ADRENAL MEDULLARY DISORDERS: PHAEOCHROMOCYTOMAS AND MORE

Transcription

1 ADRENAL MEDULLARY DISORDERS: PHAEOCHROMOCYTOMAS AND MORE DR ANJU SAHDEV READER AND CONSULTANT RADIOLOGIST QUEEN MARY UNIVERSITY AND ST BARTHOLOMEW S HOSPITAL BARTS HEALTH, LONDON, UK

2 DISCLOSURE OF CONFLICT OF INTEREST I have no disclosures Brief Introduction to the adrenal medulla Cases and discussion

3 Adult Adrenal Gland The Adult Adrenal Gland CORTEX: Glandular organ

4 DEVELOPMENT OF THE ADRENAL GLANDS The two parts of the adrenal gland develop from two different origins: The cortex: Mesodermal coelomic epithelium of the posterior abdominal wall. Mesoderm=glandular tissue=secretory glands=cortisol, aldosterone, androgens, oestrogens and progesterone Diseases affecting the cortex similar to the mesoderm elsewhere Adenomas, carcinomas, autoimmune diseases, supporting tissue diseases (AMLs, lymphoma etc) Reflected in the blood supply to the adrenal: Branches of the phrenic arteries, directly from abdominal aorta and renal artery. Venous drainage follows the arterial supply The medulla: Ectodermal develops from the neural crest cells =chromaffin adrenal cells Affected by diseases of the primitive and mature neural tissue and ganglia. Resemble neurological diseases

5 CASES

6 CASE 1 62 year old woman with hypertension. Lesion in the right adrenal detected on renal MRA?diagnosis?what further confirmatory test/tests would you perform Phaeochromocytoma

7 PHEOCHROMOCYTOMA ADRENAL PARAGANGLIOMA Carotid bodies Aortic bodies Thoracic sympathetic paraganglia Adrenal medullae Most frequent medullary tumour arising from chromaffin cells Catecholamine-secreting neoplasm Rare, but important: surgically curable form of hypertension Sporadic: ~24% have germline mutations, including mutations of RET, VHL, SDH-B, and SDH- D genes Familial (MEN, VHL, NF1, SDH, Carney s Triad) Benign or malignant Aortic sympathetic paraganglia Visceral autonomic paraganglia

8 IMAGING FEATURES: THE IMITATOR CT Pre C : >10HU Hyper vascular: Washout less than 60%* homogenous if small and heterogeneous if large MRI: No CSI signal loss

9 17% are Cystic 6HU

10 MIBG, FDG PET avid

11 VHL, MEN 1, SDHB associated small lesions, usually screen detected Higher rate of malignancy than sporadic lesions

12 Malignant Phaeochromocytoma 1. Presence of metastases only definitive criteria 2. Loco regional visceral and vascular invasion 3. Benign & malignant tumors are histologically identical; the only absolute criterion for malignancy is metastasis. 4. Ki 67 increases with increasing risk of malignancy. Ki 67>30% likely malignant

13 CASE 2: 26 year old, abdominal pain and weight loss NEUROBLASTOMA/GANGLIOBLASTOMA Most frequent malignant tumour of the medulla= Small round blue cell tumor of childhood 40% occur in the adrenal, Large, heterogeneous masses, locally invasive, and lifts the aorta/ivc, extends across mid line. Coarse amorphous calcifications in 85%

14 CASE 2 COMPANION CASE: GANGLIOBLASTOMA

15 CASE 3 16 year old female. Generally unwell and coughing. Normal CXR US abdomen showed a left solid suprarenal mass What would you do next? (imaging and clinical)

16 5HU What is your diagnosis and why? Would you do any further imaging? What? Benign (<10HU) but not characterized *wrong age for adenoma *wrong enhancement pattern for adenoma and not a cyst on CT or US MRI Endocrine profile very important here! 15HU 25HU

17 T2 T1 in phase T2 out of phase T1 fat saturated T1 fat saturated post contrast MIBG

18 What is your diagnosis and why? Would you do any further imaging? What? MIBG negative 30-40% are negative in phaeochromocytomas and paragangliomas MRI Features in keeping with phaeo: high T2, solid and enhancing Features not in keeping with phaeo: very low grade enhancement, low CT pre attenuation Biochemically inert: All catecholamines and adrenal cortex hormones normal What next? Biopsy: Ganglioneuroma

19 GANGLIONEUROMA Well Differentiated neoplasm of neural crest origin Benign Occurs in older paediatric to adult age group Pathology: Gross: Encapsulated, white, firm Micro: Ganglion cells & Schwann cells On Imaging : Associated with a negative CT contrast washout = slow, low grade progressive enhancement. Therefore delayed images have a higher attenuation than at 60s

20 8HU 22HU 45HU Resected Ganglioneuroma with low grade function Washout criteria: Absolute washout : 22-45/22-8 = -164%

21 TUMOURS: ARISING FROM NEUROBLASTIC /MATURE GANGLION CELLS Neuroblastoma Malignant Infants and children VMA and HVA Nor adrenalin VIP and other GI peptides Hypertension Palpitations Fever diarrhoea Ganglioneuroblastoma Ganglioblastoma Ganglioneuroma Neuroma Decreasing function Benign Incidental in older children and adults

22 CASE 4 32 year old woman being investigated for mild hypertension and palpitations What are the CT findings? What information do you need?

23 What are the CT findings? -bilateral adrenal enlargement including the adrenal limbs What information do you need? Biochemical changes, syndrome? -?cortisol/aldo?catecholamines Elevated cortisol/aldo Cushing s/conns Elevated catecholamines Medullary Hyperplasia Pseudopheaochromocytoma

24 MEDULLARY HYPERPLASIA: MIMIC PHAEOCHROMOCYTOMAS Defined as expansion and extension of the adrenal medulla into limbs where medullary tissue is not normally present Diffuse or nodular/symmetric or asymmetric Familial hyperplasia Associated with MEN II, Beckwith-Wiedemann syndrome Sporadic hyperplasia also exists as a distinct entity in paediatric age group, with cystic fibrosis Imaging features not distinguishable from cortical hyperplasia on CT and MRI Increased MIBG, Ga and FDG uptake on PET which may be diffuse of focal. -focal uptake mimics a phaeo

25 MEN II : HYPERPLASIA

26 CASE 5 65 year old woman with endometrial cancer Post operatively, unable to wean off ventilation and hypotensive CT indication?abdominal bleed?collection What does the CT demonstrate? Working diagnosis? How would you confirm this?

27 What does the CT demonstrate? -bilateral adrenal enlargement -irregular contour of left adrenal? Working diagnosis? -adrenal haemorrhage? How would you confirm this? -pre operative staging scan

28 Haemorrhage and Infarction Waterhouse Frederickson syndrome Post surgical ischaemia and haemorrhage Trauma History usually leads to the diagnosis Although the adrenal cortex is more susceptible to infarction, the adrenal medulla is also affected and contributes to the hypotension. ADRENAL HAEMORRHAGE

29 SUMMARY The adrenal medulla is a sympathetic ganglion made up of neural cells Diseases affecting the peripheral nervous system and the sympathetic ganglia elsewhere, also affect the adrenal medulla Phaeochromocytomas are the best known and best described lesion but other benign and malignant lesions occur in adults Of these malignant neuroblastomas and benign ganglioneuromas have specific imaging features Syndromic associations with MEN, VHL, SDH etc may be helpful in the clinical workup Patients age has a strong influence of the likelihood of pathology

30 Thank You