Volume 26, No. 3/2007(May/June)
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 Clinical Neuropathology
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Review
Clinicopathological features of medulloblastoma: an overview with an emphasis on molecular biology
T.G.K.J. de Haas and M. Kool
Abstract
T.G.K.J. de Haas and M. Kool
Departments of 1Human Genetics and 2Neuropathology, Academic Medical Center, Amsterdam, The Netherlands
Medulloblastoma is a malignant, invasive embryonal tumor of the cerebellum with preferential manifestation in children, predominantly neuronal differentiation and an inherent tendency to metastasize via CSF pathways. In this review we present an overview of the clinicopathological aspects of medulloblastoma and the recent developments in the biology and genetics of these embryonal brain tumors. Increased knowledge of the signaling pathways that play a role in normal cerebellum development and tumor formation may lead to new therapeutic approaches resulting in a higher cure rate and a better quality of life of medulloblastoma patients.Correspondence to:
Dr. M. Kool; Department of Human Genetics, Academic Medical Center,
Meibergdreef 9, 1105 AZ Amsterdam, The Netherlandsa.nl
Email: m.kool@amc.uva.nl
Tumor
Infiltrative chordoid meningioma of the pineal region: a study of 2 cases
C.M. Ida, F.J. Rodríguez, B.W. Scheithauer, N. Kois, M. Vanefsky, B.J. Andersen and B. Erickson
Abstract
C.M. Ida, F.J. Rodríguez, B.W. Scheithauer, N. Kois, M. Vanefsky, B.J. Andersen and B. Erickson
1Division of Anatomic Pathology, Hospital das Clinicas Sao Paulo University School of Medicine, Sao Paulo, Brazil, 2Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, 3Department of Pathology, Saint Alphonsus Regional Medical Center, Boise, ID, 4Department of Neurosurgery, Kaiser Permanente Medical Center, Anaheim, CA, 5Department of Neurosurgery, Saint Alphonsus Regional Medical Center, Boise, ID, 6Division of Neuroradiology, Mayo Clinic, Rochester, MN, USA
Objective: Meningiomas involving the pineal region are rare. Herein we describe two cases of chordoid meningioma with histologic evidence of pineal gland infiltration. Materials and methods: Clinical histories were abstracted from chart review and consultation letters. HE-stained slides were reviewed in both cases. Selected immunohistochemical stains were performed. Results: the patients included a 44-year-old male and a 37-year-old female who presented with symptoms of intracranial tumor referable to the pineal region. On magnetic resonance imaging (MRI), both lesions demonstrated heterogeneous contrast enhancement. Histologically, the tumors were characterized by strands and cords of meningothelial cells arranged in a mucinous stroma. In addition, obvious meningothelial cytology as well as focal osseous metaplasia (Case 1), and transitional histology (Case 2) were also noted. Tumor cells demonstrated EMA and focal S100 protein immunoreactivity, but lacked cytokeratin AE1/AE3 and glial fibrillary acidic protein (GFAP) staining. Synaptophysin and neurofilament protein highlighted the overrun pineal gland parenchyma. MIB1-proliferative index was 8.4 and 20.1%, respectively. Conclusions: Chordoid meningioma, although rare, may occur in the pineal region. The differential diagnosis of this meningioma subtype in this location is discussed.Correspondence to:
B.W. Scheithauer, MD; Mayo Clinic, Department of Laboratory Medicine and Pathology,
200 First Street, SW, Rochester, MN, USA 55905
Email: scheithauer.bernd@mayo.edu
Tumor
Papillary glioneuronal tumor: not always a benign tumor?
C. Adam, M. Polivka, A. Carpentier, B. George and F. Gray
Abstract
C. Adam, M. Polivka, A. Carpentier, B. George and F. Gray
1Service Central d’Anatomie et de Cytologie Pathologiques, 2Service de Neurochirurgie, APHP, Hôpital Lariboisière-Université Paris VII, France
Papillary glioneuronal tumor (PGNT) is a variant of ganglioglioma, characterized by a pseudopapillary structure with a single pseudostratified layer of small, cuboidal, GFAP-positive cells around hyalinized blood vessels. To date, less than 30 cases have been described with a usually benign course. We report two additional cases: a clinically, radiologically and histopathologically typical tumor in a 38-year-old man and an atypical tumor with histopathological features of anaplasia in a 74-year-old woman. The latter tumor showed the classical pseudopapillary pattern with ganglioid cells and some astrocytes between the papillae, but also had changes suggestive of anaplasia including necrosis, capillary endothelial proliferation, mitoses, dedifferentiation with loss of GFAP expression of the cuboidal cells and increased Ki-67 labeling of over 10%. Only one other case with increased proliferative activity in a minigemistocytic component has previously been described. Our cases indicate that PGNT type of ganglioglioma can have a spectrum of anaplastic changes of higher grade.Correspondence to:
Prof. F. Gray; Service Central d’Anatomie et de Cytologie Pathologiques, Hôpital Lariboisière, 2 rue Ambroise Paré, 75475 Paris cedex 10, France
Email: francoise.gray@lrb.aphp.fr
Tumor
Benign cylindroma causing transcalvarial invasion in a patient with familial cylindromatosis
J.H. Gildea, K.O. Lillehei, L.E. Golitz and B.K. Kleinschmidt-DeMasters
Abstract
J.H. Gildea, K.O. Lillehei, L.E. Golitz and B.K. Kleinschmidt-DeMasters
Departments of 1Pathology, 2Neurosurgery and 3Neurology, University of Colorado Health Sciences Center, Denver, CO, USA
Objective: To describe a case of scalp cylindroma without features of malignancy invading through the skull and dura, and producing massive intracranial extension. Tumors of epidermis and epidermal appendages rarely show bony invasion, but invasive tendency in some tumor types has been associated with increased TP53 expression. Patient and methods: Patient with familial cyindromatosis (Brooke-Spiegler syndrome) who had undergone numerous previous surgical excisions over the past 30 years of his scalp cylindromas. Light microscopic and immunohistochemical characterization of resected tumor, with TP53 immunostaining in the invasive tumor was compared with that seen in five other cutaneous, non-invasive cylindromas. Results: Tumor showed no increase in mitotic rate or increased immunostaining for TP53. Conclusion: Multiple previous surgeries down to pericranium may have contributed to local weakening of tissues and facilitated transcalvarial invasion. While an uncommon occurrence, both benign and malignant cylindromas have the capacity to invade bone, particularly in patients with the familial syndrome.Correspondence to:
B.K. Kleinschmidt-DeMasters, MD; University of Colorado Health Sciences Center, Department of Pathology, B-216 4200 E. 9th Ave. Denver, CO 80262, USA
Email: bk.demasters@uchsc.edu
Abstracts
Joint Meeting, Belgian-Dutch Neuromuscular Study Club and German Reference Center for Neuromuscular Diseases of the DGNN Vaals, The Netherlands, March 23 – 24, 2007
Organizers: M. De Baets, Maastricht (Chairman), J. Weis, Aachen (Local Organizer), P. Van den Bergh, Brussels and L. van den Berg, Utrecht in collaboration with the European Graduate School of Neuroscience (Euron)
Abstract
Organizers: M. De Baets, Maastricht (Chairman), J. Weis, Aachen (Local Organizer), P. Van den Bergh, Brussels and L. van den Berg, Utrecht in collaboration with the European Graduate School of Neuroscience (Euron)
Euro-CNS News
Society News of the European Confederation of Neuropathological Societies
Euro-CNS
