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Rhabdomyosarcoma Masquerade Syndrome

Rhabdomyosarcoma Masquerade Syndrome. LC Clarke, RS Thampy, R Ajit , L Irion , R Bonshek , S Ataullah , B Leatherbarrow Manchester Royal Eye Hospital. Rhabdomyosarcoma. Most common primary orbital malignancy of childhood. Presenting signs may mislead the unsuspecting clinician

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Rhabdomyosarcoma Masquerade Syndrome

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  1. Rhabdomyosarcoma Masquerade Syndrome LC Clarke, RS Thampy, R Ajit, L Irion, R Bonshek, S Ataullah, B Leatherbarrow Manchester Royal Eye Hospital

  2. Rhabdomyosarcoma • Most common primary orbital malignancy of childhood. • Presenting signs may mislead the unsuspecting clinician • We present a series of five consecutive patients referred to Manchester Royal Eye Hospital over the course of the last 2 years, each with a different initial diagnosis. • We highlight how rhabdomyosarcoma may mimic other orbital disorders, leading to a potentially life threatening delay in appropriate management.

  3. Occurs at sites of embryonic tissue fusion • Head ,neck, midline Classification related to prognosis Botyroid and spindle: Best Embryonal : intermediate Alveolar and pleomorphic :poor

  4. Features of Striated muscle differentiation • Bright cytoplasmiceosionophilia +/- cross striations • Immuno Panel; • MyoD1(nuclear staining) • Desmin • Myogenin and myoglobin

  5. Case 1 • FP – age 9 years. 2 week hx of right inferior fornix lesion • “Conjunctivalpapilloma”

  6. CT orbit Axial View with contrast Lesion at right medial canthus

  7. H & E Spindle to round, pleomorphic tumour cells Immunohistochemical analysis

  8. Squamouspapilloma-like appearance of at the time of biopsy. Histology showing round and spindle-shaped cells with mitoses. Demonstration of cell positivity for desmin. (d) Conjunctivalsquamouspapilloma.

  9. Local recurrence following chemotherapy 2 years prior. • Orbital exenteration, further chemo & radiotherapy • Recurrence in exenterated orbit • RIP 2010 (6 years post presentation)

  10. Case 2 • LB – age 10 years. 4 week hx of swelling right eye • “Orbital cellulitis”

  11. CT orbit Axial View with contrast CT - Upper lid local disease

  12. H & E H&E – Diffuse infiltration by poorly differentiated tumour cells

  13. Surgical debulking &chemotherapy - good response

  14. Case 3 • LR – age 10 years. 1 week hx of right proptosis and diplopia. • “Langerhans cell histiocytosis”

  15. MRI Orbit Axial MRI - Mass in lateral orbital wall and temporal fossa

  16. H & E Diffuse infiltration by poorly differentiated tumour cells

  17. Bone marrow mets. • Chemo and radiotherapy – ongoing • Lagophthalmos. • Persistent ocular surface problems

  18. Case 4 • JB – age 5 years. 2 month hx of progressive right ptosis • “Upper lid chalazion”

  19. CT Orbits Axial View CT - Upper lid local disease

  20. H&E Alveolar rhabdomyosarcoma. Clear cell component to the right

  21. Surgical debulking &chemotherapy - good response

  22. Case 5 LG – age 11 years. 3 week hx of right upper lid swelling “Eyelid papilloma”

  23. CT Orbits Axial View CT - Thickening of upper lid & regional lymphadenopathy

  24. H&E H&E – Area of alveolar architecture. Note stretched surface epithelium.

  25. Surgical debulk, chemo and radiotherapy

  26. A high index of suspicion • Following treatment, local tumor recurrence occurs in 18% of cases, metastasis in 6%, and death in 3%. • paediatric patient • history of rapidly progressive orbital , eyelid or conjunctival lesion.

  27. The diagnosis of rhabdomyosarcoma should considered until proven otherwise • to avoid unnecessary delays in diagnosis • expedite appropriate management, • optimize the outcome for this group of patients.

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