Case Presentation. Marianne Ellen Pavel. Charité University Medicine Berlin. ESMO Preceptorship on GI Neuroendocrine Tumors

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1 Case Presentation Marianne Ellen Pavel Charité University Medicine Berlin ESMO Preceptorship on GI Neuroendocrine Tumors Session 3; Singapore November 2,

2 Medical History 46-year-old man Referral to our hospital in 2008 with Recurrent nausea and abdominal pain since 09/07 Recurrent emesis Flushing episodes since January 2008 (once a week) Intermittently diarrhea Weight loss (about 34 kg in one year)

3 Additional Medical History Suspicion of Inflammatory Bowel Disease Diabetes mellitus Arterial hypertension Allergies: Penicillin Medications: Metformin Amlodipin/ Valsartan Simvastatin

4 Preliminary Medical History September 2008: Ileal segment resection for stenosis (CT finding) related to suspected Inflammatory Bowel Disease Incidental finding of neuroendocrine tumour: Well differentiated neuroendocrine tumour (nests of tumorous lesions growing in submucosa and muscularis) of the ileum with lymphangiosis carcinomatosa CgA++, Syn+; Ki-67 < 1%

5 Diagnostics Performed: Imaging 10/08 Imaging Performed Ultrasonography (abdomen) Findings Two liver lesions (segm. VIII) Steatosis hepatis CT abdomen Confirmation of multiple liver metastases segm. VIII and IVb (up to 2 cm) Suspected mesenteric lymph node metastases

6 CT Imaging in 10/2008 Prof Pavel

7 What Other Diagnostic Tests Would You Perform? A. 111 In-Octreoscan B. 68-Ga-DOTATOC- PET/CT C. Assess biochemical markers (CgA, 5-HIAA) D. Magnetic resonance imaging

8 Additional Diagnostics Analysis 10/08 Procedure Laboratory Analysis Echocardiography Findings Chromogranin A (plasma): < 48.0 ng/l (ref. value ng/l) 5-HIAA (24h-urine): 55.0 µmol/die (ref. value 10.4 to 47.1 µmol/die) No signs of cardiac heart disease 111 In-Octreoscan Suspected mesenteric lymph node metastases Single nuclide accumulation in the liver Segm 8 Nuclide accumulation in the thorax Magnetic resonance imaging Known hepatic metastases

9 Prof Pavel 111 In-Octreoscan 10/2008 Differential diagnosis? Preferred imaging for further evaluation CT Thorax 68Ga-DOTATOC-PET/CT Bone scintigraphy MRI of the thorax Other Department of Nuclear Medicine, Charité University Medicine

10 Prof Pavel 111 In-Octreoscan 10/2008 Cardiac metastasis in the interventricular septum Department of Nuclear Medicine, Charité University Medicine

11 What Would You Do with the Myocardial Metastasis? A. Watch and Wait B. Resection if symptomatic C. Resection without symptoms

12 Treatment What We Did: Wait and Watch On agreement with the German Heart Center Berlin (DHZB) resection echocardiography only if significant symptoms 1. Myocardial Metastasis is close to the coronary arteries 2. High risk of septum defect No clear indication for surgical resection of metastatic disease (R0) You can see it by echocardiography if you expect it

13 Treatment What We Did: Somatostatin analogs Date Disease Status Treatment 11/2008 Initial diagnosis and symptoms controlled Octreotide LAR therapy 20 mg/mo 04/2009 Stable Disease 11/2009 Stable Disease 05/2010 Stable Disease 11/2010 Stable Disease 05/2011 Stable Disease Octreotide LAR therapy 20 mg/mo

14 Stable Disease by MRI: Abdomen 11/09 and 11/11 11/09 11/11 (Primovist) Last follow-up 07/2012- stable disease

15 Summary of case Unusual presentation of intestinal NET mimicking Crohns disease Rare cardiac disease site depicted by all methods including echocardiography, CT thorax, SRS, 68Ga- DOTATOC-PET/CT, but first discovered by octreoscan

16 Detection of primary tumor and rare cardiac metastases 41 patients + 4 cases (own series) Mean age at Dx 57.5 yrs Females: 13, Males: 28 Primary tumor sites were - foregut in 7 - midgut in 28 - hindgut in 1 - CUP in 3 Carcinoid syndrome: 28 pts Diagnosis by -Echocardiography: 21, CT / MRI: 12, Other: 9 - Autopsy: 9 Jann et al HMR 2010 Cardiac involvement was right-ventricular only in 10 left-ventricular only in 11 biventricular in 10

17 Take home messages Clinicians treating patients with NET should be aware of the heart as a possible site of metastatic disease Somatostatin receptor imaging, preferably by PET/CT or SPECT/CT may contribute to earlier and more frequent detection MRI and echocardiography are sensitive methods for detection and preferred methods for follow-up of cardiac lesions Accurate assessment of metastatic lesions has an impact on therapeutic decision making

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