Case presentationCase presentation

Endocrine moduleEndocrine module

Jacques le Jacques le RouxRoux

20/04/201220/04/2012

Clinical pictureClinical picture 34 year male (security worker)34 year male (security worker) Presenting with spontaneous Presenting with spontaneous hypoglycemiahypoglycemia episodes episodes

from 2010from 2010 Admitted after a hypoglycemic attack which resulted Admitted after a hypoglycemic attack which resulted

in neurological symptoms that ranged from in neurological symptoms that ranged from confusion to loss of concious/coma or convulsionsconfusion to loss of concious/coma or convulsions

No ETOH useNo ETOH use. No smoker.. No smoker. No sulphonylurea overdose (not known diabetic)No sulphonylurea overdose (not known diabetic)

Special investigationsSpecial investigations Random glucose 2 lowRandom glucose 2 low Insuline levels normal , C-peptide normalInsuline levels normal , C-peptide normal Amylase normalAmylase normal TFT normal, Calsium levels normalTFT normal, Calsium levels normal ESR 59, CRP < 1, FBC normalESR 59, CRP < 1, FBC normal Renal function normalRenal function normal

Newly diagnosed RVD +Newly diagnosed RVD + No TB tests doneNo TB tests done

ImagingImaging

CXR CXR

Abdominal sonar Abdominal sonar

CT chest and abdomenCT chest and abdomen

Octreotide scanOctreotide scan

CXRCXR

SonarSonar

CTCT

ARTERIAL PORTO-VENOUS

Octreotide scanOctreotide scan

Differential diagnosisDifferential diagnosis

1) Functional Islet cell tumor of the pancreas / Insulinoma 1) Functional Islet cell tumor of the pancreas / Insulinoma [ small hypervascular pancreas lesion on the art phase, hypoglycemia] – classic[ small hypervascular pancreas lesion on the art phase, hypoglycemia] – classic

2) NH Lymphoma / AIDS related lymphoma2) NH Lymphoma / AIDS related lymphoma (mediastianl LN present, pancreas lesion not hypovascular) (mediastianl LN present, pancreas lesion not hypovascular) - will first consider infective cause for mediastinal LN’s- will first consider infective cause for mediastinal LN’s - mets not likely from insulinoma (no large lesion,no calcifications,no liver lesions,no - mets not likely from insulinoma (no large lesion,no calcifications,no liver lesions,no

peripancreatic LN’s)peripancreatic LN’s)

3) Metastatic pancreatic lesion (no primary visible, kidneys normal)3) Metastatic pancreatic lesion (no primary visible, kidneys normal)

4) Paraganglioma (not correct location)4) Paraganglioma (not correct location)

5) Other pancreas pathology,example pancreatitis (normal S-amylase)5) Other pancreas pathology,example pancreatitis (normal S-amylase)

ISLET CELL TUMORS Part of MEN I

MEN

• It is autosomal dominant conditions

• Characterized by 2 or more tumors (adenomas) in endocrine organs

• These organs come from the neural ridge

• These tumors, also called Apudomas, contain neuro-endocrine cells (APUD or Kulchitsky cells) – they produce diff. hormones

• Associated with hyperfunction and can be malignant

• Can occur alone (sporadic)

FEATURES OF MEN 1 (WERMER SYNDROME)

A) MAJOR DISEASE COMPONENTS = PPP

1) Parathyroid – hyperplasia or adenomas – 95% (common)

2) Pancreatic islet cell tumor – 40%

3) Ant. Pituitary tumor – 30%

B)ASSOCIATED TUMORS

1) Facial angiofibroma – 90%(common)

