Clinical Imaging 33 (2009) 314–317

Computed tomography appearance of spontaneous adrenal hemorrhage ina pheochromocytoma

Swati Aroraa,⁎, Scott Vargob, Anthony R. Lupetinc

aInternal Medicine, Allegheny General Hospital, Drexel University, 320-East North Avenue, Pittsburgh, PA 15212, USAbInternal Medicine, Allegheny General Hospital, Drexel University, Pittsburgh, PA, USA

cDivision of Body Imaging, Allegheny General Hospital, Pittsburgh, PA, USA

Received 1 October 2008; accepted 1 December 2008

Abstract

Pheochromocytomas are adrenal tumors that are diagnosed with time-consuming 24-h urine collection studies. Adrenal hemorrhage is arare but serious complication of pheochromocytomas that has been reported in only about 50 cases [Sutton MG, Sheps SG, Lie JT.Prevalence of clinically unsuspected pheochromocytomas. Review of a 50-year autopsy series. Mayo Clin Proc 1981;56:354–360]. We had apatient with a classic presentation of pheochromocytoma complicated with hypertensive crisis leading to spontaneous adrenal hemorrhage.We report the computed tomographic (CT) findings of ruptured pheochromocytoma that helped us in early detection and treatment of thislife-threatening complication.Published by Elsevier Inc.

Keywords: Pheochromocytoma; Adrenal tumors; Spontaneous rupture; Hemorrhage; Hypertension

1. Introduction

Pheochromocytomas are rare catecholamine producingneuro-endocrine tumors arising from chromaffin cells of thesympathetic nervous system. The annual incidence in the USis three to eight cases per million [1]. Adrenal hemorrhage isa rare but serious complication of pheochromocytomas thathas so far been reported, to our knowledge, in only about 49cases [2]. We had a patient with classic presentation ofpheochromocytoma complicated by hypertensive crisisleading to spontaneous adrenal hemorrhage. We report thecomputed tomographic (CT) findings of spontaneous adrenalhemorrhage in this uncommon condition, which can be lifethreatening if not detected early.

⁎ Corresponding author. Tel.: +1 412 359 4970; fax: +1 412 359 4983.E-mail addresses: sarora1@wpahs.org (S. Arora), svargo@wpahs.org

(S. Vargo), alupetin@wpahs.org (A.R. Lupetin).

0899-7071/09/$ – see front matter. Published by Elsevier Inc.doi:10.1016/j.clinimag.2008.12.008

2. Case report

A 54-year-old Caucasian male patient with past medicalhistory of hypertension, well-controlled with metoprolol forthe last 6 years, presented with chief complaint ofintermittent pounding headaches for 5 days associated withpalpitations, flushing, and diaphoresis. At the time ofadmission his blood pressure (BP) was 200/100 mmHgwith heart rate of 98 beats per minute. On examination, therewas no neck rigidity, jugular venous distention, or carotidbruit. Kernig's sign was negative and fundoscopic examina-tion was normal. His chest was clear to auscultation and heartsounds were normal with regular rate and rhythm. Abdomenwas soft, nontender, nondistended, no abdominal bruit, andthere was no peripheral edema.

Laboratory analysis was unremarkable with hemoglo-bin of 17.5 g/dl and creatinine of 1.5 mg/dl. Computedtomography scan of the head showed no masses or infarctor signs of intracranial bleed. CT scan of the abdomenwith contrast revealed a 5.5×5.3×7-cm heterogeneous

Fig. 1. CT scan of the abdomen (with contrast). Note the 5.5×5.3×7-cmheterogeneous left adrenal mass (black arrow), with area of hypodensitysuggesting necrosis; compared to normal right adrenal gland (white arrow).

Fig. 2. (A) Repeat CT scan of the abdomen with acute adrenal hemorrhage.Note the enlargement surrounding the left adrenal gland and hyperattenuat-ing fat (50 HU) due to hemorrhage (white arrows). (B) Repeat CT scan of theabdomen. Note the hemorrhage in the peri-adrenal retroperitoneum,extending along the cone of renal fascia as well as into the left anteriorpara-renal space (white arrow), relating to the hemorrhagic left adrenal mass(diagnosed as pheochromocytoma).

Table 1Twenty-four-hour urine collection results

Test Result Normal range

Total metanephrines 59,245 (22–180)Metanephrines 12,475 (44–261)Nor-metanephrines 46,770 (128–484)Epinephrine 3530 (0–20)Nor-epinephrine 30,366 (15–80)

315S. Arora et al. / Clinical Imaging 33 (2009) 314–317

enhancing cranio-caudal left suprarenal mass with areas ofhypodensity, suggestive of necrosis (Fig. 1). No calcifica-tion was seen. Renal arteries were grossly symmetric.Abdominal aorta was normal and liver was homogeneous.Gall bladder, pancreas, spleen, and kidneys were normal.Differential diagnosis at this point included adreno-cortical neoplasm, unilateral adrenal metastasis, or pheo-chromocytoma. A 24-h urine collection was sent forcatecholamine and metanephrine measurement to evaluatefor pheochromocytoma.

