Rare Case of Adrenal Hemangioma Discovered Incidentally during Renal Colic Investigation

Introduction

Adrenal incidentalomas were first described by Geelhoed and Druy in 1982 in the context of an incidentally discovered adrenal mass in a patient without evidence of adrenal hormone excess or malignancy [1]. For a considerable period before the advent and widespread availability of cross-sectional imaging, adrenal tumors were regarded as a rare occurrence that often went undetected except in cases in which adjacent organs were compressed or manifested as overt signs of hormone excess. From 1995 to 2017, the standardized incidence rate of adrenal tumors increased tenfold, from 4.4% to 47.8% [2].

Hemangiomas of the adrenal gland are relatively rare in clinical settings [3]. The vast majority of adrenal hemangiomas are asymptomatic and are often discovered incidentally during a routine radiographic examination, while managing a different condition. Therefore, adrenal hemangiomas in clinical practice present a significant diagnostic challenge. In 1955, Johnson and Jeppesen reported the first case of adrenal hemangioma that was surgically excised and incidentally found while investigating a case of severe hypertension [4]. Since then, a limited number of studies have reported this clinical entity.

Herein, we report a rare incidental tumor with the description of the clinical presentation, radiological features, laboratory findings, and surgical approach of a patient with a right adrenal hemangioma, in addition to a brief review of the relevant literature. The following case report has been prepared in accordance with the CAse REports (CARE) reporting guidelines.

Case Report

A 39-year-old man presented to the Emergency Department with concerns of right flank pain in November 2021. The pain began 1 week prior to presentation and was characterized by sharp, non-radiating pain with an insidious onset and intermittent occurrence sporadically throughout the day, with no discernible pattern or triggers and no association with any symptoms. However, the present episode was significantly more intense and disruptive, prompting the patient to seek medical attention. His medical and surgical history was unremarkable, his social history was non-contributory, and his family history was devoid of any relevant conditions.

A thorough review of the systems did not reveal any noteworthy findings. On examination, the patient was alert, oriented, hydrated, and afebrile, and his vital signs were within normal limits. Local abdominal examination revealed a soft, non-tender abdomen with mild discomfort on deep palpation of the right costovertebral angle but no guarding or rebound tenderness and no palpable masses. The remainder of the physical examinations were unremarkable. Due to a high suspicion of renal stones, an unenhanced computed tomography (CT) scan of the abdomen utilizing the kidney, ureter, and bladder protocol was conducted. The CT scan confirmed the presence of a 0.3-cm non-obstructive stone located at the right vesicoureteric junction, with no hydronephrosis or perinephric fat stranding bilaterally. Notably, the CT scan also identified an incidental adrenal mass, a 4.56×4.24×3.9-cm well-demarcated right-sided adrenal lesion with internal calcification and macroscopic fat, as shown in Figure 1. This finding was initially interpreted as myelolipoma by the radiologist, and for further investigation, the patient was referred to our endocrine surgery clinic.

The patient was routinely followed up with urinalysis, urine culture, electrolytes panel, urea, and creatinine levels, along with serial CT scanning for further characterization over the course of 2 years. A workup was conducted to determine the functionality of the adrenal mass by testing blood and urine samples. Tables 1 and 2 provide the serum chemistry values, complete blood count, and preoperative adrenal hormone evaluation. The patient had an average complete blood count, serum electrolytes (Na+, K+, Cl−), and urine analysis throughout the follow-up period. The dexamethasone suppression test demonstrated a suppressed cortisol response, indicating a nonfunctional adrenal mass. A follow-up CT scan, 10 months after the initial presentation, in August 2022, illustrated a heterogeneous, enhancing mass originating from the lateral limb of the right adrenal gland. The mass exhibited considerable growth, compared with the baseline scan, measuring 5×4.7×4.2 cm (Figure 2). A subsequent CT scan performed in August 2023, at 2 years after the initial presentation, documented further enlargement, with the mass measuring 5.16×4.83×4.6 cm. As shown in Figure 3, the mass was relatively stable in appearance, with hyperdense soft tissue component, fat, and foci of calcification. Figure 4 shows a heterogeneous mass with various enhancement patterns in the triphasic CT scan of the abdomen. A decision was made to perform a laparoscopic right adrenalectomy, owing to the benign nature of the preoperative diagnosis of myelolipoma and the size of the mass. The decision to delay surgical intervention 2 years following presentation was made following careful consideration of the patient’s wishes. After the initial CT scan revealed growth, the patient expressed reluctance to undergo surgery. Given this, a conservative approach of monitoring with a follow-up CT scan after 1 year was implemented, which allowed the patient time to adjust to the diagnosis and consider his options. The subsequent scan demonstrated further enlargement, prompting the renewed recommendation for surgery, which the patient ultimately accepted.

