PMID- 31118348
OWN - NLM
STAT- MEDLINE
DCOM- 20200203
LR  - 20200203
IS  - 1348-4540 (Electronic)
IS  - 0918-8959 (Linking)
VI  - 66
IP  - 8
DP  - 2019 Aug 29
TI  - A novel case of myxoid variant of adrenocortical carcinoma in a patient with 
      multiple endocrine neoplasia type 1.
PG  - 739-744
LID - 10.1507/endocrj.EJ19-0067 [doi]
AB  - Adrenocortical carcinoma (ACC) is a rare malignancy arising from adrenocortical 
      parenchymal cells. Myxoid ACC is one of the newly identified, rare, but important 
      histological variants of ACC, characterized by the presence of abundant 
      extracellular Alcian Blue-positive myxoid material. Multiple endocrine neoplasia 
      type 1 (MEN1) is an autosomal dominant cancer predisposition syndrome, and the 
      incidence of ACC in MEN1 patients has been reported to be between 1.4% and 6%. 
      Here, we report the case of a 68-year-old Japanese woman harboring the past 
      history of MEN1 associated with insulinoma, pituitary tumor, and 
      hyperparathyroidism. She presented to our hospital with hypertension and 
      hypokalemia. Imaging studies revealed a right adrenal tumor, and histological 
      examination revealed myxoid ACC. Despite surgical resection of the tumor and 
      mitotane therapy, the patient died 6 months after the surgery. To the best of our 
      knowledge, this is the first reported case of the myxoid variant of ACC in a 
      patient with MEN1. The patient's clinical course was characterized by the 
      development of both multiple endocrine and non-endocrine neoplasm, 
      hyperaldosteronism, and aggressive biological behavior. This case confirmed that 
      myxoid morphology was also associated with aggressive behavior in ACC, but 
      further studies are required to clarify the association between MEN1 and myxoid 
      ACC.
FAU - Harada, Ko
AU  - Harada K
AD  - Department of General Medicine, Okayama University Graduate School of Medicine, 
      Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan.
FAU - Yasuda, Miho
AU  - Yasuda M
AD  - Department of General Medicine, Okayama University Graduate School of Medicine, 
      Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan.
FAU - Hasegawa, Kou
AU  - Hasegawa K
AD  - Department of General Medicine, Okayama University Graduate School of Medicine, 
      Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan.
FAU - Yamazaki, Yuto
AU  - Yamazaki Y
AD  - Department of Anatomic Pathology, Tohoku University Graduate School of Medicine, 
      Miyagi 980-8575, Japan.
FAU - Sasano, Hironobu
AU  - Sasano H
AD  - Department of Anatomic Pathology, Tohoku University Graduate School of Medicine, 
      Miyagi 980-8575, Japan.
FAU - Otsuka, Fumio
AU  - Otsuka F
AD  - Department of General Medicine, Okayama University Graduate School of Medicine, 
      Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20190522
PL  - Japan
TA  - Endocr J
JT  - Endocrine journal
JID - 9313485
SB  - IM
MH  - Adrenal Cortex Neoplasms/etiology/*pathology
MH  - Adrenocortical Carcinoma/etiology/*pathology
MH  - Aged
MH  - Female
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/complications/*pathology
MH  - Myxoma/complications/*pathology
OTO - NOTNLM
OT  - Adrenocortical carcinoma
OT  - Multiple endocrine neoplasia type 1
OT  - Myxoid variant
EDAT- 2019/05/24 06:00
MHDA- 2020/02/06 06:00
CRDT- 2019/05/24 06:00
PHST- 2019/05/24 06:00 [pubmed]
PHST- 2020/02/06 06:00 [medline]
PHST- 2019/05/24 06:00 [entrez]
AID - 10.1507/endocrj.EJ19-0067 [doi]
PST - ppublish
SO  - Endocr J. 2019 Aug 29;66(8):739-744. doi: 10.1507/endocrj.EJ19-0067. Epub 2019 
      May 22.