Case Report

Educational Point-of-Care Ultrasound Discovers Adrenal Cortical Carcinoma, a Case Report: Clinical Implications and Review of the Literature

Minh Q Ngyuyen1; Moshe Bengio, DO2; Vu H Tran, MD2

Abstract

Author affiliations are listed at the end of this article.

Correspondence to: Moshe Bengio, DO (Moshe.bengio@ hcahealthcare.com)

Introduction

Point-of-care ultrasound (POCUS) has become an indispensable tool for emergency physi- cians in assessing intra-abdominal pathology due to its real-time visualization, portability, and cost-effectiveness. While POCUS is not traditionally focused on adrenal gland assess- ment, incidental findings of adrenal masses during examinations have been reported. Our patient presented with a chronic obstructive pulmonary disease (COPD) exacerbation and was found to have a large adrenal mass, discovered incidentally, during thoracic ultrasound.

Case Presentation

This case report presents the discovery of an adrenal mass in a patient with respiratory distress due to a COPD exacerbation, emphasizing the importance of sonographers’ ability to identify abnormal imaging within their POCUS framework. The clinicians promptly com- municated the incidental finding to the patient, leading to further imaging and labs while in the emergency department and subsequent hospital admission. Traumatic arteriovenous fistulas and visceral vascular injuries are rare.

Conclusion

Although adrenal glands are not the primary focus for emergent pathology, the identified mass prompted a comprehensive evaluation, ultimately revealing a nonhormone-secret- ing adrenal cortical carcinoma. The rarity of this malignancy and the discrepancy between imaging and laboratory results underscore the need for a thorough investigation, with the focus suggesting that POCUS, despite its limitations, can play a crucial role in prompting additional imaging for accurate diagnosis and informed patient management. This case also highlights the significance of transparent communication while empowering patients and guiding further investigations for a comprehensive understanding of the pathology.

Keywords

adrenocortical carcinoma; adrenal cortex neoplasms; POCUS; point-of-care ultrasound; adrenal mass; incidental finding; case reports

Introduction

Over the past several decades, there has been a significant increase in the utilization of point-of-care ultrasound (POCUS) as a vital tool for emergency physicians in patient eval- uation. Point-of-care ultrasound also serves as a rapid and noninvasive diagnostic instrument for acute cases with ambiguous symptoms, enabling clinicians to narrow the differential

diagnosis, ascertain the etiology of symptoms, and hasten the formulation of a treatment plan.1 Notably, POCUS has proven an important adjuvant in evaluating intra-abdominal pathol- ogy, occasionally detecting incidental findings, such as adrenal masses.2 The following case incidentally discovers an adrenocortical carci- noma (ACC) prompting an urgent workup. We then review the workup and diagnosis of ACC, including imaging findings, and discuss the

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management of “incidentalomas” as it pertains to this case.

Case Presentation

A 59-year-old man was brought to the emer- gency department (ED) by emergency medical services with worsening shortness of breath for the previous 3 weeks. His medical history included chronic obstructive pulmonary disease (COPD) on 2-4 L of home oxygen and a 60 pack per year tobacco use. Upon arrival, he was hy- poxic at 60% SpO2 and required 10 L of supple- mental oxygen to achieve 90% SpO2. His blood pressure was 100/79 mmHg, and his heart rate was 94 beats per minute. On physical exam, he was tachypneic and spoke in partial sentences with supraclavicular retractions. There was min- imal wheezing noted in the expiratory phase on auscultation in all lung fields and 2+ pitting ede- ma in the bilateral lower extremities that were well-perfused, nontender, and nonerythema- tous. His electrocardiogram demonstrated nor- mal sinus rhythm, right axis deviation (chronic finding), and no signs of acute ischemia.

A cardiac and pulmonary POCUS were per- formed to evaluate his respiratory distress. Car- diac POCUS with 4 views (subxiphoid, apical,

parasternal short and long axis) showed right ventricular dilation with normal left ventricular function consistent with chronic right heart dysfunction. Thoracic POCUS demonstrated small bilateral pleural effusions and B-lines suggestive of pulmonary edema on lower lung fields with normal lung sliding. A large cystic structure below the left diaphragm was inciden- tally identified and warranted further inspec- tion with renal POCUS. He denied a history of any renal or adrenal medical conditions. A left renal POCUS revealed a large complex suprare- nal structure with irregular borders containing an anechoic cystic center, as well as the pres- ence of ascites (Figure 1). The initial laborato- ry studies and arterial blood gas were within normal limits.

