Volume 29, No. 3/2010(May/June)
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 Clinical Neuropathology
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Editorial
Clinical Neuropathology, Vol. 29 – No. 3/2010
J. Hainfellner
Original contribution
Colloid cyst of the third cerebral ventricle with an embryological remnant consistent with paraphysis cerebri in an adult human
S. Nagaraju, D.G. O’Donovan, J. Cross and H. Fernandes
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (121-126)
Colloid cyst of the third cerebral ventricle with an embryological remnant consistent with paraphysis cerebri in an adult human
S. Nagaraju1, D.G. O’Donovan1, J. Cross2 and H. Fernandes3
Departments of 1Neuropathology, 2Neuroradiology, and 3Neurosurgery, Addenbrooke’s Hospital, Cambridge, UK
The histogenesis of colloid cysts of the third ventricle remains unsettled. Initial theories favored a neuroepithelial (paraphysis, ependyma, choroid plexus) origin and some investigators based on morphologic analysis have offered an alternative endodermal source. We report a case of colloid cyst of the third ventricle arising in association with a remnant which we believe corresponds to the paraphysis cerebri in man.Correspondence to:
S. Nagaraju, MBBS
Box 235, Department of Neuropathology
Addenbrookes Hospital, Hills Road
Cambridge CB2 0QQ, UK
Email: santhosh.nagaraju@addenbrookes.nhs.uk
Original contribution
Incidental pituicytoma after accidental head trauma – case report and review of literature
A. Grote, A. Kovacs, H. Clusmann, A.J. Becker and P. Niehusmann
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (127-133)
Incidental pituicytoma after accidental head trauma – case report and review of literature
A. Grote1, A. Kovacs2, H. Clusmann1, A.J. Becker3 and P. Niehusmann3
Departments of 1Neurosurgery, 2Radiology/FU Neuroradiology, and 3Neuropathology, University of Bonn Medical Center, Bonn, Germany
Objective: We report on a patient with pituicytoma, i.e. a rare neoplasm of the neurohypophysis, with unusual anamnestic manifestation. Case material: After a car accident, the patient suffered from severe persisting headaches. Diagnostic procedures revealed a minor visual impairment and restriction of the gonado- and somatotropic pituitary axis. MRI showed an architecturally solid, well demarcated and homogenous suprasellar lesion. Due to the challenging location of the lesion with a small intrasellar mass and larger suprasellar part within the hypophyseal stalk, a subtotal resection was carried out to save the pituitary function and for neuropathological assessment comprising numerous stainings and immunohistochemical reactions. We observed a highly differentiated, low proliferative, rather cellular and in individual parts moderately pleomorphic tumor with cells arranged in storiform or whorled patterns, that strongly expressed S-100 protein, microtubulus-associated protein 2 (MAP2) and vimentin. Postoperative visual field testing was inconspicuous, but pituitary malfunction was persistent. With respect to the accidental discovery of this pituicytoma, it remains unresolved whether the persisting headache was due solely to the head trauma or was additive with the effects of the pituicytoma. Conclusion: To date less than 30 bona fide examples have been described and typically present symptoms due to mass effects such as visual disturbances, hypopituitarism as well as interference with hypothalamic dopamine release, resulting in subsequent hyperprolactinemia accompanied by decreased libido and amenorrhea in females. These neoplasms represent an important differential diagnosis with respect to suprasellar lesions and a clinical and neuropathological challenge.Correspondence to:
A. Grote, MD
Clinic of Neurosurgery
University of Bonn
Medical Center
Sigmund-Freud-Str. 25
53105 Bonn, Germany
Email: alexander.grote@ukb.uni-bonn.de
Original contribution
Comprehensive review of extraventricular neurocytoma with report of two cases, and comparison with central neurocytoma
A. Furtado, M. Arantes, R. Silva, H. Romao, M. Resende and M. Honavar
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (134-140)
Comprehensive review of extraventricular neurocytoma with report of two cases, and comparison with central neurocytoma
A. Furtado1, M. Arantes2, R. Silva3, H. Romao4, M. Resende4 and M. Honavar1
