Case Report and Review

14 year old male presented as a case

of acute appendicitis and found to
have Non Hodgkin B cell Lymphoma
of the appendix
Dr. Nayef Al-Abdallat , Dr. Mahmoud Nimer ,
Dr. Omar Suleiman
 History of Presenting Illness

 A 14-year-old boy, previously medically free

and surgically free
 Arrived to our emergency room at Zarqa
New Govermental Hospital complaining of
right lower quadrant abdominal pain since 3
days

The pain has been on and off since about
one month ,but increased in severity and
became constant in the last 3 days
 History of Presenting Illness

The pain was associated with anorexia and
nausea
 Pain was felt centrally around the umbilicus most
of the time , but in the last few days the pain is felt
more in the right iliac fossa

No weight loss , no night sweats

No urinary symptoms

Constipation in the last 3 days .

In the last 3 days pain was not relieved by taking
Acetaminophen or NSAIDS
 Physical Exam

Patient was pale and tired , in pain
 Vital signs on arrival to ER
HR:95 BP:100/60 RR:13 Temperature:38 °C
 Abdominal Exam : Soft Abdomen with
+ RIF Tenderness , + Rebound Tenderness
+ Rovsing Sign
 Other Systems Exam:
Non remarkable findings
No evidence of lymphadenopathy
 Investigations & Imaging


CBC : Leuckocytosis with white cell count of
16000/mm3 with 80% shift to neutrophils .

Chemistry And Urine Analysis : Within
normal ranges

CXR : Normal

Abdominal Xrays : non significant isolated
air-fluid levels

Abdominal US: non peristaltic appendix ,
with edematous wall
 Management
 Our working diagnosis was : Acute Appendicitis
 Patient was admitted , Kept NPO , Given fluid
resuscitation
 The patient was taken to OR after consenting the
father for an open appendectomy through
traditional mcburney incision
 Operative Findings
 The appendix appeared enlarged, phlegmonous,
not perforated but with gangrene at the tip.
 Multiple adhesions with the ileum and cecum
 A 4 cm mass occupying the area between
terminal ileum and proximal cecum , not causing
obstruction
 Appendectomy was done , Mass left in place
 Post operative Period

1- Patient had smooth post operative recovery

2- Upper and lower endoscopy : Normal
3- CT scan : No masses found other than ileocecal
mass
4- Blood Film : Normal
5- Tumor markers : Not elevated
6- Patient was discharged on the fifth post operative
day pending for the histopathology results
 Histopathology

The histopathology report revealed :

- Acutely inflamed appendix
- Presence of blastic lymphoid tissue in the wall of
the appendix
- Lymphocyte B polyclonal pattern identified going
with :
Non Hodgkin B cell Lymphoma.
 After Histopathology

- PET Scan did not show metastatic deposits in

other parts of the body , with increased uptake in
the ileocecal area
- Raised LDH (lactate dehydrogenase) levels at 253
units/l (118-225 IU/l)
-Raised beta2 microglobulins at 6mg/l (1.1-2.8
mg/l).
 Decision

After discussing the case with oncology team , and

reviewing all the investigations and the images , a
Decision was made to :

1- Not to proceed to Right hemicolectomy

2- Refer the patient for Chemotherapy

 Previous Experience
Previous data from ZNGH in the past 5 years , Shows
other four cases with the following findings :
- The four  patients were between 12 and 66 years
old (mean age, 43 years).
- Three of the patients were men, and one was a
woman.
- Two patients presented with symptoms—acute right
lower quadrant pain and a fever—that were
diagnosed clinically as acute appendicitis.
 Previous Experience

- One patient presented with lower gastrointestinal

bleeding.
- And one patient presented with symptoms of a fever
and rigors that were diagnosed preoperatively as a
pelvic abscess.
- Three patients underwent only appendectomy.
- A primary right hemicolectomy was performed in one
patient.
- Non-Hodgkin's lymphoma was a new diagnosis in all
four patients.
 Discussion
- Cancer of the appendix is very rare and is typically
found incidentally on histopathology in
approximately 1% of appendectomies.
- Carcinoids are the most common 66%, cyst-
adenocarcinoma 20% , adenocarcinoma 10%.
- Then there are the rare forms of cancers which
include adenocarcinoid, signet ring, non-Hodgkin’s
lymphoma, ganglioneuroma
- The incidence of primary appendiceal lymphoma is
0.015% of appendectomy specimens
 Discussion
- The gastrointestinal tract is the most common site
for extranodal lymphoma.
- The stomach is the most common, followed by the
small intestine.
- Lymphoma of the appendix is almost exclusively
non-Hodgkin’s B-cell lymphoma
-  Men are more likely to develop appendiceal
lymphoma over women by 1.5 : 1, with a median
age onset of 18 years.
 Discussion
As with all lymphoma's, chemotherapy is the
mainstay of treatment.
- The classic combination of cyclophosphamide,
doxorubicin, vincristine, and prednisone (CHOP)
has been used for many decades.
- Rituximab, a monoclonal antibody against CD20,
was found to increase complete response rates
from 64% to 76% .
- It was also found that the CHOP plus Rituximab
increased event free states and overall survival.
 Conclusion
- Cancers of the appendix could represent a
challenge for diagnosis and management.
- No standard of care is established due to rare
frequency of occurrence
- Lymphomas of the appendix are treated with
appendectomy with/without hemicolectomy and all
require chemotherapy
- Keep High level of suspicion , and always keep an
eye for histopathology even after a simple
appendectomy
Thank You