Volume 29, No. 1/2010(Jan/Feb)
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 Clinical Neuropathology
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Editorial
Letter of the Editor
J. Hainfellner
Original contribution
Malignant transformation in craniopharyngioma after radiation therapy: a case report and review of the literature
M. Ishida, M. Hotta, A. Tsukamura, T. Taga, H. Kato, S. Ohta, Y. Takeuchi, S. Nakasu and H. Okabe
Abstract
Clinical Neuropathology, Vol. 29 – No. 1/2010 (2-7)
Malignant transformation in craniopharyngioma after radiation therapy: a case report and review of the literature
M. Ishida1, M. Hotta1, A. Tsukamura2, T. Taga2, H. Kato2, S. Ohta2, Y. Takeuchi2, S. Nakasu3 and H. Okabe1
1Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology 2Department of Pediatrics, Shiga University of Medical Science, and 3Department of Neurosurgery, Kusatsu General Hospital, Shiga, Japan
Objective: Craniopharyngioma is a benign epithelial tumor that is thought to arise from the remnant of the Rathke pouch. Malignant transformation in craniopharyngioma is extremely rare. Herein, we report a case of malignant transformation in craniopharyngioma after radiation therapy. Materials and methods: Histopathological and immunohistochemical analyses were carried out for specimens of the suprasellar tumor (from three resections, with the third surgery performed after radiation therapy). Results: The resected tumors from the first and second surgeries comprised islands of loosely cohesive aggregates of epithelial cells, so-called stellate reticulum. At the periphery of the nests, palisaded columnar epithelium was observed. Wet keratins were scattered, and few mitotic figures were seen. The third surgical specimen was composed of irregular large nests of basaloid cells that had large, round to oval nuclei with prominent nucleoli, and mitotic figures were frequently seen (21/10 high power fields). In the center of the nests, eosinophilic ghost cells, resembling wet keratin, were observed. Accordingly, the diagnosis of malignant transformation in craniopharyngioma was made. Immunohistochemical studies revealed that the p53 protein was over-expressed in the malignant component, whereas its expression was much lower in the benign component. Conclusions: Similar to the ten previously reported cases of malignant transformation in craniopharyngioma, the present case occurred after radiation therapy. p53 protein overexpression was also observed in the earlier cases of malignant craniopharyngioma as well as in the present case (6/6 cases). We concluded that radiation therapy and p53 mutations could be involved in malignant transformation in craniopharyngioma.Correspondence to:
H. Okabe, MD, PhD
Department of Clinical Laboratory
Medicine and Division of Diagnostic Pathology
Shiga University of Medical Science
Tsukinowa-cho, Seta
Otsu, Shiga, 520-2192, Japan
Email: okabe@belle.shiga-med.ac.jp
Original contribution
Intracranial extension of salivary gland tumors
F. Maiuri, M. Gangemi, A. Giamundo, G. Mariniello, A. Colella, P. Vergara and M.L. Del Basso De Caro
Abstract
F. Maiuri1, M. Gangemi1, A. Giamundo1, G. Mariniello1, A. Colella1, P. Vergara1 and M.L. Del Basso De Caro2
1Department of Neurological Sciences, Neurosurgical Clinic, and 2Department of Biomorphological and Functional Sciences, Section of Pathology, Federico II University, Naples, Italy
Objective: The aim of this report is to describe 3 cases of salivary gland tumors with intracranial extension associated to an extracerebral mass lesion, and to discuss the frequence, pathology and treatment of these very rare localizations. Clinical material: The 3 patients were 1 woman and 2 men, aged 44, 53 and 74 years, respectively. The primary tumors were an adenocarcinoma and a malignant oncocytoma of the parotid gland and an adenoid cystic carcinoma of the submandibular gland. The location of the intradural extra-axial tumor was the middle fossa and temporal region in 2 cases and the cerebellopontine angle in 1. Surgical treatment consisted in the seemingly complete removal of 2 tumors with middle fossa localization and partial removal of the cerebellopontine angle lesion. Radiotherapy was administered in all 3 cases and chemotherapy in 2. Results: 1 patient is alive and free of recurrence 32 months after removal of the intracranial tumor; 2 other patients died 28 months and 12 months postoperatively. Conclusions: The intracranial extension of salivary gland tumors is a very rare event. An aggressive surgical resection followed by radiotherapy is justified in cases with significant intracranial mass lesions and scarce bone and dural involvement.Correspondence to:
Prof. F. Maiuri
Cattedra di Neurochirurgia
Università Federico II
Via Pansini 5
80131 Napoli, Italy
Email: frmaiuri@yahoo.it
Original contribution
Oncocytic choroid plexus carcinoma: case report
