Dylan Carney, HMS III
Gillian Lieberman, MD
Giant Mucinous
Adenocarcinoma
of the Appendix
with disseminated peritoneal adenomucinosis
Dylan Carney, Harvard Medical School Year III
Gillian Lieberman, MD
January 2011
Dylan Carney, HMS III
Gillian Lieberman, MD
Overview
•
Case presentation
–
Patient presentation
–
Radiographic findings
–
Surgical procedure
–
Pathology findings
•
Neoplasms
of the appendix
–
Histologic
types
–
Menu of radiographic tests
–
Treatment and outcomes
•
Pseudomyxoma
peritonei
–
Radiographic findings
2
Dylan Carney, HMS III
Gillian Lieberman, MD
Case Presentation
•
Our patient is a previously-healthy 48 y.o. male who
presents with a 4-day history of focal, intermittent
RLQ abdominal pain that is non-radiating
•
No nausea, vomiting, fever, chills, melena,
hematochezia, hematemesis, association with PO
intake
•
Physical notable for normal vital signs, normal bowel
sounds, guarding in RLQ, tenderness to palpation
with palpable mass in RLQ
•
Labs notable for leukocytosis
of 13.9
3
Dylan Carney, HMS III
Gillian Lieberman, MD
The patient goes for urgent CT abdomen with
and without contrast…
4
Dylan Carney, HMS III
Gillian Lieberman, MD
Our patient’s CT abdomen: what do you see?
5
PACS, BIDMC Coronal CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
Out pt: appendiceal
mass
6
Cecum is displaced
medially, superiorly,
and rotated
Extension of fluid
into inguinal canal
Large bowel is
constrast‐filled
throughout with air
distal to lesion, and is
of normal to mildly‐
dilated caliber
Large, non‐enhancing
cystic mass
with rim
of enhancement
extending from
appendix –
indicating
pathology is likely
appendiceal
in origin
PACS, BIDMC Coronal CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
What do you see in this coronal section?
7
PACS, BIDMC Coronal CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
Out pt: mucinous
neoplasm
8
Cecum adjacent to
cyst
Large bowel with
contrast and air
throughout
Cystic mass is 25HU,
denser than simple
fluid and less dense
than blood –
consistent with
mucous. It is also
multi‐lobed
PACS, BIDMC Coronal CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
What do you see in this axial CT section?
9
PACS, BIDMC Axial CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
Out pt: rim calcifications and free mucus
10
Cystic mass with rim
of enhancement and
calcifications. This
portion of the mass is
intraperitoneal,
anterior the the
anterior perirenal
space, medial to the
liver and lateral to
the ascending colon
Free mucus in the
peritoneal cavity
consistent with
rupture
Note the absence of
frank ascites
or
psuedomyxoma
peritonei
“jelly
belly”, vasculature
non‐compressed
PACS, BIDMC Axial CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
What spaces is the pathology in?
11
PACS, BIDMC Axial CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
Our pt: retroperitoneal extension
12
Free mucus in the
peritoneal cavity
consistent with
rupture
Multi‐lobed cystic
mass with rim of
enhancement and
pockets of air,
indicating rupture
into intestinal lumen
(v. emphysematous
infection). Note that
the posterior lobe of
the mass is in the
anterior perirenal
space, adjacent to
the psoas.
PACS, BIDMC
Dylan Carney, HMS III
Gillian Lieberman, MD
How is the mass different on this lesion?
13
PACS, BIDMC Axial CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
Our pt: heterogeneous mucocele
14
Mucocele
displays
heterogeneity at this
level. The lobes here
are also fused and
invading the
retroperitoneum.
Thus, the mass is
singular and
continuous.
The mucocele
is now
directly abutting the
psoas.
PACS, BIDMC Axial CT + C
Dylan Carney, HMS III
Gillian Lieberman, MD
Differential Diagnosis
•
At this point it is narrow, and includes:
–
Appendiceal
neoplasm with or without pseudomyxoma
peritonei
–
Appendicitis with abscess
15
Dylan Carney, HMS III
Gillian Lieberman, MD
The patient is brought to the OR for tumor
debulking.
