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and instructed to follow-up with primary physician in his local hospital. Neurosyphilis is known as “the great impostor” because of its wide range of clinical symptoms. Once a common disease, meningovascular syphilis has become rare since the advent of antibiotics. However, with the rise in the incidence of primary and secondary syphilis,[1] meningovascular syphilis may become a more common cause for stroke. In clinical practice, neurosyphilis as the cause of ischemic stroke is often not suspected. Diagnosis of neurosyphilis has therapeutic implication and nonor delayed institution of early appropriate treatment would result in more morbidity.[2] Most frequent clinical presentation of meningovascular syphilis is stroke presentation in the middle cerebral artery territory and vertebrobasilar artery and its branches are the second most commonly involved vessels. [3,4] Syphilis can cause the cause of aortic aneurysm, however cerebral aneurysm secondary to syphilitic vasculopathy has been reported rarely.[5] Clinical suspicion and early diagnosis and early institution appropriate treatment is important to reduce the morbidity associated with the disease.

Yao Yin-Dan, Hong Wen-Ke, Guan Li-Feng Department of Neurology, Ningbo No. 2 Hospital, Ningbo, China E-mail: [email protected]

References 1. 2. 3. 4. 5.

Chen XS, Yin YP, Wang QQ, Wang BX. Historical perspective of syphilis in the past 60 years in China: Eliminated, forgotten, on the return. Chin Med J (Engl) 2013;126:2774-9. Liu LL, Zheng WH, Tong ML, Liu GL, Zhang HL, Fu ZG, et al. Ischemic stroke as a primary symptom of neurosyphilis among HIV-negative emergency patients. J Neurol Sci 2012;317:35-9. Flint AC, Liberato BB, Anziska Y, Schantz-Dunn J, Wright CB. Meningovascular syphilis as a cause of basilar artery stenosis. Neurology 2005;64:391-2. Feng W, Caplan M, Matheus MG, Papamitsakis NI. Meningovascular syphilis with fatal vertebrobasilar occlusion. Am J Med Sci 2009;338:169-71. Asdaghi N, Muayqil T, Scozzafava J, Jassal R, Saqqur M, Jeerakathil TJ. The re-emergence in Canada of meningovascular syphilis: 2 patients with headache and stroke. CMAJ 2007;176:1699-700. Access this article online Quick Response Code:

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Received: 17-01-2014 Review completed: 17-01-2014 Accepted: 08-04-2014

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A giant falcine chondrosarcoma: Case report and literature review Sir, Parafalcine chondrosarcoma is extremely rare and may be difficult to differentiate preoperatively from falx meningioma. We report yet another such rare case. A 26-year-old male presented with a 3-week history of progressive headache and right hand numbness. Neurological examination revealed decrease in proprioception in the right hand. Cranial magnetic resonance imaging (MRI) revealed a well-marginated parafalx mass (9.4 × 6.4 × 6.0 cm) occupying bilateral frontal lobes and along the falx cerebri. It was hypointense on T1-weighted and hyperintense on T2-weighted images [Figure 1a] with no perilesional edema. Contrast-enhanced MRI scan showed a lobulated heterogeneously enhanced honeycomb lesion [Figure 1b]. Preoperative diagnosis was parafalcine meningioma and MR venography (MRV) showed oppression of superior sagittal sinus by the tumor [Figure 1c]. Total resection of the tumor was performed by bicoronal craniotomy. The lesion was hard, lobulated, gray-white, with a very firm cartilaginous consistency. The tumor and the dural attachment were totally removed. Regular follow-up MRI scans at 18 months showed no recurrence [Figure 1d]. She has recovered well and is able to run again. Microscopic examination revealed lobulated appearance with variable low to moderate cellularity composed primarily of individual cells separated by a basophilic matrix. Neoplastic chondrocytes had plump and hyperchromatic nuclei with few mitotic figures [Figure 2]. Immunohistochemical studies demonstrated positivity for S-100, which is consistent with the diagnosis of a cartilaginous tumor [Figure 3]. Chondrosarcomas are malignant tumors of cartilage and may arise from bone or soft tissues.[1] They account for 6% of skull = base neoplasms and 0.15% of all intracranial tumors.[2] Approximately 75% of all cranial chondrosarcomas occurs at the base of the skull and mostly in the middle cranial fossa.[3,4] The rare locations include the following: Choroid plexus, dura mater, and intraparenchymal. Only 61 cases of dural origin have been reported to date and of them only nine were classic low-grade type.[5-8] Skull-based chondrosarcomas are most commonly classic type,[3] but about 62% dural-based chondrosarcomas are mesenchymal variant. [5] The variants of parafalcine chondrosarcomas have a strong Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2

