Table 2.
Myofibroblastic tumor possibly located in the paranasal sinuses
| Epidemiology | Location | Clinical features | Evolution | Microscopic examination | Immunohistochemistry | |
|---|---|---|---|---|---|---|
| Desmoid tumor | Children and young adults (20–40 year old) Gardner syndrome |
Neck > face > oral cavity > scalp > paranasal sinuses > orbit | Deep tumors with slow growth, infiltration and compression of neighboring structures | Recurrence: 20–50 % | Myofibroblasts in long bundles in a collagen rich matrix Lymphoid clusters |
Actin+; Calponin+; Nuclear Beta-catenin+ Desmin± |
| Nasopharyngeal angiofibroma | Male teenager Rare associations with FAP |
Rhinopharynx >>> maxillary or ethmoidal sinus | NO, recurring epistaxis, headache, facial deformation | Recurrence: 20 % Locally aggressive |
Spindle or stellate fibroblastic/myofibroblastic cells in a dense, low cellular fibrous tissue Numerous vessels and hemorrhagic areas |
Nuclear Beta-catenin+ Actin± ; Androgen receptor± |
| Solitary fibrous tumor | Mean aged adult M = W |
Orbit > lacrymal glands > thyroid > salivary > sinonasal> scalp | Slow growth tumor, symptoms depending on location. Hypoglycemia due to insulin-like growth factor secretion |
Recurrence 10–15 % Metastasis 10 % |
Two variants: fibrous/cellular Spindle or oval-shaped cells Hyaline fibrous collagen-rich area |
CD34+; STAT6+; CD99+ Bcl-2±; EMA±; Actin± |
| Myofibroblastic inflammatory tumors | Adult | Orbit > oral cavity > trachea, larynx >sinonasal > tonsil > salivary glands | NO depending on location Rare systemic symptoms |
Recurrence 25 % Metastasis 5 % |
Myofibroblasts with enlarged nuclei in a myxoid stroma Mainly lymphocytic infiltrate and plasma cells, sometimes neutrophils and eosinophils |
Vimentin+ Actin±; Desmin±; Calponin± |
| Myofibroblastic sarcoma | Mean aged adult M = W |
Mouth, tong > face > maxilla and mandible | Painless and slow growth mass | Recurrence 30 % Metastasis 7 % |
Regular spindle cells + enlarged hyperchromatic cells with atypias Inflammatory matrix made of mast cells and neutrophils |
Actin+ Calponin±; Desmin± CD34−; Cytokeratin−; pS100− |
| Adult fibrosarcoma | 45–60 years old | Nasal fossa and paranasal sinuses | Slow growth mass rarely painful. Epistaxis, NO | Recurrence 60 % Metastasis 15 %. 5-year survival 75 % |
Homogeneous beams arranged in a “herringbone” made of elongated spindle cells | Vimentin+ (non specific) |
M male, W female, NO nasal obstruction, FAP familial adenomatous polyposis