Alina Nicolae MD, PhD

CASE 2

The 11th International Course

on the Pathology of the

Digestive System

Clinical History

20-year-old female patient

Jan 2016 - acute right lower quadrant abdominal

pain, nausea, vomiting, fever

Ultrasonography – enlargement of the appendix,

no other lesions

Laparotomy - appendectomy has been performed

Macroscopically: 5cm long vermiform appendix, with

pseudomembranes on serosal surface

Dg: Acute appendicitis with periappendiceal abscess

Imaging (CT, TEP-scan):

mesenteric mass 19cm (SUV-18)

nodules (3-5 cm) peritoneal,

perihepatic, Douglas’s pouch

diffuse GI wall hyperfixation (SUV-

11) (stomach, jejunum)

thyroid nodules

inferior vena cava thrombosis,

ascites

March 2017

Rapid increase of abdominal

circumference, epigastric & lumbar pain

Biologically: hepatic cytolysis,

cholestasis, increase lipase & LDH

Clinicians asked for a retrospective

histopathological review of the

appendix

Some reflections….

What is your dg? How many of you would agree

with the dg of acute appendicitis ?

Would you ask for further IHC?

If yes, which antibodies?

CD5

CD20

CD5

CD20

Bcl-6 CD10

MUM1 Hans’ algorithm

Bcl-

2

cMyc

Ki-67 p53 p21

FISH studies

NO MYC/8q24; BCL2/18q21, BCL6/3q27 gene rearrangments

Primary appendicular DLBCL, NOS

GC phenotype (Hans’ algorithm)

FISH 8q24 break-apart probe

Absence of MYC translocation,

fusion signal pattern

BL/DLBCL morphology

BL-like phenotype

BL

HGBCL, NOS

DLBCL, NOS

HGBCL w R

MYC+BCL2+/-BCL6

Revised diagnosis

Apr 2017: Core needle biopsies of mesenteric

mass were performed to confirm the dg

CD3 CD20

Further clinical work-up

Flow cytometry peripheral blood and bone

marrow - negative for lymphoma

Bone marrow biopsy – absence of infiltration

Cytology LCR - negative

Ann Arbor Stage IV

(digestive, peritoneal)

aaIPI – 2 (LDH, Stage)

Apr 2017: COP treatment for debulking (remarkable

regression of tumor mass) R-COPADEM, FISH results

neg switched to R-CHOP 14 (GAINED study)

No ovarian cortex cryopreservation (emergency treatment)

Follow-up

May 17

Aug 17

PET scan Sept

2017 CR

CR – 13 months

after ASCT

Rare, <250 cases, 1.7% of appendiceal tumors

Mean age 48y (range 4-70), M:F - 1.5:1, most White

Most pts no relevant medical history, immunocompetent

Non-specific clinical findings, often signs and symptoms

suggestive of acute appendicitis

Right hemicolectomy confers no survival benefit over

appendectomy CHT primary treatment modality

Journal of Surgical Research 2017

Markedly homogeneous

enlargement (2.5-4cm) of the

appendix

Usually preserved vermiform

morphology

Stranding of the

periappendiceal fat :

superimposed inflammation

or tumor extension

Coexisting abdominal LAD or

aneurysmal dilatation of the

appendiceal lumen specific

for lymphoma

Pickhardt et al AJR Am J Roentgenol. 2002

CT scan

Axial images from

unenhanced CT

Contrast enhanced CT

Pathological examination

Diffuse, circumferential wall

thickening w obliteration of the lumen

Diffuse lymphocytic infiltration of the

appendiceal wall

Periappendiceal inflammation,

necrosis, and/or lymphomatous

extension into adjacent fat

Lymphoma types: DLBCL (34%), BL

(26%, young age), FL (15%)

Pickhardt et al AJR Am J Roentgenol. 2002

Ayud et al. J Surg Res 2017

Primary lymphomas of the appendix exist and can

affect young pts

All appendectomies should be sent for HP dg

Appendix with >2.5 cm diameter suspicious for

neoplasm extensive sampling

Awareness is crucial to achieve the correct dg;

clinical signs of appendicitis, young age and acute

inflammation are pitfalls in recognizing lymphomatous

appendiceal involvement

Take home message