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Case 18

Case 18. 40 year old woman with a slowly enlarging mass of the right maxillary sinus associated with bone destruction. Myxoma. ALCIAN BLUE. RET. IPEX. Vimentin -----Strongly positive MSA-----------Occasionally positive Desmin---------Infrequently positive Cytokeratin----Negative

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Case 18

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  1. Case 18 40 year old woman with a slowly enlarging mass of the right maxillary sinus associated with bone destruction.

  2. Myxoma

  3. ALCIAN BLUE

  4. RET

  5. IPEX • Vimentin -----Strongly positive • MSA-----------Occasionally positive • Desmin---------Infrequently positive • Cytokeratin----Negative • S-100-----------Negative • GFAP-----------Negative

  6. TRICHROME

  7. Myxoma of Bone • Marked predilection for jaws • Occurs primarily between 20-40 years of age; rare in patients less than 10 or over 50 years • Mandible more often involved ( 55-65%) than maxilla, especially posterior area.

  8. Myxoma of Bone • May be asymptomatic or cause pain, nasal obstruction, proptosis, facial asymmetry • Unilocular or multilocular on x-ray with or without bone destruction • Surgery treatment of choice. 26% local recurrence following curettage

  9. DP

  10. DP

  11. DP

  12. Myxoma of Soft Tissue Any age but predominately 30-60 year age group Less aggressive, not restricted to one area of the body Often intramuscular, occasionally subcutaneous tissue, neurovascular bundles

  13. Distribution of 43 Soft Tissue Myxomas of the Head & Neck* Site Number of Cases Palate 10 • Parotid 6 Lateral neck 4 Scalp 3 Cheek 3 • Lip 3 Mandibular alveolar ridge 2 Floor of mouth 2 Oropharynx 2 Chin 1 Face 1 Tongue 1 • Larynx 1 Interdental gingiva 1 Buccal mucosa 1 Retromolar pad 1 Geniohyoid muscle 1 *Tse JJ, Vander S, Head Neck Surg 7:479, 1985

  14. PA

  15. MUC

  16. VCN

  17. VCN

  18. Myxoma of Soft Tissue • Most common intramuscular sites are thigh, pelvic girdle area, and shoulder • Rarely exceeds 5 cm. in head and neck • Conservative but complete excision is TOC • 3-8% local recurrence • 5% are multiple; think of fibrous dysplasia

  19. Myxomas and Fibrous Dysplasia • Only associated with intramuscular myxomas • Mazabraud syndrome • Myxomas are generally multiple; usually 3-4 but sometimes up to 9 per patient • 80% have polystotic fibrous dysplasia; almost half have Albright’s syndrome

  20. Myxomas and Fibrous Dysplasia Myxomas appear years to decades after discovery of the bone lesions. They generally occur in the same vicinity as the abnormal bones No relationship between extent of fibrous dysplasia and number of myxomas

  21. Fibrous Dysplasia GNAS 1 (chromosome 20) GSA (cystine or histidine substituted for arginine at position 201) Excess cAMP Endocrine activity Cells in bone Melanocytes

  22. Myxomas and Gsa Mutations • Present in intramuscular myxomas with or without fibrous dysplasia • Absent in odontogenic myxomas • Absent in cardiac myxomas

  23. Syndrome of Myxomas, Spotty Pigmentation, And Endocrine Overactivity (N=58) 71% Cardiac myxomas 66% Spotty pigmentation 41% Cutaneous myxomas 33% Pigmented nodular adrenocortical disease 22% Myxoid fibroadenomas 21% Cushing’s syndrome 9% Acromegaly 7% Intraoral myxomas 44% Testicular tumors

  24. Carney’s Complex • Multiple neoplasia syndrome • Autosomal dominant • Genes mapped to 2p16 and 17q22-24

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