A. Sav, B.W. Scheithauer, C.A. Mazzola, S.R.P. Ketterling, S.J. Thompson and M.H. Reilly
Abstract
A. Sav1, B.W. Scheithauer1, C.A. Mazzola2, S.R.P. Ketterling1, S.J. Thompson3 and M.H. Reilly4
1Department of Pathology, Mayo Clinic, Rochester, Minnesota, Departments of 2Neurosurgery, 3Oncology and 4Pathology, The Valley Hospital, Ridgewood, New Jersey, USA
Herein, we report an unusual choroid plexus carcinoma with extensive oncocytic transformation. A 13-month-old girl presented with acute lethargy which quickly progressed to coma. A CT scan of the head revealed impending herniation due to hemorrhage within an intracranial tumor. An MRI scan showed a large, partly cystic and highly vascular left lateral ventricular mass. A near total resection was achieved. Microsections revealed a WHO Grade III choroid plexus carcinoma with extensive oncocyti c transformation. A minor portion of the moderately to poorly differentiated tumor exhibited classical microscopic features of choroid plexus carcinoma, including marked nuclear atypia, brisk mitotic activity (78/10 HPF), a high MIB-1 labeling index (44%) and zones of necrosis. In contrast, the large, eosinophilic, cytologically malignant but granular-appearing oncocytes comprising the majority of the lesion showed scant (1/10 HPF) mitotic activity and only a low MIB-1 labeling index (5%). A subsequent recurrence at 1 year consisted entirely of non-oncocytic tumor. Choroid plexus carcinoma with oncocytic transformation has not been previously reported. The remarkable extent of this alteration and its clinical significance remains to be determined.Correspondence to:
B.W. Scheithauer, MD
Mayo Clinic
Department of Laboratory Medicine and Pathology
200 First Street, SW
Rochester, MN 55905, USA
Email: scheithauer.bernd@mayo.edu
Original contribution
Primary angioleiomyoma in the sellar region: a case report and literature review
Y. Xu, Y. Jing, S. Ma, F. Ma, Y. Wang, W. Ma and Q. Li
Abstract
Y. Xu1, Y. Jing1, S. Ma1, F. Ma1, Y. Wang1, W. Ma2 and Q. Li1
1Department of Pathology, 2Department of Radiology, Xijing Hospital, Fourth Military Medical University, Xi’an, PR China
Objective: Angioleiomyoma (vascular leiomyomas, angiomyoma) is a rare, benign soft tissue tumor which consists of a mixture of well-differentiated smooth muscle cells and thick-walled vessels. Here we report a case of angioleiomyoma in the sellar region, which is the first case report of angioleiomyoma at this anatomical location. Clinical presentation: A 53-year-old man was admitted with a medical history of three months episodic headache and one month visual deterioration, both of which progressed gradually. The neurological examination showed nothing remarkable except a slightly decreased adrenocorticotrophic hormone (ACTH) level. Preoperative magnetic resonance imaging revealed a pituitary apoplexy. Gross total resection of the tumor was then performed. Pathological findings: Histological and immunohistochemical studies showed a typical microscopic appearance of an angioleiomyoma present in a rather uncommon place. The pertinent literature regarding the features of this tumor was reviewed and discussed. Conclusions: Intracranial angioleiomyoma is a benign soft tissue tumor with excellent prognosis. Early diagnosis of this tumor is difficult.Correspondence to:
Prof. Q. Li, MD, PhD
Department of Pathology
Xijing Hospital
Fourth Military Medical University
Xi’an, 710032, P.R. China
Email: liqing@fmmu.edu.cn
Original contribution
Lymphocytic hypophysitis with normal pituitary function mimicking a pituitary adenoma: a case report and review of literature
M. Rumana, A. Kirmani, N. Khursheed, S. Besina and M. Khalil
Abstract
M. Rumana1, A. Kirmani2, N. Khursheed2, S. Besina1 and M. Khalil1
Departments of 1Pathology and 2Neurosurgery, SKIMS, Kashmir, India
Lymphocytic hypophysitis (LYH) is a rare autoimmune inflammatory disorder of the pituitary gland usually affecting young women, often in pregnancy or post partum period. It is rare in non-pregnant females and in men. Patients present with symptoms of an expanding pituitary mass and/or varying degrees of pituitary dysfunction. We, hereby report a case of lymphocytic hypophysitis in a 28-year-old non-pregnant female who presented with clinical and radiological features of a pituitary tumor with normal pituitary hormones which on histopathological examination revealed features of lymphocytic hypophysitis. The case is presented here for its rarity and its unique presentation as an expanding pituitary mass with normal pituitary function. Peculiar clinical, radiological and histopathological features of this uncommon entity are discussed.Correspondence to:
Dr. R. Makhdoomi
Assistant Professor
Department of Pathology
Sher-i-Kashmir Institute of Medical Sciences
Srinagar-190 011, India
Email: rumanahamid@rediffmail.com
Original contribution
Role of dentate gyrus alterations in mesial temporal sclerosis
