96. Jahrestagung der Deutschen Gesellschaft für Pathologie e. V ...

chemotherapy was recommended and continuing the follow-up observation
of the B-cell CLL with no current need for a specific treatment.
Conclusions. Despite a challenging combination of simultaneous neoplasias
of different and/or the same entit(ies)y, in addition with complicating
factors (2 primary tumor manifestations of the same entity with
the need of an extended standard surgery due to tumor sites, subileus,
perioperatively persisting, initially diagnosed hemoblastosis), the treatment
was successful with curative intention and preserved mid- to longterm
curative potential by abdominal surgery with a low complication
rate. This confirms the indicated primary surgical care of the GI cancers,
also because of the coincidence of a colonic carcinoma at 2 sites. The rare
transversal-colon intussusception in this age, flanked by a carcinoma of
the right and left flexure, is the first report of this type in the literature.
FR-P-055
Seltenes Langerhans-Zell-Sarkom der Milz mit ungewöhnlicher
klinischer Manifestation
J . Arend1 , D . Küster2 , H . Lippert1 , F . Meyer1 1 2 University Hospital, Dept . of Surgery, Magdeburg, University Hospital,
Institute of Pathology, Magdeburg
Aims. Abklärung eines pathohistolischen Zufallsbefundes nach Splenektomie
im Rahmen einer Operation bei Leberparenchymblutung (primär
V. a. akute Cholangitis bei Choledochocystolithiasis).
Methods. Zunächst therapeutische ERCP mit Stentimplantation im
D. hepatocholedochus nach Papillotomie unter antibiotische Abschirmung.
In der Folge-CT multiple Leberherde, die zur histologischen
Abklärung sonographiegestützt bioptiert wurden. Komplikation: intra-
und perihepat. Hämatom, welches bei symptomatischer Größenzunahme
und drohendem sept. KH-Bild (Ursache nur teils durch Staphylokokkenkolonisation
eines i.v.-Portsystems zur Chemotherapie bei
Mamma-Ca erklärt) eine notfallmäßige expl. Laparotomie zur sept.
Fokussanierung erforderte: i) Intraabdominal kein sept. Fokus; ii) Entlastung
des Leberkapselhämatoms und lokale Thermokoagulation/
Hämostyptikaapplikation; iii) intraop. multiple, unklare, teils massiv
blutende fokale Milzläsionen (vulnerable Parenchymoberfläche), die zur
Blutungsbeherrschung und pathohistologische Abklärung die Splenektomie
erforderten; i.v.) Explantation des infizierten i.v.-Ports.
Results. Bei Staphylokokkensepsis (zusätzl. Bakteriämie durch Streptokokkus
hominis) und multiplen chologenen Leberabszessen zunächst
kalkulierte und später resistenzgerechte Antibiotika (keine klein. Befundbesserung).
Im Leberbiopsiepräparat multiple kleine Abszesse mit
schaumzellig-histozytärer, resorptiver Entzündungskomponente. Das
Splenektomiepräparat war diffus mit zytologisch malignen Zellen bei
aufgehobener Milzarchitektur durchsetzt mit einer Zellproliferationsfraktion
(Ki-67-Ag) von ca. 50% (pos. Immunhistologie für S-100, CD1a
und teils CD68; Lysozym, CD3, Cd20, CD30, Alk 1, Myeloperoxidase und
Chlorazetatesterase hingegen negativ). Trotz techn. Operationserfolgs
tolerierte die Patientin die Intervention nur mäßig, durch zunehmende
AZ-Verschlechterung keine weiterführend ggf. diagnosespezifisch indizierte
Therapie möglich. Trotz max. int.-therapeut. Maßnahmen erlag
die Patientin 13 Tage nach stationärer Aufnahme dem foudroyanten Verlauf
im MOV.
Conclusions. Die zur Aufnahme nicht bekannte, seltene Erkrankung
eines Langerhans-Zell-Sarkoms trug wesentlich zum fulminanten Cholangitis-Verlauf
bei. Zusätzliche Komplikationen wie Portinfektion und
Blutung nach Leberpunktion verschlechterten die Prognose weiter. Offen
bleibt, ob klinischer Verdacht oder Sarkomfrühdiagnose den KH-
Verlauf suffizient beeinflusst hätte. Die Seltenheit der Erkrankung und
damit fehlende Erfahrungen in Diagnostik und Therapie erschweren
eine individuelle Therapie massiv.
