Primary Occipital Ewing’s Sarcoma with Subsequent Spinal Seeding

Abstract

Ewing's sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing's sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT) imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI) showed cerebrospinal fluid (CSF) seeding from the L5 to the S4 vertebrae. Primary cranial Ewing's sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing's sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing's sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

Optimum treatment for primary intracranial Ewing sarcoma

“…Although primary intracranial extraosseous ES with leptomeningeal dissemination has been rarely described in the literature, no other cases share the extent of central nervous system metastasis present in this patient. [5][6][7] Radiation and chemotherapeutic management strategies for primary intracranial ES with metastasis are illustrated in Table 1. [5][6][7][8][9] A study of 14 cases from a single institution revealed that patients who underwent adjuvant radiotherapy had statistically significant improvement in 1-and 2-year survival and median survival time compared to patients without radiotherapy.…”

“…[5][6][7] Radiation and chemotherapeutic management strategies for primary intracranial ES with metastasis are illustrated in Table 1. [5][6][7][8][9] A study of 14 cases from a single institution revealed that patients who underwent adjuvant radiotherapy had statistically significant improvement in 1-and 2-year survival and median survival time compared to patients without radiotherapy. 6,10 Radiation regimens are typically initiated postoperatively with the standard craniospinal irradiation protocol of 36 Gy in 21 fractions followed by a boost to 54 Gy.…”

Malignant spindle cell tumors of the posterior fossa in children: case series and review of management

“…The most common symptom at presentation was headache and emesis (10/12, 83.3%). 2,3,6,7,[9][10][11][12][13][14][15] Extracranial spread was uncommon, occurring in only two patients. Management typically requires a multimodal approach to include resection, radiotherapy, and intensive multiagent chemotherapy.…”