Let’s look at the intraoperative
findings…
16
Dylan Carney, HMS III
Gillian Lieberman, MD
Our pt: visceral mucosal implants
17
Mucosal Implants
Courtesy of Dr. James Hurst, Acute Care Surgery
Dylan Carney, HMS III
Gillian Lieberman, MD
Our pt: resection cavity
18
Resection cavity
Courtesy of Dr. James Hurst, Acute Care Surgery
Dylan Carney, HMS III
Gillian Lieberman, MD
19
Ascending Colon
Mucocele
Courtesy of Dr. James Hurst, Acute Care Surgery
Gross Specimen: mucocele
removed en bloc with ascending
colon and transverse colon
Dylan Carney, HMS III
Gillian Lieberman, MD
Mucocele, ileum, ascending, transverse colon
and mucosal implants are sent to pathology.
Let’s see the findings…
20
Dylan Carney, HMS III
Gillian Lieberman, MD
Histology: infiltrating glandular tissue
Infiltrating glandular
tissue
Courtesy of Dr. Robert Najarian
and Dr. Shu‐Hsien
Sheu, BIDMC Pathology 21
Dylan Carney, HMS III
Gillian Lieberman, MD
Histology: neoplastic
epithelium
Neoplastic
epithelium with enlarged,
hyperchromatic
nuclei, numerous mitotic
figures, abundant intraluminal
mucin
Courtesy of Dr. Robert Najarian
and Dr. Shu‐Hsien
Sheu, BIDMC Pathology 22
Dylan Carney, HMS III
Gillian Lieberman, MD
Histology: Pools of Mucin
Pools of mucin
outside lumen
Courtesy of Dr. Robert Najarian
and Dr. Shu‐Hsien
Sheu, BIDMC Pathology 23
Dylan Carney, HMS III
Gillian Lieberman, MD
Pathology Report: Mucinous
Adenocarcinoma
•
Well-differentiated mucinous
adenocarcinoma
of
the appendix
•
0 of 18 lymph nodes involved
•
Ileum through transverse colon normal with clear
margins
•
Acellular
mucosal studding along serosal
surface
consistent with disseminated peritoneal
adenomucinosis
24BIDMC Pathology
Dylan Carney, HMS III
Gillian Lieberman, MD
The patient recovered well post-operatively
and was discharged on hospital day #6.
He was seen in clinic 2 weeks later and
reported resolution of symptoms with minimal
residual pain.
25
Dylan Carney, HMS III
Gillian Lieberman, MD
Let’s learn more about neoplasms
of the
appendix…
26
Dylan Carney, HMS III
Gillian Lieberman, MD
Appendiceal
Neoplasms
•
Common presentations: abdominal pain, appendicitis,
ascites, obstruction
•
Incidental finding on 1% of appy
specimens
•
Overall incidental 0.12 cases / 1M pt-years
•
Diagnosis
–
Contrast-enhanced CT (gold standard) –
shows non-enhancing,
mucous-filled, rim-enhancing lesion of appendix (because most
patients present with appendicitis, this is usually the test of choice)
–
Plain films –
may see air-fluid level if there is rupture, or mass effect
on nearby organs
–
Barium enema –
cecal
impaction, non-filling of appendix
–
U/S –
layering (“onion skin”), nodular mural thickening indicates
malignancy (indicated in children, pregnant women)
27
Cerame
MA. A 25‐year review of adenocarcinoma
of the appendix. A frequently perforating carcinoma. Dis
Colon Rectum 1988;31:145‐50.
Connor SJ, Hanna GB, Frizelle
FA. Appendiceal
tumors: retrospective clinicopathologic
analysis of appendiceal
tumors from 7,970 appendectomies. Dis
Colon Rectum 1998;41:75‐80.
Ito H, Osteen RT, Bleday
R, Zinner
MJ, Ashley SW, Whang
EE. Appendiceal
adenocarcinoma: long‐term outcomes after surgical therapy. Dis
Colon Rectum 2004;47:474‐80.
