51 research outputs found
Tussen schijn en werkelijkheid
Het gebeurt niet vaak dat een patholoog contact heeft met patiënten of familie
van patiënten. Enige tijd geleden werd ik gebeld door de vader van een baby die
was geboren met een grote hersentumor die de hersenkamers voor een deel innam.
Op het door de neurochirurg verwijderde weefsel stelde ik de diagnose carcinoom
uitgaande van de plexus chorioideus. De plexus chorioideus is een structuur die in de
hersenkamers thuishoort en het hersenvocht, ook wel de liquor geheten, produceert.
Mijn diagnose was gebaseerd op enige gelijkenis van het tumorweefsel met die
plexus chorioideus. Carcinomen van de plexus chorioideus zijn uitermate zeldzaam.
Hoog in het lijstje van alternatieve diagnoses stond de zogenaamde AT/RT (afkorting
van atypical teratoid/rhabdoid tumor), een tumor die onduidelijk is gedefi nieerd
en waarvan het microscopisch beeld zeer variabel is. Ik had echter, afgezien van
genoemde gelijkenis van het tumorweefsel met de plexus, nog een aantal argumenten
om de tumor niet als een AT/RT te beschouwen. Omdat de chemotherapie, die wordt
gegeven nadat chirurgische verwijdering heeft plaatsgevonden, anders is in het geval
van een plexuscarcinoom dan van AT/RT begon de druk vanuit de kinderkliniek toe
te nemen om een zekere diagnose te stellen.Rede, in verkorte vorm uitgesproken
ter gelegenheid van het aanvaarden
van het ambt van bijzonder
hoogleraar in de Neuropathologie
aan het Erasmus MC, faculteit van de
Erasmus Universiteit Rotterdam
op 2 oktober 200
Tumor characteristics and biological behavior of oligodendroglioma
Whereas in astrocytomas grading results yielded satisfying clinico-pathological
correlations, grading procedures were without acclaim in the oligodendroglia! tumor
group. The reason for this might be that only small series of this uncommon neoplasm
were studied, and delineation of oligodendroglia! tumors from mixed gliomas or astrocytic
tumors was hampered with difficulties.
In order to study the relationship between tumor size and survival, as well as tumor
size and histopathologic grade, tumor volumes were calculated from the CT-scans of 43
oligodendrogliomas (paper 1). Although a good correlation between grade and survival
was shown, no correlation between tumor size and survival or histopathologic grade was
obtained.
In paper 2 a comparison was made between the traditional grading system of
Kernahan, and the recently developed grading system for oligodendrogliomas of Smith. In
a retrospective study on 72 patients the grading results of both systems were related to the
survival times of the patients. It was found that grading according to both systems yielded
three groups of patients with significant differences in survival. Nevertheless, the grading
system of Smith is preferred because of its lower inter-observer error.
The prognostic value of DNA-flow cytometry and mitotic count was tested
retrospectively on the paraffin-embedded material of 85 oligodendrogliomas (paper 3).
The results of the DNA-flow cytometry neither correlated with survival, nor with the
histopathological grades. The mitotic count, however, had relevance for the prognosis.
Thus, the mitotic count is a valid parameter for grading oligodendrogliomas
Approved CAR T cell therapies
Two autologous chimeric antigen receptor (CAR) T cell therapies (Kymriahâ„¢ and Yescartaâ„¢) were recently approved by the FDA. Kymriahâ„¢ is for the treatment of pediatric patients and young adults with refractory or relapse (R/R) B cell precursor acute ly
The pleomorphic xanthoastrocytoma and its differential diagnosis: A study of five cases
__Abstract__
Five brain tumors with the histopathologic features of pleomorphic xanthoastrocytomas (PXAs) are presented. Computed tomography scans showed a remarkable homology. Two cases had atypical localizations for a PXA, while one 46-year-old patient did not conform to the normal age distribution of this tumor. Nevertheless, in these cases, the histopathology was always characteristic for PXA, a remarkable pleomorphism, in addition to simultaneous expression of glial fibrillary acidic protein and histiocytic markers in the various tumor cells. In one of the presented tumors, however, clusters of neoplastic neuronal cells were also found. In this particular case, differential diagnostic criteria to distinguish between a PXA and a desmoplastic infantile ganglioglioma are lacking
Breakthroughs in modern cancer therapy and elusive cardiotoxicity: Critical research-practice gaps, challenges, and insights
To date, five cancer treatment modalities have been defined. The three traditional modalities of cancer treatment are surgery, radiotherapy, and conventional chemotherapy, and the two modern modalities include molecularly targeted therapy (the fourth modality) and immunotherapy (the fifth modality). The cardiotoxicity associated with conventional chemotherapy and radiotherapy is well known. Similar adverse cardiac events are resurging with the fourth modality. Aside from the conventional and newer targeted agents, even the most newly developed, immune-based therapeutic modalities of anticancer treatment (the fifth modality), e.g
Papillary meningioma with pleural metastasis: Case report and literature review
Papillary meningiomas are rare meningeal tumors which are associated with a grim prognosis. These tumors usually recur locally and in some cases they metastasize. The clinical, radiological and histopathological features of a case of a papillary meningioma with a pleural metastasis in a 13-year-old boy are presented. The literature on metastasizing papillary meningiomas is reviewed. Up to now, 131 cases of papillary meningioma have been reported in the literature. Only 8 cases gave rise to metastases outside the central nervous system. The preferential site of metastasis appeared to be the lung. This is the first report of a papillary meningioma giving rise to a metastasis in the pleura
Molecular misreading: The occurrence of frameshift proteins in different diseases
Neuronal homoeostasis requires a constant balance between biosynthetic and catabolic processes. Eukaryotic cells primarily use two distinct mechanisms for degradation: the proteasome and autophagy of aggregates by the lysosomes. We focused on the UPS (ubiquitin-proteasome system). As a result of molecular misreading, misframed UBB (ubiquitin B) (UBB+1) is generated. UBB+1 accumulates in the neuritic plaques and neurofibrillary tangles in all patients with AD (Alzheimer's disease) and in the neuronal and glial hallmarks of other tauopathies and in polyglutamine diseases such as Huntington's disease. UBB+1 is not present in synucleinopathies such as Parkinson's disease. We showed that UBB+1 causes UPS dysfunction, aggregation and apoptotic cell death. UBB+1 is also present in non-neurological cells, hepatocytes of the diseased liver and in muscles during inclusion body myositis. Other frequently occurring (age-related) diseases such as Type 2 (non-insulin-dependent) diabetes mellitus are currently under investigation. These findings point to the importance of the UPS in diseases and open new avenues for target identification of the main players of the UPS. Treatment of these diseases with tools (e.g. viral RNA interference constructs) to intervene with specific targets is the next step
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