G. Marucci, G. Rubboli and M. Giulioni
Abstract
G. Marucci1, G. Rubboli2 and M. Giulioni3
1Section of Pathology, Department of Hematology and Oncology of the University of Bologna, 2Division of Neurology and 3Division of Neurosurgery, Bellaria Hospital, Bologna, Italy
Drug-resistant chronic temporal lobe epilepsy is the most common type of epilepsy that undergoes surgical treatment. To verify if dentate gyrus alterations may play a role in patients with mesial temporal sclerosis (MTS), 14 patients, submitted to epilepsy surgery, were selected. Only cases with MTS alone were included. Granule cell dispersion (GCD) was observed in 7 cases (50%). A statistically significant correlation between GCD and the mean number of seizures/month was evidenced. The percentage of patients who did not achieve seizure relief (i.e. they were not in Engel class 1A) was 57.14% in patients without GCD, whereas that percentage dropped to 14.29% in patients with GCD. The association between a more favorable postsurgical epileptogenic outcome and granule cell pathology in patients with MTS has been observed, thus suggesting that dentate gyrus alterations may play a role in drug-resistant TLE.Correspondence to:
G. Marucci, MD
Sezione di Anatomia Patologica “M. Malpighi”
Ospedale Bellaria
Via Altura 3
40139 Bologna, Italy
Email: gianluca.marucci@ausl.bologna.it
Original contribution
Late onset glycogen storage disease Type II with “reducing body”-like inclusions
N. Gayathri, T.C. Yasha, S. Vani, A.B. Taly, A. Nalini and S.K. Shankar
Abstract
N. Gayathri1, T.C. Yasha1, S. Vani1, A.B. Taly2, A. Nalini2 and S.K. Shankar1
1Department of Neuropathology and 2Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India
Skeletal muscle tissue from 3 patients with clinical diagnosis of limb girdle muscular dystrophy revealed a vacuolar myopathy with glycogen storage and lysosomal activity. A diagnosis of late onset GSD Type II was considered. An interesting finding was the presence of round to oval eosinophilic inclusions which reduced on menadione linked a-glycerophosphate dehydrogenase (MAG). There are only two reports in the literature describing similar inclusions in late onset GSD II. We report morphological findings of this rare disorder and compare the findings with earlier two reports.Correspondence to:
Dr. N. Gayathri
Associate Professor
Electron microscope Laboratory
Department of Neuropathology, NIMHANS
Bangalore 560 029, India
Email: gayathrin12@rediffmail.com
Original contribution
Neuropathological biomarker candidates in brain tumors: key issues for translational efficiency
J.A. Hainfellner and H. Heinzl
Abstract
J.A. Hainfellner1 and H. Heinzl2
1Institute of Neurology and 2Core Unit for Medical Statistics and Informatics, Medical University of Vienna, Vienna, Austria
Brain tumors comprise a large spectrum of rare malignancies in children and adults that are often associated with severe neurological symptoms and fatal outcome. Neuropathological tumor typing provides both prognostic and predictive tissue information which is the basis for optimal postoperative patient management and therapy. Molecular biomarkers may extend and refine prognostic and predictive information in a brain tumor case, providing more individualized and optimized treatment options. In the recent past a few neuropathological brain tumor biomarkers have translated smoothly into clinical use whereas many candidates show protracted translation. We investigated the causes of protracted translation of candidate brain tumor biomarkers. Considering the research environment from personal, social and systemic perspectives we identified eight determinants of translational success: methodology, funding, statistics, organization, phases of research, cooperation, self-reflection, and scientific progeny. Smoothly translating biomarkers are associated with low degrees of translational complexity whereas biomarkers with protracted translation are associated with high degrees. Key issues for translational efficiency of neuropathological brain tumor biomarker research seem to be related to (i) the strict orientation to the mission of medical research, that is the improval of medical practice as primordial purpose of research, (ii) definition of research priorities according to clinical needs, and (iii) absorption of translational complexities by means of operatively beneficial standards. To this end, concrete actions should comprise adequate scientific education of young investigators, and shaping of integrative diagnostics and therapy research both on the local level and the level of influential international brain tumor research platforms.Correspondence to:
J.A. Hainfellner
Institute of Neurology
Medical University of Vienna
Waehringer Guertel 18-20
1097 Vienna, Austria
Email: johannes.hainfellner@meduniwien.ac.at
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