Dylan Carney, HMS III
Gillian Lieberman, MD
CT Appearance of Appendiceal
Mucoceles
Compared to Acute Appendicitis
•
Findings in appendicitis and small appendiceal
mucoceles
often overlap overlap, making preoperative
diagnosis of mucoceles
tricky
•
CT features found to be associated with mucoceles
–
Cystic dilation of the appendix
–
Mural calcification
–
Luminal diameter > 1.3cm (71% sensitive and 95% specific)
•
Non-specific CT findings: appendicolith, fat stranding,
periappendiceal
fluid, lymphadenopathy
28Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Dylan Carney, HMS III
Gillian Lieberman, MD
CT Findings: Appendicitis with
and without Mucocele
29Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Dylan Carney, HMS III
Gillian Lieberman, MD
Let’s see some other examples of appendiceal
mucoceles
30
Dylan Carney, HMS III
Gillian Lieberman, MD
Companion Patient 1: Mucocele
with Air
and Mural Calcifications
31Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Air
Calcifications
Dylan Carney, HMS III
Gillian Lieberman, MD
Companion Patient 2: Mural Calcifications
32Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Calcifications
Dylan Carney, HMS III
Gillian Lieberman, MD
Companion Patient 3: Mural Calcifications
and Fat Stranding
33Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Fat Stranding
Calcifications
Dylan Carney, HMS III
Gillian Lieberman, MD
Companion Patient 4: Fat Stranding
34Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Fat Stranding
Dylan Carney, HMS III
Gillian Lieberman, MD
Companion Patient 5: Fat Stranding
35Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Fat Stranding
Dylan Carney, HMS III
Gillian Lieberman, MD
Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Companion Patient 5: Appendicolith
36
Appendicolith
Dylan Carney, HMS III
Gillian Lieberman, MD
Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis. AJR Am J Roentgenol
2009;192:W103‐10.
Companion Patients 5: Appendicitis!
37
These last two are actually examples of
acute appendicitis with appendicolith
–
there is no mucocele!
Dylan Carney, HMS III
Gillian Lieberman, MD
Appendiceal
Neoplasms: Histologic
Types
•
Histologic
types
–
Carcinoid
–
Epithelial (appendiceal
mucoceles)
•
Mucosal hyperplasia (20%)
•
Simple cyst (18%)
•
Mucinous
adenoma (10%)
•
Mucinous
adenocarcinoma
(52%)
–
Other (Signet Ring, lymphoma)
38
Higa
E, Rosai
J, Pizzimbono
CA, Wise L. Mucosal hyperplasia, mucinous
cystadenoma, and mucinous
cystadenocarcinoma
of the appendix. A re‐evaluation of appendiceal
"mucocele". Cancer 1973;32:1525‐41.
Isaacs KL, Warshauer DM. Mucocele of the appendix: computed tomographic, endoscopic, and pathologic correlation. Am J Gastroenterol. 1992;87(6):787‐9.
Landen
S, Bertrand C, Maddern
GJ, et al. Appendiceal
mucoceles
and pseudomyxoma
peritonei. Surg
Gynecol
Obstet
1992;175:401‐4.
Qizilbash AH. Mucoceles of the appendix. Their relationship to hyperplastic polyps, mucinous cystadenomas, and cystadenocarcinomas. Arch Pathol. 1975;99(10):548‐55.
Dylan Carney, HMS III
Gillian Lieberman, MD
Complications of Appendiceal
Neoplasms
•
“Mucocele”
encompasses any mucinous
epithelial
neoplasm of the appendix, from benign to malignant
•
Complications
–
Bowel obstruction
–
Perforation
–
Infection
–
Pseudomyxoma
Peritonei
(PMP)
•
Disseminated peritoneal mucinous
tumors arising from perforation of
true appendiceal
neoplasms
(adenoma or adenocarcinoma) and
subsequent omental/peritoneal implantation of well-differentiated
mucin-secreting epithelium
•
Implantation occurs in a gravity-dependent fashion: the pouch of
Douglas, retrohepatic
space, paracolic
gutters, and the fossa
created
by ligament of Trietz
39
Sugarbaker PH, Ronnett BM, Archer A, Averbach AM, Bland R, Chang
D, Dalton RR, Ettinghausen SE, Jacquet P, Jelinek J, Koslowe P,
Kurman RJ, Shmookler B, Stephens AD, Steves MA, Stuart OA, White S, Zahn CM,
Zoetmulder FA. Pseudomyxoma peritonei syndrome. Adv Surg. 1996;30:233‐80.
