Alveolar rhabdomyosarcoma

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Alveolar rhabdomyosarcoma
Classification and external resources
ICD-O:	M 8920/3
MeSH	D018232

Alveolar rhabdomyosarcoma is a type of rhabdomyosarcoma characterized by its appearance, which is similar to the alveoli of the lungs.

It can be associated with a fusion protein between PAX3 and FKHR (now known as FOXO1).^[1]^[2]

It can also be associated with PAX7-FKHR.^[3]^[4]

References

^ Fredericks WJ, Galili N, Mukhopadhyay S, et al. (March 1995). "The PAX3-FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3". Mol. Cell. Biol. 15 (3): 1522–35. PMC 230376. PMID 7862145. http://mcb.asm.org/cgi/pmidlookup?view=long&pmid=7862145.
^ Mercado GE, Xia SJ, Zhang C, et al. (June 2008). "Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant target". Genes Chromosomes Cancer 47 (6): 510–20. doi:10.1002/gcc.20554. PMID 18335505.
^ Mercado GE, Barr FG (February 2007). "Fusions involving PAX and FOX genes in the molecular pathogenesis of alveolar rhabdomyosarcoma: recent advances". Curr. Mol. Med. 7 (1): 47–61. doi:10.2174/156652407779940440. PMID 17311532. http://www.bentham-direct.org/pages/content.php?CMM/2007/00000007/00000001/0005M.SGM.
^ Laé M, Ahn EH, Mercado GE, et al. (June 2007). "Global gene expression profiling of PAX-FKHR fusion-positive alveolar and PAX-FKHR fusion-negative embryonal rhabdomyosarcomas". J. Pathol. 212 (2): 143–51. doi:10.1002/path.2170. PMID 17471488.

Fibromatous (8810–8839)	Fibroma/fibrosarcoma: Dermatofibrosarcoma · Dermatofibrosarcoma protuberans · Desmoplastic fibroma Fibroma/fibromatosis: Aggressive infantile fibromatosis · Aponeurotic fibroma · Collagenous fibroma · Diffuse infantile fibromatosis · Familial myxovascular fibromas · Fibroma of tendon sheath · Fibromatosis colli · Infantile digital fibromatosis · Juvenile hyaline fibromatosis · Plantar fibromatosis · Pleomorphic fibroma · Oral submucous fibrosis Histiocytoma/histiocytic sarcoma: Benign fibrous histiocytoma · Malignant fibrous histiocytoma · Atypical fibroxanthoma Solitary fibrous tumor

Myxomatous (8840–8849)	Myxoma/myxosarcoma (Cutaneous myxoma, Superficial acral fibromyxoma) · Angiomyxoma · Ossifying fibromyxoid tumour

Fibroepithelial (9000–9039)	Brenner tumour · Fibroadenoma · Phyllodes tumor

Synovial-like (9040–9049)	Synovial sarcoma · Clear-cell sarcoma

Lipomatous (8850–8889)

Lipoma/liposarcoma (Myelolipoma, Myxoid liposarcoma) · PEComa (Angiomyolipoma)

Chondroid lipoma · Intradermal spindle cell lipoma · Pleomorphic lipoma · Benign lipoblastomatosis · Spindle cell lipoma · Hibernoma

Myomatous (8890–8929)

general: Myoma/myosarcoma

smooth muscle: Leiomyoma/leiomyosarcoma

skeletal muscle: Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma (Sarcoma botryoides) · Alveolar rhabdomyosarcoma

Leiomyoma · Angioleiomyoma · Angiolipoleiomyoma · Genital leiomyoma · Leiomyosarcoma · Multiple cutaneous and uterine leiomyomatosis syndrome · Multiple cutaneous leiomyoma · Neural fibrolipoma · Solitary cutaneous leiomyoma

Complex mixed and stromal (8930–8999)

Adenomyoma · Pleomorphic adenoma · Mixed Müllerian tumor · Mesoblastic nephroma · Wilms' tumor · Rhabdoid tumour · Clear-cell sarcoma of the kidney · Hepatoblastoma · Pancreatoblastoma · Carcinosarcoma

Mesothelial (9050–9059)

Mesothelioma · Adenomatoid tumor

see also Template:Connective tissue

M: MUS, DF+DRCT

anat (h/n, u, t/d, a/p, l)/phys/devp/hist

noco(m, s, c)/cong(d)/tumr, sysi/epon, injr

proc, drug (M1A/3)

Pathology: chromosome abnormalities (Q90–Q99 · 758)

Autosomal

X/Y linked

Monosomy	Turner syndrome (XO)

Trisomy/tetrasomy, other karyotypes/mosaics	Klinefelter's syndrome (47,XXY) · 48,XXYY · 48,XXXY · 49,XXXYY · 49,XXXXY Triple X syndrome (47,XXX) · 48,XXXX · 49,XXXXX 47,XYY · 48,XYYY · 49,XYYYY 46,XX/XY

Translocations

Leukemia/lymphoma

Lymphoid	Burkitt's lymphoma t(8 MYC;14 IGH) · Follicular lymphoma t(14 IGH;18 BCL2) · Mantle cell lymphoma/Multiple myeloma t(11 CCND1:14 IGH) · Anaplastic large cell lymphoma t(2 ALK;5 NPM1) · Acute lymphoblastic leukemia

Myeloid	Philadelphia chromosome t(9 ABL; 22 BCR) · Acute myeloblastic leukemia with maturation t(8 RUNX1T1;21 RUNX1) · Acute promyelocytic leukemia t(15 PML,17 RARA) · Acute megakaryoblastic leukemia t(1 RBM15;22 MKL1)

Other

Ewing's sarcoma t(11 FLI1; 22 EWS) · Synovial sarcoma t(x SYT;18 SSX) · Dermatofibrosarcoma protuberans t(17 COL1A1;22 PDGFB) · Myxoid liposarcoma t(12 DDIT3; 16 FUS) · Desmoplastic small round cell tumor t(11 WT1; 22 EWS) · Alveolar rhabdomyosarcoma t(2 PAX3; 13 FOXO1) t (1 PAX7; 13 FOXO1)

Other

Fragile X syndrome · Uniparental disomy · XX male syndrome

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