1Anatomic Pathology Service, 2Neuroradiology Service, 3Neurology Service and 4Neurosurgery Service, Pedro Hispano Hospital, Matosinhos, Portugal
Two new cases of extraventricular neurocytoma are reported. The first concerns an 18-year-old female with a left frontal lobe lesion and the second occurred in a 54-year-old female in the thoracic spinal cord. The first patient is free of disease after 18 months of follow up. The second patient is stable after 16 months of follow up. Both were operated – total excision in Case 1 and partial resection in Case 2. The latter underwent adjuvant radiotherapy due to atypical histological features. Cases of extraventricular neurocytoma are reviewed. Differences and similarities between extraventricular and central neurocytoma are outlined.Correspondence to:
Dr. A. Furtado
Anatomia Patológica
CHVNG-E
Rua Conceição Fernandes
4434-502 Vila Nova de Gaia, Portugal
Email: antoniafurtado@gmail.com
Original contribution
Metastatic oligodendroglioma: a case report and incidence in The Netherlands
J.L.M. Krijnen, R.E.M. Fleischeur, M. van Berkel and P.J. Westenend
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (141-146)
Metastatic oligodendroglioma: a case report and incidence in The Netherlands
J.L.M. Krijnen1, R.E.M. Fleischeur2, M. van Berkel3 and P.J. Westenend1
1Laboratory of Pathology, Dordrecht, 2Department of Pathology, Elisabeth Hospital, Tilburg and 3Department of internal medicine, Beatrix Hospital, Gorinchem, The Netherlands
Oligodendroglioma is a tumor of the central nervous system which rarely metastasizes. The diagnosis of oligodendroglioma is based on histomorphology with limited use of immunohistochemistry. However, recently a specific 1p/19q codeletion has been found which can be demonstrated by in situ hybridization. We report a case of a 58-years-old man with a 31-months history of oligodendroglioma presenting with fatigue and anemia. A bone marrow biopsy demonstrated massive localization of oligodendroglioma which was confirmed by in situ hybridization for the 1p/19q deletion. In addition we studied data from PALGA, the nationwide network and registry of histo- and cytopathology in the Netherlands and found an incidence of approximately 2 in 1,000 for metastasis of oligodendroglioma outside the central nervous system.Correspondence to:
P.J. Westenend, MD
Laboratory of Pathology
Laan van Londen 1800
3317DA, Dordrecht, The Netherlands
Email: pwestenend@paldordt.nl
Original contribution
Metastatic gliosarcoma with a unique presentation and progression: case report and review of the literature
F.B. Mesfin, E.M. Deshaies, R. Patel, S. Weaver, P. Spurgas and A.J. Popp
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (147-150)
Metastatic gliosarcoma with a unique presentation and progression: case report and review of the literature
F.B. Mesfin1, E.M. Deshaies1, R. Patel3, S. Weaver2, P. Spurgas1 and A.J. Popp1
1Division of Neurosurgery, 2Department of Neurology and 3Department of Pathology, the Neurosciences Institute, Albany Medical Center, Albany, NY, USA
A 51-year-old right-handed woman initially presented with generalized tonic-clonic seizures. MRI showed abnormal signal hyperintensity of the right temporal lobe that was thought to be consistent with ischemic stroke. Three years later, she developed an intensely enhancing centrally necrotic tumor in the right temporal-parietal lobes. A craniotomy was performed with gross total resection of the tumor followed by chemotherapy and radiation treatments. Histological examination demonstrated a gliosarcoma. A year later, she had a recurrence of the intra-axial gliosarcoma requiring a second craniotomy for tumor resection and placement of Gliadel wafers. Postoperatively, she developed plural effusions. A pulmonary workup revealed lung lesions that were biopsied and found to be gliosarcoma. After the second surgery, she underwent pleurodesis and one cycle of modified mesna, doxorubicin, ifosfamide, and dacarbazine (MAID) chemotherapy, but died 5 months later from progression of the lung metastases. There are fewer than 20 reported cases of extracranial metastases of gliosarcoma. This is the first report of gliosarcoma with prolonged survival (over 2 years) and death from non-CNS metastatic gliosarcoma.Correspondence to:
