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| Micro = large cells (~30-50 μm) with abundant eosinophilic cytoplasm and an eccentric nucleus +/-nucleolus, arranged in nest/separated by thin septa - vaguely resembles alveoli (at low power) | | Micro = large cells (~30-50 μm) with abundant eosinophilic cytoplasm and an eccentric nucleus +/-nucleolus, arranged in nest/separated by thin septa - vaguely resembles alveoli (at low power) | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[alveolar rhabdomyosarcoma]] | | LMDDx = [[paraganglioma]], [[clear cell renal cell carcinoma]], [[alveolar rhabdomyosarcoma]] | ||
| Stains = PAS +ve (cytoplasm) | | Stains = PAS +ve (cytoplasm) | ||
| IHC = TFE3 +ve (suggestive of translocation) | | IHC = TFE3 +ve (suggestive of translocation) | ||
| EM = | | EM = intracytoplasmic crystal lattice with ~5 nm fibres spaced 10 nm apart | ||
| Molecular = t(X;17) | | Molecular = t(X;17) | ||
| IF = | | IF = | ||
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| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = usu. slow growing mass | ||
| Signs = | | Signs = | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = | | Prevalence = rare | ||
| Bloodwork = | | Bloodwork = | ||
| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = poor | | Prognosis = ultimately poor | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = | ||
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*Arranged in nest/separated by thin septa; vaguely resembles alveoli (at low power). | *Arranged in nest/separated by thin septa; vaguely resembles alveoli (at low power). | ||
*Large cells (~30-50 μm) with abundant eosinophilic cytoplasm. | *Large cells (~30-50 μm) with abundant eosinophilic cytoplasm. | ||
**May be focally cleared. | |||
*An eccentric nucleus. | *An eccentric nucleus. | ||
*+/-Nucleolus. | *+/-Nucleolus, prominent. | ||
*+/-Multi-nucleation (common). | |||
DDx: | DDx: | ||
*[[Rhabdomyosarcoma]], alveolar - usu. does not have sheets of rhabdoid cells. | *[[Paraganglioma]]. | ||
*[[Clear cell renal cell carcinoma]] - areas with cytoplasmic clearing. | |||
*[[Rhabdomyosarcoma]], alveolar - usu. does not have sheets of rhabdoid cells, no cross striations. | |||
===Images=== | ===Images=== | ||
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*HMB45 -ve. | *HMB45 -ve. | ||
*Melan‐A -ve. | *Melan‐A -ve. | ||
*Chromogranin A -ve. | |||
*Synaptophysin -ve. | |||
==Molecular== | ==Molecular== |
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