Adrenocortical carcinoma is an uncommon disease found in 0.5–2 per million people, accounting for only 0.02% of all malignant tumors [1-3]. Approximately 40% of adrenocortical carcinomas have been reported to be non-functional [1-3]. In contrast, Cushing’s syndrome is often present in cases of functioning tumors [1], with dehydroepiandrosterone sulfate as a tumor marker. The prognosis of patients with adrenocortical carcinoma is poor, with a 5-year survival rate of 16–35% [8], largely attributable to the fact that approximately 80% of cases are discovered at an advanced stage [9]. In the present case, however, although the tumor was large, no extracapsular invasion or metastasis had occurred and complete resection was accomplished successfully.
CT and MRI are useful modalities for diagnosing adrenocortical carcinoma [10-12]. Tumors ≥5 cm are highly likely to be malignant, and non-uniform imaging effects/necrosis in the center accompanied by calcification have been reported to be a common finding of malignant tumors [10]. Typical adrenocortical cancer presents with low signals on T1 intensity MRI images and non-uniform high signals on T2 intensity images [11,12]. The diagnosis of non-functional adrenocortical carcinomas using adrenal cortex scintigraphy is generally regarded as difficult. However, Weiss criteria are often used to differentiate between malignant adrenocortical tumors and benign tumors pathologically [13,14]. Nine criteria are assessed, including a high nuclear grade, >5 mitoses per 50 HPF, atypical mitotic figures, <25% clear cells, a diffuse architecture, necrosis, venous invasion, sinusoidal invasion, and capsular invasion. The diagnosis in the present case was made based on four criteria: nuclear grade, mitotic index, diffuse architecture, and necrosis.
Surgical resection is the treatment of choice in patients with adrenocortical carcinoma, and survival can be prolonged significantly among patients able to undergo radical excision [1,14]. The steroidogenesis repressor mitotane is often used in cases of advanced disease, or as postoperative adjuvant therapy. Previous reports also found that the administration of postoperative adjuvant therapy following radical surgery prolonged relapse-free survival [15]. However, no universal consensus has been reached regarding the efficacy of mitotane as adjuvant therapy [4,16,17].
Adrenal hemorrhage is an uncommon entity. Although trauma is the most common cause, non-traumatic etiologies have also been reported. Large and progressively growing tumorous lesions, such as adrenocortical carcinomas, pheochromocytomas, metastatic tumors, and myelolipomas, may carry a risk of tearing or rupture, either alone or in combination with hemorrhagic diathesis, stress, or other idiopathic causes [4,16,17]. Massive adrenal hemorrhage remains rare. With respect to its treatment, follow-up is therefore often sufficient if the patient’s systemic condition is stable and findings for tumorous lesions are negative [17]. However, if the patient’s systemic condition is unstable or bleeding from a tumorous lesion is suspected, as in the current case, adrenalectomy, including the hematoma, is required [17,18]. The effective application of transcatheter embolization therapy has been reported, including in the present case, suggesting its potential value in patients with an unstable systemic condition [19-21].
We experienced an unusual case of acute massive adrenal hemorrhage caused by the rupture of a non-functional adrenocortical carcinoma, which was treated successfully with ambulatory transcatheter embolization therapy and elective surgery.