Ewing’s sarcoma

Ewing’s sarcoma

Ewing’s sarcoma: aggressive malignant tumor with a peak incidence during the second decade of life. Spine metastases are more common than primary spine lesions.

Treatment

Treatment is mostly palliative: radical excision followed by RTX (very radiosensitive) and chemotherapy 1).

The use of stereotactic body radiotherapy (SBRT) is well-accepted 2).

SRS for spine metastases from Ewing sarcoma can be considered as a treatment option in adolescent and young adult patients and is associated with acceptable toxicity rates. Further studies must be conducted to determine long-term local control and toxicity for this treatment modality 3).

Complications

SIADH.

Combined intracranial/extracranial lesions.


see Ewing’s Sarcoma peripheral primitive neuroectodermal tumor

Case reports

Intradural Extramedullary Ewing’s Sarcoma in the Cervical Region 4).


A 21-year-old woman presenting with quadriplegia which was initially diagnosed with an epidural abscess in view of her MR scan and raised inflammatory marker levels. Histology revealed an epidural extra-osseous Ewing’s sarcoma (EES). Epidural location of EES is a very rare condition which can be very challenging to diagnose. Early diagnosis and surgical excision followed by chemotherapy represent the main stem of management 5).

References

1)

Grubb MR, Currier BL, Pritchard DJ, et al. Primary Ewing’s Sarcoma of the Spine. Spine. 1994; 19:309– 313
2)

Karimi AM, Campbell SR, Parsai S, Angelov L, Scott J, Qi P, Anderson P, Chao ST, Murphy ES. Aggressive Local Control With Multisite Stereotactic Body Radiation in Metastatic Ewing Sarcoma: A Literature Review and Case Report. Anticancer Res. 2020 Feb;40(2):951-955. doi: 10.21873/anticanres.14028. Review. PubMed PMID: 32014939.
3)

Parsai S, Juloori A, Angelov L, Scott JG, Krishnaney AA, Udo-Inyang I, Zhuang T, Qi P, Kolar M, Anderson P, Zahler S, Chao ST, Suh JH, Murphy ES. Spine radiosurgery in adolescents and young adults: early outcomes and toxicity in patients with metastatic Ewing sarcoma and osteosarcoma. J Neurosurg Spine. 2019 Nov 29:1-8. doi: 10.3171/2019.9.SPINE19377. [Epub ahead of print] PubMed PMID: 31783349.
4)

Warade AC, Jha AK, Pattankar S, Madiwale C, Rekhi B, Desai K. A Rare Case of Intradural Extramedullary Ewing’s Sarcoma in the Cervical Region. Neurol India. 2019 Nov-Dec;67(6):1543-1544. doi: 10.4103/0028-3886.273622. PubMed PMID: 31857557.
5)

Bailey M, Mccabe M, Pal P, Agushi E, Karabatsou K. Cervical epidural extra-osseous Ewing sarcoma mimicking an epidural abscess. Br J Neurosurg. 2016 Feb;30(1):113-114. Epub 2015 Sep 7. PubMed PMID: 26982951.

Facial nerve schwannoma

Facial nerve schwannoma

Facial nerve schwannoma may arise in any portion of the facial nerve, with a predilection for the geniculate ganglion 1) 2).

They can occur anywhere from the internal auditory canal to the parotid gland. Schwannomas arising from the greater superficial petrosal nerve are exceedingly rare 3).

Clinical

Even in these tumors, hearing loss tends to precede facial paresis. Hearing loss may be sensorineural from VIII cranial nerve compression from tumors arising in the proximal portion of VII cranial nerve (cisternal or internal auditory canal (IAC) segment), or it may be conductive from erosion of the ossicles by tumors arising in the second (tympanic, or horizontal) segment of VII. Facial palsy (peripheral) may also develop, usually late 4).

Diagnosis

Computed tomography (CT) of the temporal bone is important for evaluating the impact on the surrounding structures 5).

Treatment

Treatment for intracranial facial nerve schwannomas depends on clinical presentation, tumor size, preoperative facial, and hearing function.

Conservative management is recommended for asymptomatic patients with small tumors. Stereotactic radiosurgery may be an option for smaller and symptomatic tumors with good facial function. If tumor is large or the patient has facial paralysis, surgical resection should be indicated. If preservation of the facial nerve is not possible, total resection with nerve grafting should be performed for those patients with facial paralysis, whereas subtotal resection is best for those patients with good facial function 6).

see Middle Fossa Approach for Facial Nerve Schwannoma.


These tumors must be assessed with imaging studies, incisional biopsy is not recommended. The treatment is surgical resection in symptomatic patients with facial paralysis greater than grade III of House-Brackmann, with immediate reconstruction of the nerve 7).

Case series

Facial nerve schwannoma case series.

Case reports

Facial nerve schwannoma case reports.

References

1) , 4)

Inoue Y, Tabuchi T, Hakuba A, et al. Facial Nerve Neuromas: CT Findings. J Comput Assist Tomogr. 1987; 11:942–947
2)

Tew JM, Yeh HS, Miller GW, Shahbabian S. Intratemporal Schwannoma of the Facial Nerve. Neurosurgery. 1983; 13:186–188
3)

Sade B, Lee JH. Recovery of low-frequency sensorineural hearing loss following resection of a greater superficial petrosal nerve schwannoma. Case report. J Neurosurg. 2007 Jul;107(1):181-4. PubMed PMID: 17639892.
5)

Loos E, Wuyts L, Puls T, Foer B, Casselman JW, Bernaerts A, Vanspauwen R, Offeciers E, Dinther JV, Zarowski A, Somers T. Cochlear Erosion due to a Facial Nerve Schwannoma. J Int Adv Otol. 2019 Jul 9. doi: 10.5152/iao.2019.5304. [Epub ahead of print] PubMed PMID: 31287431.
6)

Xu F, Pan S, Alonso F, Dekker SE, Bambakidis NC. Intracranial Facial Nerve Schwannomas: Current Management and Review of Literature. World Neurosurg. 2017 Apr;100:444-449. doi: 10.1016/j.wneu.2016.09.082. Epub 2016 Sep 28. Review. PubMed PMID: 27693767.
7)

Prado-Calleros HM, Corvera-Behar G, García-de-la-Cruz M, Calderón-Wengerman Ó, Prado A, Pombo-Nava A. Tympanic-mastoid and parotid schwannomas of the facial nerve: clinical presentation related to the anatomic site of origin. Cir Cir. 2019;87(4):377-384. doi: 10.24875/CIRU.18000449. PubMed PMID: 31264987.

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