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Appendicular Tumors
Neoplasms of the appendix are not often
suspected before surgery and are found either
intraoperatively or on pathologic examination
Historical background
Appendiceal tumours can be broadly classified
as:
? Epithelial
? Nonepithelial tumors
Epithelial tumors
? Adenoma: mild-to-moderate atypia, mitosis, no
stromal invasion, perforation with mucin
? Mucinous tumor of uncertain potential: adenoma
with positive margin, mucin present within the wall
? Mucinous tumor?low malignant potential: adenoma
with neoplastic cells in peritoneum
? Adenocarcinoma: invasive mucinous tumor
Low grade appendiceal Mucinous Neoplasm
Carcinoid tumor
Nonepithelial tumors
Endocrine tumors
? Classic appendiceal endocrine tumors
? Goblet cell carcinomas
Lymphoma
Sarcoma
Carcinoids
MC located at the tip of the appendix
Two types:
? insular type
? tubular
Management
? Tumors 1 cm: appendectomy.
? 1 cm to 2 cm (without involvement of the base of appendix):
appendectomy
Right hemicolectomy depends on grade, mitotic activity,
invasion of mesoappendix, or lymphovascular invasion.
? 2 cm are at risk for lymph node or distant metastasis and a
right hemicolectomy is indicated.
Epithelial tumors
Presentation:
? Incidental finding in the appendectomy specimen
? Appendicitis
? Pelvic mass
? Peritoneal carcinomatosis with or without ascites.
Diagnosis
Biochemistry
Chromogranin A can be used as tumour marker in appendiceal
endocrine tumours and is useful to differentiate the tumour from goblet
cel carcinoids. It is indicated in metastatic disease as a biochemical
parameter for follow-up
CT scan
Histopathology
Staging
Grade of epithelium beyond appendiceal mucosa
Low-grade mucinous carcinoma
High-grade mucinous carcinoma
Tumor stage
T1: Tumor involves submucosa
?
T2: Tumor invades muscularis propria
?
T3: Tumor invades subserosa or mesoappendix
?
T4a: Tumor penetrates serosa, including tumor in the right lower
quadrant
Distant metastases
?
M1a: Intraperitoneal metastases beyond right lower quadrant
?
M1b: Extra-peritoneal metastases
Pre operative CT scan
Treatment
Surgery
? Appendiceal tumours can be cured by appendectomy if
the tumour is located at the tip of the appendix, the
tumour diameter is <2 cm and no deep meso
appendiceal invasion is observed
? Right hemicolectomy, is indicated if:
? tumour diameter >2 cm
? deep mesoappendiceal invasion
? positive surgical margins
Medical therapy
? No medical therapy is indicated in patients with resected
appendiceal endocrine tumours.
? Chemotherapeutic options are not available on an evidence-
based level, nor are data to recommend peptide
radioreceptor therapy (PRRT).
? PRRT may be an option in a somatostatin receptor-positive,
metastasized, inoperable appendiceal endocrine tumour.
Systemic chemotherapy in appendiceal
tumor
? Preoperative (neoadjuvant)
? Postoperative (adjuvant)
? Postoperative after suboptimal cytoreduction
with residual bulky disease
? Palliative in unresectable or progressive and
metastatic disease
Minimal Consensus Statement on Diagnostic
Procedures for Fol ow-Up
? For wel -differentiated tumours, diagnosed incidental y, with a
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
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
performed.
? Factors believed to argue for follow-up investigations are a high
proliferation marker, vascular involvement, deep mesoappendiceal
infiltration, and possibly location at the base of the appendix
This post was last modified on 08 April 2022