Volume 28, No. 3/2009(May/June)
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 Clinical Neuropathology
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Tumor
Efficient systemic therapy of rat glioblastoma by nanoparticle-bound doxorubicin is due to antiangiogenic effects
Abstract
T. Hekmatara1*, C. Bernreuther2*, A.S. Khalansky3, A. Theisen4, J. Weissenberger5, J. Matschke2, S. Gelperina6, J. Kreuter1 and M. Glatzel2
1Institute of Pharmaceutical Technology, J.-W. Goethe-University, Frankfurt/Main, 2Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany, 3Institute of Human Morphology, Moscow, Russia, 4Zentrale Forschungseinrichtung, Klinikum Goethe-University, 5Neuro Science Center, Exp. Neurochirurgie, Frankfurt/Main, Germany, and 6Nanosystem Ltd., Moscow, Russia
The objective of this study was to investigate the therapeutic effects of doxorubicin bound to polysorbate-coated nanoparticles that had previously been shown to significantly enhance survival in the orthotopic rat 101/8 glioblastoma model. Tumor-bearing animals were subjected to chemotherapy using doxorubicin in solution (Dox-sol) or doxorubicin bound to polysorbate 80-coated poly(butyl cyanoacrylate) nanoparticles (Dox-np) injected intravenously on Days 2, 5 and 8 post tumor implantation. The antitumor effect was assessed on Days 10, 14 and 18 post tumor implantation. Tumors showed signs of malignancy including invasion of brain tissue, brisk mitotic activity, microvascular proliferation, necrosis and increased proliferation resembling human glioblastoma. Dox-np produced a considerably more pronounced antitumor effect exhibited as a reduced tumor size, lower proliferation, and a decreased necrotic area compared to Dox-sol and to untreated control groups. A drastic effect of Dox-np on vascularization indicated an antiangiogenic mode of action.Correspondence to:
M. Glatzel, MD; Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Martinistraße 52, 20246 Hamburg, Germany
Email: m.glatzel@uke.uni-hamburg.de
Tumor
Glioblastoma multiforme with an unusual location and clinical course
Abstract
É. Gömöri1, J.D. Halbauer2, G. Kasza3, D. Varga4, Zs. Horvath5 and S. Komoly4
1Department of Pathology, 3Department of Surgery, 4Department of Neurology, 5Department of Neurosurgery, Faculty of Medicine, Pécs University, Hungary, and 2Department of Psychiatry, Stanford University, Palo Alto, CA, USA
We present a unique case of a brain tumor patient with atypical location and progression. He was initially presented with mood and anxiety type symptoms together with aphasia and left-sided paraesthesias. Magnetic resonance imaging and CSF were negative and the patient was diagnosed with PTSD as he recently experienced a small motor vehicle accident. Two months after the first presentation, MRI revealed multifocal juxtacortical, leptomeningeal hyperdensities in the bilateral frontal lobes. MRI-guided frameless stereotactic biopsy defined a diagnosis of GBM 1 week prior to death which occurred within 4 months. Postmortally, formalin-fixed brain demonstrated that the main tumor mass was located in the fornix, infiltrating the ventricular system and disseminating over the cortex, cerebellum and spinal cord. The authors recommend closer scrutiny of psychiatric patients presenting CNS symptomatology, negative MRI, CT and CSF.Correspondence to:
É. Gömöri, MD, PhD; Department of Pathology, Faculty of Medicine, Pécs University, 7624 Pécs,
Szigeti út 12., Hungary
Email: eva.gomori@kk.pte.hu
Tumor
Rosenthal fiber-rich glioblastoma: a case report
Abstract
H. Takei and S.Z. Powell
Department of Pathology, The Methodist Hospital, Houston, TX, USA