F.B. Mesfin, MD, PhD
Albany Medical College
47 New Scotland Ave.
Albany, NY 12209, USA
Email: mesfinfb@mail.amc.edu
Original contribution
Intracranial inflammatory pseudotumor: case report and review of the literature
D. Yavuzer, S. Dalbayrak, B. Oz, M. Yilmaz and G. Akansel
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (151-155)
Intracranial inflammatory pseudotumor: case report and review of the literature
D. Yavuzer1, S. Dalbayrak2, B. Oz3, M. Yilmaz2 and G. Akansel4
1Department of Pathology, 2Department of 1. Neurosurgery, Dr. Lutfi Kirdar Kartal Training and Research Hospital, 3Department of Pathology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, and 4Department of Radiology, Kocaeli University School of Medicine, Izmit, Turkey
Objective: Inflammatory pseudotumor is an uncommon lesion of unknown etiology most frequently involving the lungs and orbits. Primary intracranial inflammatory pseudotumors are exceptionally rare. Herein, we report a case of inflammatory pseudotumor that arises from the central nervous system in a 25-year-old man. Material and method: The patient presented with numbness in his right arm and right leg. Computed Tomography and Magnetic Resonance Imaging demonstrated a left fronto-parietal lobulated mass with intense contrast enhancement and perilesional edema mimicking a high grade glioma or metastasis. The lesion was removed by complete surgical resection. Results: Pathologic examination showed spindle cell proliferation in a collagenous background with dense infiltrates of mononuclear inflammatory cells.The spindle cells were diffusely immunopositive for vimentin and focally positive smooth muscle actin but the cells did not show glial fibrillary acidic protein, epithelial membrane antigen, synaptophysin, S-100 protein, anaplastic lymphoma kinase-1 protein and CD1a immunoreactivity. Based on the morphologic and immunohistochemical findings, the diagnosis of inflammatory pseudotumor was made. After surgery, the symptoms had disappeared. No recurrence was observed at the eleven-month follow-up. Conclusion: Although rare, inflammatory pseudotumor of central nervous system is important in the differential diagnosis of the tumor-like intracranial lesions. We discuss the etiopathogenetic, diagnostic and therapeutic issues related to this entity, and review the literature.Correspondence to:
Dr. D. Yavuzer
Dr. Lutfi Kirdar Kartal Training and Research Hospital
Pathology Department
34865 Cevizli, Istanbul, Turkey
Email: dilekyavuzer@yahoo.com
Original contribution
Neurosarcoidosis presenting as an isolated intrasellar mass: case report and review of the literature
M.M. Kimball, J.J. Wind, K.-E.T. Codispoti, R.V. Jones and J.W. Leiphart
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (156-162)
Neurosarcoidosis presenting as an isolated intrasellar mass: case report and review of the literature
M.M. Kimball1, J.J. Wind2, K.-E.T. Codispoti3, R.V. Jones3 and J.W. Leiphart2
1Department of Neurosurgery, University of Florida, Gainesville, FL, 2Department of Neurosurgery and 3Department of Pathology, The George Washington University, Washington, DC, USA
Objective: Isolated neurosarcoidosis without evidence of extracranial manifestation continues to be a rare phenomenon. This case report and others in the literature demonstrate the difficulty in making the diagnosis of isolated neurosarcoidosis, as it may be indistinguishable from other pathologies on radiographic and laboratory studies. This case report and review of the literature will emphasize the need for clinical suspicion for neurosarcoidosis in patients with intrasellar lesions and the appropriate clinical history. Case history: A 37-year-old female presented with visual field changes and a headache unresponsive to nonsteroidal anti-inflammatory medications. A history of Bell’s palsy, hypothyroidism, and a history of sarcoidosis in the patient’s father were noted. Imaging revealed an intrasellar mass resembling a pituitary macroadenoma. Routine neuroendocrine laboratory studies were consistent with hypopituitarism, and all other standard laboratory tests were