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female preponderance, clinical symptoms at onset were similar to falx meningiomas and the preoperative diagnosis was frequently meningioma.

a

b

c

d

Figure 1: Imaging appearances of the intracranial chondrosarcoma. (a) Axial T2-weighted image reveals a bilateral frontal mass and has high signal intensity relative to cortex, with the low signal areas representing probable calcification (b) Axial contrast-enhanced T1-weighted image shows a lobulated heterogeneously enhanced tumor (c) MRV shows a part of the superior sagittal sinus that was oppressed by the tumor (d) There was no sign of recurrence at 18-month follow-up MRV = Magnetic resonance venography

Figure 2: High-power view of the surgical specimen shows neoplastic chondrocytes with moderate cellular atypia with identifiable nuclei

Figure 3: The cartilaginous portion of the tumor was positive for S-100 protein

Neurology India | Mar-Apr 2014 | Vol 62 | Issue 2

Chondrosarcomas are classified into the classic, mesenchymal, and myxoid subtypes based on the histologic architecture; these variants signifi cantly differ in clinical presentation and prognosis. Classic chondrosarcoma is easily distinguished from the mesenchymal variant by its well-differentiated structure with few or no mesenchymal components and from the myxoid variant, by the absence of myxoid stroma. The mesenchymal subtypes are the most malignant of the three, illustrated by a strong tendency for intradural and cerebral growth, and occasionally distant metastases.[3] On computed tomography (CT) scans, these lesions are usually isodense to hyperdense, with variable degrees of heterogeneous enhancement. These tumors have varying amounts of histologic and radiologic calcifications, but falcine chondrosarcomas show lesser calcification compared with skull-base variants. MRI offers better demarcation of the lesion and tumor characterization. These tumors are frequently hypointense on T1-weighted images and extremely hyperintense on T2-weighted images. They show some mild or moderate degree of contrast enhancement and may have a honeycomb appearance.[9] The absence of a dural tail, minimal vasogenic edema, and relative avascularity on angiography could be the characteristics for differential diagnosis. The most important differentials include falx or parasagittal meningioma, hemangiopericytoma, metastasis, and vascular malformations. Higher signal intensity on the T2-weighted MRI, islands of low signal intensity despite lack of gross calcification on all the sequences due to well-differentiated cartilage, “honeycomb” appearance on contrast enhancement, and lack of perfusion may differentiate chondrosarcomas from meningiomas.[9] The relative absence of edema can also be used to distinguish it from tumors such as glioblastomas and metastases. Lack of vascularity and absence of flow voids differentiate it from a vascular malformation or the mesenchymal variety. To specially mention, none of the MRI or CT features appear to be useful for differentiating chordomas from chondrosarcomas preoperatively.[10] Radical surgical resection is the preferred treatment modality. The widest possible resection seems to be a significant prognostic factor. Chondrosarcomas at the skull base have a strong tendency for local recurrence due to the difficulty of complete resection. In a report by Korten et al., [3] 53% of patients had 215

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recurrence attributed to partial resection due to the proximity of the lesion to critical neuronal and vascular structures. However, no recurrences have been reported to date in dural-based classical chondrosarcomas. [5-8] Mesenchymal and myxoid subtype indicates worse clinical behavior and poorer prognosis than conventional histology. About 50% of the mesenchymal and nearly all the myxoid variants showed recurrence. [3] Some authors recommend adjuvant radiation therapy to prevent recurrence, but it is difficult to tell if this mode of adjuvant therapy is effective because the few cases prohibit drawing any conclusions about such treatment.

Crooke's cell adenoma of the pituitary: A histological, immunocytochemical, and electron microscopic study of a rare case

1.

