Pathologica 4-07.pdf - Pacini Editore

PATHOLOGICA 2007;99:214-224
Patologia dell’apparato digerente
Telangiectatic focal nodular hyperplasia
(FNH) of the liver currently classified as
hepatocellular adenoma (HCA) variant with
ductular differentiation? A problem area and
report of a paradigmatic case
M. Bisceglia, A. Gatta * , A. Tomezzoli ** , M. Donataccio ***
Departments of Pathology and * Pediatrics, IRCCS “Casa
Sollievo della Sofferenza” Hospital, San Giovanni Rotondo;
Departments of ** Pathology and *** Surgery, Ospedale Civile
Maggiore di Verona, Italy
Introduction. FNH and HCA are benign liver tumors. In
1999 two categories of FNH were defined: the classical type
(~ 80%), with or without gross central scar, histologically
showing architectural nodular distortion, malformed arterial
vessels, and bile ductular reaction, and the non-classical type,
lacking nodular architecture or malformed vessels, but always
presenting bile ductules, the hallmark of the lesion 1 .
FNH was further subdivided into the telangiectatic FNH
(TFNH) (~ 15%), the mixed hyperplastic and adenomatous
FNH (1-2%), and the FNH with cytologic atypia (2-3%). In
2004 molecular studies displayed that TFNH is closer to
HCA than to FNH and the term of telangiectatic HCA (HCA-
TFNHtype) was suggested 2 . This latter datum was soon corroborated
by others 3 4 , and TFNH is now included in the
monoclonal spectrum of HCA as a separate entity (HCA variant),
due to the peculiar morphology and the absence of
known gene mutation. This year 2007 new diagnostic criteria
came out in regard to HCA, and 4 variants have been delineated
in addition to the classical. Variant-3 (with or without
inflammatory infiltrates) is TFNH (“progressive FNH”), and
may contain ck7+ bile ductules (adenoma with duct differentiation).
The other variants of HCA have incorporated the rest
of non-classical FNH, and FNH is now represented by the
classical or solid form only. Still, the diagnosis (dx) of TFNH
remains problematic and overlaps FNH.
Case report. Young Italian girl had a twisted pedunculated
liver mass, which was surgically resected in emergency in
1999 at the age of 17. No “pill”, no Fanconi anemia, no
glycogen storage disease, no familial adenomatous polyposis,
no diabetes mellitus was recorded. At histology, based on
the presence of a seeming central scar, dystrophic vessels,
and patchy ductular proliferation, the lesion was diagnosed
as FNH. Peliotic, hemorrhagic and acute necrotic changes
were ascribed to the torsion. At surgery another 3 cm liver
mass located on the dome was noted but left alone till the end
of 2006, by which time had grown to 7 cm. While planning
the second surgical intervention, many slides of the 1 st lesion
were sent in consultation to 7 specialized liver centers, and
diverse dx came out, ranging from FNH to HCA-TFNH type
to classical HCA (w.d. HCC also considered; concern expressed
for the 2 nd ). The 2 nd tumor was resected: no central
scar was seen, the lesion was ill-delimited with some zonation
of clear ballooned hepatocytes with steatosis arranged
around thin-walled venules, alternated with smaller
eosinophilic cells disposed along arterial branches; bile ducts
and ductules were noted; multiple minute hyperplastic nodular
foci of clear/steatotic hepatocytes were also seen in the
adjacent host liver. Slides were sent to 5 of the previous cen-
ters: the dx received from 3 were classic HCA, HCA with
ck7+ biliary ductules, and adenomatous hyperplasia (exclusive
of HCA due to the presence of ductules), repsectively;
no answer from 2. With the previous history available, 2 of
those who answered also suggested the dx of adenomatosis.
Finally, on request 2 more pathologists reviewed the entire
case and the dx were adenomatosis with different types of
HCA (TFNH type and stetatotic-type), and multiple HCA
with duct differentiation (progressive FNH type), respectively.
Of interest one of these interpreted the “central scar” of
the first tumor 5 as the result of socalled congestive hepatopathy.
Results. Malignancy was excluded based on morphology
(absence of atypia, intact reticulin framework, and regular
disposition of 1-2 thick-layered trabeculae) and immunostainings
(Glypican3 was negative, CD34 showed minimal sinusoidal
staining, MIB1 labeled very occasional nuclei).
Conclusions. Given the absence of any significant clinical
context, the final diagnosis was spontaneous multiple adenomas
(adenomatosis). The clinical management is difficult but
requires regular follow-up: removal of larger tumors at risk
of bleeding is recommended.
References
1 Nguyen BN, et al. Am J Sur Pathol 1999;23:1441-54.
2 Paradis V, et al. Gastroenterology 2004;126:1323-9.
3 Bioulac-Sage P, et al. Gastroenterology 2005;128:1211-8.
4 Zucman-Rossi J, et al. Hepatology 2006;43:515-24.
5 Bioulac-Sage, et al. J Hepatol 2007;46:521-7.
Fatal venous systemic air embolism following
endoscopic retrograde
cholangiopacreatography (ERCP). A case
report
M. Bisceglia, A. Simeone * , R. Forlano ** , A. Andriulli ** , A.
Pilotto ***
Departments of Pathology, * Radiology, ** Gastroenterology
and Gastrointestinal Endoscopy, and *** Geriatrics, IRCCS
“Casa Sollievo della Sofferenza” Hospital, San Giovanni
Rotondo, Italy
Introduction. Air embolism (AE) is a rare complication of
gastrointestinal (GI) endoscopy, resulting from penetration of
gas into the portal veins. Risk factors associated with air embolism
in this setting include situations where the mucosa is
damaged or where high pressures are generated in the GI
tract. Thus this complication can be seen in the context of
various pathologies, including acute mesenteric ischemia,
chronic inflammatory GI diseases, GI infections, acute gastric
dilatation, caustic ingestion, superior mesenteric artery
syndrome with duodenal dilatation, ileus, blunt abdominal
trauma, duodeno-caval fistulas, and invasive diagnostic procedures,
such as double-contrast barium enema, endoscopic
sphincterotomy (ES), and ERCP. The likely mechanism by
which ES and ERCP cause AE is intramural dissection of insufflated
air into the portal venous system via venous duodenal
radicles which are inadvertently injured or transected. AE
is an ominous sign and may be fatal (mortality rate of 75%),
but may also be reversible or cured by surgery depending on