RIGHT ILIAC FOSSA

MASS: APPENDICULAR
CARCINOMA
TEAM FORSSMAN
Dr. Amanpreet Kaur JR3
Dr. Divyanti Mishra JR3
Dr. Aditi Solanki JR2
Dr. Aman Pnkaj JR2
Dr. Ashutosh JR1
Dr. Anmol JR1
Dr. Akhil JR1
ANATOMY OF APPENDIX

Most common type is Retrocecal

2nd most common position of appendix is
Pelvic
Retrocecal> pelvic> post ileal> pre ileal
•The appendix is a midgut organ and is first identified at 8 weeks of
gestation as a small outpouching of the cecum. As gestation progresses ,
the appendix becomes more elongated and tubular as the cecum rotates
medially and becomes fixed in the right lower quadrant of the
abdomen.

•The appendiceal mucosa is of the co-lonic type, with columnar

epithelium, neuroendocrine cells, and mucin-producing goblet cells
lining its tubular structure. Lymphoid tissue is found in the submucosa
of the appendix, leading
Because the appendix is an outpouching of the cecum, the flora within the
appendix is similar to that found within the colon. Infections associated
with appendicitis should be considered polymicrobial, and antibiotic
coverage should include agents that address the presence of both gram-
negative bacteria and anaerobes. Common isolates include Escherichia
coli, Bacteroides fragilis, enterococci, Pseudomonas aeruginosa, Klebsiella
pneumoniae, and others
BLOOD SUPPLY OF APPENDIX

Appendicular artery is a branch of ileo-colic

artery.
Accessory appendicular is a branch of
posterior caecal artery.
Accessory Appendicular Artery is a branch of
posterior caecal Artery.
 Histology
The appendix has abundant lymphoid tissue. There are very few crypts and the lamina propria
is the dominant feature of the mucosa.

The appendix is composed of:

1) Mucosa- columnar epithelium with neuroendocrine cells and mucin-producing goblet cells
lining its tubular structure. Neither villi nor plicae circularis are present.
Crypts - very few in number.

2) Lamina Propria - comprises almost the entire mucosa.

Nodules fill the lamina propria.
Plasma cells, lymphocytes, eosinophils and macrophages are present.

3) Submucosa - lymphoid tissue found

4) Muscularis Externa - two orthogonal layers of smooth muscle (inner circular and outer
longitudinal).

5) Serosa
 ' 5 Main histopathological subtypes

Non epithelial 1. Neuro endocrine neoplasm

tumors

2. Mucinous neoplasms

3. Goblet cell adenocarcinomas

Epithelial
tumors 4. Colonic type (non mucinous)
adenocarcinoma

5. Signet ring cell adeno carcinoma

 NEOPLASMS OF
APPENDIX
1). Neoplasms of the appendix are found in 1% of appendicectomy
specimens, with the vast majority being and incidental finding.

2). Most tumours involving the appendix may be classified as either

carcinoid or epithelial, with the latter group accounting for approximately
three quarters of all cases.
 Carcinoid Tumour ( Synonym
Argentaffinoma)
• Carcinoid tumours arise in argentaffin tissue ( Kulchitsky cells of the
crypts of Liberkuhn) and are most common in the vermiform appendix.

• MC site- GIT> Resp > Genitourinary

• Most common site of carcinoid tumour-

a). Midgut( M.C)- are Argentaffin cells ( Take up silver staining)
b). Hindgut- Argyrophilic cells ( take up silver staining but in reduced
form)
c). Foregut- Argyrophilic cells
• In GIT Appendix > Ileum> Rectum> Stomach

• Carcinoid tumour is found onces in every 300-400 appendicies

subjected to histological examination.

• The tumour can occur in any part of the appendix, but it is frequently
found in the distal third.
Carcinoid Tumour: Gross and on histology
• The neoplasm feels moderately hard and on sectioning the appendix, it
can be seen as a yellow tumour between the intact mucosa and the
peritoneum.

Microscopically- The tumour cells are small, arranged in small nests

within the muscle and have a characteristic pattern using
immunohistochemical stain for chromogranin B.
 Carcinoid syndrome
• Main symptoms- Pain, flushing and diarrhoea.
• Flushing is more common than pain and diarrhea
• Flushing is caused by serotonin. It is known as carcinoid crisis.
• In GIT we get carcinoid syndrome only when there is malignant
carcinoid with liver secondaries.
• Carcinoid causes niacin deficiency and thus plegra like skin patches
• Niacin deficiency occurs as the trypsin which has to make niacin,
forms inflammatory mediators instead of niacin.
TREATMENT OF CARCINOID TUMOUR
• Appendicectomy has been shown to be sufficient treatment unless the
caecal wall is involved.

