KEGG   DISEASE: Primary peritoneal carcinomaHelp
Entry
H01665                      Disease                                

Name
Primary peritoneal carcinoma
Description
Female primary peritoneal carcinoma (PPC) has the characteristic of diffuse involvement of the peritoneum by papillary carcinoma in the absence of an obvious primary site and grossly normal ovaries, or minimal involvement. It is histologically indistinguishable from primary epithelial ovarian carcinoma (EOC) and is diagnosed in the absence of another identifiable primary. Most reported PPC cases are primary peritoneal serous papillary carcinoma (PPSPC), while peritoneal mixed epithelial carcinoma (MEC) and malignant mixed Mullerian tumor (MMMT) are rarely reported. Germline BRCA1 mutations in 26% of PPSPC patients was detected. BRCA1 mutation carriers had a higher overall incidence of p53 mutations than wildtype BRCA1 case subjects. It is suggested that p53 has a unique role in the development and progression of BRCA1-related PPSPC.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of soft tissues and bone
   H01665  Primary peritoneal carcinoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant neoplasms of retroperitoneum, peritoneum or omentum
     2C51  Malignant neoplasms of peritoneum
      H01665  Primary peritoneal carcinoma
BRITE hierarchy
Gene
BRCA1 (mutation) [HSA:672] [KO:K10605]
Drug
Bevacizumab [DR:D06409]
Niraparib [DR:D10140]
Comment
ICD-O-3: 8461/3 Serous surface papillary carcinoma (C56.9)
Other DBs
ICD-11: 2C51
ICD-10: C48.1
MeSH: D010534
Reference
  Authors
Cormio G, Di Vagno G, Di Gesu G, Mastroianni M, Melilli GA, Vimercati A, Putignano G, Loverro G, Selvaggi L
  Title
Primary peritoneal carcinoma: a report of twelve cases and a review of the literature.
  Journal
Gynecol Obstet Invest 50:203-6 (2000)
DOI:10.1159/000010311
Reference
  Authors
Pentheroudakis G, Pavlidis N
  Title
Serous papillary peritoneal carcinoma: unknown primary tumour, ovarian cancer counterpart or a distinct entity? A systematic review.
  Journal
Crit Rev Oncol Hematol 75:27-42 (2010)
DOI:10.1016/j.critrevonc.2009.10.003
Reference
  Authors
Schorge JO, Muto MG, Lee SJ, Huang LW, Welch WR, Bell DA, Keung EZ, Berkowitz RS, Mok SC
  Title
BRCA1-related papillary serous carcinoma of the peritoneum has a unique molecular pathogenesis.
  Journal
Cancer Res 60:1361-4 (2000)
Reference
PMID:21352070 (drug)
  Authors
Slomovitz BM, Worley MJ, Markman M, Coleman RL
  Title
Emerging therapeutics for primary peritoneal cancer.
  Journal
Expert Opin Emerg Drugs 16:71-84 (2011)
DOI:10.1517/14728214.2011.526600
Reference
PMID:18543395 (drug, ICD-O-3)
  Authors
Zhang C, Li XP, Cui H, Shen DH, Wei LH
  Title
Advanced primary peritoneal carcinoma: clinicopathological and prognostic factor analyses.
  Journal
J Zhejiang Univ Sci B 9:435-40 (2008)
DOI:10.1631/jzus.B0820051
Reference
PMID:25315029 (drug)
  Authors
Keating GM
  Title
Bevacizumab: a review of its use in advanced cancer.
  Journal
Drugs 74:1891-925 (2014)
DOI:10.1007/s40265-014-0302-9

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