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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 6  |  Page : 655-656

Neuroblastoma presenting as hypertrophic pachymeningitis in a young female


1 Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
2 Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
3 Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

Date of Web Publication29-Dec-2012

Correspondence Address:
Ritu Verma
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.105211

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How to cite this article:
Singh P, Verma R, Paliwal VK, Neyaz Z. Neuroblastoma presenting as hypertrophic pachymeningitis in a young female. Neurol India 2012;60:655-6

How to cite this URL:
Singh P, Verma R, Paliwal VK, Neyaz Z. Neuroblastoma presenting as hypertrophic pachymeningitis in a young female. Neurol India [serial online] 2012 [cited 2019 Dec 16];60:655-6. Available from: http://www.neurologyindia.com/text.asp?2012/60/6/655/105211


Sir,

Neuroblastoma, a childhood tumour, may metastasise to different organ systems. Central nervous system (CNS) metastasis is not uncommon and usually presents as dural, leptomeningeal or intra-parenchymal masses. [1] Since the common causes of dural metastasis (prostrate, breast, lungs and stomach cancers) affect middle-aged/elderly patients, dural metastasis is seldom thought of as a cause of hypertrophic pachymeningitis in young adults. [2]

An 18-year-old female presented with low-grade fever, headache and vomiting of 2-month duration. Initially she had double vision for 1 month, followed soon by reduced vision in the left eye. There was no other relevant history. The patient's mother had pulmonary tuberculosis in the past. The patient was on empirical anti-tubercular treatment for 3 weeks before this admission. On examination, she had pallor and inguinal lymphadenopathy. Visual acuity in the left eye was 6/36 and fundus revealed bilateral papilloedema and left secondary optic atrophy. Bilateral lateral rectus palsy was evident. Complete blood picture and blood biochemistry revealed low haemoglobin at 6.5 gm% Cerebrospinal fluid showed normal biochemistry and cells at 20/mm 3 , all lymphocytes, and was negative for cryptococcal antigen, polymerase chain reaction (PCR) tuberculosis and malignant cells. Ultrasound of abdomen revealed a right hypochondrial mass with solid/cystic components on computer tomography (CT) scan of the abdomen. Ultrasound-guided needle biopsy from the mass was suggestive of small round-cell tumour and immunohistochemistry showed tumour cells positive for neuron-specific enolase and synaptophysin, and negative for creatine kinase, leucocyte common antigen and CD99, confirming diagnosis of neuroblastoma [Figure 1]. Bone marrow biopsy confirmed tumour metastasis to marrow. Magnetic resonance imaging revealed enhancing pachymeningeal thickening along the cerebral convexities [Figure 2]. The patient was planned for meningeal biopsy followed by chemotherapy. However, the patient could not afford the cost of treatment. Subsequently, the patient developed multiple scalp nodules, proptosis and progressive unconsciousness, and succumbed to her illness.
Figure 1: Bone marrow biopsy. Nests/alveolar pattern of small round/ oval cells having hyper&# 8209;chromatic nucleus and scant cytoplasm (haematoxylin and eosin (H and E): 20) (a) similar cells from abdominal mass in fibrillary matrix with areas of necrosis (H and E): 20) (b) tumour cells positive for neuron‑specific enolase (c) synaptophysin (d) and negative for CD99 (e) and LCA (f)

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Figure 2: Axial post‑gadolinium T1‑weighted magnetic resonance image showing enhancing pachymeningeal thickening over bilateral cerebral convexities

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Headache, visual loss and other cranial neuropathies with features of raised intracranial pressure are often the presenting features of hypertrophic pachymeningitis of different causes. [3] Hypertrophic pachymeningitis is usually considered tubercular in India and many patients receive anti-tubercular drugs on empirical basis. Other causes of hypertrophic pachymeningitis include sarcoidosis, mycosis, meningeal carcinomatosis, collagen vascular disease, en plaque meningioma, lymphoma/leukaemia and repeated intrathecal drug administration. [3] Although neuroblastoma can metastasise to the CNS in more than 50% of patients with recurrent disease, dural metastasis presenting as hypertrophic pachymeningitis is less common at the time of diagnosis. [4] In view of this, children with hypertrophic pachymeningitis should be evaluated for neuroblastoma. Since dural metastasis by neuroblastoma occurs possibly by haematogenous spread (as suggested by involvement of bone marrow), absence of malignant cells in the cerebrospinal fluid does not exclude malignancy as a cause.

 
  References Top

1.D'Ambrosio N, Lyo JK, Young RJ, Haque SS, Karimi S. Imaging of metastatic CNS neuroblastoma. AJR Am J Roentgenol 2010;194:1223-9.  Back to cited text no. 1
[PUBMED]    
2.Laigle-Donadey F, Taillibert S, Mokhtari K, Hildebrand J, Delattre JY. Dural metastases. J Neurooncol 2005;75:57-61.  Back to cited text no. 2
[PUBMED]    
3.Sylaja PN, Cherian PJ, Das CK, Radhakrishnan VV, Radhakrishnan K. Idiopathic hypertrophic cranial pachymeningitis. Neurol India 2002;50:53-9.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Kramer K, Kushner B, Heller G, Cheung NK. Neuroblastoma metastatic to the central nervous system. The Memorial Sloan-Kettering Cancer Center experience and a literature review. Cancer 2001;91:1510-9.  Back to cited text no. 4
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