• If the tumour is 2cm or more in size or involved lymph nodes are

found, right hemicolectomy is indicated.
EPITHELIAL TUMOURS OF THE
APPENDIX
• Epithelial neoplasms are found in 0.6% of appendicectomy specimens.
• Recently, following a modified Delphi consultation process, a group of
international experts proposed an updated classification system for
appendiceal epithelial neoplasms ( Carr et al., 2016)

• Tumours may be classified as mucinous or non mucinous (intestinal

type) and according to the degree of cytological atypia and
architectural features ( infiltrative versus pushing invasion )
Classification of epithelial neoplasia
Low grade appendiceal mucinous neoplasm
with mucocoele formation
Low- grade appendiceal neoplasm with
mucocoele formation that had redistributed
causing secondary low-grade mucinous
peritonei, mainly of the ovary.
 Primary MAN

W/o evidence of infiltrative (destructive) invasion Without evidence of infiltrative

invasion

Low grade High grade Mucinous appendiceal

adenocarcinoma

- *risk for lymph node

metastasis

i) moderately differentiated
ii) poorly differentiated, often
with signet ring cells
 Mucinous neoplasms

• >50% off tumors volume is composed of extra

cellular mucin
• may present as an unruptured mucin filled
appendices with peritoneal metastasis
• peritoneal spread is now
• Goblet cell carcinoid , a rare tumour of the appendix, which shows
both gland forming and neuroendocrine features, has now been
reclassified as goblet cell tumour and may be of a mucinous and non
mucinous subtype.

• The relevance of appendiceal epithelial tumours, particularly when of

the mucinous subtype, lies in their propensity to disseminate causing
the syndrome known as pseudomyxoma peritonei (PMP).
Management

Appendicectomy + right hemicollectomy- grade 2 and

3 Man/ + pre post op chemotherapy

Cytoreductive surgery with intra peritoneal for

pseudomyxoma pertonii
PSEDOMYXOMA PERITONEI
• PMP is a rare condition typified by progressive peritoneal tumour
deposits, mucinous ascites, omental cake and ovarian involvement in
females.
• The vast majority of cases arise as a result of perforation of a
mucinous appendiceal tumour. This association was first described by
Fraenkel in 1901.
• Patients typically present with progressive and massive abdominal
distension, anorexia and symptoms of bowel dysfunction. The
condition is invariably fatal without intervention.
• The overall risk of developing pseudomyxoma following removal of
an appendix harbouring epithelial tumour is approximately 9%, with
the risk varying according to the tumour subtype and the mode of
presentation.

• Following removal of a non mucinous neoplasm the risk of PMP is as

low as 3%, while it may ne as high as 30-50% in the case of a
mucinous adenocarcinoma of the appendix.

• PMP is classified according to the degree of cytological atypia within

the peritoneal deposits and its grading may differ from that of the
Pseudomyxoma peritonei
(PSOGI)/grade G1, well differentiated (AJCC 8th
edition); PMP with high-grade cytohistologic features
5 year survival rates = 91%

(PSOGI)/grade 2, moderately differentiated (AJCC

8th edition); and PMP with signet ring cells
5 year survival rates = 61%

(PSOGI)/grade 3, poorly differentiated (AJCC 8th

edition)
5 year survival rates = 23%
Treatment
1). Appendiceal epithelial tumour without PMP-

- In patients with an incidental finding of an epithelial neoplasm and no

current evidence of established PMP, subsequent treatment is dependent
dependent on the degree of cytological atypia within the primary tumour
and the estimated future risk of developing PMP.

- Low grade epithelial neoplasms have no evidence of mucin or

epithelial cells beyond the appendix.
Investigations
•1). Colonoscopy – to exclude associated colonic epithelial
lesions.
•2). Patients to be enrolled in surveillance protocol for atleast 5
years.
•3). Clinical review
•4). Annual low dose abdominalpelvic CT scan
•5). Appendix related tumour markers ( CEA, CA 199, CA
125)
TREATMENT OF APPENDICEAL EPITHELIAL
TUMOUR WITH ESTABLISHED PMP
• The standard accepted treatment for established PMP is cytoreductive
surgery (CRS) combined with heated intraperitoneal chemotherapy
HIPEC ( Hyperthermic intraperitoneal chemotherapy surgery).

• HIPEC surgery is a two step procedure that treats certain cancers in the
abdomen. Cancerous tumours are surgically removed, and then heated
chemotherapy drugs are applied directly inside the abdomen to
eliminate the remaining cancerous cells.
• HIPEC approach combines multiple peritonectomy procedures with
multvisceral resections as required to achieve a complete surgical
clearance of the tumour.

• The combined surgery may require total abdominopelvic

peritonectomy, greater and lesser omentectomy, bilateral salpingo-
oophorectomy, hysterectomy, cholecystectomy, splenectomy, partial
gastrectomy, colectomy and anterior resection of the rectum.
• Following a complete cytoreduction 5 and 10 year survival rates of
87% and 70%, can be achieved.

• Poor outcomes are seen in males, patients with elevated tumour

markers and following resection of tumour showing high- grade or
invasive features.