Dylan Carney, HMS III
Gillian Lieberman, MD
Appendiceal
Mucocele:
Treatment and Outcomes
•
Debate over appendectomy versus hemicolectomy,
with trend towards hemicolectomy
•
Andersson
et al, 1976
–
Of 7 cases of mucinous
adenocarcinoma, 6 were treated with
appendectomy alone, 5 of whom survived beyond 5 years
•
Edmonds et al, 1984
–
Of 10 cases, 5-year survival was significant longer with right
hemicolectomy
versus appendectomy (60% versus 20%)
•
Nitechki
et al, 1994
–
Of 94 cases, 5-year survival with right hemicolectomy
was 68%
versus 20% with appendectomy alone.
–
Secondary right hemicolectomy
led to tumor downstaging
and
improved outcomes
40
Andersson
A, Bergdahl
L, Boquist
L. Primary carcinoma of the appendix. Ann Surg
1976;183:53‐7.
Edmonds P, Merino MJ, LiVolsi
VA, Duray
PH. Adenocarcinoid
(mucinous
carcinoid) of the appendix. Gastroenterology 1984;86:302‐9.
Nitecki
SS, Wolff BG, Schlinkert
R, Sarr
MG. The natural history of surgically treated primary adenocarcinoma
of the appendix. Ann Surg
1994;219:51‐7.
Dylan Carney, HMS III
Gillian Lieberman, MD
What about pseudomyxoma
peritonei?
Let’s see some examples…
41
Dylan Carney, HMS III
Gillian Lieberman, MD
Companion Patient 6: Pseudomyxoma
Peritonei
•
Non-contrast CT of the
abdomen showing large
cavity with an air fluid
level (indicating frank
rupture), compressing
the small and large
bowel
42Naidu LS, Jobling
JC. Chronic colonic perforation in pseudomyxoma
peritonei. Br J Radiol
2007;80:e94‐7.
Dylan Carney, HMS III
Gillian Lieberman, MD
Companion Patient 7: Pseudomyxoma
Peritonei
•
Contrast-enhanced CT
showing abundant
intraperitoneal
mucin
with non-enhancing,
fluid-filled cysts
scalloping the liver and
spleen
43Naidu LS, Jobling
JC. Chronic colonic perforation in pseudomyxoma
peritonei. Br J Radiol
2007;80:e94‐7.
Dylan Carney, HMS III
Gillian Lieberman, MD
Pseudomyxoma
Peritonei
-
Treatment
•
Systemic and intraperitoneal
chemotherapy
•
Debulking
•
Peritonectomy
•
Outcomes –
typically poor
–
25% 5-year survival
–
Mortality frequently secondary to intestinal or renal obstruction
44Landen
S, Bertrand C, Maddern
GJ, et al. Appendiceal
mucoceles
and pseudomyxoma
peritonei. Surg
Gynecol
Obstet
1992;175:401‐4.
Dylan Carney, HMS III
Gillian Lieberman, MD
Summary
•
Appendiceal
tumors are rare gastrointestinal neoplasms
•
Histological subtypes include carcinoid, epithelial (hyperplasia, cystic,
mucinous
adenoma and mucinous
adenocarcinoma), signet ring and
lymphomas
•
Patients frequently mimic acute appendicitis and tumors are frequently
discovered on CT, intraoperatively, or via postoperative pathology
•
Complete debulking, usually with right hemicolectomy, should be
performed with care to avoid rupture
•
Rupture of adenomas and adenocarcinoma
can lead to peritoneal
implants and subseuqent
pseudomyxoma
peritonei
(PMP)
•
PMP is treated with debulking, intraperitoneal
chemotherapy or
peritonectomy, and overall 5-year prognosis is poor
45
Dylan Carney, HMS III
Gillian Lieberman, MD
Bibliography
•
Andersson
A, Bergdahl
L, Boquist
L. Primary carcinoma of the appendix. Ann Surg
1976;183:53-7.
