Adrenal hemangioma in a 19-year-old female.

Adrenal masses are being detected with increasing frequency due to the widespread use of computed tomography, magnetic resonance imaging and even ultrasonography for the evaluation of diseases with abdominal involvement. It is estimated that adrenal masses are an accidental finding in 1% to 5% of all abdominal CT scans performed. Adrenal hemangiomas are rare and nonfunctioning benign tumors and their differential diagnosis preoperatively is rather challenging. Adrenal hemangiomas are most usually cavernous, unilateral lesions of the adrenal glands; bilateral involvement has been reported twice, which appears between the ages 50 and 70 years, with a 2:1 female-to-male ratio. Approximately 60 surgical cases have been reported in the literature. In general, this tumor is large, and all cases reported were treated with open surgery or retroperitoneoscopic procedure. We report a case of a 19-year-old female patient with adrenal hemangioma that was removed by laparoscopic adrenalectomy.

Adrenal masses are being detected with increasing frequency due to the widespread use of computed tomography (CT), magnetic resonance imaging (MRI) and even ultrasonography for the evaluation of diseases with abdominal involvement. Uncommon adrenal masses include cystic lesions (hydatid cyst, endothelial cyst), solid lesions (hemangioma, ganglioneuroma, angiosarcoma, primary malignant melanoma) and solid fatty lesions (myelolipoma, collision tumor). Adrenal hemangiomas are rare and nonfunctioning benign tumors. To the best of our knowledge, approximately 60 surgical cases have been reported in the literature. In general, this tumor is large, and all cases reported were treated with open surgery or a retroperitoneoscopic procedure.1–8 The first case of adrenal hemangioma resected surgically was reported in 1955.9 We report a case of large adrenal hemangioma that was removed by laparoscopic adrenalectomy.

CASE

A 19-year-old, single, Saudi female was evaluated for adrenal incidentaloma. She was asymptomatic, and physical examination was unremarkable. Her endocrinology tests revealed all parameters to be within the normal range; the results were as follows: aldosterone 379 pmol/L; renin 8.2 pg/mL; adrenaline 22 nmol/24 h; noradrenaline 142 nmol/24 h; metanephrine 0.45 μmol/24 h; normetanephrine 0.78 μmol/24 h; dopamine 2116 nmol/24 h; chromogranin A 83 μg/L; adrenocorticotrophic hormone (ACTH) 3.0 pmol/L; DHEA-S 3.94 μmol/L; 17-OH-progesterone 6.5 nmol/L; serum cortisol (60 minutes after 250 μg ACTH stimulation test) 732 mmol/L. Abdominal ultrasound showed a cystic multiseptated lesion in the right adrenal gland, measuring 6.5×4.2 cm, with evidence of focal calcification (). An intravenous contrast CT scan of the abdomen showed a well-defined lobulated multicystic lesion with internal septation, measuring about 4.3×7.3×5.4 cm in the right adrenal gland, which showed faint marginal enhancement as well as septal enhancement (). MRI showed right adrenal well-defined lobulated mass measuring 5.1×4.3×4.4 cm, multiseptated with spoke wheel appearance (Figures ). She underwent laparoscopic right adrenalectomy with no complications, and postoperative histopathology showed the presence of a hemangioma.

Abdominal ultrasound showed cystic multiseptated lesion in the right adrenal region, measuring 6.5×4.2 cm (white arrow), with evidence of focal calcification.

An intravenous contrast CT scan of the abdomen showed a well-defined lobulated multicystic lesion, with some having internal septation measuring about 4.3×7.3×5.4 cm (white arrow), originating from the right adrenal gland, which shows faint marginal enhancement as well as septal enhancement.

MRI axial T1-weighted FLASH sequence with TR/TE 118/5.2 showed right adrenal well-defined lobulated mass measuring 5.1×4.3× 4.4 cm (white arrow), multiseptated with spoke-wheel appearance.

MRI coronal T2-weighted HASTE sequence with TR/TE 1100/114 showed right adrenal well-defined lobulated mass (white arrow).

DISCUSSION

Adrenal hemangiomas are rare, nonfunctioning benign tumors that can present in young patients. The indication for excision is based on tumor size. Adrenal incidentalomas larger than 6 cm in diameter must be excised because the risk of adrenal cancer is 35% to 98%. For lesions measuring 4 to 6 cm, other imaging features, a history of extra-adrenal malignancy, patient preference, age and comorbidities should be taken into consideration. Adrenalectomy and follow-up with imaging are both acceptable in such cases.10 Most adrenal hemangiomas reported so far have been treated surgically due to their size. Other indications for surgery include mass-effect type symptoms from neighboring organs and complications such as hemorrhage. In our case, the risk in terms of morbidity and mortality of merely watching the incidentaloma was probably not high, but potentially more serious conditions such as carcinoma can occur, and such adrenal masses should be resected. The evolution of radiological imaging resulted in an increased detection rate of adrenal incidentalomas. It is estimated that adrenal masses are an accidental finding in 1% to 5% of all abdominal CT scans performed.

Adrenal hemangiomas are extremely rare, and their differential diagnosis preoperatively is rather challenging. Adrenal hemangiomas are most usually cavernous, unilateral lesions of the adrenal glands; bilateral involvement has been reported twice, which appears between the ages 50 and 70 years, with a 2:1 female-to-male ratio.310–12 Our patient seemed to be the youngest of patients with similar cases that have been reported in the literature. In other cases, tumor size ranged from 2 to 25 cm in diameter, with the majority measuring more than 10 cm, probably because most of these tumors are incidental findings and are usually asymptomatic unless pain is caused by hemorrhage or mechanical mass effects of the tumor on associated structures.13–15 They are most commonly nonfunctioning tumors, with only 3 cases of hormone-secreting adrenal hemangiomas being reported to date.5716 In 2 cases, adrenal hemangiomas presented with spontaneous life-threatening retroperitoneal hemorrhage.1017

Hemangiomas usually involve the adrenal cortex; the tumor is well delimited and encapsulated. Multiple cavities are seen at the periphery, whereas complex modifications involve necrosis, calcifications, fibrosis, thrombosis and hemorrhage. Microscopic analysis reveals that hemangiomas are usually cavernous and rarely capillary.18 Plain radiographs demonstrate calcifications in 64% of cases. The ultrasound appearance is nonspecific, hypoechoic, hyperechoic or of mixed echogenicity. A non-enhanced CT scan showed a hypoattenuating mass with necrotic areas.19 Preoperative angiograms can demonstrate pooling of contrast media within this mass, strongly suggesting a diagnosis of hemangioma. The radiological finding of calcifications, when present, along with the characteristic angiographic appearance of a hemangioma, allows the radiologist to correctly diagnose this benign tumor preoperatively.1 The preoperative diagnosis of adrenal hemangioma is difficult but should be kept in mind as being part of the differential diagnosis of adrenal tumors.