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(→Rhabdomyosarcoma: subd..) |
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#Intermediate (rarely metastasizing). | #Intermediate (rarely metastasizing). | ||
#Malignant. | #Malignant. | ||
=Other= | |||
==Neurofibromatosis== | |||
Comes in two flavours: | |||
#NF1 (peripheral). | |||
#NF2 (central). | |||
===NF1=== | |||
Features (need 2/7 to diagnose):<ref>URL: [http://emedicine.medscape.com/article/1177266-overview http://emedicine.medscape.com/article/1177266-overview]. Accessed on: 3 May 2010.</ref> | |||
*Two or more neurofibromas or one plexiform neurofibroma. | |||
*Café-au-lait spots. | |||
*Freckles in axilla or inguinal area. | |||
*Optic nerve glioma. | |||
*Iris hamartomas (Lisch nodules). | |||
*Sphenoid dysplasia or typical long-bone abnormalities (e.g. bowing). | |||
*First-degree relative with NF1. | |||
===NF2=== | |||
Features (need 1/3 to diagnose):<ref>URL: [http://emedicine.medscape.com/article/1178283-overview http://emedicine.medscape.com/article/1178283-overview]. Accessed on: 3 May 2010.</ref> | |||
#Bilateral CNVIII masses on imaging. | |||
#Unilateral CNVIII mass + first-degree relative with NF2. | |||
#First-degree relative with NF2 ''and'' 2/4 of the following: | |||
## Meningioma. | |||
## Glioma. | |||
## Schwannoma. | |||
## Juvenile cataract. | |||
==Small round blue cell tumours (SRBCT)== | |||
A group of tumours that has a similar histologic appearance. It is a group of tumours that is seen more often in childhood than adulthood. | |||
===DDx=== | |||
*Neuroblastoma. | |||
*Wilm's tumour. | |||
*Alveolar rhabdomyosarcoma. | |||
*Ewing sarcoma/PNET - this entity is dealt with in the ''[[bone]]'' article. | |||
*Lymphoma (diffuse large B cell lymphoma). | |||
*Retinoblastoma. | |||
*Hepatoblastoma. | |||
*Desmoplastic small round cell tumour. | |||
===Microscopic=== | |||
Features: | |||
*Sheets of cells, very cellular. | |||
*Small cells ~ 2X RBC diameter. | |||
*Scant cytoplasm. | |||
*Coarse chromatin. | |||
*Nucleolus (???). | |||
*+/-Vascular. | |||
=Adipocytic tumours= | =Adipocytic tumours= | ||
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===IHC=== | ===IHC=== | ||
*Factor VIII +ve. | *Factor VIII +ve. | ||
=Skeletal muscle tumours= | =Skeletal muscle tumours= |
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