Background: Rosenthal fibers (RFs) are thick, elongated, brightly eosinophilic structures occurring within astrocytic processes. Although the presence of abundant RFs within brain tumors is most closely associated with a low-grade astrocytoma, particularly pilocytic astrocytoma (PA), a few RFs are recognized to occur, although rarely, in glioblastoma (GBM). We report a very rare case of GBM with abundant RFs. Case report: A 60-year-old woman presented with a 6-month history of difficulty coordinating her right hand, occasional intermittent diplopia, and occasional dizzy spells. An MRI showed a heterogeneously enhancing, infiltrating mass lesion with a cystic component involving the left midbrain, thalamus, and posterior basal ganglia. Biopsy was performed. Cytologic touch imprints revealed fibrillary astrocytic cells possessing oval nuclei and long delicate processes with abundant RFs. Histologic sections showed diffusely infiltrating astrocytoma with prominent RFs diffusely distributed throughout the tumor, brisk mitotic activity, vascular proliferation, and small areas of necrosis, as seen in a GBM. The Ki-67 (MIB-1) labeling index was 7.1%. P53 immunoreactivity was not seen. A follow-up MRI study performed 3 months after the biopsy showed a considerable tumor progression with extension into the left superior cerebellar peduncle and progressive hydrocephalus. Discussion: This is a case of RF-rich GBM (primary or de novo type). The differential diagnosis includes PA and anaplastic PA. For the histological diagnosis, infiltrating astrocytoma with abundant RFs should be carefully examined in light of clinical information (e.g., patient age, evolution of the symptoms) and neuroimaging studies.Correspondence to:
H. Takei, MD; Department of Pathology, The Methodist Hospital, 6565 Fannin Street, Houston, TX 77030, USA
Email: takei327@aol.com
Tumor
Simultaneous conus medullaris ependymoma and cerebellar astrocytoma in the same patient
Abstract
T. Marinovic1, G. Grahovac1, M. Habek3, S. Lambasa2 and D. Tomac1
Departments of 1Neurosurgery and 2Pathology, University Hospital Dubrava, and 3University Department of Neurology, Zagreb School of Medicine and University Hospital Center, Zagreb, Croatia
Multiple primary tumors in the central nervous system of different histological cell types are uncommon. We report a patient who had pilocytic astrocytoma in the cerebellum and ependymoma in the cauda equina region, occurring simultaneously. The suggested mechanism of this association is that primitive multipotent cells might have been displaced in the different CNS areas and developed in different tumor cells. Multiplicity of primary CNS tumors should be considered in certain occasions, when clinical symptoms and signs are pointing in that direction.Correspondence to:
G. Grahovac; Department of Neurosurgery, University Hospital “Dubrava”, Av. G. Suska 6, 10000 Zagreb, Croatia
Email: ggrahov@mef.hr
Tumor
Late development of craniopharyngioma following surgery for Rathke’s cleft cyst
Abstract
Y.S. Park1, J.Y. Ahn1, D.S. Kim1, T.S. Kim2 and S.H. Kim1
1Department of Neurosurgery, Brain Research Institute, and 2Department of Pathology, Yonsei University College of Medicine, Seoul, Republic of Korea
Objective: Rathke’s cleft cyst (RCC) may transform to papillary type craniopharyngioma (CP) after squamous metaplasia: this is referred to as ciliated CP. We encountered a case involving a patient who had late development of adamantinomatous CP following surgery for RCC, the details of which may shed light on the histogenesis of CP in general. Patient: A 41-year-old man presented to our institution with visual disturbance, and magnetic resonance imaging (MRI) showed a cystic mass in the suprasellar region. The patient underwent a biopsy via a transsphenoidal approach and was diagnosed as having a RCC. 34 months after the initial surgery, the patient revisited our hospital for a rapidly aggravating visual disturbance and underwent neuroendoscopic biopsy and tumor removal via a bifrontal craniotomy. Histologically, the tumor was shown to be an adamantinomatous CP. No nuclear beta-catenin accumulation was detectable in the previous RCC specimen, but nuclear beta-catenin accumulation was found in the recent CP specimen, restricted to whorl-like structures or surrounding ghost cells. Conclusions: Our case of adamantinomatous CP that developed long after removal of the RCC, diagnosed by beta-catenin staining, supports the hypothesis that CPs may develop from RCCs directly due to beta-catenin mutations. However, it still does not prove that a histogenetic connection can be shown between the two lesions which are clonally unrelated. Our case is reported as two consecutive lesions; this in itself is a rare situation.Correspondence to:
J.Y. Ahn, MD, PhD; Department of Neurosurgery, Yongdong Severance Hospital, Yonsei University College of Medicine, 146-92, Dogok-dong, Kangnam-gu, Seoul, 135-720, Korea
Email: jyahn@ymc.yuhs.ac
Tumor
Plurihormonal gonadotroph cell pituitary adenoma: report of a unique case
Abstract
M. Al-Sharim1, B.W. Scheithauer4, E. Horvath1, K. Kovacs1, H. Smyth2, C. Coire2, R.V. Lloyd4, R. Jastania1 and M. Al-Gahtany3
Departments of 1Laboratory Medicine and 2Neurosurgery, St. Michael’s Hospital, 3Department of Neurosurgery, Toronto Western Hospital, University of Toronto, Ontario, Canada, and 4Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
Objective and importance: Pituitary adenomas producing primarily FSH and to a lesser extent GH, LH, alpha-subunit, TSH and PRL without clinical or laboratory evidence of increased hormone release have not previously been reported. Our aim was to obtain some insight into the possible cytogenesis of this unusual tumor. Clinical presentation: A 65-year-old woman presented with headaches. Magnetic resonance imaging (MRI) demonstrated a sellar mass. Pituitary hormone assays showed normal blood levels. The tumor was removed by the transsphenoidal approach. Result: By light microscopy, the adenoma was chromophobic, weakly PAS-positive, and immunoreactive mainly for FSH (85%) and to a lesser extent for GH (30%), LH (15%), alpha-subunit (3%), TSH (2%), and PRL (1%). Although double immunostaining showed hormone reactivities to be localized largely in separate distinct cells, the tumor was ultrastructurally monomorphous, i.e., consisted of a single-cell type, resembling gonadotrophs. Conclusion: The cytogenesis of plurihormonal pituitary adenomas is not fully understood. Further investigations are required to clarify the basis for their plurihormonality despite an ultrastructural gonadotroph phenotype.Correspondence to:
B.W. Scheithauer, MD; Department of Pathology and Laboratory Medicine Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA
Email: scheithauer.bernd@mayo.edu
Tumor
Occult leptomeningeal large cell medulloblastoma in an adult
Abstract
E.J. Rushing1, A.B. Smith2,3, J.G. Smirniotopoulos2,3, A.F. Douglas4, W. Zeng5 and N. Azumi5
Departments of 1Neuropathology and Ophthalmic Pathology and 2Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC, 3Department of Radiology, Uniformed Services University of the Health Sciences, Bethesda, MD, 4Departments of 4Neurosurgery and 5Pathology, Georgetown University Hospital, Washington, DC, USA
Objective and importance: Large cell medulloblastoma is an uncommon malignancy of childhood that often pursues an aggressive clinical course. We report the first case of this entity in an adult that proved to be an unsuspected primary leptomeningeal tumor. Clinical presentation: A 30-year-old man complained of worsening neck pain over the course of 3 months. Neck pain increased a few days prior to admission and a cervical spine CT revealed tonsillar herniation. Cervical spine MRI performed the day prior to admission confirmed the diagnosis of Chiari I malformation and C3 – 4 disk herniation without spinal cord compression. On the day of admission, the patient became unresponsive and resuscitative measures were unsuccessful. Postmortem examination of the brain was notable for necrotic cerebellar tonsils, but demonstrated no evidence of an intraparenchymal mass lesion. Microscopic examination of the cerebellum revealed discohesive neoplastic cells, which showed characteristic dot-like immunoreactivity for synaptophysin, diagnostic of large cell medulloblastoma within the subarachnoid space. Conclusions: Our experience with this unique case illustrates the challenges of diagnosing a primary leptomeningeal neoplasm. This case also underscores the importance of maintaining a high degree of suspicion for leptomeningeal neoplasms in patients who present with imaging studies suspicious for Chiari I malformation.Correspondence to:
