✍️ Most common primary orbital malignancy of children
✍️ Most common soft tissue malignancy of childhood
✍️ Most common mesenchymal tumor of orbit
✍️ Malignant spindle cell tumor with loose myxomatous matrix
✍️ Average age at diagnosis is 8 years old (90% before age 16)
✍️ Cell of origin is an undifferentiated, pluripotent cell of the soft tissue
✍️ does not originate from the extraocular muscles
✍️ usually Unilateral superonasal portion of orbit
✍️ More common in males (5:3)
✍️ Aggressive local spread through orbital bones
✍️ hematogenous spread to lungs and cervical lymph nodes
✍️ most common location for metastasis is chest
✍️ Rhabdomyosarcoma clinical presentation:
☝️ rapidly progressive proptosis
☝️ reddish discoloration of eyelid
☝️ ptosis
☝️ tortuous retinal veins
☝️ choroidal folds
☝️ optic nerve edema
✍️ Rhabdomyosarcoma Types
☝️ Embryonal
• most common (70%), usually occurs in children
☝️ Pleomorphic
least common with best prognosis , usually occurs in adults, rare involvement of orbit
☝️ Alveolar
• poorest prognosis, usually found in inferior orbit, generally arises in extremities (adolescents).
✍️ Rhabdomyosarcoma Investigations:
✍️ CT scan
☝️ well-circumscribed orbital mass with possible extension into adjacent orbital bones or sinuses, bony destruction
✍️ A-scan ultrasound
☝️ orbital mass with medium internal reflectivity
✍️ Treatment
☝️ urgent biopsy
☝️ radiation (100% local control with 6000 cGy)
☝️ 30% mortality due to metastases
☝️ chemotherapy for any microscopic metastases
☝️ surgical debulking
✍️ Rhabdomyosarcoma Prognosis:
☝️ with chemotherapy and XRT, 3-year
survival 90%
☝️ cure rate close to 100% with localized orbital tumor
☝️ 60% if invasion of adjacent structures
☝️ Tumors arising in the orbit, bladder, and prostate 77% disease-free survival at 2 years
☝️ Intrathoracic tumors: worst prognosis, 24% disease-free survival at 2 years.