The patient was treated with nebulized albuter- ol/ipratropium, corticosteroids, broad-spec- trum antibiotics, and noninvasive ventilation. Once stabilized, a computed tomography (CT) angiogram of the chest was performed to rule out pulmonary embolus, and a CT abdomen/ pelvis was done to evaluate the left upper quad- rant mass. The patient’s CT scan showed signs of right heart strain, trace right-sided pleural effusion, severe diffuse centrilobular emphy-

Figure 1. An ultrasound showed the left inferior lateral thoracic view. Inferior to the left hemidia- phragm (the orange arrow points to the diaphragm), there was a large complex structure with- out clear borders but with a central, irregularly bordered, anechoic, cystic-like structure (yellow arrow). The spleen and kidney were unidentified in this window. Above the diaphragm, a small anechoic pleural effusion was noted in the costophrenic angle. Color doppler demonstrated vas- cularity within the structure, consistent with a malignant mass (inset).

GE

Superior

Inferior

sematous changes, and diffuse bilateral axillary, hilar, and mediastinal lymphadenopathy without pulmonary embolus. The dedicated CT of the abdomen/pelvis (Figure 2) confirmed a 12 cm x 11 cm x 11 cm mass with a hemorrhagic and ne- crotic core concerning for ACC. Moderate asci- tes were also present. A bilateral lower extrem- ity venous doppler ultrasound demonstrated a nonocclusive right femoral vein thrombosis. Anticoagulation for the deep vein thrombosis was deferred given the suspected hemorrhage in the adrenal mass, and surgical oncology was consulted for inpatient management.

The inpatient workup included labs to further elucidate the adrenal mass activity, as follows (normal values): serum renin 4.415 ng/ml/hr (0.167-5.380 ng/ml/hr), serum aldosterone 11.9 ng/dL (0.0-30.0 ng/dL), 17-hydroxyprogesterone less than 10 ng/dL (27-199 ng/dL), dehydroepi- androsterone less than 20 ng/dL (21-402 ng/ dL), testosterone 52.9 ng/dL (241-827 ng/dL), metanephrine 68.6 pg/mL (0.0-88.0 pg/mL), and carcinoembryonic antigen (CEA) 6.2 ng/ml (0.0-5.0 ng/ml). Based on these findings, it was determined that the mass was nonhormone secreting. A pathology report of the ascitic fluid revealed a hypocellular specimen consisting of a few macrophages, histiocytes, and scattered mixed leukocytes.

The surgical oncology team next recommended a biopsy; however, the patient refused the bi- opsy or additional invasive procedures and was ultimately discharged to hospice. There were multiple unsuccessful attempts over the next 2 months to follow up with the patient via the

provided contact information. Eventually, it was confirmed that he passed away.

Discussion Background

Adrenal tumors typically manifest as small, be- nign, nonfunctional adrenocortical adenomas, affecting 3% to 10% of the human population.3 In contrast, ACC is a rarer condition, with a prevalence of 1 to 2 cases per million per year.4 Approximately 20% to 30% of ACC cases are incidentally diagnosed.4-6

The existing clinical practice guideline for ACC provided by the European Society of Endocri- nology (ESE) advocates for a comprehensive hormone workup to identify potential auton- omous excess glucocorticoids, sex hormones, mineralocorticoids, and adrenocortical steroid hormone precursors.7

Current data indicate that 85% of adrenal incidentalomas are nonfunctional, while 15% exhibit functional characteristics.8 The most frequent presentation involves hypercorti- solism, such as plethora, acute-onset diabetes mellitus, muscle weakness/atrophy, and os- teoporosis (Cushing syndrome). The second most common presentation is characterized by the secretion of adrenal androgens, leading to rapid-onset male pattern baldness, hirsutism, virilization, and menstrual irregularities.9

Adrenocortical carcinomas typically present as large tumors, averaging 10 x 13 cm, charac- terized by internal hemorrhage, necrosis, and calcifications, resulting in frequent tumor het-

Figure 2. A computed tomography image showed a large necrotic adrenal mass (yellow arrows) in the transverse (A) and coronal (B) views.

A

B

H

M

F

erogeneity.9 Although “cannot miss” diagnoses like pheochromocytomas are rare, their identi- fication is crucial, particularly when presenting with newly decompensated congestive heart failure and persistent hypertension, as these conditions can be fatal.2

The ESE guidelines suggest incorporating a chest CT, in addition to abdominal-pelvic cross-sectional imaging (CT or magnetic res- onance image [MRI]), in cases where there is a high suspicion of ACC.7 Particularly in a prima- ry tumor exceeding 4 cm, a chest CT or MRI with contrast is important in the evaluation of metastatic disease and local invasions.7 On contrast-enhanced CT scans, ACCs typically display irregular peripheral enhancement with a centrally nonenhancing area, indicating hemor- rhage or necrosis.1º Approximately 30% of ACC cases exhibit calcification, manifested as either coarse or microcalcification, usually centrally located and identified by high attenuation foci. Metastases to regional and para-aortic lymph nodes, lungs, livers, and bones are best iden- tified through contrast-enhanced CT scans in ACCs, facilitating accurate disease staging.11 They exhibit an isointense to hypointense ap- pearance distinct from the liver parenchyma on T1-weighted imaging and appear hyperintense on T2-weighted images.” Lastly, the ESE guide- lines discourage adrenal biopsy unless there is evidence of metastatic disease that precludes surgical intervention.7