normal. An endonasal transsphenoidal resection of the intrasellar lesion was done. The tissue was inconsistent with a typical adenoma. Intraoperative pathology reported non-caseating granulomatous disease. Based on the patient’s history and intraoperative pathology she was diagnosed with neurosarcoidosis, which was confirmed by final pathologic analysis. Minimal debulking was performed to decompress the optic chiasm. The patient was then placed on corticosteroids and methotrexate and responded well to medical therapy. Conclusion: If isolated neurosarcoidosis is diagnosed early it will save a costly and invasive work-up. Radiographic and laboratory studies may aid in diagnosis but no studies are pathognomonic. Neurosarcoidosis is diagnosed by a combination of imaging, diagnostic tests, and good clinical suspicion.Correspondence to:
J.W. Leiphart, MD, PhD
Assistant Professor
Director of Stereotactic and Functional Neurosurgery
Department of Neurosurgery
The George Washington University
2150 Pennsylvania Avenue, NW, Suite 7-420
Washington, DC 20037, USA
Email: jleiphart@mfa.gwu.edu
Original contribution
Giant chordoid meningioma symptomatic immediately after pregnancy: report of a rare case
B. Bakar, A. Sav and I.H. Tekkok
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (163-168)
Giant chordoid meningioma symptomatic immediately after pregnancy: report of a rare case
B. Bakar1, A. Sav2 and I.H. Tekkok3
1Kirikkale University, Faculty of Medicine, Department of Neurosurgery, Kirikkale, 2Acibadem Hospital, Department of Pathology, Istanbul, and 3MESA Hospital, Department of Neurosurgery, Ankara, Turkey
Background: Chordoid meningioma is a very rare subtype of meningioma with less than 90 cases reported in the literature. Meningiomas related with a pregnancy are also very rare. Herein, we present a pregnancy-associated case of a chordoid meningioma and briefly discuss possible mechanisms. Case History: Approximately 10 hours after a Caesarean section, a 37-year-old woman became stuporous with a Glasgow coma scale score of 9 – 10 and the right pupil became dilated without reaction to light. An emergency computed tomography scan showed a right frontotemporal lesion measuring 8 × 7 × 6 cm which enhanced moderately and homogenously after intravenous contrast injection. The patient’s hemoglobin level was slightly lower than normal level but there was no dysgammaglobulinemia or lymphoid hyperplasia. The patient was immediately taken to the operating theater and the tumor was gross totally removed. The postoperative period was uneventful; and histopathological diagnosis was chordoid meningioma. Conclusion: The exact mechanism causing a pregnancy-associated increase in the size of meningiomas and the amount of peritumoral edema has not been clearly elucidated. As for this case of chordoid meningioma associated with pregnancy, we think mucin accumulation in tumor could be involved in an increase in the tumor size while delivery procedures with the common anaesthetic and sedative drugs may also have enhanced the peritumoral edema by causing a relative decrease in the cerebral blood flow.Correspondence to:
B. Bakar, MD
Kirikkale University
Faculty of Medicine
Department of Neurosurgery
71100 Kirikkale, Turkey
Email: bulentbanrs@yahoo.com
Original contribution
Primary intradural chordoma: report on three cases and review of the literature
M. Bergmann, Y. Abdalla, U. Neubauer, H.-U. Schildhaus and S. Probst-Cousin
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (169-176)
Primary intradural chordoma: report on three cases and review of the literature
M. Bergmann1, Y. Abdalla2, U. Neubauer3, H.-U. Schildhaus4 and S. Probst-Cousin5
1Department of Neuropathology, Klinikum Bremen-Mitte, Bremen, 2Department of Neurosurgery, Nordwest-Krankenhaus Sanderbusch, Sande, 3Department of Neurosurgery, Klinikum Bremen-Mitte, Bremen, 4Department of Pathology, University Bonn, Bonn, and 5Department of Neurology, Klinikum Bremen-Ost, Bremen, Germany