Poster: GI-Trakt: GIST, Dünndarm, Kolorektum
FR-P-056
Value of epithelioid histomorphology and PDGFRA immunostaining
patterns for prediction of PDGFRA mutated genotype
in GISTs
A . Agaimy1 , C . Otto2 , H . Geddert 3 , I .-M . Schaefer4 , A . Braun2 , R . Schneider-
Stock1 , F . Haller2 1 2 Friedrich Alexander University, Institute of Pathology, Erlangen, Albert
Ludwigs University, Institute of Pathology, Freiburg, 3St . Vincentius Hospital,
Institute of Pathology, Karlsruhe, 4Georg August University, Institute of
Pathology, Göttingen
Aims. A majority of gastrointestinal stromal tumors (GISTs) carry mutations
in the receptor tyrosine kinases KIT or PDGFRA. In a recent
study, we demonstrated upregulated expression of KIT in KIT mutated
GISTs, in contrast to upregulated PDGFRA expression in PDGFRA mutated
GISTs, on mRNA (qRT-PCR) and protein (Western BloT) level.
However, most routinely processed GISTs are formalin-fixed and paraffin-embedded;
thus, these methods are not applicable in daily pathology
routine. Reliable determination of PDGFRA expression by immunohistochemistry
might help to identify GISTs with PDGFRA mutation,
without the necessity for complete genotyping of KIT and PDGFRA by
mutational analysis. The aim of the current study was to evaluate the
predictive value of a combination of histomorphology and PDGFRA immunohistochemistry
in comparison to mutational analyses.
Methods. In order to conduct a tissue microarray, 109 surgically resected
GISTs with known mutation status of KIT (74%) and PDGFRA (16%)
were used. The histomorphological phenotype (spindled, epithelioid,
mixed growth pattern) was determined on H&E sections without knowledge
of the genotype. The staining intensity (negative, 1–25%, 26–50%,
>50%) and the staining pattern (paranuclear, cytoplasmic, membranous)
of PDGFRA were determined without knowledge of the genotype.
Results. PDGFRA-mutated GISTs were significantly more often of epithelioid
phenotype and had a significantly higher expression of PDGFRA
protein, compared to KIT-mutated GISTs. The paranuclear stainig pattern
was almost exclusively observed in PDGFRA mutated GISTs. A
combination of histomorphology, staining intensity and staining pattern
of PDGFRA was a reliable predictor for PDGFRA genotype.
Conclusions. A combination of histomorphology and PDGFRA immunostaining
is a reliable predictor of PDGFRA genotype. The use of this
PDGFRA genotype predictor may help to reduce costs and shorten processing
time of GIST genotyping by excluding KIT mutational analysis
in PDGFRA-overexpressing GISTs. This might be even more important
in less developed countries with restricted health budgets.
FR-P-057
Expression of CD34 in GIST is site-dependent and genotypeassociated
A . Braun1 , C . Otto1 , H . Geddert2 , D .J . Zhang3 , A . Agaimy4 , Ö . Sahin3 , F . Haller1 1 2 Albert Ludwigs University, Institute of Pathology, Freiburg, St . Vincentius
Hospital, Institute of Pathology, Karlsruhe, 3German Cancer Research Center,
Heidelberg, 4Friedrich Alexander University, Institute of Pathology, Erlangen
Aims. Gastrointestinal stromal tumors (GISTs) are the most common
mesenchymal tumors of the gastrointestinal tract. In addition to immunopositivity
for KIT (CD117), 60–70% of cases are positive for CD34.
CD34 is a cell surface glycoprotein, which is expressed especially in early
hematopoietic stem and progenitor cells. Its function is still not yet
sufficiently clarified. Our aim was to gain a better understanding of the
regulation of CD34 expression in GISTs.
Methods. From the archives of our institutes, 109 surgically resected
GISTs were compiled. Mutation analyses of KIT (exon 9, 11, 13 and 17),
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