E.J. Rushing, MD; Department of Neuropathology, Armed Forces Institute of Pathology, Washington DC 20306-6000, USA
Email: rushinge@afip.osd.mil
Tumor
Multiple ganglioneuromas: a report of a case and review of the ganglioneuromas
Abstract
X. Dai1, R. Zhang1, Y. Li2 and G. Wu1
1Cancer Center, Union Hospital, Tong Ji Medical College, Huazhong University of Science and Technology, Wuhan, China, and 2Institute of Pathology, Tong Ji Hospital, Tong Ji Medical College, Huazhong University of Science and Technology, Wuhan, China
Background: Ganglioneuromas are rare, benign, slowly growing and well-differentiated tumors of neurogenic origin, which are composed of ganglioid cells and Schwann cells. They usually present in patients under 30 years of age with a slight female predominance. They are most commonly localized in the posterior mediastinum (41.5%). Moreover, ganglioneuroma with multiple tumor masses in several organs is unusual. Case presentation: Here, we report and discuss a case of multiple ganglioneuromas in the maxillary sinus, liver, adrenal and mediastinum of a 30 years old female patient.Correspondence to:
Prof. G. Wu; Cancer Center, Wuhan Union Hospital, Wuhan, 430024, China
Email: wugangzr@yahoo.com.cn
Tumor
A case of idiopathic hypereosinophilic syndrome with leptomeningeal dissemination and intraventricular mass lesion: an autopsy report
Abstract
M. Kanamori1, H. Suzuki2, I. Sato3, K. Ohyama4, F. Tezuka2 and R. Katakura1
1Department of Neurosurgery, Miyagi Cancer Center, Natori, 2Department of Pathology and Laboratory Medicine, Sendai Medical Center, Sendai, 3Department of Pathology, Miyagi Cancer Center, Natori, and 4Department of Otolaryngology, Tohoku Rosai Hospital, Sendai, Miyagi, Japan
A 43-year-old female presented with idiopathic hypereosinophilic syndrome (HES) manifesting as an intraventricular mass lesion and leptomeningeal and cerebral parenchymal infiltration by eosinophils, lymphocytes and macrophages. She had no history of either malignancy or allergic disorder. She complained of hearing disturbance caused by eosinophilic otitis media. Eosinophilia was detected in the peripheral blood. Hearing disturbance and eosinophilia improved with corticosteroid treatment. Six months later, she was admitted with disturbances of consciousness. Magnetic resonance imaging revealed a mass lesion in the right lateral ventricle and leptomeningeal involvement around the brain stem. Her symptoms deteriorated rapidly, and she died of brain stem malfunction. Autopsy demonstrated significant infiltration by eosinophils and lymphocytes into the mass lesion in the ventricle, subarachnoid space, perivascular space and parenchyma of the medulla oblongata. Histological examination of the bone marrow and other organs did not detect any evidence of parasites, malignancies, or systemic disorders in any organ. The final diagnosis was idiopathic HES. The present case shows that leptomeningeal dissemination and infiltration by eosinophils into the cerebral ventricles and brain stem should be considered in the course of idiopathic HES.Correspondence to:
R. Katakura MD, PhD; Department of Neurosurgery, Miyagi Cancer Center, 47 Medeshima, Natori, Miyagi 981-1293, Japan
Email: katakura-ry843@pref.miyagi.jp
Infectious disease
Do cyst wall thickness and cyst size have any effect on the intra-operative inadvertent rupture of echinococcal hydatid cyst of central nervous system?
