Cervical chordoma. A case report and differential diagnosis

VOLUME 2 - No. 5 - MARCH 15, 2012

CENTAURO S.r.l., BOLOGNA

Fortnightly

ISSN 2239-7493

Vol. 2 No. 5 Official Journal of:

AINR - Associazione Italiana di Neuroradiologia

and: The Neuroradiologists of Alpe-Adria ANRS - Albanian Neuroradiological Society PANRS - Pan Arab NeuroRadiology Society Radiological Society of Saudi Arabia, Division of Neuroradiology Egyptian Society of Neuroradiology ISNR - Indian Society of Neuroradiology Indonesian Society of Neuroradiology Neuroradiology Section of the Radiology Society of Iran Israeli Society of Neuroradiology College of Radiology Malaysia

Neuroradiology Section - Pakistan Psychiatry Research Center Section of Neuroradiology - Polish Radiological Society The Neuroradiologists of Romania Section of Neuroradiology of Serbia and Montenegro SILAN - Sociedad Ibero Latino Americana de Neurorradiologia Neuroradiology Section of Singapore Radiological Society Slovenian Society of Neuroradiology The Neuroradiological Society of Taiwan TSNR - Turkish Society of Neuroradiology

Official Journal of:

AINR - Associazione Italiana di Neuroradiologia and The Neuroradiologists of Alpe-Adria ANRS - Albanian Neuroradiological Society PANRS - Pan Arab NeuroRadiology Society Radiological Society of Saudi Arabia, Division of Neuroradiology Egyptian Society of Neuroradiology ISNR - Indian Society of Neuroradiology Indonesian Society of Neuroradiology Neuroradiology Section of the Radiology Society of Iran Israeli Society of Neuroradiology College of Radiology Malaysia Neuroradiology Section - Pakistan Psychiatry Research Center Section of Neuroradiology - Polish Radiological Society The Neuroradiologists of Romania Section of Neuroradiology of Serbia and Montenegro SILAN - Sociedad Ibero Latino Americana de Neurorradiologia Neuroradiology Section of Singapore Radiological Society Slovenian Society of Neuroradiology The Neuroradiological Society of Taiwan TSNR - Turkish Society of Neuroradiology

Index

Radiological Findings in Gliosarcoma. A Single Institution Experience

173

Diagnostic Ability of Fluid-Attenuated Inversion Recovery MR Imaging to Detect Remnant or Recurrent Meningiomas after Resection

181





H. Mori, A. Kunimatsu, O. Abe, H. Sasaki, H. Takao, T. Nojo, K. Kawai, N. Saito, K. Ohtomo



Xanthogranuloma of the Sellar Region. A Case Report

A. Agarwal, K. Agarwal, H.K. Lee

194

Primary Skull Osteosarcoma: MDCT Evaluation and Histopathological Correlation in Two Cases

197

NRJ Digital - Corso ECM FAD - Formazione a Distanza

202

Instructions for Authors

206

R. Conforti, G. Taglialatela, F. Rinaldi, E. Quaranta, M. Cirillo, G. Paolisso, S. Cirillo

M.Y. Swaidan, M. Hussaini, I. Sultan, A. Mansour



Cervical Chordoma. A Case Report and Differential Diagnosis

K. Gangadhar, D. Santhosh

190

Cover: John Singer Sargent: Daughters of Edward Darley Boit (detail) - Oil painting. 1882. - By deflam - http://www.flickr.com

Indexed in: Google Scholar (http://scholar.google.com) EMBASE (http://www.scopus.com) Scopus Overview: Scopus is the largest abstract and citation database of research literature and quality web sources. It's designed to find the information scientists need. Quick, easy and comprehensive, Scopus provides superior support of the literature research process. Updated daily, Scopus offers. • Nearly 18,000 peer-reviewed journals from more than 5,000 publishers, including coverage of: 1) 16,500 peer-reviewed journals (inc > 1200 Open Access journals); 2) 600 trade publications; 3) 350 book series; 4) Extensive conference coverage (3,6 million conference papers). • 38 million records, of which: 1) 19 million records include references going back to 1996 (78% include references); 2) 19 million pre-1996 records go back as far as 1823. • Results from 435 million scientific web pages. • 23 million patent records from 5 patent offices. • "Articles-in-Press" from over 3,000 journals.• Seamless links to fulltext articles and other library resources. • Innovative tools that give an at-a-glance overview of search results and refine them to the most relevant hits. • Alerts to keep you up-to-date on new articles matching your search query, or by favorite author. Scopus is the easiest way to get to relevant content fast. Tools to sort, refine and quickly identify results help you focus on the outcome of your work. You can spend less time mastering databases and more time on research. ISSN 2239-7493

