- Department of Surgery, Geisel School of Medicine at Dartmouth College, Hanover, New Hampshire,
- Department of Surgery, Section of Otolaryngology- Head and Neck Surgery,
- Department of Pathology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.
Department of Surgery, Section of Otolaryngology- Head and Neck Surgery,
DOI:10.25259/SNI_415_2019Copyright: © 2020 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Derrek Schartz, Prashanthi Divakar, Laura Tafe, Joseph Paydarfar. Primary Ewing’s sarcoma of the petroclival bone: A case report and literature review. 10-Jan-2020;11:6
How to cite this URL: Derrek Schartz, Prashanthi Divakar, Laura Tafe, Joseph Paydarfar. Primary Ewing’s sarcoma of the petroclival bone: A case report and literature review. 10-Jan-2020;11:6. Available from: https://surgicalneurologyint.com/surgicalint-articles/9837/
Background: Primary Ewing’s sarcoma (ES) is typically seen within the long bones, vertebrae, or pelvis. Uncommonly, it can be found within the cranium among the rarest locations for primary ES are the skull base, in particular, the petroclival bone.
Case Description: The patient is a 68-year-old female with past medical history of Stage III breast cancer who presented with severe retro-orbital headache and diplopia due to a cranial nerve VI palsy. Magnetic resonance imaging (MRI) revealed a mass at the left petroclival bone with extension into the adjacent left petrous apex and into the posterior aspect of the left cavernous sinus proximal to the carotid artery. The patient subsequently underwent an endoscopic transsphenoidal biopsy. Pathological and molecular analysis supported a diagnosis of ES. The patient then underwent neoadjuvant chemotherapy and radiotherapy. At 12 month-follow-up, her petroclival ES demonstrated significant interval decrease in size on MRI surveillance imaging.
Conclusions: This is the third case of primary ES of the petroclival bone to be reported in the literature. In this patient, management consisted of surgical incisional biopsy followed by adjuvant radiation and chemotherapy. Knowledge and awareness of this type of tumor is important for the skull base surgeon.
Keywords: Cranial Ewing’s sarcoma, Petroclival bone, Skull base
Ewing’s sarcoma (ES) is a bone malignancy that is usually seen in the pediatric population, but it may also present in adults. ES is commonly found in the pelvis, femur, tibia, vertebrae, and humerus, and pathologically manifests as small, round, and blue cells with a poor prognosis.[
A 68-year-old female with past medical history of Stage III breast cancer treated in 2011 presented in 2017 with severe retro-orbital headache primarily on the left side around her temple and posterior neck. The headache lasted a few days and resolved; however, soon after, she developed associated symptoms of diplopia. She denied any concurrent adenopathy, trismus, numbness, nasal congestion, recent epistaxis, or any prior diplopia symptoms. Her physical exam was notable for leftward gaze restriction suggestive of a left-sided CN VI palsy. She initially presented to her primary care provider with these symptoms and underwent magnetic resonance imaging (MRI) which revealed a mass at the left petroclival bone with extension into the adjacent left petrous apex and the posterior aspect of the left cavernous sinus concerning for recurrence/metastatic disease of her previously treated breast cancer [
Frozen section pathology demonstrated small round blue cell tumor consistent with primitive neuroectodermal tumor (PNET)/ES [
At her postoperative follow-up visit, the patient’s CN symptoms had improved with ocular movements closer to baseline and reduction in headache severity. The patient was then treated with induction chemotherapy consisting of dactinomycin/cyclophosphamide alternating with ifosfamide/etoposide for four cycles. This was followed by radiotherapy and seventeen cycles of consolidative chemotherapy for cure. One month after completion of her chemotherapy and radiotherapy, MRI surveillance imaging demonstrated a significant interval decrease in enhancement and size of the mass [
Approximately 90% of cases of primary ES occur in patients younger than 20-years-old and it most commonly involves the long bone shafts, pelvic bones, or within ribs or vertebrae.[
Histopathologic analysis of the lesion biopsy demonstrated small, malignant, round blue cells consistent with ES or PNET [
On literature review, only two prior cases have been documented of primary petroclival ES.[
In our case, transsphenoidal biopsy worked to functionally debulk the lesion as the patient’s diplopia and headache improved postoperatively even before the initiation of chemotherapy and radiotherapy. Aggressive initial surgical resection was deferred due to the tumor’s extension into the cavernous sinus and involvement of the carotid artery. Postchemoradiation, the patient’s symptoms have not returned and the most recent imaging has not demonstrated disease progression or recurrence. Therefore, we did not pursue additional surgical resection due to the patient’s tumor stability and risk of surgical morbidity due to surrounding vascular structures. However, prior studies have shown that radical tumor excision, if surgically appropriate, is recommended to reduce tumor mass and increase adjuvant therapy effectiveness.[
Primary ES of the petroclival bone is a rare entity. However, awareness and knowledge of management of this lesion is important for the skull base surgeon. Successful treatment can be facilitated by resection or debulking, which can be successfully done transsphenoidally followed by adjuvant radiotherapy and chemotherapy.
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