saeger

2018

Pathologe

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Ectopia of nodular adrenocortical tissue is very frequently found in the periadrenal region. It corresponds to accessory adrenal tissue and is a normal variant. Ectopia in more distant regions are found in inner male or female genitalia. Strong long-lasting hyperstimultions induce hyperplasia and simulate true tumors ("adrenal rest tumors"). Real autonomic tumors from ectopic adrenal tissue with or without hyperfunction are rare. Ectopia of adrenomedullary tissue are nearly exclusively found in the surroundings of normal medulla. Ectopia in the adrenals corresponds almost exclusively with thyroid tissue. Ectopic secretion of hormones, mostly ACTH, can be found in pheochromocytomas and induces hyperfunction (Cushing's syndrome).

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Spinal adrenal cortical adenoma associated with Beckwith-Wiedemann syndrome: case report and review of the literature

Cited by 5 publications

References 15 publications

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors

Adrenal Cortical Neoplasm with Uncertain Malignant Potential Arising in the Heterotopic Adrenal Cortex in the Liver of a Patient with Beckwith-Wiedemann Syndrome

Ectopia of the adrenal

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