NRJ Digital - The Neuroradiology Journal 2: 173-180, 2012

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Radiological Findings in Gliosarcoma A Single Institution Experience

M.Y. SWAIDAN, M. HUSSAINI, I. SULTAN, A. MANSOUR King Hussein Cancer Center; Amman, Jordan

Key words: gliosarcoma, radiological features, glioblastoma multiforme

SUMMARY – Gliosarcomas are rare tumors with a poor prognosis composed of intermingled malignant glial and sarcoma elements with an estimated incidence of 1.8-8.0% of all malignant astrocytic neoplasms. We aimed to review the imaging findings in eight patients with gliosarcoma who were treated in our center between 2002 and 2010. The diagnosis was confirmed by morphological and immunohistochemical stains. This study, to the best of our knowledge, is the largest describing the imaging manifestations of this tumor. Although our study revealed no unique radiological features for gliosarcoma, it is important to note that they all demonstrated either dural or ependymal involvement or both. Calcification, hemorrhage or cystic components are described with a tendency for a ring enhancement pattern. Interestingly pre-existing benign looking lesions and associated remotely located small meningiomas are also described.

Introduction Gliosarcoma (GSa) is a very rare primary biphasic central nervous system (CNS) neoplasm, classified by the World Health Organization (WHO) 2007 as a variant of glioblastoma (GBM), therefore a grade IV tumor 1. It represents approximately 1.8% of all cases of GBM 2. GSa shows a biphasic tissue pattern with glial and mesenchymal differentiation. The age of presentation ranges from the fourth to the sixth decades of life with a mean age of 53 years. However, cases have been described in children with tumors originating in the cerebral hemispheres 3-5. Gliosarcoma is usually situated supratentorially 8. The primary site of predilection is reported to be in the temporal lobe 1,2,7-8. GSa was initially hypothesized to originate from neoplastic transformation of blood vessels in a preexisting glioblastoma 9. Recent immunohistochemical and genetic studies failed to support this theory, suggesting a monoclonal origin for both histological components 1. GBM and GSa are reported to share significant clinical and genetic similarities 10. No significant differences between GSa and GBM

could be demonstrated with regard to age, sex, pretreatment Karnofsky performance status, tumor location, size, unifocality, median survival time, nor to the rarity of extra-cranial metastasis 11. None of the treatment regimens used, which included various combinations of radiation therapy and chemotherapy, improved the survival of GSa over GBM 2. We describe eight cases of pathologically proven GSa that were managed at our institution during the period from 2002 to 2010. Special emphasis is placed on radiological and pathological findings. Patients and Methods We reviewed the clinical data and radiological imaging features of five female and three male patients with ages ranging from 19 to 66 years (median age: 51 years) diagnosed with GSa over a period of eight years (from January 2002 to December 2010). Two radiologists reviewed the images retrospectively. Our clinical and epidemiological findings are summarized in Table 1. 173

Radiological Findings in Gliosarcoma

M.Y. Swaidan

could be identified in only one case, which happened to be the same patient (Figure 2). MRI showed variable appearance on both T1 and T2 weighted images. In general, the tumors had a tendency for hypointense signal on T1 and hyperintense signal on T2 (Figure 3). Six tumors (75%) showed intense ring-like enhancement (Figure 4) and two heterogeneous enhancement of a mainly solid tumor. Associated dural (62%) and/or ependymal involvement (62%) was present in all

Results Five of eight patients (62%) were females. All lesions were located supratentorially. In five cases (62%) the tumor was located in the parietal lobe (Figure 1). The other three were in the occipital lobe, temporal lobe (Figure 2) and occipitoparietal lobes. Tumors were iso- or hyperdense on CT with evidence of calcification in three of four cases who had brain CT scan done (Figure 1). Cystic components and hemorrhage

Table 1 Summary of the epidemiological, clinical and radiological findings in the eight patients diagnosed at the King Hussein Cancer Center.