•
Bennett GL, Tanpitukpongse
TP, Macari
M, Cho KC, Babb JS. CT diagnosis of mucocele
of the appendix in patients with acute appendicitis.
AJR Am J Roentgenol
2009;192:W103-10.
•
Cerame
MA. A 25-year review of adenocarcinoma
of the appendix. A frequently perforating carcinoma. Dis
Colon Rectum 1988;31:145-50.
•
Connor SJ, Hanna GB, Frizelle
FA. Appendiceal
tumors: retrospective clinicopathologic
analysis of appendiceal
tumors from 7,970
appendectomies. Dis
Colon Rectum 1998;41:75-80.
•
Edmonds P, Merino MJ, LiVolsi
VA, Duray
PH. Adenocarcinoid
(mucinous
carcinoid) of the appendix. Gastroenterology 1984;86:302-9.
•
Higa
E, Rosai
J, Pizzimbono
CA, Wise L. Mucosal hyperplasia, mucinous
cystadenoma, and mucinous
cystadenocarcinoma
of the appendix. A
re-evaluation of appendiceal
"mucocele". Cancer 1973;32:1525-41.
•
Isaacs KL, Warshauer
DM. Mucocele
of the appendix: computed tomographic, endoscopic, and pathologic correlation. Am J Gastroenterol.
1992;87(6):787-9.
•
Ito H, Osteen RT, Bleday
R, Zinner
MJ, Ashley SW, Whang
EE. Appendiceal
adenocarcinoma: long-term outcomes after surgical therapy. Dis
Colon Rectum 2004;47:474-80.
•
Landen
S, Bertrand C, Maddern
GJ, et al. Appendiceal
mucoceles
and pseudomyxoma
peritonei. Surg
Gynecol
Obstet
1992;175:401-4.
•
McCusker
ME, Cote TR, Clegg LX, Sobin
LH. Primary malignant neoplasms
of the appendix: a population-based study from the surveillance,
epidemiology and end-results program, 1973-1998. Cancer 2002;94:3307-12.
•
Naidu LS, Jobling
JC. Chronic colonic perforation in pseudomyxoma
peritonei. Br J Radiol
2007;80:e94-7.
•
Nitecki
SS, Wolff BG, Schlinkert
R, Sarr
MG. The natural history of surgically treated primary adenocarcinoma
of the appendix. Ann Surg
1994;219:51-7.
•
Qizilbash
AH. Mucoceles
of the appendix. Their relationship to hyperplastic
polyps, mucinous
cystadenomas, and cystadenocarcinomas. Arch
Pathol. 1975;99(10):548-55.
•
Sugarbaker
PH, Ronnett
BM, Archer A, Averbach
AM, Bland R, Chang D, Dalton RR, Ettinghausen
SE, Jacquet
P, Jelinek
J, Koslowe
P,
Kurman
RJ, Shmookler
B, Stephens AD, Steves
MA, Stuart OA, White S, Zahn
CM, Zoetmulder
FA. Pseudomyxoma
peritonei
syndrome. Adv
Surg. 1996;30:233-80.
•
Sugarbaker
PH. The natural history, gross pathology, and histopathology of
appendiceal
epithelial neoplasms. Eur
J Surg
Oncol
2006;32:644-7.
•
Tsai CJ. Ultrasound features of disseminated adenomucinosis
(pseudomyxoma). Br J Radiol
1998;71:564-6.
46
Dylan Carney, HMS III
Gillian Lieberman, MD
Acknowledgements
47
Giant Mucinous Adenocarcinoma of the Appendix�with disseminated peritoneal adenomucinosis
Overview
Case Presentation
The patient goes for urgent CT abdomen with and without contrast…
Our patient’s CT abdomen: what do you