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12. Sonneland PR, Scheithauer BW, LeChago J, Crawford BG, Onofrio BM. Paraganglioma of the cauda equina region. Clinicopathologic study of 31 cases with special reference to immunocytology and ultrastructure. Cancer 1986;58:1720‑35. Access this article online Quick Response Code:

Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144456

Received: 10-07-2014 Review completed: 07-08-2014 Accepted: 03-10-2014

Primary plasmacytoma of the anterior skull base: A rare case

Figure 1: T1 axial post contrast MRI showing the contrast enhancing tumor involving the anterior cranial fossa base extending from the frontal and ethmoidal sinuses to the roof of the left orbit

Sir, Skull base plasmacytoma is a rare condition and accounts for 10% of all plasmacytomas. The anterior skull base plasmacytomas are extramedullary plasmacytomas which have better prognosis with surgery and adjuvant therapy. Only about 42 cases of skull base plasmacytomas have been reported till date. We present a case of skull base plasmacytoma without evidence of multiple myeloma. A 30‑year‑old man presented with progressive protrusion of the left eye with headache since six months. Examination showed left extra‑axial proptosis with displacement of eyeball downwards. Visual acuity and fundus examination were normal. Magnetic resonance imaging (MRI) showed large lesion isointense in T1 weighted images and slightly hyperintense in T2 weighted images. It was homogenously enhancing with gadolinium [Figures 1 and 2]. It was involving the anterior cranial fossa base extending from the frontal and ethmoidal sinuses to the roof of the orbit in the extradural plane. Left fronto‑temporal craniotomy was performed. The tumor was soft to firm and was eroding the skull base in the extradural plane and was extending from the frontal and ethmoidal sinuses to the roof of the entire orbit upto the optic canal. The tumor could be excised completely. Histopathological examination showed sheets of neoplastic plasma cells including binucleate forms, suggestive of plasmacytoma [Figure 3]. Bone marrow aspiration did not show evidence of myeloma. Skeletal survey was normal. Patient has undergone post‑operative radiotherapy. He is doing fine at 15 months follow‑up. Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5

Figure 2: T1 coronal post contrast MRI showing the contrast enhancing tumor involving the roof of left orbit in the extradural plane

Figure 3: Histopathology picture of the tumor (H and E, ×400) showing sheets of neoplastic plasma cells including bi-nucleate forms

Plasmacytoma is a focal form of plasma cell tumor. Only 36 cases have been documented till 2002 and 545

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another six more cases since then.[1‑6] Wein et al. have divided the plasmacytoma of the skull base into anterior (nasopharyngeal) group and central (sphenoid, clivus, petrous apex) group. This is of prognostic importance because in the central group, the rate of progression to multiple myeloma is 63.6%, which is much higher than that in the anterior group (9.5%). Moreover plasmacytomas are also classified as extramedullary plasmacytoma (EMP) or solitary plasmacytoma of bone (SPB).[1] Most of the anterior group of skull base plasmacytoma fall into EMP group and the central skull base plasmacytoma fall into SPB group. Since solitary plasmacytoma represents only one end of the spectrum of plasma cell tumors and represents a distinct manifestation of a disease continuum, progression to multiple myeloma is almost invariable. Hence adjuvant therapy is essential to arrest further progression of the disease. This patient had involvement of frontal and ethmoidal sinuses and eroding the floor of the anterior cranial fossa and most probably falls into the group of EMP. Hence, though the tumor had been excised totally adjuvant radiotherapy was given to the patient. The possibility of plasmacytoma has to be kept in mind in any osteolytic tumor of the skull base.

Acknowledgement We thank Prof. K. Ramesh Rao, Department of Pathology, Chettinad Superspeciality Hospital, Chennai, for providing the histopathology pictures.

Ramesh Ganesan Vengalathur, Karthikeyan Veerasamy Kavindapadi, Balasubramanian Chandramouli Department of Neurosurgery, Chettinad Superspeciality Hospital, Chettinad Health City, Kelambakkam, Chennai, Tamil Nadu, India E‑mail: [email protected]

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Wein RO, Popat SR, Doerr TD, Dutcher PO. Plasma cell tumors of the skull base: Four case reports and literature review. Skull Base 2002;12:77‑86. Ustuner Z, Basaran M, Kiris T, Bilgic B, Sencer S, Sakar B, et al. Skull base plasmacytoma in a patient with light chain myeloma. Skull Base 2003;13:167‑71. Singh AD, Chacko AG, Chacko G, Rajshekhar V. Plasma cell tumors of the skull. Surg Neurol 2005;64:434‑8. Pancholi A, Raniga S, Vohra PA, Vaidya V, Prajapati A, Mansingani S. Imaging features of extramedullary plasmacytoma of skull base with multiple myeloma—A rare case. Indian J Radiol Imaging 2006;16:29‑32. Yamaguchi S, Terasaka S, Ando S, Shinohara T, Iwasaki Y. Neoadjuvant therapy in a patient with clival plasmacytoma associated with multiple myeloma: A case report. Surg Neurol 2008;70:403‑7. Guinto‑Balanzar G, Abdo‑Toro M, Aréchiga‑Ramos N, Leal‑Ortega R, Zepeda‑Fernández E, Nambo‑Lucio Mde J. Plasma cell tumor of the clivus: Report of two cases. Cir Cir 2012;80:171‑6.

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Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144457

Received: 16-07-2014 Review completed: 26-08-2014 Accepted: 03-10-2014

An unusually aggressive clinical behavior in a case of atypical subependymal giant cell astrocytoma Sir, Subependymal giant cell astrocytoma (SEGA) is a benign tumor that mostly arises in the wall of the lateral ventricle.[1] Though it has been classified as a low grade, WHO Grade 1 tumor, aggressive lesions with metastasis and intratumoral hemorrhages have been described.[2,3] High grade features like mitoses, focal necrosis, and endothelial proliferations are distinctly rare, but few cases have been reported in the literature with these unusual findings of high‑grade tumor, termed as atypical SEGA.[4,5] Despite anaplasia in histology, the clinical behavior of these patients have been benign, same as typical SEGA. A few cases of atypical SEGA may behave aggressively.[5] We report a case of atypical SEGA mimicking malignant glioma with an unusually aggressive clinical course. A 5‑year‑old male child presented with 1 month history of worsening headache with right hemiparesis and facial weakness. Magnetic resonance imaging (MRI) showed a large lesion in the left posterior thalamic region abutting the atrial wall with peripheral enhancement with edema [Figure 1a and b]. There were no markers of tuberous sclerosis complex (TSC). The patient underwent a parietooccipital craniotomy and near total resection of the tumor through transcortical, transventricular route [Figure 1c and d]. Immediate postoperative period was uneventful. The histology showed spindle‑shaped to large polygonal tumor cells with many multinucleated giant cells scattered in between [Figure 2]. Large necrotic areas were observed. Few mitotic figures were identified with Ki‑67 labelling index around 2-3%. The tumor also showed glial fibrillary acidic protein (GFAP) positivity. The overall features were suggestive of SEGA with atypical features. Within 3 weeks of primary surgery, the patient presented with altered sensorium and progressively worsening right hemiparesis. MRI showed a small Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5

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Primary plasmacytoma of the anterior skull base: a rare case.

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