Daniela Faivovich K., MS VII Universidad de Chile Gillian Lieberman, MD Harvard Medical School

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1 Daniela Faivovich K., MS VII Universidad de Chile Gillian Lieberman, MD Harvard Medical School May 21st, 2010

2 56 year old male patient History of hypertension, hyperlipidemia and insulin-resistance 2009: developed non specific abdominal discomfort During a workup for iron deficiency anemia he was noted to have an abdominal mass on PE As part of his workup, an abdominal US and CT scan were obtained

3 Our patient: abdominal ultrasound (12/2009)

4 PACS, BIDMC * Space occupaying, solid, rounded, heterogeneous mass, with anechoic lesions within it. Abdominal US Color doppler images reveal very little flow within the mass. Abdominal US

5 Our patient: abdominal CT (12/2009)

6 Abdominal CT/axial/ C+ PACS, BIDMC Abdominal CT/axial/ C+ Large, well defined mass, probably arising from the left adrenal gland. It is an heterogeneous mass, which compresses the left kidney and has some calcifications in it.

7 Large heterogeneous mass, with central calcifications, measuring 18.4 cm and apparently arising from the left adrenal gland

8 Hypertension (+) Blushing (-) Headache (ocasional) Palpitations (-) Anxiety (ocasional) Weight loss (-) Night sweats (-) Abdominal pain (-) Elevated catecholamines (+) & metanephrines in plasma or through 24h urine collection Sympathetic nervous system hyperactivity

9 An abdominal MRI was also performed, in order to define in a better way the features of the previously described mass

10 Our patient: abdominal MRI (01/2010)

11 PACS, BIDMC * * Abdominal MRI/axial/C- * * Abdominal MRI/sagital/C+ Abdominal MRI/coronal/C+ Well circumscribed mass, arising from the left adrenal gland, with heterogeneous signal characteristics. After gadolinium, enhancing tissue is demonstraded around the mass, which compresses the left kidney and displaces the stomach. The left renal vein remains patent.

12 1) 19-cm cystic left adrenal mass consistent with pheochromocytoma. No definite invasion into the stomach or pancreas. No other retroperitoneal masses or evidence of metastatic disease. 2) Two small subcentimeter hepatic hemangiomata and hepatic cyst. 3) Cholelithiasis.

13 Our patient: MIBG scan (02/2010)

14 MIBG was performed to look for other potencial foci and to rule out metastasis. MIBG is a compound resembling norepinefrin, that is taken up by adrenergic tissue. PACS, BIDMC

15 Discussion

16 The widespread use of crosssectional imaging has increased the detection of incidental adrenal masses. Approximately 3-10% of adrenal incidentalomas prove to be pheochromocytomas CT scanning offers good spacial localization, but findings are not specific enough to distinguish masses caused by pheochromocytoma from other adrenal masses. With MRI it is possible to characterize some adrenal lesions by means of their signal characteristics on different pulse sequences or by their enhancement characteristics. Sometimes, it may be difficult to determine the exact organ of origin of mass. The capability of obtaining images in innumerable planes is an advantage of MRI. Gary M. Israel, Glenn A. Krinsky, MR imaging of the kidneys and adrenal glands, Radiol Clin N Am 41 (2003)

17 Pheochromocytoma Adrenal adenoma Adrenal carcinoma Adrenal metastases Others (myelolipoma, hematomas, cysts) Adrenal adenoma * Gary M. Israel, Glenn A. Krinsky, MR imaging of the kidneys and adrenal glands, Radiol Clin N Am 41 (2003)

18 It is critical to make the differential diagnosis between pheochromocytoma and cortical carcinoma, because their imaging features may be identical and failure to do so may result in catastrophic consequences during surgery.

19 Pheochromocytomas are neoplasms of the adrenal medulla that produce catecholamines Equal frequency in men and women 3rd and 4th decades of life Pheochromocytomas are extra-adrenal, bilateral or malignant 10% of the time. Pheochromocytomas may be associated with other syndromes including MEN-2, von Hippel-Lindau disease, and neurofibromatosis Symptoms are nonspecific, and include palpitations, headache, sweating, and hypertension Pheochromocytomas account for the cause of hypertension in < 1% of patients Gary M. Israel, Glenn A. Krinsky, MR imaging of the kidneys and adrenal glands, Radiol Clin N Am 41 (2003)

20 Pheochromocytomas may have variable signal on T2W sequences, especially when they are greater than 5 cm. MRI is more useful in identifying an adrenal mass in a patient who is clinically thought to have a pheochromocytoma than in characterizing an adrenal mass as a pheochromocytoma. Furthermore, MRI is useful in identifying extraadrenal pheochromocytomas (paragangliomas) in the retroperitoneum along the paraspinal muscles. Gary M. Israel, Glenn A. Krinsky, MR imaging of the kidneys and adrenal glands, Radiol Clin N Am 41 (2003)

21 Occurs in the 4th to 5th decades of life Equal prevalence in men and women They are typically large (> 5 cm) at presentation, may contain varying degrees of hemorrhage and necrosis, and often contain calcium Some adrenal cortical carcinomas are hypersecreting (most commonly cortisol) Generally heterogeneous on T1W and T2W sequences (necrosis and hemorrhagic components). After the administration of gadolinium, the viable portion of the tumor enhances. Adrenal cortical carcinoma has a predilection to invade the adrenal veins, grow into the renal vein and inferior vena cava Gary M. Israel, Glenn A. Krinsky, MR imaging of the kidneys and adrenal glands, Radiol Clin N Am 41 (2003)

22 * Abdominal MRI/sagital/C+ Large, heterogeneous, enhancing adrenal neoplasm, displacing the left kidney inferiorly Abdominal MRI/coronal/C+ Enhancing tumor trombus growing into the left adrenal vein and the IVC Gary M. Israel, Glenn A. Krinsky, MR imaging of the kidneys and adrenal glands, Radiol Clin N Am 41 (2003)

23 Surgical resection of the tumor is the treatment of first choice, either by open laparotomy or laparoscopy. Either surgical option requires prior treatment with alpha/beta adrenoceptor blockers. Doing so permits the surgery to proceed while minimizing the likelihood of severe intraoperative hypertension.

24 THANK YOU

25 Dr. Gillian Lieberman Dr. Dennis Boparai Maria Levantakis

26 Gary M. Israel, Glenn A. Krinsky, MR imaging of the kidneys and adrenal glands, Radiol Clin N Am 41 (2003) Motta-Ramirez, GA, Remer, EM, Herts, BR, et al. Comparison of CT findings in symptomatic and incidentally discovered pheochromocytomas. AJR Am J Roentgenol 2005; 185:684 Grumbach, MM, Biller, BM, Braunstein, GD, et al. Management of the clinically inapparent adrenal mass ("incidentaloma"). Ann Intern Med 2003; 138:424 Manger, WM, Gifford, RW. Pheochromocytoma. J Clin Hypertens (Greenwich) 2002; 4:62.