Seminario de Patología “Salvados por la campana”

Seminario de Patología “Salvados por la campana”

Seminario de Patología
“Salvados por la campana”
Fátima Carneiro
IPATIMUP
&
Medical Faculty/Centro Hospitalar São João
Porto, Portugal
Case 1
Female, 18-year old, 4-month history
of abdominal pain
CT scan showed a tumour in the gastric wall
with cystic areas and calcification. A distal
gastrectomy was performed.
CT scan
Endoscopic ultrasound
FNAB – not conclusive
Surgical specimen
H12/257
HE 40X
H12/257
HE 100X
H12/257
H12/257
HE 200X
HE 200X
H12/257
H12/257
HE 200X
HE 200X
H12/257
HE 400X
H12/257
HE 400X
H12/257
HE 400X
Unfortunately, no bell was ringing!
• It was not any gastric tumour that I had seen before
• Apparently it was a (malignant?) biphasic neoplasia
• Several hypotheses were advanced and IHC studies
were performed
• GIST
• Synovial sarcoma
• Carcinosarcoma
• Teratoma
• Other
CD117 (c-Kit)
AE1/AE3
Vimentina
200X
200X
200X
CD56
(Epithelial)
CD56
(spindle cells)
CD10
200X
200X
200X
Immunohistochemistry
Epithelial component
Spindle cell component
+
+
+
+
+
CD117 (c-Kit)
AE1/AE3
Chromogranin
Synaptophysin
Vimentin
Desmin
S100
CD34
Calretinin
CD56
CD10
+
(multifocal)
Molecular study
• Screening of KIT mutations – negative
• Search of synovial sarcoma t(X;18) - absent
• GIST
• Synovial sarcoma
• Carcinosarcoma
• Teratoma
• Other
A bell was finally ringing...
Am J Surg Pathol 33:1370,2009
J Clin Pathol 63: 270, 2010
Clinicopathologic features
Case Age/sex
1
19/M
Size
(cm)
Mitotic
index
5x4x2.5
30
(free of
disease)
Greater
Sub-total
3.5 years
curvature, body gastrectomy
Greater
curvature, body Sub-total
gastrectomy
dor abdominal
27/F
6x4x3,5
4
3
30/M
15x12
1
Antrum
Anemia
4
9/M
9x6,5
0
Antrum,
abdominal pain
Sub-total
gastrectomy
9 months
5
18/F
10,5x8,7x
5,5
5
Antrum,
abdominal pain
Distal
gastrectomy
1 year
Shin
(2010)
Our case
Treatment
2
(2009)
et al
Localization
(50 HPF)
Miettinen
et al
Follow-up
5 years
Tumorectomy,
14 years
radiotherapy
Immunohistochemistry
CD117 (c-Kit)
Miettinen et al (2009)
Shin et al (2010)
Our case
-
+ (epithelial component)
-
+ (epithelial component) + (epithelial component)
+ (epithelial component)
AE1/AE3
Cromogranina
Sinaptofísina
Vimentina + (spindle cell component) + (spindle cell component) + (spindle cell component)
Desmina
ND
S100
CD34
Calretinina
CD56
CD10
+ (epithelial component;
ND
faint in the spindle cell
component)
+ (spindle cell component)
+ (focal, spindle cell
component)
NA – not available; +: positive; -: negative
+ (both components)
+ (both components)
Gastroblastoma
• Young adults and children
• Symptoms: abdominal pain; can be asymptomatic
• Macro: solid and cystic, with haemorragic areas
• Micro: biphasic tumor – epithelial and spindle cell
component, without necrosis; low mitotic index
• Immunohistochemistry: epithelial component – keratins
and CD56; spindle cell component – vimentin and CD10
• Prognosis: very few cases described so far and limited
follow-up
Gastroblastoma in a 28-year-old man
with nodal metastases
Proof of malignant potential
Wey et al; Arch Pathol Lab Med, 136: 961, 2012
TAKE HOME LESSONS
Never try to fit into a previously known histological
category cases that do not fit in.
In this case, the bell has been the temporo-spacial
memory that pathologists share with chess players.
Thanks for your attention
Save the Date
25th European
Congress of Pathology
31 August – 4 September 2013
in Lisbon, Portugal
www.esp-congress.org