The 11th International Course on the Pathology of the ...
Transcript of The 11th International Course on the Pathology of the ...
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Alina Nicolae MD, PhD
CASE 2
The 11th International Course
on the Pathology of the
Digestive System
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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
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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
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Clinicians asked for a retrospective
histopathological review of the
appendix
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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?
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CD5
CD20
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CD5
CD20
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Bcl-6 CD10
MUM1 Hans’ algorithm
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Bcl-
2
cMyc
Ki-67 p53 p21
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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
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Apr 2017: Core needle biopsies of mesenteric
mass were performed to confirm the dg
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CD3 CD20
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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)
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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
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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
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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
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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
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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