2) Adrenal cortical tumor – 40%

3) Foregut carcinoid – 3% e.g. thymus, bronchus, stomach

- Colon polyps, thymoma

Usually functional, small and multiple adenomas

In order of frequency

1) INSULINOMAS Hypoglycemia 10% Occur with gastrinomas 10% Malignant

2) GASTRINOMAS Associated with Zollinger Ellison Syndrome: (Presents with PU and diffuse stomach wall

thickening) and multiple duod. microgastrinomas 60% Can be become malignant

3) GLUCAGONOMAS DM and glossitis 80% Malignant

4) VIPOMA (Vasoactive intest peptide) WDHA syndrome (watery diarrhea, hypokalemia, achlorhydrin) 60% malignant

5) SOMATOSTATINOMA DM (block insulin) 80 % malignant

IMAGING - Choice

1) CT = MRIHypervascular – CE +

Signs of malignancy (must do follow up)• Large tumor• Ca⁺⁺• Hypervasc. mets to liver

2) NM• Octreoscan ¹¹¹ indium• Is somatostatin receptor. scan

- Blocks insulin and growth hormone

SONAR eg.• Transabdominal – Low sensitivity ( 70%) or

• Endoscopic – nearly 100% sensitivity, but invasive

• Intraoperative

Islet cell tumors are hypoechoic masses

Endoscopic a) in duod wall

b) In pancreatic body

Intraoperative c) in pancreatic body

Hypoecchoic lesion in pancreas on sonar

CT – 80% sensitive

• Most widely used for localization, local spread and liver involvement

• Hypervascular – will enhance (art phase)

• Large tumors and calcifications suggest malignancy

A - insulinomaB - non functional islet cell tumor

A B

MRI – Greater sensitivity than CT for small adenomas

• SOLITARY ADENOMA (PANCREAS)a) T₁ – low signalb) T₂ Fat-saturated – high signalc) T₁ with contrast – enhance

• MULTIPLE ADENOMAS (PANCREAS)d) T₂ high signal lesions

Nuclear medicine

SRS (Somatostatin receptor scintigraphy)

• Some islet cell tumors have these receptors

• It is a whole body technique

• Use indium

• gastrinomas will show increase uptake

PET

• Currently insufficient evidence for routine use (tumors have low metabolic rate)

PANCREATIC ANGIOGRAPHY• If no functional tumor is detected do:

Art stimulation with Ca⁺⁺ (will cause secretion of hormones – catheter

into splenic art and do venous sampling with catheter into R hepatic

vein and will get rise in hormone concentration

Octreotide scan with islet cell tumor and liver mets- high uptake

Thickened gastric wall – gastrinoma with Zollinger Ellison

Patient with prolactinoma – MEN 1 associated with multiple pancreas islet cell tumors, usually non functional

Further managementFurther management

Patient must be followed up, lesions may become malignant Patient must be followed up, lesions may become malignant (6 months initially)(6 months initially)

Exclude other features of MEN1 (PPP, colon Exclude other features of MEN1 (PPP, colon polyps,thymoma,carsinoid) – NM, colonoscopy / imaging ,MRI polyps,thymoma,carsinoid) – NM, colonoscopy / imaging ,MRI brain, bloodtests (HPT, Calsium)brain, bloodtests (HPT, Calsium)

Family screeningFamily screening

* For this case - Mediastinoscopy with biopsy to determine * For this case - Mediastinoscopy with biopsy to determine cause of mediastinal pathology ? Infective/ ? malignantcause of mediastinal pathology ? Infective/ ? malignant

REFERENCES

1. Brandt W E, Helms C A. Fundamentals Of Diagnostic Radiology 3 rd ed. Phicadelphia: Lippincott, 2006: 786-788, 147-148.

2. Adam A. Grainger and Allison's Diagnostic Radiology. Churchill Livingstone, 2008: 1719-1731.

3. Dähnert W. Radiology Review Manual 6th ed. Lippincott, 2007: 732-733.

4. Scarsbrook A F. Multiple Endocrine Neoplasia. Radiographics 2006; 26: 433-451.

5. Lewis R. Pancreatic Endocrine Tumors : Radiological Clinicopathologic Correlation. Radiographics 2010; 30 : 1445-1464.