Case 1

Case 2

Case 3

Case 4

Case 5

Case 6

Case 7

Case 8

36

19

64

37

66

58

48

54

Female

Male

Female

Female

Female

Male

Male

Female

NF

–

–

–

Hypertension

DM and IHD

Epilepsy

–

Location of tumor

Parietal

Parietal

Parietal

Parietal

Occipital

Parietal

Temporal

Occipital and parietal

Outline

infiltrative

welldefined

infiltrative

infiltrative

infiltrative

infiltrative

infiltrative

infiltrative

Dural involvement

+

–

+

–

+

–

+

+

Ependymal involvement

–

+

–

+

–

+

+

+

Edema

+++

++

+

++

++

+++

+++

++

Calcification

NA

+

–

NA

NA

+

+

NA

Hemorrhage

–

–

–

–

–

–

+

–

Cystic component

–

–

–

–

–

–

+

–

Ring

Ring

Heterogeneous

Ring

Ring

Ring

Heterogeneous

Ring

Pre-existing lesion

+*

–

+

–

–

–

+

–

Associated meningioma

–

–

–

–

+

–

–

+

NA

Hyper

NA

NA

NA

Iso

Iso

NA

T1

Hypo

Iso

Hypo

Hyper

Hypo to Hyper

Hypo

Hyper

Hypo

T2

Hyper

Iso

Hyper

Hypo

Hypo to Hyper

Hyper

Hypo

Hyper

Age (yrs) Sex Pre-existing clinical conditions

Enhancement pattern

Density on CT Signal intensity on MRI

NF: neurofibromatosis, DM: diabetes mellitus, IHD: ischemic heart disease, NA: not available, *: unknown pathology

174

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NRJ Digital - The Neuroradiology Journal 2: 173-180, 2012

Figure 1 A 19-year-old male patient (case 2). Non-enhanced CT scan in November 2006 showing a 5 cm deeply seated parietal lobe mass with coarse calcification (black arrow), welldefined outline, moderate surrounding edema (white arrow), sulcal effacement and left lateral ventricular trigonal compression (small arrowhead) and minimal midline shift to the right side (large arrowheads). It appears homogenously hyperdense.

cases (Figures 2 and 3) and moderate to severe edema was evident in seven (85%) (Figure 1). The outline of the tumor was infiltrative in seven cases (85%) (Figure 3). Interestingly, three cases had a pre-existing lesion at the same site of the tumor at least two of which had benign radiological appearance (Figures 2 and 3). Two other female patients had associated small meningiomas (Figure 4). Three of our cases died six, 17, 18 months after diagnosis, three patients are lost to follow-up, two of which after documented tumor progression by brain MRI. The last two patients are still alive five and 12 months after surgery but with evidence of tumor progression on brain MRI. Data from the Jordan cancer registry were reviewed to get an idea of the incidence of GBM and gliosarcoma in Jordan. Accurate registration of cases could only be obtained since July 2006. The total number of GBM cases in adults for the period July 2006 till June 2010 was 162: 48 (30%) females and 114 (70%) males. On the other hand, the total number of GSa cases in the same period was seven 7: four (57%) females and three (43%) males. The percentage of the GBM cases from the total number of primary brain tumors in adults, which is 462, was calculated to be about 35% for this period and that for GSa about 1.5%.

Pathological Findings Microscopically, all tumors but one showed a mixture of glial and mesenchymal elements, with mesenchymal elements occupying 30-80% of the slides examined. The most frequent sarcomatous pattern was spindle cell fibrosarcoma (6/8 cases), while two cases showed malignant fibrous histiocytoma pattern, with malignant giant cells scattered. In addition, chondroidlike differentiation and calcification was detected in two cases each. One case showed evidence of a pre-existing lesion in the form of benign fibroblastic/meningeal proliferation admixed with blood vessels and calcification suggesting meningoangiomatosis. Mitosis was identified in both glial and sarcomatous components. Reticulin stain highlighted the difference in both components as it was positive in the sarcomatous but not the glial component. Immunostains were performed on all cases. The malignant glial component was positive in all cases examined, while the sarcomatous component showed patchy staining with GFAP in only one case, with negative staining in all other cases. Vimentin was positive in all cases in both components. In a single case, focal staining with desmin was detected in the sarcomatous component. P53 was performed on four 175

Radiological Findings in Gliosarcoma

M.Y. Swaidan

A

B

C

D

176

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cases and was either strongly or diffusely positive (three cases), or completely negative (one case) in both components. Discrepant staining for P53 between the glial and sarcomatous component was not noted in any of the cases. Discussion GSa is an uncommon primary central nervous system tumor composed of intermingled malignant glial and sarcomatous elements. It was first described in 1895 by Stroebe 12 and gained general acceptance after it was redescribed by Feigin in 1955 13 and Robinstein e year later 14. GSa has an estimated incidence of 1.8-8.0% of all malignant astrocytic neoplasms 2,8 . This study is unique in combining the radiological findings and the clinical course in eight cases of GSa seen at the primary oncology referral center in Jordan over a period of eight years making this report the largest in the literature to date. In doing so, we have tried to overcome some of the limitations associated with studying rare tumors, such as the long periods required to assimilate a large number of cases. Interestingly, three out of our eight patients (37%) were rather young (