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Appendiceal Neuroendocrine Neoplasms (ANENs)

Appendiceal Neuroendocrine Neoplasms (ANENs). Krystallenia Alexandraki Endocrinologist. Endocrine Department , Pathophysiology Clinic , “Laikon” Hospital , National and Kapodistrian University of Athens. NEN of the Appendix (ANEN)- Epidemiology. Incidence 0.15 (SEER)/ 10 6 / yr

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Appendiceal Neuroendocrine Neoplasms (ANENs)

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  1. AppendicealNeuroendocrineNeoplasms (ANENs) Krystallenia Alexandraki Endocrinologist Endocrine Department, Pathophysiology Clinic, “Laikon” Hospital, National and Kapodistrian University of Athens

  2. NEN of the Appendix (ANEN)- Epidemiology Incidence 0.15 (SEER)/ 106/ yr 0.4-0.6/106/ yr Incidence of appendectomies - 3-9/1000 procedures ENETS Consensus, 2012

  3. Patient 1, A. K. 27 year-old woman, previously in good health 2/2004 - abdominal pain Appendicectomy: Small tumor noted at base of appendix

  4. 1.1 cm WDNEN. penetrates the serosa Do not extend into the mesoappendiceal fat Not evidence of angioinvasion Ki-67 < 1%. Tumour close to the surgical margins Chromogranin Patient 1, A.K. - histopathology

  5. Patient 2, F. S. 25 year-old woman, previously in good health. 7/05- abdominal pain Appendicectomy: Small tumor noted at tip of appendix

  6. 1.2 cm WDNEN. the tumor penetrates through all layers of the appendix and and invades the mesoappendiceal fat. Ki-67 = 1% Surgical margins - free of tumor. immunohistochemistry: CgA (+)ve, NSE (+)ve, συναπτοφυσίνη(+)ve, CD56 (+)ve Tumor Meso Mesoappendiceal invasion Mesoappendiceal invasion Patient 2, F.S. - histopathology

  7. Patient 3, K. T. 19 year-old woman, previously in good health. 7/06- chronic abdominal pain Appendicectomy: Small tumor noted at the base of appendix and close to the surgical margins

  8. 0.8 cm WDNEN. the tumor penetrates through all layers of the appendix and the muscular wall. Low Ki-67 (few cells) Surgical margins - free of tumor. immunohistochemistry: CgA (+)ve, NSE (+)ve, Synaptophysin (+)ve, CD56 (+)ve Chromogranin Patient 3, K.T. - histopathology

  9. Patients A. K. - F. S. K.T.:What next? Nothing? Right hemicolectomy?

  10. Histopathology characteristics Feature A. K. F. S. Size (cm.) 1.1 1.2 Penetration to serosa Yes Yes Mesoappendiceal involvement no Yes lymph/angioinvasion no No Perineural involvement No no Surgical margins close Free 1 Ki-67 index (%) 1 K. T. Appendix) base Tip base 0.8 Yes no no no close few cells Alexandraki, J Endocrinol Invest 2011

  11. “Probability of metastasis in appendiceal NET is related to tumor size” Histopathology Characteristics in Presented Patients: Tumor Size <10% 5-25% > 2 cm <1 cm 1-2 cm Less clear-mets in 10% or none % metastasis: ~0 25-40% ? No Further surgery: Rt. Hemicolectomy Cured by simple appendicectomy; exception: base/ MAI>3mm /incomplete resection

  12. Histopathology Characteristics in Presented Patients: Penetration to Serosa • “Serosa involvement is demonstrated to be present in up to 70% of all malignant NETs, but is judged to be unrelated to outcome in the published literature”, Stinner and Rothmund, 2005. • “Invasion of the serosa is not correlated with lymph node metastasis and has no impact on survival”, ENETS Consensus, 2008.

  13. Histopathology Characteristics in Presented Patients: Localisation • “Lesions at the base of the appendix are more likely to produce local recurrence than those at the tip if treated only by simple appendicectomy”, Sutton, 2003. • “Incomplete resection after appendicectomy mets NEN at the base: more aggressive therapy in NEN 1-2cm in size”, ENETS Consensus, 2012.

  14. Histopathology Characteristics in Presented Patients: Mesoappendiceal invasion (MAI) • “in up to 20% of adults and 40% of children’ Rossi, 2003 • “The depth of invasion beyond 3 mm reflects the aggressiveness” ENETS Consensus, 2012.

  15. Histopathology Characteristics in Presented Patients: Additional criteriaKi-67 index • “The role of proliferation markers such as Ki-67 and mitotic activity is not precisely defined for appendiceal NETs….it might justified to use these items even for NETs of the appendix regarding the basic biological principles of proliferating tissue, although this reflects a very low level of evidence”, Stinner and Rothmund, 2005 • “Additional criteria such as ki-67 of ≥3% or angioinvasion aid decision making”, ENETS Consensus, 2012

  16. A. K.: 0/36 LNs – persistent disease in proximity of the primary F. S.: 1/10 LN-metastatic NEN K.T.: 1/14 LN-metastatic NEN Histopathology after RHC Tumor LN H & E SYN

  17. Follow-up • K. T.: 25 yrs old • Post-operative complication:ileus – re-operated • Abdominal MRI every 2-3 yrs- 8 yrs free of - A. K.-34 yrs old • Post-operative complication: ileus – re-operated • abdominal CT and MRI 10 years later free of disease- 1 full-term pregnancy F. S.:32 yrs old • No post-operative complications • Abdominal ultrasound and abdominal CT every 5 yrs- 9 yrs free of disease- nulliparous

  18. BIOCHEMICAL PROFIL

  19. 5-HTP PET SCAN A.K. (-)ve Indium-111-pentetreotide scintigraphy- A.K. False (+)ve result

  20. Three patients summary & Aims: • Three patients with Appendiceal NENs had: • “Grey Zone” tumors with respect to size • Mesoappendiceal invasion (MAI) and base localisation • Regional lymph node metastasis and residual disease • Patients with MAI and 1-2cm in max diameter might have a higher risk of local metastasis than previously considered. • ANEN <1cm, simple appendicectomy is curative and sufficient; only exception NEN at the base of the appendix and incompletely resected or MAI 3 mm

  21. Acknowledgments Thank you Neuroendocrine Tumor Unit Team, Endocrinology & Metabolism Service, Department of Medicine, Hadassah-Hebrew University Medical Center,  Jerusalem, Israel Department of Surgery, Shaare Zedek Medical Center, Jerusalem, Israel Prof. P. Reissman Prof. D. J. Gross Dr S. Grozinsky-Glasberg Mrs. D. Barak

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