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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1015-1017

Primary Ewing's sarcoma of the squamous part of temporal bone in a young girl treated with adjuvant volumetric arc therapy

1 Department of Radiation Oncology, Apollo Gleneagles Hospital Limited, Kolkata, West Bengal, India
2 Department of Medical Oncology, Medica Superspeciality Hospital, Kolkata, West Bengal, India

Date of Web Publication03-Nov-2015

Correspondence Address:
Moujhuri Nandi
Department of Radiation Oncology, Apollo Gleneagles Hospital Limited, 58 Canal Circular Road, EM Bypass, Kolkata - 700 054, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.168995

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 > Abstract 

Ewing's sarcoma (ES)/peripheral primitive neuroectodermal tumors usually arise in the long bones of children and young adults. Primary ES of the cranium is unusual. Treatment involves multi-modality therapy incorporating surgery, radiotherapy and chemotherapy; outcomes are similar to those arising from long bones. We report a case of Primary ES of the squamous part of temporal bone with intracranial extension in a 9-year-old girl who was treated with surgery, chemotherapy followed by adjuvant radiotherapy by volumetric arc therapy. Post 1-year of treatment the girl is performing well in her classes.

Keywords: Cranium, Ewing′s sarcoma, radiotherapy, temporal bone, volumetric arc therapy

How to cite this article:
Nandi M, Bhattacharya J, Goswami S, Goswami C. Primary Ewing's sarcoma of the squamous part of temporal bone in a young girl treated with adjuvant volumetric arc therapy. J Can Res Ther 2015;11:1015-7

How to cite this URL:
Nandi M, Bhattacharya J, Goswami S, Goswami C. Primary Ewing's sarcoma of the squamous part of temporal bone in a young girl treated with adjuvant volumetric arc therapy. J Can Res Ther [serial online] 2015 [cited 2020 Feb 19];11:1015-7. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1015/168995

 > Introduction Top

Ewing's sarcoma (ES) family of tumors are a group of small round blue cell neoplasms that include ES of bone, primitive neuroectodermal tumor (PNET) of bone, Askin's tumor (PNET of chest wall), and extraosseous ES. [1] Typically, these tumors occur in young adults and adolescents commonly arising from pelvic bones, long bones, and bones of the chest wall. [2] The skull bones are rarely involved; incidence being 2%. [3] The successful treatment of patients requires systemic chemotherapy in conjunction with either surgery or radiation therapy or both for local tumor control. [4],[5]

We report a case of ES arising from the squamous part of left temporal bone with involvement of temporal lobe in a 9-year-old girl who was treated with surgery, chemotherapy, and postoperative radiotherapy by volumetric arc therapy along with concurrent chemotherapy.

 > Case report Top

A 9-year-old girl child presented with complaints of left sided scalp swelling and occasional headache for 2 months. Swelling was gradually progressive; headache was intermittent in nature without affecting her daily activities.

On examination, a diffuse swelling (~3 cm × 3 cm) was noted at the left temporal region; fixed, bony hard in consistency, and nontender. There was no redness, increased vascularity, or fixity of the overlying skin. No discharge was noted from the external acoustic meatus; her vision and hearing abilities were within normal limits and there was no associated neurodeficit.

Computed tomography (CT) scan of the brain revealed an expansile vascular tumor arising from the squamous part of left temporal bone [Figure 1]. Subsequent magnetic resonance imaging (MRI) showed a 5 cm × 5.3 cm × 4 cm lobulated lesion; arising from the left temporal bone and extending upward to compress the left temporal lobe, brainstem and the temporal horn of lateral ventricle. Laterally, the tumor was limited by the dura. The lesion was hypointense on T1, hyperintense on T2, and exhibited heterogeneous enhancement on T1 gadolinium contrast [Figure 2].
Figure 1: Computed tomography scan brain showed an expansile vascular tumor arising from the squamous part of the temporal bone

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Figure 2 : Preoperative magnetic resonance imaging (T2 sequences) - showed a hyperintense 5 cm lobulated lesion arising from the left temporal bone, extending upward and compressing the left temporal lobe. Laterally, the tumor was limited by the dura

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Routine blood tests were normal. Metastatic work-up with CT scan of thorax, whole body bone scan, and bone marrow biopsy were negative.

She underwent left temporal craniotomy with excision of the mass at an outside hospital preceded by a frozen section which was called out as malignant round cell tumor. Final histopathology reported it as suggestive of ES or PNET. Immunohistochemical staining confirmed the diagnosis of primary ES as it was positive for vimentin, CD99, and friend leukemia integration 1 transcription factor [Figure 3].
Figure 3: (a) Depicts solid sheet of cells with small monotonous round nuclei; rosette formation. (b) Depicts CD99

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Postoperative CT scan of the brain showed a residual tumor. The patient received 8 cycles of chemotherapy with alternate vincristine, adriamycin, cyclophosphamide (VAC) -ifosfamide, and etoposide (IE) regime over next 6 months in our hospital which was followed by chemoradiation to the local area.

