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Primary ewing sarcoma of the breast in a male adolescent - a case report
Joie Sheen Bastian
Philippine Children's Medical Center, Philippines
INTRODUCTION:
Ewing sarcoma of the breast is a rare disease among the extraosseous type with less than 1% of cases reported in literature. There are 16 cases of breast Ewing sarcoma documented worldwide, with only 1 out 16 diagnosed in the pediatric age group. None of the published cases were from the Philippines. This will be the first reported case of Ewing sarcoma of the breast in a pediatric patient from the locality.
CASE PRESENTATION:
A 14-year-old male consulted due to a rapidly growing right breast mass. Tissue biopsy revealed malignant round blue cell tumor, immunohistochemical staining positive for CD 99 and NKX2.2 as well as EWSR1 break apart in chromosome 22q12 on fluorescent in situ hybridization (FISH) test, favoring the diagnosis of Ewing sarcoma. Chest computed tomography showed a large, exophytic, right anterior chest wall mass with intrathoracic extension measuring 16.8x18.1x9.5cm, involving 2nd to 6th anterior ribs and multiple enlarged axillary lymph nodes. PET/CT revealed no other metastatic disease. The patient received neoadjuvant chemotherapy using the PNET/Ewing sarcoma protocol. Imaging post neoadjuvant chemotherapy showed a significant decrease of the mass, now measuring 3.9x12.2x6.5cm. This was followed by wide excision of the tumor with en-bloc anterior 5th rib resection, frozen section of margins, axillary lymph node dissection and latissimus dorsi flap reconstruction. The patient tolerated the treatment with no recurrence of the tumor upon follow up post-surgery.
CONCLUSION:
Ewing sarcoma of the breast is rare, and complete immunohistomorphologic work up including FISH test is necessary to confirm the diagnosis. Current guidelines have not reached a consensus in the treatment of this malignancy due to the scarcity of number of cases reported. A disease more commonly diagnosed in the pediatric age group but conspicuously seen in adults on this specific anatomical location may be of interest for future investigation.