Abstract
N. Balak1, Ç. Bayindir2 and E. Uzuner2
1Department of Neurosurgery, Göztepe Education and Research Hospital, and 2Division of Neuropathology, University of Istanbul, Medical Faculty of Istanbul, Istanbul, Turkey
Our primary aim was to investigate whether there is any association between thickness of the hydatid cyst’s wall and size and whether it has any effect on intact removal of the cyst, independent of the surgeons’ technique. Sixteen cases of echinococcal hydatid cyst of the central nervous system, operated on between 1991 and 2007 by various surgeons, were examined. Cyst diameter was directly measured on the specimen or calculated on radiological scans. Histological preparations were made identically by cutting 5-µm-thick longitudinal sections from paraffin blocks of formalin-fixed tissue and stained with HE. Wall thickness was measured using a micrometer by the same pathologist blinded to the measurements of cyst diameter. Cyst diameter and wall thickness correlated negatively (the larger the cyst the thinner the cyst wall); however this was not statistically significant. Neither the cyst size nor the wall thickness proved to be statistically significant as a factor having an effect on intact removal of the cyst. In conclusion, the cyst size and wall thickness do not have any effect on the intact removal of hydatid cyst. It seems that the use of appropriate surgical techniques play the most effective role in outcome of hydatid cyst surgery.Correspondence to:
N. Balak, MD; Goztepe Education and Research Hospital, Department of Neurosurgery, Kadiköy,
Istanbul 34730, Turkey
Email: naci.balak@attglobal.net
Infectious disease
Persistent toxoplasma bradyzoite cysts in the brain: incidental finding in an immunocompetent patient without evidence of a toxoplasmosis
Abstract
L. Pusch1, B. Romeike1, M. Deckert2 and C. Mawrin1,3
1Department of Neuropathology, Friedrich-Schiller University, Jena, 2Department of Neuropathology, University of Cologne, Cologne, and 3Institute of Neuropathology, Otto-von-Guericke-University, Magdeburg, Germany
We report on a 72-year-old patient in whom autopsy demonstrated incidentally intracerebral Toxoplasma gondii cysts, locally restricted in the occipital lobe, in association with only a few CD4+ and CD8+ T cells and a mild microglial activation. The patient was HIV-negative. Serologically, there was no evidence for an active inflammation (Toxoplasma gondii specific antibody IgG-positive, IgM-negative). This unusual observation may indicate that in patients with sepsis, who may yield to a state of immunodysbalance, a focal reactivation of parasites may ensue in the absence of conditions predisposing for opportunistic infection.Correspondence to:
Prof. Dr. med. C. Mawrin; Institute of Neuropathology, Otto-von-Guericke-University, Leipziger Straße 44, 39120 Magdeburg, Germany
Email: christian.mawrin@med.svgn.de
Neuropathy
Adult fulminant subacute sclerosing panencephalitis: pathological and molecular studies. A case report
Abstract
J.B. Souraud1, A. Faivre2, D. Waku-Kouomou3, T. Gaillard1, N. Aouad4, E. Meaudre5, F.T. Wild3, B. Fouet1 and R. Soulard1
1Department of Pathology, 2Department of Neurology, HIA Sainte-Anne, Toulon, 3INSERM U 404, Université Claude Bernard Lyon, IFR128 BioScience Lyon-Gerland, Centre National de Référence pour la Rougeole, Lyon, and 4Department of Neurosurgery and 5Department of Anesthesiology and Intensive Care, HIA Sainte-Anne, Toulon, France
Subacute sclerosing panencephalitis is an uncommon progressive neurological disorder caused by a persistent defective measles virus, typically affecting children. We describe a case of fulminant subacute sclerosing panencephalitis in a 25-year-old male. Brain tissue biopsy showed histologic evidence of encephalitis with eosinophilic intranuclear inclusion bodies (Cowdry Type A and B), intracytoplasmic inclusion bodies, perivascular lymphoplasmacytic infiltration and gliosis. Immunohistochemical studies were positive using an anti-measles antibody. Reverse transcriptase-PCR detected measles virus RNA and phylogenetic analysis indicated a C2 genotype. The rare adult-onset form is often atypical and difficult to diagnose and should be included in the differential diagnosis of subacute “unexplained” neurological diseases and uncommon infectious disorders.Correspondence to:
J.B. Souraud, MD; Department of Pathology, HIA Sainte-Anne, Toulon 83800, France
Email: jsouraud@hotmail.com
Abstracts
Joint Meeting: XLV. Congress of the Italian Association of Neuropathology (AINP) Xand XXV. Congress of the Italian Association for Research on Brain Aging (AIRIC)
Euro-CNS News
Society News of the European Confederation of Neuropathological Societies