2.1. Hospital course

The patient was admitted to the intensive care unit andstarted on clonidine and labetalol. His BP remained labilefluctuating between 70s to 250s systolic, necessitatingfrequent titration of BP medications. On Day 2, the patientdeveloped an acute abdomen with diffuse tenderness,rebound, and guarding. BP was found to be 350/150 viaarterial line. A stat CBC showed a drop of hemoglobin from16.1 to 12.7g/dl. Stat repeat CT scan of the abdomen wasordered. It demonstrated interval development of hemor-rhage relating to the left adrenal mass (Fig. 2A and B).Relatively dense blood was seen in the peri-adrenal retro-peritoneum, extending along the cone of renal fascia as wellas into the left anterior para-renal space as far caudal as theleft iliac fossa. The pancreas was displaced anteriorly by theretroperitoneal hemorrhage. No bowel wall hemorrhage orobstruction was seen. No localized or diffuse abnormalitywas seen in the liver, gall bladder, or spleen. Low attenuationascites was seen in the pelvis.

General surgery and endocrine were consulted for furthermanagement of ruptured adrenal mass. The patient subse-quently underwent left adrenalectomy 2 days later afteradequate alpha blockade with phenoxybenzamine. Surgicalremoval of pheochromocytoma is a high-risk surgery due to

massive release of catecholamines secondary to manipula-tion of the tumor during resection. Therefore it isrecommended to control the blood pressure adequately forat least 7 to 14 days with alpha blockers preoperatively [3].In our patient, due to the acute adrenal hemorrhage andhemodynamic instability, resection was done in emergency

316 S. Arora et al. / Clinical Imaging 33 (2009) 314–317

after only 48 h of alpha blockade. Postoperatively, the patientwas continued on Phenoxybenzamine taper and recoveredwell with control of blood pressure and symptoms.

Twenty-four-hour urine collection results came back 4days later which were consistent with pheochromocytoma(Table 1). Pathological analysis of the surgical specimenshowed left adrenal gland pheochromocytoma, 11 cm ingreatest dimension, with pleomorphism and bizarre hyper-chromatic nuclei.

3. Discussion

Adrenal hemorrhage is a rare but life-threatening eventthat may occur as a complication of underlying adrenalpathology, physiological stress, blunt trauma, sepsis, or acoagulopathic state. It may lead to acute adrenal crisis,shock, and death unless it is recognized promptly and treatedappropriately [4,5].

3.1. Incidence

Adrenal hemorrhage has been reported in 0.3–1.8% ofunselected cases in autopsy studies. Adrenal adenomas,adreno-cortical carcinomas, and pheochromocytoma areprimary adrenal tumors that may lead to spontaneous adrenalhemorrhage [6–8]. Massive adrenal hemorrhage secondaryto a previously undiagnosed pheochromocytoma may belethal in 50% of cases [9–11]. Early suspicion and diagnosiswith imaging modalities and appropriate treatment may leadto improved survival [2].

3.2. Imaging

Abdominal imaging studies are necessary for earlydiagnosis of adrenal hemorrhage in cases of pheochromo-cytoma. The confirmatory laboratory tests such as 24-hurine collection and analysis for metanephrines/catechola-mines can take up to 3–4 days before giving a definiteanswer. In this case, the patient was started on phenox-ybenzamine and underwent emergency adrenalectomybased on the CT scan findings before 24-h urine test resultswere available.

CT scan, magnetic resonance imaging (MRI), or ultra-sound can be utilized to diagnose an adrenal hemorrhage. CTscan is preferred in all age groups except for neonates lessthan 6 months old where ultrasound may be more beneficial[12]. Noncontrast CT is usually performed due to its highsensitivity and specificity in diagnosing early hemorrhage[13]. Adrenal hemorrhage may present on CT scan asinfiltrating hyper-attenuating foci with Hounsfield unit (HU)in the range of +50 to +90 with distortion of inverted v-shapeof the adrenal gland, by a round or oval mass [12,14,15]. Ahomogeneous density greater than 50 HU has beensuggested by Lee et al. [16] as a specific sign of hematomaespecially in cases with acute hemorrhage [17]. However,

chronic or subacute adrenal hematomas cannot be distin-guished clearly from other lesions on the basis of density onCT scan [18].

Additional findings in case of adrenal hemorrhage mayinclude peri-adrenal fat stranding with thickening of theadjacent diaphragmatic crura. The addition of enhancedimages allows demonstration of active bleeding sites.Adrenal hematomas decrease in size and attenuation overtime and most resolve spontaneously although hemorrhagiccysts may occur [9].

The diagnosis of adrenal hemorrhage based on CTfindings is challenging because a large number of benign ormalignant conditions might mimic its appearance on theimaging studies. Important differential diagnosis to consideris leaking aortic aneurysm, bleeding from retroperitonealorgans, renal vein thrombosis, and abdominal abscess.Renal abscess usually appears as poorly defined, wedge-shaped hypodense area with low attenuation (0 to +20 HU)which does not enhance on contrast administration [19,20].Leaking aortic aneurysm, aortic dissection, or renal veinthrombosis is better visualized with contrast CT. It alsohelps in providing details about surrounding abdominalstructures and their relationship to the bleed [21]. In ourpatient, we got an opportunity to see the classic clinicalpresentation suggestive of pheochromocytoma, a largeadrenal mass on CT and interval development of sponta-neous hemorrhage in the adrenal mass which helped indirecting our treatment.

