PMID- 20403035
OWN - NLM
STAT- MEDLINE
DCOM- 20100715
LR  - 20131121
IS  - 1440-1827 (Electronic)
IS  - 1320-5463 (Linking)
VI  - 60
IP  - 4
DP  - 2010 Apr
TI  - Multiple endocrine neoplasia type 1-associated cystic pancreatic endocrine 
      neoplasia and multifocal cholesterol granulomas.
PG  - 321-5
LID - 10.1111/j.1440-1827.2010.02516.x [doi]
AB  - A novel combination of tumors was found in a 68 year-old female with Multiple 
      Endocrine Neoplasia type-1 (MEN 1) that included a cystic pancreatic endocrine 
      neoplasm (CPEN), a pituitary adenoma, and multifocal cholesterol granulomas 
      (MCGs) in the breast, pleura, and the extremities. The pancreatic tumor displayed 
      a single central locule surrounded by a thin rim of neoplastic parenchyma. The 
      tumor showed heterogeneity in the architecture that included glandular, 
      trabecular and solid patterns. The tumor cells of the pancreas were 
      immunohistochemically positive for both endocrine and pancreatic acinar markers 
      including chromogranin A, synaptophysin, glucagon, lipase, and reg protein. 
      Electron microscopy revealed that there were numerous smaller dense-cored 
      neurosecretory granules, larger zymogen-like granules and microvilli on the 
      apical side of the tumor cells. The pancreatic tumor was diagnosed as CPEN with 
      acinar cell features. Analysis of the DNA extracted from the tissues revealed 
      that there is a MEN1 germline mutation in exon 10 codon 527, and somatic mutation 
      in exon 2 codon 32 in the pancreatic tumor, and one base pair deletion in exon 2 
      codon 79 in the pituitary adenoma. Here, we report the case and discuss possible 
      pathogenesis of CPEN and MCGs in a patient with MEN 1.
FAU - Kimura, Noriko
AU  - Kimura N
AD  - Department of Pathology, Japan National Hospital Organization, Hakodate Hospital, 
      Hakodate, Japan. kimura-path@hnh.hosp.go.jp
FAU - Komuro, Kazuteru
AU  - Komuro K
FAU - Uchino, Shinya
AU  - Uchino S
FAU - Yagihashi, Soroku
AU  - Yagihashi S
FAU - Ishidate, Takuzo
AU  - Ishidate T
FAU - Ishizaka, Masanori
AU  - Ishizaka M
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Australia
TA  - Pathol Int
JT  - Pathology international
JID - 9431380
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
RN  - 97C5T2UQ7J (Cholesterol)
SB  - IM
MH  - Adenoma/genetics/*pathology
MH  - Aged
MH  - Breast Neoplasms/genetics/*pathology
MH  - *Cholesterol
MH  - Female
MH  - Granuloma, Foreign-Body/genetics/*pathology
MH  - Humans
MH  - Immunohistochemistry
MH  - Multiple Endocrine Neoplasia Type 1/genetics/*pathology
MH  - Mutation
MH  - Pancreas/*pathology
MH  - Pancreatic Neoplasms/genetics/*pathology
MH  - Pituitary Neoplasms/genetics/*pathology
MH  - Proto-Oncogene Proteins/genetics
MH  - Reverse Transcriptase Polymerase Chain Reaction
EDAT- 2010/04/21 06:00
MHDA- 2010/07/16 06:00
CRDT- 2010/04/21 06:00
PHST- 2010/04/21 06:00 [entrez]
PHST- 2010/04/21 06:00 [pubmed]
PHST- 2010/07/16 06:00 [medline]
AID - PIN2516 [pii]
AID - 10.1111/j.1440-1827.2010.02516.x [doi]
PST - ppublish
SO  - Pathol Int. 2010 Apr;60(4):321-5. doi: 10.1111/j.1440-1827.2010.02516.x.