Primary Ewing’s sarcoma of the petroclival bone: A case report and literature review

Abstract

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.

Primary intracranial extraosseous Ewing’s sarcoma of the skull base in an elderly adult: illustrative case

“…1 Our English MEDLINE online literature review identified only 13 cases of primary intracranial Ewing's sarcoma in patients of age 50 years or older (Table 1). 5,8,10,[16][17][18][19][20][21][22][23][24] The mean (standard deviation) age of these patients was 59.2 (8.2) years. Male to female ratio was 8:5 (61.6%:38.4%) Of these patients, only 3 (23%) were of age similar to or older than our patient.…”

Surgical management of primary Ewing’s sarcoma of the petroclival bone extend into the sphenoid sinus: A case report and review of literatures

“…Primary ESs of skull base involvement are extremely rare, and to date, only three cases of primary ES of the petroclival bone have been reported [ Table 1 ]. [ 1 , 7 , 8 ] The four reported cases include two male and two female patients. Most of patients are younger with a median age of 23 years (14–72).…”

“…Regarding surgical management, previous reports have recommended that tumor resection should be as radical as possible. [ 1 , 6 , 7 ] However, radical tumor resection may be difficult in skull base lesions due to the inclusion of or proximity to critical structures. Among the current treatment protocols for primary ES, multimodal treatment including a combination of radiation therapy, chemotherapy, and surgery can result in a cure rate of ≥50%.…”

“…[ 2 ] Moreover, adjuvant multimodal treatment may lead to recovery or improvement of CN palsy after several months. [ 1 , 7 ] Safe and minimally invasive surgery for diagnosis of the pathology and subsequent multimodal treatment without delay is a valuable strategy for patients with primary petroclival ES.…”

“…Only three cases of primary ES of the petroclival bone have been reported; therefore, the etiology is not well known. [ 1 , 7 , 8 ] Here, we report a rare case of primary ES of the petroclival bone extending into the sphenoid sinus that was successfully treated by endoscopic transsphenoidal surgery, adjuvant chemotherapy, and radiation, and discuss the clinical features and surgical management.…”