Intraoperatively, the patient was positioned in the right lateral decubitus position. A 10-mm port was placed midline at the subcostal margin, and another 10-mm port laterally to the umbilicus. Two 5-mm ports were added, one in the epigastric quadrant and one in the right lower quadrant. The triangular ligament of the liver was mobilized, and the right hepatic lobe was rotated and elevated. Dissection of the right border of the inferior vena cava commenced by incising the overlying peritoneal reflection with a hook and harmonic device, extending from the diaphragm to the suprarenal area, while ensuring right hepatic vein integrity. In the right suprarenal space, retroperitoneal fat was elevated off its superior attachment to the diaphragm. The adrenal gland was visualized as medial and superior to the kidney. Due to the nature of the mass, we encountered bleeding during the manipulation of the mass, which was controlled by a harmonic device. Posterior dissection using the harmonic device ensured the complete removal of the adrenal gland and surrounding suprarenal tissue, reaching the quadratus lumborum. Following the operation, the patient experienced an uneventful recovery, with no perioperative complications, and was discharged discharge home in a good condition, with a follow-up appointment.

Histopathological studies reported that the resected mass was composed of an ovoid piece of brown-tan tissue measuring 5×4×4 cm in size and weighing 30 g, with an attached fibro-fatty tissue measuring 6 cm in depth. Serial sectioning revealed a relatively encapsulated lesion with heterogeneous solid and cystic surfaces. Light microscopy examination showed predominantly dilated and congested vascular channels lined by flattened endothelium. Focal mature adipose tissue was seen. No extramedullary trilineage hematopoietic cells, myoid spindle cells, or thick-walled hyalinized blood vessels were seen. The pathologist determined that the findings were consistent with adrenal hemangioma. The hematoxylin and eosin stain of the specimen is shown in Figure 5.

The timeline for the presented case is summarized in Figure 6.

Discussion

Hemangiomas of the adrenal gland are rare benign non-functional tumors that arise from the gland’s vascular endothelium. In the present case report, we aim to provide valuable insight into the presentation of this unusual clinical entity. To the best of our knowledge, based on an extensive review of the literature, between 1955 and 2024, a total of 180 cases were reported.

Adrenal hemangiomas do not exhibit any specific symptoms or findings. Clinical diagnosis of these tumors is made only when their size causes symptoms because of mass effects. In light of a comprehensive review of the medical literature, most adrenal hemangiomas are discovered incidentally, and patients are typically asymptomatic at the time of discovery, as was the case with our patient. Conversely, symptomatic patients typically have vague abdominal discomfort along with pressure in the lower abdominal region that extends to the flanks, and some patients have syncope and anemia [5]. Furthermore, the presentation of a spontaneous life-threatening retroperitoneal hemorrhage of adrenal hemangiomas has been reported in 2 cases [6,7]. Muslim et al reported an adrenal cavernous hemangioma in a 76-year-old man with a concomitant infra-renal abdominal aorta fusiform aneurysm associated with mural thrombosis [8]. There is no clear association between our case and those presented in the literature regarding factors predisposing patients to the development of adrenal hemangiomas. A common etiology or risk factor for developing these unique masses cannot be demonstrated within the cases, owing to the lack of unified clinical characteristics or patterns.

