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|Year : 2018 | Volume
| Issue : 5 | Page : 1517-1518
Medulloblastoma with extensive nodularity: A rare variant with a favorable outcome
Saumya Jakati1, Rani Y Jyotsna2, AK Purohit3, Challa Sundaram1
1 Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
2 Department of Radiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
3 Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
|Date of Web Publication||17-Sep-2018|
Dr. Challa Sundaram
Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad - 500 082, Telangana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jakati S, Jyotsna RY, Purohit A K, Sundaram C. Medulloblastoma with extensive nodularity: A rare variant with a favorable outcome. Neurol India 2018;66:1517-8
A 3-year old female child was admitted with a history of listlessness for 3 months and vomiting for 15 days. On examination, there was bilateral papilloedema and no focal neurological deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a characteristic nodular (grape-like) mass in the fourth ventricle with hydrocephalus. The post-contrast CT scan showed enhancement of the nodules at the periphery. The isointense lesion on T1- and T2-weighted MRI images showed a heterogeneous contrast enhancement [Figure 1]. Craniotomy and near-total excision of the mass was performed. Histology showed multiple small nodules with pale staining areas composed of neuropil and few small round cells in a laminar pattern, which were strongly positive with synaptophysin, and had a low Ki 67 index [Figure 2]. The internodular areas were narrow, cellular, had a high Ki 67 index and were Bcl2 (b-cell lymphoma-2) positive. With a diagnosis of medulloblastoma with extensive nodularity (MBEN), she was treated with radiotherapy.
|Figure 1: (a) Post contrast T1weighted sagittal section and (b) post contrast CT scan of the brain axial section show a large lobulated heterogeneously enhancing mass at the floor of fourth ventricle with multiple peripherally enhancing nodules causing gross hydrocephalus|
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|Figure 2: Photomicrographs of MBEN show (a) multiple small nodules, [hematoxylin and eosin (H and E) ×40]; (b) intranodular small round cells in a laminar pattern with neuropil background (H and E, ×400); (c) nodules were highlighted by the reticulin stain (reticulin stain ×100); (d and e) small round cells were positive for synaptophysin (synaptophysin ×100, ×400); (f) neurofilament positivity was seen in the background neuropil (neurofilament ×400)|
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MBEN constitutes 3% of medulloblastomas (MBs), and occurs in children less than 3 years of age.,, The characteristic MRI findings permit the establishment of a preoperative diagnosis., The histopathology is a very strong and independent prognostic factor for young children with desmoplastic/nodular MB and MBEN; however, there is overlap between the two., MBEN differs in the age, location, and prognosis, but shares nodularity and activation of the sonic hedgehog pathway with desmoplastic MB. The excellent survival for MBEN is attributed to surgical resectability and extensive neuronal differentiation. It is important to recognize MBEN as it is associated with a favorable outcome and de-escalation of treatment may be considered.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]