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Clinical Neuropathology, Vol. 31 – No. 1/2012 (1-6)

Clinical Neuropathology, Vol. 31 – No. 1/2012

J.A. Hainfellner

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Clinical Neuropathology, Vol. 31 – No. 1/2012 (7-23)

Processing of nerve biopsies: A practical guide for neuropathologists

Joachim Weis¹, Sebastian Brandner², Martin Lammens³, Claudia Sommer⁴ and Jean-Michel Vallat⁵

¹Institute of Neuropathology, Medical Faculty, RWTH Aachen University, Aachen, Germany, ²Division of Neuropathology, National Hospital for Neurology and Neurosurgery, and Department of Neurodegenerative Disease, UCL Institute of Neurology, London, UK, ³Departments of Pathology and Neurology, Radboud UMC Nijmegen, Nijmegen, The Netherlands, ⁴Department of Neurology, University Hospital of Würzburg, Würzburg, Germany, and ⁵Neurology Department, National Reference Center: “Rare peripheral neuropathies”, University Hospital, Limoges, France
Nerve biopsy is a valuable tool in the diagnostic work-up of peripheral neuropathies. Currently, major indications include interstitial pathologies such as suspected vasculitis and amyloidosis, atypical cases of inflammatory neuropathy and the differential diagnosis of hereditary neuropathies that cannot be specified otherwise. However, surgical removal of a piece of nerve causes a sensory deficit and – in some cases – chronic pain. Therefore, a nerve biopsy is usually performed only when other clinical, laboratory and electrophysiological methods have failed to clarify the cause of disease. The neuropathological work-up should include at least paraffin and resin semithin histology using a panel of conventional and immunohistochemical stains. Cryostat section staining, teased fiber preparations, electron microscopy and molecular genetic analyses are potentially useful additional methods in a subset of cases. Being performed, processed and read by experienced physicians and technicians nerve biopsies can provide important information relevant for clinical management.Correspondence to:
Univ.-Prof. Dr. Joachim Weis
Institute of Neuropathology
RWTH Aachen University Hospital
Pauwelsstraße 30
52074 Aachen, Germany
Email: jweis@ukaachen.de

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Clinical Neuropathology, Vol. 31 – No. 1/2012 (24-30)

Ectopic pituitary adenoma of the clivus presenting with apoplexy: case report and review of the literature

Pamela A. Mudd¹, Samantha Hohensee², Kevin O. Lillehei⁴, Todd T. Kingdom¹ and Bette K. Kleinschmidt-DeMasters²,³,⁴

From the Departments of ¹Otolaryngology, ²Pathology, ³Neurology, and ⁴Neurosurgery, The University of Colorado Health Sciences Center, Aurora, CO, USA
Ectopic pituitary adenomas usually occur within sphenoid sinus or nasopharynx, and seldom within the clivus. There is only a single reported example of ectopic adenoma with clinical apoplexy, albeit not from clivus. We report a 78-year-old male with known prostate carcinoma admitted with acute onset of blurred vision, suggestive of apoplexy. Work-up revealed unilateral cranial nerve VI palsy and neuroimaging showed a mass confined to the clivus; sellar region was normal. Preoperative considerations included chordoma, chondrosarcoma, or metastatic prostate carcinoma to bone. Resection was via endoscopic transsphenoidal approach to the clivus. An ectopic null cell pituitary adenoma with bland infarction was identified as the cause of the patient’s clinical apoplexy. No antecedent precipitating factors for apoplexy were present; specifically the patient had not received leuprolide preoperatively, a known precipitant of pituitary apoplexy in prostate cancer patients who receive drug. We review the literature on ectopic clival pituitary adenomas, apoplexy in ectopic adenomas, and the link between apoplexy and leuprolide usage.Correspondence to:
Bette 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@ucdenver.edu

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Clinical Neuropathology, Vol. 31 – No. 1/2012 (31-38)

Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: review of 2 cases with chromosome 1p/19q and IDH1 analysis

Ji Xiong¹, Ying Liu², Shu-Guang Chu³, Hong Chen¹, Hai-Xia Chen¹, Ying Mao⁴ and Yin Wang¹

