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Year : 2013  |  Volume : 9  |  Issue : 4  |  Page : 741-742

Isolated skull base primary Ewing's sarcoma: An extremely rare location

1 Department of Neurosurgery, JSS Medical College and Hospital, Mysore, Karnataka, India
2 Department of Neurosurgery, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605006, India
3 Dr. Achanta Lakshmipathi Neurosurgical Centre, Post Graduate Institute of Neurological Surgery, V.H.S Hospital, Taramani, Chennai, India

Date of Web Publication11-Feb-2014

Correspondence Address:
Goutham Cugati
Department of Neurosurgery, JSS Medical College and Hospital, Mysore, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.126479

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 > Abstract 

A 16-year-old boy presented with history of headache and blurring of distant vision. He had UMN facial paresis and papilledema. Imaging revealed a heterogenous space occupying lesion in the middle cranial fossa base with mass effect and midline shift and non homogenous contrast enhancement with erosion of the petrous bone. He underwent gross total excision of the lesion. Histopathology and immunohistochemistry were suggestive of skull base Ewing's sarcoma. Bone scan was done to rule out primary involvement of other extracranial location. We have discussed the epidemiology, presentation, management and prognosis of such cases.

Keywords: Immunohistochemistry, primary Ewing′s sarcoma, skull base

How to cite this article:
Cugati G, Singh M, Pande A, Symss NP, Chakravarthy VM, Ramamurthi R. Isolated skull base primary Ewing's sarcoma: An extremely rare location. J Can Res Ther 2013;9:741-2

How to cite this URL:
Cugati G, Singh M, Pande A, Symss NP, Chakravarthy VM, Ramamurthi R. Isolated skull base primary Ewing's sarcoma: An extremely rare location. J Can Res Ther [serial online] 2013 [cited 2020 Nov 30];9:741-2. Available from: https://www.cancerjournal.net/text.asp?2013/9/4/741/126479

 > Introduction Top

Primary Ewing's sarcoma of the skull bones is a rare entity. Involvement of the skull base present with unique set of complaints and the management of such lesion pose a challenge to the treating surgeon. As most of these patients are teenagers, we need to understand the tumor characteristic and the appropriate way to handle these tumors, so that a prolonged good quality of life can be achieved.

 > Case Report Top

This 16-years-old boy presented with history of right sided fronto-temporal headache for one month. He had blurring of distant vision for 15 days. There was no history of loss of consciousness, seizures or vomiting. On examination, he had a mini mental scale of 28/30. His neurological examination was normal expect for left UMN facial paresis and bilateral papilledema. Magnetic resonance imaging (MRI) scan of the brain showed a heterogenous space occupying lesion (55x67x54mm) in the middle cranial fossa base, which was predominantly isointense on T1W and hyperintense on T2W with mass effect and midline shift and non homogenous contrast enhancement [Figure 1]. CT scan brain with bone window showed evidence of right petrous temporal bone erosion. CT scan thorax, abdomen and bone scintigraphy shows no evidence of primary or metastasis in chest, abdominal organs and bone outside skull.

He underwent right pterional craniotomy and gross total excision of the right temporal lesion. The involved bone was drilled and removed. There was no dural breach by the tumor. Histopathology showed compactly arranged round cell tumor, with cells are having clear cytoplasm. Small spicules of bone were seen enclosed within tumor tissue suggesting bone destruction. Immunohistochemistry showed strongly positivity for CD-99 and negative for GFAP. This picture confirmed a pathological diagnosis of Ewing's sarcoma. Technetium 99 bone scan was performed and extracranial Ewing's sarcoma was ruled out. Postoperative MRI brain plain and contrast showed complete excision of lesion with postoperative changes [Figure 2]. He received 50 Gy of radiation and was started on chemotherapy (vincristine, adriamycin, cyclophosphamide, and actinomycin-D).
Figure 1: Preoperative MRI of the Brain showing a large extra-axial lesion in the right middle cranial fossa base which is predominantly iso-intense in T1W (A), and predominantly hyper-intense in T2W (B). On contrast administration there is heterogenous enhancement with cavitations seen within the tumor (C and D). There is significant mass effect with compression of brain stem and midline shift to the opposite side

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Figure 2: Post operative MRI of the brain showing total excision of the lesion with post operative changes (A and B)

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 > Discussion Top

Malignant bone tumors affecting children and young adults account for 6-9% of primary malignant bone tumors. [1],[2],[3] Ewing's sarcoma is a malignant bone tumor, which is commonly seen in pelvis, chest, femur, tibia, vertebrae and humerus. Cranial Ewing's sarcoma is an extremely rare. [1],[2],[3],[4] While most of these tumors are metastatic Ewing's sarcoma, primary Ewing's sarcoma of the skull is much rarer. Ewing's sarcoma occurs most frequently in teenagers, with a male/female ratio of 1.6:1. Frontal, parietal convexities are the common sites in skull. Less common locations are facial bones, temporal, sphenoid and clival bones. Skull base tumors present with symptoms like cranial nerve involvement, unlike cranial vault tumors where swelling and headache are predominant. In this present case, the tumor was involving petrous temporal bone and had completely grown into the cranial cavity. Such tumors present like an intracranial lesion with headache and signs of raised intracranial pressure. [1],[2],[3],[4] CT scan shows the location of the tumor, as well as bone destruction. MRI will show marked heterogenous lesion, with areas of hemorrhage and necrosis. The extent of tumor is better shown in MRI, which helps in surgical planning. The largest series of primary cranial Ewing's sarcoma was reported by Desai et al in 2000. [1] Five year survival of more than 57% was noted in their series. Skull base location of these tumors make then difficult to achieve total excision. Irrespective of the location the primary surgical goal is to achieve total excision as this is an important factor for long term survival. [1],[4] Removal of the involved bone is to be done. Reconstruction of the bone defect has been advised by few authors. [2] A four-drug regimen (vincristine, adriamycin, cyclophosphamide, and actinomycin-D) of chemotherapy is described to be superior to other regimens. [2],[5] Aggressive multidrug chemotherapy and radiotherapy have a significant role in preventing local recurrences or systemic metastasis (especially to lungs). [2]

 > References Top

1.Desai KI, Nadkarni TD, Goel A, Muzumdar DP, Naresh KN, Nair CN. Primary Ewing′s sarcoma of the cranium. Neurosurgery 2000;46:62-8.  Back to cited text no. 1
2.Agrawal A, Dulani R, Mahadevan A, Vagaha SJ, Vagha J, Shankar SK. Primary Ewing′s sarcoma of the frontal bone with intracranial extension. J Cancer Res Ther 2009;5:208-9.  Back to cited text no. 2
3.Garg A, Ahmad FU, Suri A, Mahapatra AK, Mehta VS, Atri S, et al. Primary Ewing′s sarcoma of the occipital bone presenting as hydrocephalus and blindness. Pediatr Neurosurg 2007;43:170-3.  Back to cited text no. 3
4.Bricha M, Jroundi L, Boujida N, El Hassani MR, Chakir N, Jiddane M. Primary Ewing sarcoma of the skull vault. J Radiol 2007;88:1899-901.  Back to cited text no. 4
5.Thacker MM, Temple HT, Scully SP. Current treatment for Ewing′s sarcoma. Expert Rev Anticancer Ther 2005;5:319-31.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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