Teaching Case

A 78-year-old female patient was admitted to the emergency department with chest pain, diarrhoea and anorexia. She had no relevant medical history other than cardiovascular risk factors. However, due to the persistence of symptoms, a thoracoabdominal-pelvic CT was performed.

Unenhanced CT revealed a large mass in the left adrenal gland location. It is a heterogeneous lesion, with hypoattenuating areas, calcifications and areas of macroscopic fat (Fig. 1).  In the dynamic CT scan with an acquisition in the venous phase, this lesion exhibits a heterogeneous peripheral enhancement (Fig. 2). With multiplanar reconstructions, the lesion dimensions are assessed as 83x55 mm in the axial plane and 74 mm in the longitudinal axis, demonstrating well-defined margins. It contacts and exerts mass effect on the spleen, left kidney, stomach and pancreas, as well as the splenic and renal vessels, but without apparent signs of invasion of these structures. No enlarged lymph nodes or peritoneal effusion is detected. Not fully characterized by this imaging method, a better characterization with the histological study was suggested.

All laboratory tests were within the normal range indicating a non-functional adrenal tumour.

Adrenal cavernous hemangiomas are extremely rare benign vascular tumours arising from endothelial cells lining the blood vessels. Only approximately 90 cases have been reported in the literature. [1,2]

They are usually discovered incidentally on abdominal imaging. Adrenal hemangiomas generally present in the sixth to seventh decade of life, with a 2: 1 female-to-male predilection. They are unilateral in most cases and tend to be asymptomatic non-functioning tumours. Only a few secreting adrenal hemangiomas have been described in the literature. [2,3]

Ultrasonography is not helpful in differentiating cavernous hemangiomas from other adrenal lesions. Abdominal CT and MRI are valuable diagnostic tools for hemangiomas. [2]

On contrast-enhanced CT, peripheral patchy and centripetal enhancements and highly dense peripheral rim are characteristic for adrenal hemangioma.  Our case showed only thin rim enhancement without centripetal enhancement, although no delayed phase was obtained. This finding is inespecific and can be seen in other adrenal tumours, making diagnosis more difficult. [4] 

The other findings of adrenal hemangioma on CT include a hypodense, heterogeneous lesion with calcifications, as was seen in this patient. Characteristic calcifications appear speckled throughout the entire lesion due to phleboliths in dilated vascular spaces. However, calcifications lack specificity, are also seen in a variety of other adrenal lesions. [5]  There are some cases reported in the literature with small well-demarcated islets of intralesional fat, like our case, but it is a rare finding. [6]

MRI usually shows low signal intensity on T1 weighted images, with possible focal areas of T1 hyperintensity on T1-weighted corresponding to focal haemorrhage. On T2 weighted images, it generally has high signal intensity. These findings can also be seen in other adrenal tumours and are not pathognomonic of adrenal hemangioma. However, observing the combination of these findings can lead to the correct diagnosis. [5]

Adrenalectomy is, usually, recommended for a non-functioning adrenal mass > 4 cm due to concern for adrenal cortical cancer or metastasis. [7]

In this case, the patient underwent a left adrenalectomy, and the histological specimen showed a cavernous hemangioma of the adrenal gland.

In conclusion, despite its rarity, one should be familiar with the diagnosis of adrenal hemangioma and include it in the differential of an adrenal gland lesion when typical imaging findings are present, even if small intralesional islets of fat are found like in this case.

Written informed patient consent for publication has been obtained.