¹Department of Neuropathology, Institute of Neurology, Huashan Hospital of Fudan University, ²Department of Pathology, Shanghai Medical college of Fudan University, ³Department of Radiology and ⁴Department of Neurosurgery, Huashan Hospital of Fudan University, Shanghai, China
Dysembryoplastic neuroepithelial tumor (DNT)-like neoplasms of the septum pellucidum are extremely rare. We reviewed 2 cases of DNT-like neoplasm of the septum pellucidum and specifically studied the immunohistochemical features and chromosomes 1p and 19q deletions. One case was a 26-year-old woman who complained of aggravated headache for 2 weeks. The other case was a 31-year-old female presenting with double vision for a month. Histological examinations showed that the lesions were composed of uniform oligodendrocytelike cells (OLCs) with obvious floating neurons in a mucin-rich background. Immunohistochemical studies revealed that both tumors were diffusely positive for Synaptophysin and Olig2. Sporadic neurons were clearly positive for NeuN. Loss of chromosome 1p/19q and isocitrate dehydrogenase 1(IDH1) mutations were not identified in both cases. It might indicate that these OLCs of DNT-like neoplasms were genetically different from glial tumors, although they showed close morphological similarities.Correspondence to:
Yin Wang, MD, PhD
Department of Neuropathology
Institute of Neurology and Huashan
Hospital of Fudan University
NO.12 the Middle Wu Lu Mu Qi Road
Shanghai 200040, China
Email: yinwang88@hotmail.com

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Clinical Neuropathology, Vol. 31 – No. 1/2012 (39-43)

Paraganglioma of the cauda equina: a report of two cases with unusual histopathological features

Miki Matsumoto¹, Kuniko Abe¹, Hideo Baba², Naoe Kinoshita¹, Taishi Yamauchi¹, Masaya Shiraishi³, Makoto Osaki² and Tomayoshi Hayashi¹

¹Department of Pathology, ²Department of Orthopedics, Nagasaki University Hospital, and ³Department of Orthopedics, Nagasaki Prefecture Shimabara Hospital, Nagasaki, Japan
Paragangliomas of the cauda equina are very rare. Preoperatively, they are usually diagnosed as schwannoma. We report 2 cases that exhibited unusual histopathologic structure as well as a typical Zellballen pattern with a highly vascular stroma. The unusual features included papillary, ribboning, and pseudorosette patterns, ganglion cell nests, and melanin pigment. Pathological diagnosis of paraganglioma may be especially difficult when such unusual histopathological features are seen. Correct histopathological diagnosis is important as paraganglioma has a better prognosis than other tumors included in the differential diagnosis for this location.Correspondence to:
Kuniko Abe, MD, PhD
Department of Pathology
Nagasaki University Hospital
1-7-1 Sakamoto
Nagasaki 852-8501, Japan
Email: abek@nagasaki-u.ac.jp

Abstract

Clinical Neuropathology, Vol. 31 – No. 1/2012 (44-50)

Septo-optic dysplasia: an autopsy study of a 23-week fetus

Dilip P. Jethwani¹, Anita Mahadevan¹, B.S. Ramamurthy², R. Mangala³ and S.K. Shankar¹

¹Department of Neuropathology, National Institute of Mental Health and Neuroscience, ²Consultant Ultrasonologist and Radiologist, Srinivasa Ultrasound Scanning Center, and ³Obstetrician and Gynecologist, Sevakshetra Hospital, Bangalore, India
Septo-optic dysplasia is a rare congenital anomaly with an incidence of 1 in 10,000 live births. Recognizing this anomaly early can help parents take an informed decision. There are very few reports of pathological descriptions of this anomaly. We present neuropathological findings at autopsy following medical termination of pregnancy of a 23-week fetus with septo-optic dysplasia (SOD), optic nerve hypoplasia, absent septum pellucidum with aplastic posterior pituitary.Correspondence to:
Prof. Dr. S.K. Shankar, MD
Dpartment of Neuropathology
National Institute of Mental
Health and Neuroscience
Bangalore, India, 560029
Email: shankarsk2004@gmail.com

Abstract

Combined rhabdoid, papillary and adenocarcinomatous-like elements in a malignant meningioma – a potential diagnostic problem

Elena Martinez-Saez¹, Irfan Malik², Stephen Connor³ and Andrew King¹

Departments of ¹Clinical Neuropathology, ²Neurosurgery and ³Neuroradiology, King’s College Hospital, London, UK
Correspondence to:
Dr. Andrew King
Department of Clinical Neuropathology
Academic Neuroscience Building
King’s College Hospital
Denmark Hill, London SE5 9RS, UK
Email: andrewking@nhs.net

Abstract

Pleomorphic xanthoastrocytoma in two siblings with neurofibromatosis Type 1 (NF-1)

Matthew T. Neal¹, Thomas L. Ellis¹ and Constance A. Stanton²

¹Department of Neurosurgery and ²Department of Neuropathology, Wake Forest University School of Medicine, Winston-Salem, NC, USA
Correspondence to:
Thomas L. Ellis, MD
Wake Forest University
Department of Neurosurgery
Winston-Salem, NC 27157, USA
Email: tlellis@wfubmc.edu

Abstract

Society News of the European Confederation of Neuropathological Societies

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Volume 31, No. 1/2012(January/February)