Ewing’s Sarcoma Multifocal Metastases to Temporal and Occipital Bone: A Rare Presentation

Abstract

An 11-year-old male patient was referred to the ENT department at Lok Nayak Hospital with the complaints of right ear discharge, facial weakness, inability to close right eye, and a gradually progressive occipital swelling for one month. In addition, he complained of decreased hearing from right ear, as well as headache. The patient was a known case of Ewing's sarcoma of the left pelvic bones, with involvement of iliac bone, ischium and sacroiliac joint. He had presented initially with the complaints of pain in the left hip and a swelling in the groin and was started upon five cycles of multidrug chemotherapy that included ifosfamide, etoposide, vincristine, actinomycin-D and cyclophosphamide. This was followed by radiotherapy, with a total body dose of 55 Gy over 30 cycles. In view of persistent disease, patient further received 3 cycles of chemotherapy (CT) with ifosfamide, carboplatin and etoposide. It was during one of the follow up visits that patient complained of right sided ear discharge, facial weakness, and an occipital swelling, and was referred to ENT clinic for evaluation.On examination, patient had a red polypoidal mass in the right bony external auditory canal (EAC) with some overlying sero-sanguinous discharge on the floor. The mass was found to be extending into the middle ear, with disruption of lower part of tympanic membrane. It was friable and bled on probing. He also had right sided facial weakness with history of deviation of angle of mouth towards left, suggestive of a right sided lower motor neuron (LMN) type facial nerve palsy . However, patient's right eye was close at the time of examination due to conjuctival chemosis and oedema. The patient had an associated 6 x 6 cm swelling present over the ABSTRACTEwing's sarcoma (ES) is a common malignant bone tumour seen to involve long bones, flat pelvic bones and ribs and vertebrae in majority of cases. Here, we present a rare case of aggressive primary ES of pelvic bones with multifocal metastases to temporal bone and occipital bone. The patient presented with facial palsy and an occipital swelling, and was referred for chemotherapy.occipital bone [Table /Fig-1b]. The swelling was non tender, immobile, and hard in consistency. The overlying skin appeared stretched, and there were multiple dilated scalp veins over the swelling and the adjoining area.The patient underwent computed tomography scan of temporal bone and head revealing soft tissue attenuation contents in the right mastoid air cells and middle ear, with erosion of right ear ossicles, tegmen tympani and scutum [Table/ Fig-2a&b]. The facial canal was eroded at the genu and tympanic segment. Scans through the head revealed an isodense lesion involving occipital bone, heterogeneously enhancing after contrast administration. There was no brain involvement. The patient was taken up for biopsy of the mass which revealed fibro collagenous tissue infiltrated by small round cells, having high nucleus: cytoplasm ratio and few mitoses. The tumour cells were positive for vimentin, neuron speci...

Ewing`s Sarcoma of Temporal Bone : Case Report

“…In the literature, headache, localized swelling, and signs of raised intracranial pressure were the most common symptoms of skull ES 1,3,5,6,7,9 , this could be explained as they found that, the Intracranial extension was present in the majority of primary cases of the skull ES 4 . The main presentation of ES in the temporal bone was facial paralysis, hearing loss and ear discharge 5,7 . Even though it may differ from case to case depending on the location and spread of the tumor, but some researchers did not find significant difference in the presentation of primary and metastatic tumors 5 .…”

“…ES is the second most common malignant bone tumor in young patients 3,4,5 even though it accounts for only 1% of all childhood cancers 8 . Primary ES is more likely to affect long bones, pelvic bones, ribs, and vertebrae 1,2,5,6,7,8 presented most commonly in the second decade of life 2,3,4,5,7 with a male to female ratio of 1.6:1 1,3,5,7 .…”

“…The main presentation of ES in the temporal bone was facial paralysis, hearing loss and ear discharge 5,7 . Even though it may differ from case to case depending on the location and spread of the tumor, but some researchers did not find significant difference in the presentation of primary and metastatic tumors 5 .…”

“…Primary Ewing Sarcoma (ES) is small round cell tumor of neuroectodermal origin 1,2 . the second most common malignant bone tumor in young patients 3,4,5 . It is more likely to affect long bones, pelvic bones, ribs, and vertebrae 1,2,5,6,7,8 and present most commonly in the second decade of life 2,3,4,5,7 .…”

“…the second most common malignant bone tumor in young patients 3,4,5 . It is more likely to affect long bones, pelvic bones, ribs, and vertebrae 1,2,5,6,7,8 and present most commonly in the second decade of life 2,3,4,5,7 . Primary ES of the skull is rare 3,4,6,7,9 and appears in different bones, the most common site is temporal bone 2,3 , as in this present case.…”