Adenoma-Hemangioma Adrenal Collision Tumor Associated with Mild Autonomous Cortisol Secretion: A Case Report

Miguel A. Ortiz Rivera, MD; Frances T. Lee, MD, FACS

Department of Surgery, Southern Illinois University School of Medicine, Springfield, Illinois

Introduction

Adrenal collision tumors (ACTs) are rare masses of the adrenal

gland where two histologically distinct tumors coexist within

an adrenal gland. Due to their complex appearance on

imaging, accurate preoperative diagnosis can be challenging,

often requiring a multimodal imaging approach.

Adenoma-hemangioma ACTs have been reported very few

times in the literature. To the best of our knowledge, this is

one of two hormonally active adenoma-hemangioma ACT

reported in the literature.

Presentation

A 65-year-old female was evaluated in the endocrine surgery

clinic due to an incidentally found adrenal tumor during chest

computed tomography (CT) for cardiac scoring.

Follow-up CT of the abdomen and pelvis:

- Pre-contrast: 4 x 5.5 cm left adrenal tumor without local invasion

or calcification but with heterogeneous density.

- Arterial phase (35s): punctate foci of enhancement.

- Portal venous (70s): increasing enhancement compared to the

arterial phase.

- Delayed (4 min): Persistent nodular enhancement.

Impression: heterogenous mass concerning for

pheochromocytoma vs adrenocortical carcinoma.

Local symptoms: no

Symptoms of excess catecholamines: no

Symptoms of hypercortisolism: osteopenia

Symptoms of hyperaldosteronism: no

Physical exam: unremarkable.

Histories: Thalassemia, osteoarthritis, glaucoma, family

history of colorectal cancer.

Workup

Biochemical Profile:

Test Result Reference Range

24 hr urine free cortisol 62.6 mcg/dL <45

Dexamethasone suppression test (1g) 4.4 mcg/dL (10:04 AM) <1.8

Adrenocorticotropic Hormone 5.7pg/mL 7-63

24 hr urine catecholamines

- Epinephrine

- Norepinephrine

- Metanephrine

- Normetanephrine

Potassium 3.7mmol/L 3.5-5.1

Serum Aldosterone 5.9 ng/dL 3-35

Plasma Renin Activity 0.4 ng/mL/Hr 0.2-1.6 (supine)

DHEA-S 7 mcg/dL 24-244

 

Surgery & Recovery

Laparoscopic Left Adrenalectomy

- R lateral decubitus positioning, OG tube, Foley catheter.

- Veress insufflation at Palmer’s point. Ports: three 5mm ports

(paramedian supraumbilical, epigastric and left flank), and one 12mm

port (left subcostal midclavicular line),

- Splenic flexure mobilization.

- Lateral to medial mobilization of the pancreas, spleen, greater

curvature of the stomach.

- Mass identified: no desmoplastic reaction or local tissue invasion.

- The left inferior phrenic vein was traced down to its left adrenal vein

junction and renal vein termination.

- The adrenal vein was ligated twice on the stay side and a bipolar

energy device used to transect the vein on the specimen side.

- Dissection continued in the cephalad and lateral directions until the

specimen was freed & removed from the enlarged 12mm port site.

Post Operative Course

- Hemodynamics were stable during overnight observation.

- AM cortisol was low (12.1 mcg/dL, N >15).

- Follow up cosyntropin stimulation test indicated adrenal

insufficiency. (12.1 mcg/dL @ 30 min, 14.7 @ 60 min; N >18).

- A corticosteroid regimen with 10mg of hydrocortisone qAM and 5 mg

of hydrocortisone qPM was started.

- Discharge on POD 1.

- No complaints on 2 week post operative evaluation.

- The patient continues on a steroid regimen (approximately 11

months post op).

Pathology

Figure 1: Preoperative imaging of the left adrenal mass. CT axial views in the portal venous

(a) and delayed (b) phases showing heterogeneous enhancement and persistent nodular

enhancement, respectively. FDG PET coronal view (c) showing mildly hypermetabolic left

adrenal activity (SUVmax 2.6, white arrow). MRI without contrast axial view (d) showing

medial high T2 signal (red arrow) with and lateral intralesional fat (yellow arrow).

Multidisciplinary Tumor Board Discussion:

- Mass more likely benign than malignant.

- Low concern for metastatic disease.

- Surveillance could be an option.

Given size, hormonally active tumor, and patient desire for

definitive pathological evaluation, surgery was elected.

1. 2. 3. 4. 5. Discussion

- Although rare, a wide gamut of ACTs have been described in

the literature with benign, metastatic, and primary adrenal

malignant components described.

- To our knowledge, <10 adenoma-hemangioma ACTs have

been described in the literature. Their potential biochemical

profile has not been well described.

- Due to their heterogeneous appearance, diagnosis of ACTs

based on imaging can be challenging

- Hemangioma components classically have early peripheral

nodular enhancement with progressive centripetal filling on

contrast enhanced imaging (CT and MRI).

- Adenoma components will be hypodense lesions with <10 HU on

non-contrast CT, or have rapid washout on the 15 min delay

phase. MRI will show a signal drop on out-of-phase T2 images.

- While surveillance can be considered for benign appearing

adrenal lesions, hormonal activity and evolution should

assessed.

- Functional tumors carry long-term risk of morbidity, even if the

patient is otherwise “asymptomatic”.

- Adrenal masses >4-6 cm have higher chance of malignant

evolution.

Conclusion

ACTs should undergo thorough workup for signs of malignancy

and function. Even in otherwise asymptomatic patients in

which malignancy has been ruled-out, consideration for

adrenalectomy should be given to hormonally active tumors

due to long-term risk of medical morbidity.