Sir, Crooke's hyaline change, first described by Crooke, represents intracytoplasmic accumulation of cytokeratin (CK), usually noted in non-tumorous corticotrophs in response to excess glucocorticoids.[1] Rarely this change is seen in corticotroph adenomas, termed Crooke’s cell adenomas (CCA). CCA is a rare form of adrenocorticotropic hormone (ACTH)-producing adenoma, known for its aggressive behavior and tendency to recur. Few cases are reported in literature, with the largest series comprising 36 cases, reported by George et al.[2] In this study, the recurrence rate was in 61%, suggesting the aggressive nature of this tumor. We report an additional case of CCA, probably the first case from India.

Received: 22-09-2013 Review completed: 17-12-2013 Accepted: 31-03-2014

A 58-year-old lady presented with features of Cushing’s disease of 2-years duration. Magnetic resonance imaging (MRI) revealed a lobulated, partially enhancing, solid, and cystic sellar/suprasellar tumor [Figure 1a-c]. Serum ACTH level was 102 pg/ml (normal 7.2-63 pg/ml). She underwent a transethmosphenoidal microsurgical excision of the pituitary tumor. No mass lesion was noted on ultrasound examination of abdomen. Histology revealed features of an ACTH-secreting pituitary adenoma. Most tumor cells exhibited characteristic Crooke’s hyaline [Figure 2a], which was positively stained for pan cytokeratin (AE1/AE2), variably for CK-7 [Figure 2b and c], and displaced the ACTH secretory granules to the periphery of the cells [Figure 2d]. These findings matched with the study of Rotondo and colleagues.[3] The tumor showed a variable expression of markers of tumor aggressiveness such as MIB-1 [Figure 3a], topoisomerase-2 alpha [Figure 3b], and p53 [Figure 3c], suggesting that these markers may not consistently indicate the inherent aggressive nature of CCAs. On the other hand, diffuse staining for p27 and O6 methyl guanine methyl transferase (MGMT) was noted [Figure 3d and e]. Even though most studies have shown decreased p27 expression in aggressive pituitary adenomas, in the present study and in the study of Rotondo et al., a high expression of p27 was noted in CCA, suggesting its dual tumor suppressor and oncogenic role. [4] Our case was immunopositive for MGMT, probably suggesting that CCAs are not good candidates for temozolomide (TMZ) treatment. Ultrastructurally, Crooke’s hyaline corresponds to ‘microfilaments’, as we

Yan-Wu Yang, Shuang Liu, Xiang Wang, Qing Mao Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, PR China E-mail: [email protected]

References Alvira MM, McLaurin RL. Asymptomatic subdural chondrosarcoma. Case report. J Neurosurg 1978;48:825-8. 2. Kretzschmar HA, Eggert HR, Beck U, Furmaier R. Intracranial chondroma. Case report. Surg Neurol 1989;32:121-5. 3. Korten AG, ter Berg HJ, Spincemaille GH, van der Laan RT, Van de Wel AM. Intracranial chondrosarcoma: Review of the literature and report of 15 cases. J Neurol Neurosurg Psychiatry 1998;65:88-92. 4. Sorimachi T, Sasaki O, Nakazato S, Koike T, Shibuya H. Myxoid chondrosarcoma in the pineal region. J Neurosurg 2008;109:904-7. 5. Krishnan SS, Panigrahi M, Varma D, Madigubba S. Falcine and parasagittal intracranial chondrosarcomas of the classical variant: Report of two cases with review of literature. Neurol India 2011;59:451-4. 6. Kan Z, Li H, Zhang J, You C. Intracranial mesenchymal chondrosarcoma: Case report and literature review. Br J Neurosurg 2012;26:912-4. 7. Lin HS, Tsai CC, Chang CK, Chen SJ. Giant intracranial mesenchymal chondrosarcoma with uncal herniation. Fo J Surg 2012;45:93-6. 8. Altinbo AA, Yalcin N, Akbulut M, Coskun E, Duzcan SE. Intracranial chondrosarcoma in a 22-years old woman: Report of a case. J Neurological S (Turk) 2011;28:614-8. 9. Güneş M, Günaldi O, Tuğcu B, Tanriverdi O, Güler AK, Cöllüoğlu B. Intracranial chondrosarcoma: A case report and review of the literature. Minim Invasive Neurosurg 2009;52:238-41. 10. Pamir MN, Ozduman K. Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas. Eur J Radiol 2006;58:461-70.

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A giant falcine chondrosarcoma: case report and literature review.

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