Gangliocytic Paraganglioma

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Retrieved

2021-01-18

Source

Wikipedia

Trials

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Genes

SDHD, SDHB, VHL, SDHC, EPAS1, SDHAF2, TMEM127, EGLN1, MDH2, BAP1, TP53, SSTR2, PTEN, SDHA, ATRX, CCND1, ATM, DLST, SLC25A11, DAXX, MAX, RET, SARDH, SST, C11orf65, SORD, SDS, FH, HIF1A, CHGA

SDHD, SDHB, VHL, SDHC, EPAS1, SDHAF2, TMEM127, EGLN1, MDH2, BAP1, TP53, SSTR2, PTEN, SDHA, ATRX, CCND1, ATM, DLST, SLC25A11, DAXX, MAX, RET, SARDH, SST, C11orf65, SORD, SDS, FH, HIF1A, CHGA, MEN1, S100A1, NF1, MIR210, SMUG1, VEGFA, CRYGD, TH, S100B, CDKN1B, SLC6A2, CDKN2A, MPZL2, PPY, PROM1, SLC26A4, PGR, PNMT, PPARA, SLC18A2, INSM1, ISCU, ERBB2, FLT1, CALCA, IDH2, FGFR1, SYP, IDH1, MERTK, TTR, MIB1, CDC73, CAMKMT, VCAM1, TET1, TWIST1, RASSF5, EGLN2, CNTN4, CXCR4, TICAM2, LINC01194, MIR21, MIR382, TMED7-TICAM2, LINC01672, RAB4B-EGLN2, H3P23, PRDM2, NLGN4X, FGF21, KMT2D, CD274, PDE11A, TMED7, TRAP1, SCO2, BMS1, MTA1, AIP, PDP1, ARTN, PHOX2B, MAML3, PDE8B, ZNRD2, CMAS, GPR68, DCTN6, NES, SDHAF3, RASSF1, ACACA, TRBV20OR9-2, HRAS, GUSB, GSK3B, GRN, GNA12, GDNF, GATA3, FOLH1, FGFR3, ENPEP, ENO2, ENG, EDNRA, TYMP, DNMT3A, DBH, CSF2RA, COL11A2, CHGB, CDKN1C, CD44, KRIT1, CALM3, CALM2, CALM1, BRAF, BCL2, AGRP, HPT, HTC2, SNAI1, IFI27, CX3CL1, RPS6KB1, RBP1, PTGS2, PSMD9, PARP1, PPP2R1B, POMC, PDGFRB, PDGFRA, PCNA, SLC22A18, NRTN, NNMT, NME1, MYO5B, MYC, MUC1, MKI67, MITF, MGMT, SMAD4, STMN1, KRAS, KIT, JAG2, IGH, H3P10

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A gangliocytic paraganglioma is a rare tumour that is typically found in the duodenum and consists of three components: (1) ganglion cells, (2) epithelioid cells (paraganglioma-like) and, (3) spindle cells (schwannoma-like).

Symptoms and signs

The most common presentation is gastrointestinal bleed (~45% of cases), followed by abdominal pain (~43% of cases) and anemia (~15% of cases).

Pathology

GP consist of three components (1) ganglion cells, (2) epithelioid cells (neuroendocrine-like), and (3) spindle cells (schwannoma-like). The microscopic differential diagnosis includes poorly differentiated carcinoma, neuroendocrine tumour and paraganglioma.

GPs may be sporadic or arise in the context neurofibromatosis type 1.

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Gangliocytic Paraganglioma

Symptoms and signs

Pathology

See also