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

FR-P-168
Autopsy findings in a 2-year-old boy with EHEC/HUS in the 2011
German outbreak
M . Kuhlmann 1 *, C . Baier 1 *, J .U . Becker 1 , C . Hartmann 2 , F .-C . Bange 3 , T . Ahlenstiel
4 , H . Kreipe 1
1 MH Hannover, Institute of Pathology, Hannover, 2 Ruprecht-Karls University
Heidelberg, Institute of Pathology, Department Neuropathology, 3 MH
Hannover, Department of Medical Microbiology and Hospital Epidemiology,
4 MH Hannover, Department of Pediatric Nephrology
Aims. Demonstration of histopathological findings in a 2-year-old boy
with EHEC/HUS in the 2011 German outbreak.
Methods. Routine autopsy procedure including gross and microscopic
examination with histochemical and immunhistochemical stainings
(MSB, CD 61, GFAP, CD 68), and review of clinical data.
Results. A previously healthy 2-year-old boy was diagnosed with EHEC/
HUS (serotype O104:H4, Shiga-toxin 2 positive) and developed in the
clinical course severe acute renal and heart failure as well as neurological
complications. Our main histopathological findings: Heart: heart weight
was increased above the 90 percentile. Histology revealed rather fresh
focal necrosis of heart muscle cells and thrombocyte rich thrombi in cardial
capillaries. Kidney: on cross sections the kidneys appeared darkly
red and no distinction between renal cortex and medulla was possible.
Histologic examination revealed luminal thrombocyte rich thrombi in
glomeruli and in afferent arterioles, with negligible amounts of stainable
fibrin. Moreover, glomeruli exhibited severe endothelial swelling and
severe mesangiolysis with microaneurysms. Brain: supra- and infratentorial
multiple hemorrhagic infarctions (stage I and II) with severe brain
edema as well as a laminar necrosis of the cortex (stage I) were detectable.
Furthermore there was a severe gliosis with multiple reactive astrocytes
and activated microglia in the white matter. Within the complete central
nervous system no thrombi indicating TMA were found.
Conclusions. In the kidneys endothelial swelling was dominant reflecting
endothelial damage caused by Shiga-toxin 2. Endothelial damage induced
formation of thrombocyte microthrombi without stainable fibrin
content in the heart and in the kidneys with resultant ischemia. While
in previously published cases the thrombi contained fibrin depositions
besides thrombocytes, in this case we only found minimal or no fibrin
with MSB stain. Another key feature of our renal histology was a severe
mesangiolysis, which is described as a rare finding in typical D+ HUS
whereas it is more often seen in D- negative HUS cases. Regarding the
cerebral findings the severe gliosis of the white matter without similar
changes in the cortex could indicate a prior non-ischemic, possibly toxic
damage to the white matter, as is discussed in the literature. Our case
with cerebral, myocardial and renal involvement causing failure of all
three organs argues against an overly rigid clinical separation of HUS
and thrombotic-thrombocytopenic purpura.
*These authors contributed equally to the study .
FR-P-169
Synchronous presentation of gastrointestinal stromal tumor of
the stomach, ganglioneuroma of the adrenal gland and adenomas
of the colon
N . Pawlaczyk1 , K . Neumann1 , J . Knolle1 , H . Zühlke2 1 2 Institute of Pathology, Dessau-Rosslau, Department of General,
Visceral and Vascular Surgery, Lutherstadt Wittenberg
Aims. Gastrointestinal stromal tumor (GIST) is the most common mesenchymal
tumor of the gastrointestinal tract. Its carcinogenesis is driven
by activating mutations of the KIT or PDGFRA gene but a small
subset of GISTs has a negative mutation status. These wild type-GISTs
may develop as part of a multi-neoplastic disease. We present a case with
concurrent presentation of GIST of the stomach, Ganglioneuroma (GN)
of the adrenal gland and adenomas of the colon. To our knowledge, the
synchronous existence of these neoplasias has not yet been reported.
Methods. We describe an 85-year-old male patient with initially acute upper
gastrointestinal bleeding. Explorative laparotomy and subsequently
pathologic analysis of resected specimen revealed three distinct neoplasias:
GIST of the stomach (5,5×3,7×3,8 cm, T,
Gly12Cys). GNs are rarely seen in patients with neurofibromatosis (NF)
type 1. An association between the development of GIST and type 1 NF
has also been established. Our patient showed no further clinical features
of NF. GISTs arising in the setting of type 1 NF are usually KIT- and
PDGFRA-wild type and the tumor suppressor gene Neurofibromin is
inactivated. Testing for a potential silencing of Neurofibromin is underway.
Conclusions. The synchronous manifestation of GIST and other neoplasms
is a common observation. In our case, the co-occurence of GIST,
GN and adenomas of the colon could represent a syndromal setting. Molecular
analysis revealed no amino acid changing mutations of the KIT
and PDGFRA genes what differs from sporadic GISTs. The role of alternative
oncogenes or pathways in the carcinogenesis of wild type-GISTs
as well as in their presentation in a multi-neoplastic context requires
further examination.
FR-P-170
Pleural malacoplakia caused by Rhodoccocus equi infection in a
patient after stem cell transplantation because of a T-PLL
C .L . Behnes1 , S . Neumann2 , S . Schweyer1 , H .-J . Radzun1 1 2 University of Göttingen/Institute of Pathology, University of Göttingen/
Institute of Onkology
Aims. Malakoplakia is a disease especially of the urinary tract with typical
plaques most frequently observed in the bladder’s mucosa, consisting
of accumulated macrophages. The reason for this disease is an impaired
lysosomal degradation of bacteria, especially E. coli. In the context of immunosuppression
malakoplakia can also occur in other organs such as
the prostate, kidney and lung. To our knowledge, affection of the pleura
by malacoplakia has not yet been documented.
Methods. Case report: a 60-year-old man was admitted to the hospital
because of a generalized lymphadenopathy, hepatosplenomegaly and
suspicion of pneumonia. Based on a lymph node biopsy the diagnosis
of a T-cell-prolymphocytic-leukemia was confirmed and treated with
an allogenic stem cell transplantation. A half year later the patient was
admitted to the hospital with retrosternal pain and a reduced state of
condition. The clinical examinations revealed a 12 cm in diameter and
well circumscribed focus within the right upper pleura.
Results. The macroscopical examination of the upper lobe of the right
lung showed a 12 cm in diameter tumor adherent to the pleura, displacing
the lung and showing a gray-white cut surface with central necrotic,
disintegrated areas. The microscopical examinations revealed a tumor
consisting of a monomorphic cell population, which was predominantly
composed of macrophages. Granulomas, multinucleated giant cells, significant
cellular atypia or increased proliferation could not be observed.
The immunohistochemical examinations revealed numerous Ki-M1P/
CD68 positive macrophages with intracellular PAS positive deposits,
which could be identified as calcifications by Kossa staining (Michaelis-
Gutmann-Bodies) being pathognomonic for Malacoplakia. A microbio-
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