intraoperatively or on pathologic examination
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Historical background
Appendiceal tumours can be broadly classified
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as:
? Epithelial
? Nonepithelial tumors
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Epithelial tumors
? Adenoma: mild-to-moderate atypia, mitosis, no
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stromal invasion, perforation with mucin
? Mucinous tumor of uncertain potential: adenoma
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with positive margin, mucin present within the wall? Mucinous tumor?low malignant potential: adenoma
with neoplastic cells in peritoneum
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? Adenocarcinoma: invasive mucinous tumor
Low grade appendiceal Mucinous Neoplasm
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Carcinoid tumor
Nonepithelial tumors
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Endocrine tumors? Classic appendiceal endocrine tumors
? Goblet cell carcinomas
Lymphoma
Sarcoma
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CarcinoidsMC located at the tip of the appendix
Two types:
? insular type
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? tubularManagement
? Tumors 1 cm: appendectomy.
? 1 cm to 2 cm (without involvement of the base of appendix):
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appendectomyRight hemicolectomy depends on grade, mitotic activity,
invasion of mesoappendix, or lymphovascular invasion.
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? 2 cm are at risk for lymph node or distant metastasis and a
right hemicolectomy is indicated.
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Epithelial tumorsPresentation:
? Incidental finding in the appendectomy specimen
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? Appendicitis? Pelvic mass
? Peritoneal carcinomatosis with or without ascites.
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DiagnosisBiochemistry
Chromogranin A can be used as tumour marker in appendiceal
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endocrine tumours and is useful to differentiate the tumour from gobletcel carcinoids. It is indicated in metastatic disease as a biochemical
parameter for follow-up
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CT scan
Histopathology
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StagingGrade of epithelium beyond appendiceal mucosa
Low-grade mucinous carcinoma
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High-grade mucinous carcinoma
Tumor stage
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T1: Tumor involves submucosa?
T2: Tumor invades muscularis propria
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?T3: Tumor invades subserosa or mesoappendix
?
T4a: Tumor penetrates serosa, including tumor in the right lower
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quadrant
Distant metastases
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?M1a: Intraperitoneal metastases beyond right lower quadrant
?
M1b: Extra-peritoneal metastases
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Pre operative CT scan
Treatment
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Surgery
? Appendiceal tumours can be cured by appendectomy if
the tumour is located at the tip of the appendix, the
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tumour diameter is <2 cm and no deep meso
appendiceal invasion is observed
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? Right hemicolectomy, is indicated if:? tumour diameter >2 cm
? deep mesoappendiceal invasion
? positive surgical margins
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Medical therapy? No medical therapy is indicated in patients with resected
appendiceal endocrine tumours.
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? Chemotherapeutic options are not available on an evidence-based level, nor are data to recommend peptide
radioreceptor therapy (PRRT).
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? PRRT may be an option in a somatostatin receptor-positive,
metastasized, inoperable appendiceal endocrine tumour.
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Systemic chemotherapy in appendicealtumor
? Preoperative (neoadjuvant)
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? Postoperative (adjuvant)
? Postoperative after suboptimal cytoreduction
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with residual bulky disease? Palliative in unresectable or progressive and
metastatic disease
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Minimal Consensus Statement on DiagnosticProcedures for Fol ow-Up
? For wel -differentiated tumours, diagnosed incidental y, with a
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maximum diameter < 1 cm and R0 resection, no follow-up is required.
? For wel -differentiated tumours of 1 to < 2 cm and R0 resection there
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are no sufficient data for a clear-cut decision.? In cases with deep mesoappendiceal infiltration or angioinvasion, CT
of the abdomen and somatostatin receptor scintigraphy may be
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performed.
? Factors believed to argue for follow-up investigations are a high
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proliferation marker, vascular involvement, deep mesoappendicealinfiltration, and possibly location at the base of the appendix