The diagnostic dilemma of benign adrenal endothelial tumors: clinical, radiological, and pathological analysis of 20 rare cases

Kimberly Coscia; Cristina Mosconi; Lorenzo Tucci; Giacomo Colombin; Francesca Donnarumma; Caterina Balacchi; Saverio Selva; Dalmazi Guido Di; Velentina Vicennati; Uberto Pagotto; Leo Antonio De

doi:10.1530/endoabs.99.EP232

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Endocrine Abstracts (2024) 99 EP232 | DOI: 10.1530/endoabs.99.EP232

ECE2024 Eposter Presentations Adrenal and Cardiovascular Endocrinology (155 abstracts)

The diagnostic dilemma of benign adrenal endothelial tumors: clinical, radiological, and pathological analysis of 20 rare cases

Kimberly Coscia ^1,2 , Cristina Mosconi ³ , Lorenzo Tucci ^1,2 , Giacomo Colombin ^1,2 , Francesca Donnarumma ^1,2 , Caterina Balacchi ³ , Saverio Selva ⁴ , Guido Di Dalmazi ^1,2 , Velentina Vicennati ^1,2 , Uberto Pagotto ^1,2 & Antonio De Leo ⁵

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¹Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; ²Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy; ³Radiology Unit, Department of Specialized, Diagnostic and Experimental Medicine (DIMES), Alma Mater Studiorum University of Bologna, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; ⁴Division of Pancreatic Surgery, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; ⁵Pathology Unit - Azienda USL di Bologna, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum University of Bologna, Bologna, Italy

Background: Adrenal endothelial tumors are mainly represented by adrenal cavernous hemangiomas (ACHs) and adrenal cystic lymphangiomas (ACLs). They are rare incidental findings, with about 160 cases of both ACHs and ACLS reported in the literature. They are usually unilateral, benign, non-functioning, and asymptomatic, although abdominal pain and life-threatening retroperitoneal hemorrhage may occur if tumor size increases. Radiological features often overlap with malignant adrenal tumors, therefore ruling out malignancy becomes mandatory. We analyzed clinical, radiological, and histopathological data to identify specific characteristics of adrenal endothelial tumors.

Methods: We conducted a retrospective review of patients admitted to our Department between 2007 and 2022 with ACHs or ACLs confirmed by histopathology. All clinical and radiological data were collected retrospectively from electronic health records.

Results: A total of 20 patients underwent elective adrenalectomy and were diagnosed with adrenal hemangiomas (n=10), adrenal cystic lymphangiomas (n=7), or undefined/combined adrenal cystic lesions (n=3). Six patients had no available information about preoperative workup. More than half of the cases were incidentally discovered (n=13) and only 2 cases were identified correctly by pre-operative imaging studies. Mean age at the diagnosis was 53 years. Sex was evenly distributed (females n=9, males n=11). All tumors were unilateral. Mean preoperative tumor size was 45 mm. An upward trend in tumor size was observed in 7 cases. All tumors showed CT density values on the non-contrast series >10 Hounsfield Units (HU). Calcifications were found in 5 patients. Functional evaluation was performed in 13 patients, whereas 6 patients had no available information about hormone secretion. Hormonal studies revealed non-functioning adrenal masses in 9 cases and autonomous cortisol secretion in 2 patients, assessed by 1 mg dexamethasone suppression test. Elevated urinary metanephrines were found in 2 cases.

Conclusions: ACHs and ACLs represent a diagnostic dilemma in clinical practice due to their rarity and their misleading imaging features. Because of the heterogeneous clinical and radiological pictures, treatment should be targeted to the patient’s characteristics.