Rhabdoides Meningeom

[Ulrich]

Link: http://link.springer.de/link/service/journals/00292/contents/02/00537/s00292-002-0537-1ch002.html

© Springer-Verlag 2002
Rhabdoides Meningeom - Ein neuer maligner Subtyp
Rhabdoid meningioma - A new malignant subtype

R. Klein1, 4, [Contact Information] , M. Bendszus2, J. Perez3 and W. Roggendorf1
(1) Pathologisches Institut, Abteilung Neuropathologie, Julius-Maximilians-Universität Würzburg
(2) Abteilung Neuroradiologie, Julius-Maximilians-Universität Würzburg
(3) Neurochirurgische Klinik, Julius-Maximilians-Universität Würzburg
(4) Pathologisches Institut der Universität, Abteilung Neuropathologie, Josef-Schneider-Straße 2, 97080 Würzburg

Zusammenfassung (Zitat):

Von den zahlreichen morphologischen Spielarten des Meningeoms haben nur wenige, darunter das rhabdoide Meningeom, eine prognostische Relevanz. Rhabdoide Meningeome wurden erstmals 1998 als ungewöhnliche Variante mit dem Hinweis auf eine erhöhte Proliferation beschrieben. Als eigener Meningeomsubtyp mit dem Risiko erhöhter Rezidivneigung und aggressiverem Wachstumspotenzial wurden sie 2000 in die revidierte WHO-Klassifikation aufgenommen und einem WHO-Grad III zugeordnet. Wir berichten über eine 21 Jahre alte Frau, die nach kurzer Anamnese an einem scheinbar intrazerebral gelegenen Tumor operiert wurde, der sich klinisch zunächst als Oligodendrogliom darstellte. Der Tumor zeigte histologisch Kennzeichen eines Meningeoms mit rhabdoider Zellmorphologie, angiomatöser Komponente sowie Verkalkungen und wurde als rhabdoides Meningeom eingeordnet. Nach der Operation verblieb ein kleiner Tumorrest, der nach Bestrahlung in Kontrollen nach 4 und 8 Monaten regredient war. Anhand des vorliegenden Falles diskutieren wir die Differenzialdiagnosen und prognostische Relevanz des Erkennens eines rhabdoiden Meningeoms.

Abstract

Of the numerous morphological variants of meningiomas only few, and among these the rhabdoid meningioma, have prognostic importance. Rhabdoid meningiomas were described for the first time in 1998 as an unusual variant with increased proliferative activity. In 2000 they have been included in the revised WHO classification of tumours of the CNS as a subtype of meningiomas with increased risk of recurrence and more aggressive growth, corresponding to WHO grade III. We report the case of a rhabdoid meningioma in a 21-year-old woman presenting as a intracerebral tumour mimicking an oligodendroglioma. The tumour showed features of a meningioma and a rhabdoid morphology with angiomatous components and was considered to be a rhabdoid meningioma. After surgery a small residual tumour remained. The patient received postoperative radiotherapy resulting in regression of the residual tumour in control examinations after 4 and 8 months. Using the presented case we discuss the differential diagnosis and prognostic significance of recognition of a rhabdoid meningioma.

[Ulrich]

Meningioma with Rhabdoid Transformation
Singapore Med J 2000 Vol 41(9) : 464-467

MENINGOTHELIAL MENINGIOMA WITH LIPOMATOUS AND RHABDOID DIFFERENTIATION.

Meningiomas are well known entities with frequent histological variants, such as meningothelial, fibroblastic, syncytial, and transitional, among others. However, unusual aggressive subtypes such as papillary meningiomas and rhabdoid meningiomas might constitute a problem in the differential diagnosis with other central nervous system tumours, mainly ependymomas, PNETs, oligodendrogliomas and teratoid/rhabdoid tumors of the CNS (1,2,3,4).

The rhabdoid differentiation, defined as sheets of loosely arranged cells with eccentric nuclei and eosinophilic cytoplasm containing hyaline paranuclear inclusions, seems to constitute a hallmark of malignancy (5,6).

Lipotamous and lipoblastic meningiomas constitute a controversial group among the metaplastic meningiomas (1,2,3,4). Ferracini et al (7) reported the presence of pluripotential primitive cells of the neural crest in perivascular areas of the leptomeninges, which are the probable source of metaplastic mesenchymal differentiation in meningiomas. Lipomatous meningiomas affect adults, and their biological behavior is similar to the classic meningothelial meningiomas (8). However, the recognition of this metaplastic variant is important, since they can be misdiagnosed as liposarcomas, metastatic mucinous adenocarcinomas and chordomas (1,2,3,4).

The rhabdoid differentiation was first described in a renal tumor affecting the kidney of infants (5). Since that description, this morphology has been described in several other types of tumors outside the kidney, such as intracranial gliomas, carcinomas, sarcomas and skin melanomas. It is inferred that this differentiation, occurring at extrarenal sites, might represent poorly-differentiated tumors with a common phenotype. Recently, Keppes et al (6) and Perry et al (5) described a series of the meningothelial meningiomas with rhabdoid differentiation, and suggested that it might constitute a hallmark of malignant progression in meningeal neoplasms, due to the high recurrence rate, documented extracranial metastases, as well as the presence of rhabdoid features only after the first recurrence in 40% of all reported cases (5). In the present case, the rhabdoid morphology was observed only after the third recurrence, supporting the concept of malignant progression. This phenomenon might be linked to the radiotherapy performed prior to the last biopsy.