Objective: Chordomas are rare malignant bone tumors of the skull base or sacrococcygeal region. They derive from notochordal remnants and usually have a chronic progressive course. Even rarer, intradural chordomas with a better biological behavior have also been reported. We present 3 further primary intradural extraosseous chordomas with a favorable clinical outcome. Clinical presentation: Two patients, a 38-year-old man and a 44-year-old woman, presented with neck pain. In these, intradural extraspinal tumors within intervertebral foramina were found. Both tumors were totally removed and the patients have been free of disease for 7 years and 1 year, respectively. The other patient, a 76-year-old man suffered from an unspecific gait disorder and diplopia as a result of a prepontine space-occupying lesion. In this case, only an incomplete tumor resection was possible but progression has not occurred for 5 years. Materials and methods: Paraffin blocks from all cases were examined with classical histopathological stainings and immunohistochemistry for pancytokeratin, CK7, CK8/18, CK19, EMA, CEA, vimentin, S100, aktin, desmin, GFAP, CD117, PDGF-receptor α and β, collagen-type-IV, p63, and Ki67. Fluorescent in situ hybridization was used to exclude EWS translocation. Results: All cases showed the typical histological picture with physaliphorous cells in a myxoid matrix and the characteristic immunohistochemical profile with positivity for vimentin, pancytokeratin, CK19, EMA, and S100. Staining for P63 and type IV collagen was consistently negative. Myxoid extraskeletal chondrosarcoma was excluded by in-situ-hybridization of the EWS gene. Conclusion: Considering our cases in context with so far published literature, we conclude that intradural chordomas are rare and in this location usually have a better prognosis compared to classical intraosseous chordomas.Correspondence to:
PD Dr. S. Probst-Cousin
Department of Neurology
Klinikum Bremen-Ost
Züricher Straße 40
28325 Bremen, Germany
Email: stefan.probst@klinikum-bremen-ost.de
Original contribution
The history of neuropathology in Italy
D. Schiffer
Abstract
Clinical Neuropathology, Vol. 29 – No. 3/2010 (177-181)
The history of neuropathology in Italy
D. Schiffer
CNBO - Policlinico di Monza Foundation, University of Turin, Vercelli, Italy
The history of Italian Neuropathology begins in the XIX century with Lombroso with his studies of criminal and prostitutes, inspired by the positivism of the era, and on the brain of epileptic patients. It reached its peak at the beginning of XX century with Camillo Golgi, Nobel laureate for his impregnation of neurons and the theory of the diffuse neuronal net. Neuropathology was then cultivated in Asylums and Universities where the main subject of interest were dementias and degenerative diseases, followed by vascular and inflammatory diseases. Some Laboratories arose in the country, especially in neurological institutes and some people later began to emigrate, especially to France and Germany and then to USA in order to improve their Neuropathology. Starting in the late fifties of the 20th century there was a progressive enrichment of Neuropathology with histochemistry, electron microscopy, immunohistochemistry and then molecular biology and the number of Laboratories increased consistently. As in other developed countries, Neuropathology with the enlargement of its scientific fields, began to split in sub-disciplines. It remained as a wide spectrum of knowledge, but neuropathologists were obliged to specialize in specific areas of the discipline. The continuous change of the set up of the university studies in the country in the last 20 years did not favor Neuropathology from which, moreover, some new independent disciplines originated.Correspondence to:
D. Schiffer
CNBO – Policlinico di Monza Foundation
University of Turin
Via Pietro Micca, 29
Vercelli 13100, Italy
Email: davide.schiffer@unito.it
Abstracts
Joint Meeting: XLVI Congress of the Italian Association of Neuropathology (AINP), XXXVI Congress of the Italian Association for Research on Brain Aging (AIRIC) – Squillace, Catanzaro, Italy, May 23 – 25, 2010
Euro-CNS News
Society News of the European Confederation of Neuropathological Societies