MRI findings specific for adrenal hemorrhage are highsignal intensity on T1-weighted images and heterogeneouslow signal serpiginious areas on T2-weighted images [22].MRI is most useful to confirm the presence and chronicityof hemorrhage associated with an adrenal mass. In thechronic phase, both hemosiderin and calcification result inlow signal on T1- and T2-weighted images. CT seems tohave advantage over MRI in emergency situations asquick scanning and imaging of CT are more feasible andreadily available.

4. Conclusion

Since there is a time lapse in diagnosis of pheochromo-cytoma based on definitive urine tests, utilization of imagingstudies and greater awareness of manifestations of sponta-neous hemorrhagic pheochromocytoma amongst radiolo-gists and clinicians will help in early recognition andtreatment of this emergent and life-threatening situation.

References

[1] Sutton MG, Sheps SG, Lie JT. Prevalence of clinically unsuspectedpheochromocytomas. Review of a 50-year autopsy series. Mayo ClinProc 1981;56:354–60.

[2] Kobayashi T, Iwai A, Takahashi R, Ide Y, Nishizawa K, Mitsumori K.Spontaneous rupture of adrenal pheochromocytoma: review andanalysis of prognostic factors. J Surg Oncol 2005;90:31–5.

317S. Arora et al. / Clinical Imaging 33 (2009) 314–317

[3] Kocak S, Aydintug S, Canakci N. Alpha blockade in preoperativepreparation of patients with pheochromocytomas. Int Surg 2002;87:191–4.

[4] Rao RH, Vagnucci AH, Amico JA. Bilateral massive adrenalhemorrhage: early recognition and treatment. Ann Intern Med 1989;111(2):185.

[5] Vella A, Nippoldt TB, Morris JC. Adrenal hemorrhage: a 25-yearexperience at the Mayo Clinic. Mayo Clin Proc 2001;76(2):161–8.

[6] Clark OH, Hall AD, Schambelan M. Clinical manifestations of adrenalhemorrhage. Am J Surg 1974;128:219.

[7] Will CH, Hoeppener HJ, Beutler W. Post-traumatic hematomasimulating a left-sided adrenal gland tumor. Bildgebung 1990;57:39.

[8] Wilms G, Marchal G, Baert A, Adisoejoso B, Mangkuwerdojo S. CTand ultrasound features of post-traumatic adrenal hemorrhage.J Comput Assist Tomogr 1987;11:112.

[9] Jacobs LM, Williams LF, Hinrichs HR. Hemorrhage into apheochromocytoma. JAMA 1978;239:1156.

[10] Nicholls K. Massive adrenal haemorrhage complicating adrenalneoplasm. Med J Aust 1979;17:560–2.

[11] Kawashima A, Sandler CM, Ernst RD, Takahashi N, Roubidoux MA,Goldman SM, Fishman EK, Dunnick NR. Imaging of nontraumatichemorrhage of the adrenal gland. Radiographics 1999;19:949–63.

[12] Dunnick NR. Hanson lecture. Adrenal imaging: current status. AJRAm J Roentgenol 1990;154(5):927–36.

[13] Witteles RM, Kaplan EL, Roizen MF. Sensitivity of diagnostic andlocalization tests for pheochromocytoma in clinical practice. ArchIntern Med 2000;160(16):2521–4.

[14] Dunnick NR, Korobkin M. Imaging of adrenal incidentalomas: currentstatus. AJR Am J Roentgenol 2002;179(3):559–68.

[15] Dunnick NA. Metabolic abnormalities of the adrenal cortex. In:Pollack H, editor. Clinical urography. Philadelphia: Saunders, 1990. p.2313–37.

[16] Lee MJ, et al. Benign and malignant adrenal masses: CT distinctionwith attenuation coefficients, size, and observer analysis. Radiology1991;179(2):415–8.

[17] Moulton JS, Moulton JS. CT of the adrenal glands. Semin Roentgenol1988;23(4):288–303.

[18] Bowen AD, et al. Adrenal hemorrhage after liver transplantation.Radiology 1990;176(1):85–8.

[19] Dalla Palma L, Pozzi-Mucelli F, Ene V. Medical treatment of renal andperirenal abscesses: CT evaluation. Clin Radiol 1999;54(12):792–7.

[20] Kawashima A, et al. Renal inflammatory disease: the current role ofCT. Crit Rev Diagn Imaging 1997;38(5):369–415.

[21] Asghar M, et al. Renal vein thrombosis. Eur J Vasc Endovasc Surg2007;34(2):217–23.

[22] Hoeffel C, et al. Spontaneous unilateral adrenal hemorrhage:computerized tomography and magnetic resonance imaging findingsin 8 cases. J Urol 1995;154(5):1647–51.