While CT and ultrasound sonography findings for hemangiomas are typically described as nonspecific, some authors have suggested that contrast-enhanced CT has been proposed as a tool that can provide sufficient information for accurate diagnosis. The presence of calcification within the mass can indicate a hemangioma; however, cysts and carcinomas of the adrenal gland can exhibit this characteristic as well [9]. Peng et al conducted a study to evaluate the CT findings of adrenal cavernous hemangiomas in 10 histopathologically confirmed cases [3]. The study concluded that in cases in which an adrenal neoplasm is incidentally discovered, adrenal cavernous hemangiomas should be considered for differential diagnosis, especially when it appears as a well-defined, heterogeneous mass with calcifications and various enhancement patterns, which can include heterogeneous enhancements with progressive partial filling-in or a lack of enhancement in any phase. Regarding magnetic resonance imaging manifestations, adrenal hemangiomas are similar to liver and spleen hemangiomas, characterized by a slight hypointense signal on T1-weighted images and a hypointensity on T2-weighted images [10]. In addition, the 2 imaging features that can contribute to diagnosing this disease are heterogeneous density on plain scanning and progressive partial filling, with early peripheral enhancement. Most adrenal hemangiomas exhibit this post-enhancement appearance, which is considered a diagnostic feature.

A thorough review of the literature was conducted by Toklu et al, who identified 138 reported cases of adrenal hemangioma in the English literature [11]. According to this review, a slight female predominance was observed. This was similar to other studies, which concluded a 2: 1 ratio of females to males [7,12–14]. Additionally, contrary to the average age reported in the literature, which is 60 years, our patient presented at a much younger age (39 years). However, he falls within the age range of the previous cases (19–84 years). According to the case presented by Aljabri et al, the youngest patient with adrenal hemangioma previously reported in English-language medical literature was a 19-year-old woman [14]. Regarding laterality, 53.7% of cases are presented on the right side, which is in accordance with our case [11].

Given the rarity of adrenal hemangiomas, appropriate treatment guidelines are yet to be determined. Adrenal hemangiomas are treated according to the size of the tumor. The study by Matsui et al described the case of a 73-year-old man with chronic abdominal pain [15]. The patient had a large retroperitoneal cavernous hemangioma measuring 35 cm, underwent total tumor resection, and recovered well postoperatively. Based on the current reported literature, active surgical treatment is recommended when the tumor diameter exceeds 4 cm. Patients can be closely observed in the case of adrenal hemangiomas smaller than 3.5 cm; however, surgery can be considered if the tumor increases in size [16–20]. Resection can be considered in cases of indeterminate imaging, tumors with mass effect, substantial growth on surveillance, or hemorrhage [21]. Surgical removal of adrenal hemangiomas using laparoscopic techniques is feasible and safe, owing to the fibrotic capsule that surrounds adrenal cavernous hemangiomas, thereby reducing the risk of bleeding caused by surgical manipulation [17,22,23].

A retrospective study conducted at a single institution in China between January 1998 and December 2018 revealed that among the 601 patients who underwent adrenalectomy, only 8 were diagnosed with adrenal cavernous hemangioma [16]. According to this study, there was no evidence of a disease recurrence at the median follow-up interval of 38.37 months (ranging from 8 to 60 months).

The current understanding of adrenal hemangiomas remains limited due to scarcity and the type of existing research. Most evidence relies on case reports and small case series, which, while offering valuable insights, are inherently restricted in their generalizability. This paucity of large-scale studies hinders our ability to draw definitive conclusions about various aspects of this tumor, including its precise clinical presentation, optimal diagnostic/therapeutic approaches, and long-term outcomes. Consequently, a pressing need exists for high-quality studies employing rigorous methodologies and larger patient cohorts. We aim to add to the limited pool of documented cases and improve our understanding of the presentation, workup, and potential management approaches. While our case shares some characteristics with previously published reports, to the best of our knowledge, our case represents the first reported instance of intra-operative tumor bleeding associated with this rare condition. This unreported complication expands the current understanding of the clinical presentation and potential surgical challenges associated with this condition.

Conclusions

In conclusion, we reported a rare case of adrenal incidentaloma in a 39-year-old man. The infrequent occurrence of adrenal hemangiomas and their nonspecific clinical and radiological presentation results in a considerable diagnostic challenge.

In most cases, surgical resection is necessary to exclude malignant disease, alleviate pressure-related symptoms, and decrease the risk of hemorrhage within the retroperitoneum. These lesions are associated with a good prognosis. One limitation of this report is the lack of preoperative magnetic resonance imaging of the incidental adrenal mass.