PMID- 36654999
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230120
IS  - 2376-0605 (Electronic)
IS  - 2376-0605 (Linking)
VI  - 9
IP  - 1
DP  - 2023 Jan-Feb
TI  - Adrenal Liposarcoma: A Novel Presentation of Multiple Endocrine Neoplasia Type 1.
PG  - 10-12
LID - 10.1016/j.aace.2022.11.003 [doi]
AB  - BACKGROUND/OBJECTIVE: Multiple endocrine neoplasia type 1 (MEN1) syndrome results 
      from genetic sequence variations of the tumor suppressor MEN1 gene, which codes 
      for the protein menin. Individuals with MEN1 are prone to developing multiple 
      tumors involving the endocrine and nonendocrine organs. MEN1 associated with 
      liposarcomas has not been documented previously. We highlight a case of MEN1 
      presenting with a metastatic adrenal liposarcoma. CASE REPORT: A 41-year-old 
      Hispanic man with a history of nephrolithiasis and skin lesions presented to the 
      emergency department with abdominal pain. He was found to have a right adrenal 
      mass measuring 7.9 cm with extension into the liver and primary 
      hyperparathyroidism. He had multiple paternal first-degree relatives with similar 
      skin lesions, hypercalcemia, and tumors of the brain, thoracic cavity, abdomen, 
      and thyroid. The mass was identified as a metastatic pleiomorphic adrenal 
      liposarcoma on surgical pathology. Genetic testing revealed a germline pathogenic 
      sequence variation of the MEN1 gene. DISCUSSION: Liposarcomas are rare malignant 
      tumors with an annual incidence of 2.5 cases per 1 million. Although lipoma 
      formation is a commonly described manifestation of MEN1, liposarcomas have not 
      been associated with MEN1 previously. A potential mechanism of this association 
      is through the role of menin in inducing adipocyte differentiation via peroxisome 
      proliferator-activated receptor-gamma activation, a highly expressed protein in 
      liposarcomas. CONCLUSION: Liposarcomas should be included in the differential of 
      MEN1-related tumors.
CI  - (c) 2022 Published by Elsevier Inc. on behalf of the AACE.
FAU - Duro, Teodor
AU  - Duro T
AD  - Department of Endocrinology, University of New Mexico Health Sciences Center, 
      Albuquerque, New Mexico.
FAU - Gonzales, Kristen L
AU  - Gonzales KL
AD  - Department of Endocrinology, University of New Mexico Health Sciences Center, 
      Albuquerque, New Mexico.
LA  - eng
PT  - Case Reports
DEP - 20221113
PL  - United States
TA  - AACE Clin Case Rep
JT  - AACE clinical case reports
JID - 101670593
PMC - PMC9837086
OTO - NOTNLM
OT  - MEN1
OT  - MEN1, multiple endocrine neoplasia type 1
OT  - PPAR-gamma, peroxisome proliferator-activated receptor-gamma
OT  - adrenal tumor
OT  - liposarcoma
EDAT- 2023/01/20 06:00
MHDA- 2023/01/20 06:01
PMCR- 2022/11/13
CRDT- 2023/01/19 02:00
PHST- 2022/08/27 00:00 [received]
PHST- 2022/10/28 00:00 [revised]
PHST- 2022/11/08 00:00 [accepted]
PHST- 2023/01/19 02:00 [entrez]
PHST- 2023/01/20 06:00 [pubmed]
PHST- 2023/01/20 06:01 [medline]
PHST- 2022/11/13 00:00 [pmc-release]
AID - S2376-0605(22)00081-5 [pii]
AID - 10.1016/j.aace.2022.11.003 [doi]
PST - epublish
SO  - AACE Clin Case Rep. 2022 Nov 13;9(1):10-12. doi: 10.1016/j.aace.2022.11.003. 
      eCollection 2023 Jan-Feb.