Point-of-Care Testing

While the primary training of traditional POCUS training does not focus on assessing the adrenal glands, there have been reports of incidental findings ranging from benign cysts to pheochromocytomas.2 When imaging a mass along the upper pole of the kidneys, it is recommended to use a curvilinear trans- ducer with a frequency of 2-5 MHz in both longitudinal and transverse planes. Preferably, anterior transverse scanning is employed for identifying small adrenal masses, and the use of color flow doppler is considered to assess for vascular involvement, which is significantly more common in malignant lesions.2,8,12 Normal adrenal gland tissues are expected to appear hypoechoic, but can be difficult to identify since they often exhibit a similar echogenicity to retroperitoneal fat.13 They normally mea- sure less than 3 cm in size. In cases where the

etiology remains unclear with POCUS, fur- ther imaging modalities, such as an abdominal CT or MRI, are warranted. While sonography demonstrates high sensitivity and specificity in detecting the presence of adrenal masses, differentiation between benign and malignant masses cannot be solely based on size. Con- trast-enhanced sonography, while comparable in sensitivity to CT/MRI for discerning adrenal masses as benign versus neoplastic, is also not reliable for distinguishing between various his- topathologic nonadenomatous lesions.8

The ability of an emergency physician to iden- tify pathology early on using POCUS offers a comprehensive approach to patient manage- ment. When a mass is detected, emergency physicians should consider pursuing follow-up imaging for further clarification.2,14 However, reporting of incidental findings varies on expe- rience, documentation, and by institution. The range of these findings were as little as only 1.6% of scans up to 26% of routine POCUS.15 The extreme end of this range is based on a study of renal and biliary scans by emergency medicine residents.16 Even in rapid and less thorough exams, providers were able to identi- fy 137 incidental findings over 1452 (9.4%) fo- cused assessment with sonography in trauma (FAST) exams during a 15-month period.15,17 In another study of 137 patients, 31 (22.6%) of these patients had an incidental finding in the ED. Shockingly, only 6 of these patients were informed of these findings.15,17

The ability to steer diagnosis, treatment, and future follow-up should allow for transparent communication of incidental findings, grant- ing patients autonomy over their health. The American College of Radiology and Fleischner Society recommend for all incidental findings a standardized protocol that follows evi- dence-based guidelines to capitalize on cost effectiveness and inter-rater reliability.15,18-20 Hence, medical providers and their trainees performing POCUS require evidence-based guidelines for when additional imaging is nec- essary while considering health care costs and patient wellbeing. Each individual institution should be responsible for formulating these protocols, piloting discussions, and conducting training.15

Conclusion

In an ACC, rigorous workup is essential, involv- ing the measurement of steroid hormones pro- duced by the mass, contrast-enhanced CT/MRI imaging, and biopsy consideration to determine the secretory nature of the mass. In our case, a thoracic POCUS initially detected a suprarenal mass, requiring a clarifying abdominal CT scan, which the intended chest CT scan may have incompletely identified, requiring a potentially unstable patient to return for imaging again and substantially delaying proper identification. The patient was promptly informed about the incidental finding, leading to further imaging and labs during their admission to ascertain an accurate diagnosis. Although the definitive diagnosis was elusive, this information enabled informed decisions, supporting patient auton- omy. This case is a reminder of how POCUS has the potential to identify incidental findings unrelated to the initial scan indication, offering significant clinical value in patient care. In cases where emergency physicians come across inci- dental POCUS findings, there is a need to relay this information and assess whether additional imaging is warranted. Developing a clear fol- low-up plan becomes essential when necessary. There is room for improvement in EDs, partic- ularly in enhancing the clarity of follow-up rec- ommendations and effectively communicating ultrasound results to patients. Currently there are no guidelines on management of incidental findings on POCUS. The clinician performing the study should follow hospital specific guide- lines, document them to avoid malpractice risks, and ensure timely follow-up and workup.

Conflicts of Interest

The authors declare they have no conflicts of interest.

Drs Bengio and Tran are employees of HCA Florida Aventura Hospital, a hospital affiliated with the journal’s publisher.

This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare-affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.

Author Affiliations

1. West Virginia School of Osteopathic Medicine, Lewisburg, West Virginia

2. HCA Florida Aventura Hospital, Aventura, Florida

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