Volumetric arc therapy was delivered in two phases; gross tumor volume delineated from fused preoperative MR scan was prescribed 45 Gy in 25 fractions at 1.8 Gy per fraction in phase I and postoperative residual tumor volume was boosted in phase II to a further 10.8 Gy in 6 fractions to deliver a total dose of 55.8 Gy in 31 fractions. Contouring and treatment planning were done on Treatment Planning System (Eclipse 3D version 11; Varian Medical Systems Inc., Palo Alto, USA) and rapid arc therapy was delivered with Novalis Tx [Figure 4].
Figure 4: Treatment with rapid arc therapy shows adequate target coverage with sparing of surrounding critical structures

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She received chemotherapy (IE) concurrently with radiotherapy during the first and last weeks of treatment. During radiation, she developed Grade 1 (Radiation Therapy Oncology Group) skin toxicity (pigmentation and dry desquamation of left temporal region). She also had excoriation of left external auditory canal and conjunctival congestion in the left eye in the last 2 weeks of treatment. The symptoms were managed conservatively. She tolerated radiotherapy well and completed treatment without any interruption.

Her follow-up MRI at 6 months revealed no residual mass lesion suggesting a complete response to treatment. Post 1-year of treatment she remains disease-free and is performing well in her classes [Figure 5].
Figure 5: Posttreatment magnetic resonance imaging at 1-year shows gliosis only, no residual or recurrent disease

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 > Discussion Top

ES of the cranium, though infrequent, primarily affects the frontal and parietal bones; temporal, orbital, and maxillary bones may be affected as well. [6]

Cranial Ewing's usually presents with scalp swelling and features of increased intracranial tension (headache, vomiting). These tumors respect dural planes; thus, present with large lesions with an intracranial extension or impending neurological deficit warranting immediate surgical exploration. Proximity of vital structures leads to incomplete surgery. Despite the adversity in the location of these tumors, their prognosis do not seem to differ much from the extremity or pelvic tumors as distant metastasis from cranial tumors is rare. Moreover, they seem to respond favorably to radio and chemotherapy. [6],[7],[8],[9],[10]

To the best of our knowledge, this is the second case being reported of primary ES arising from the squamous temporal bone. Earlier case reports document petrous part as the site of origin with only one reported case from the squamous part of temporal bone. [8] Steinbok et al. [3] in 1986 and Desai et al. in 1998 [6] reported 2 cases of ES involving the petrous bone in a 3-year-old girl and in an 18-month-old child, that were both treated by radical surgery followed by adjuvant radiation and chemotherapy. In the case of a 5-month-old boy reported by Carlotti et al. in 1999, [7] surgical intervention and chemotherapy were performed without radiation. Interestingly, all the cases of primary petrous bone ES presented with very large lesions and signs of raised intracranial pressure requiring urgent surgical intervention. Nevertheless, despite aggressive surgery, the lesions were resected subtotally in all the cases but showed a favorable response to polychemotherapy and radiation. None of the children showed signs of metastatic spread.

Our case was a 9-year-old girl presenting with a diffuse swelling with occasional headaches. She underwent subtotal resection, received adjuvant chemotherapy according to EURO-EWING 99 protocol; which is considered superior to the three regimen protocol. Most of the cases have delivered 54 Gy in 30 fractions; we prescribed 55.8 Gy in 31 fractions by volumetric arc therapy which was achieved respecting the dose constraints of the critical organs. Post 1-year of treatment the girl remains disease-free and is performing well in her classes.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Delattre O, Zucman J, Melot T, Garau XS, Zucker JM, Lenoir GM, et al. The Ewing family of tumors - a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994;331:294-9.  Back to cited text no. 1
Bhansali SK, Desai PB. Ewing sarcoma observation on 107 cases. J Bone Joint Surg 1963;45:541-53.  Back to cited text no. 2
Steinbok P, Flodmark O, Norman MG, Chan KW, Fryer CJ. Primary Ewing's sarcoma of the base of the skull. Neurosurgery 1986;19:104-7.  Back to cited text no. 3
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. 4
Dunst J, Schuck A. Role of radiotherapy in Ewing tumors. Pediatr Blood Cancer 2004;42:465-70.  Back to cited text no. 5
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. 6
Carlotti CG Jr, Drake JM, Hladky JP, Teshima I, Becker LE, Rutka JT. Primary Ewing's sarcoma of the skull in children. Utility of molecular diagnostics, surgery and adjuvant therapies. Pediatr Neurosurg 1999;31:307-15.  Back to cited text no. 7
Souvirón Encabo R, Arenas Britez O, Gil R, Pérez-Luengo E, Scola Yurrita B. Primitive neuroectodermal tumor/extraosseus Ewing sarcoma of the temporal bone. Acta Otorrinolaringol Esp 2005;56:38-40.  Back to cited text no. 8
Harman M, Kiroglu F, Kösem M, Unal O. Primary Ewing's sarcoma of the paranasal sinus with intracranial extension: Imaging features. Dentomaxillofac Radiol 2003;32:343-6.  Back to cited text no. 9
Bhatoe HS, Deshpande GU. Primary cranial Ewing's sarcoma. Br J Neurosurg 1998;12:165-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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