PMID- 38200366
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20240113
IS  - 2198-7793 (Print)
IS  - 2198-7793 (Electronic)
IS  - 2198-7793 (Linking)
VI  - 10
IP  - 1
DP  - 2024 Jan 11
TI  - Familial multiple endocrine neoplasia type 1 with intrathoracic low-grade 
      fibromyxoid sarcoma.
PG  - 16
LID - 10.1186/s40792-024-01809-w [doi]
LID - 16
AB  - BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary tumor 
      syndrome characterized by endocrine tumors with mainly a parathyroid, pancreatic, 
      or anterior pituitary origin. Low-grade fibromyxoid sarcoma (LGFMS) is a rare 
      low-grade soft tissue tumor. There is one known report of a patient with MEN1 
      complicated by LGFMS, which is very rare. Our report represents the second 
      documented case, providing valuable insights. CASE PRESENTATION: A 31-year-old 
      man with the chief complaint of a cough underwent chest contrast-enhanced 
      computed tomography, which revealed a giant hypoabsorptive tumor with a maximum 
      diameter of 23 cm in the left thoracic cavity. The patient was diagnosed with 
      MEN1, as he also possessed a pancreatic neuroendocrine tumor and parathyroid 
      tumor, and because his father had been found to have MEN1. To control 
      hypercalcemia, surgery for the parathyroid tumor was initially performed, 
      followed by surgical resection of the giant thoracic tumor for diagnosis and 
      treatment. Histopathological examination findings of the tumor resulted in a 
      diagnosis of LGFMS. CONCLUSION: We experienced a very rare MEN1 with LGFMS. 
      Although endocrine tumors generally occur more frequently in MEN1, non-endocrine 
      tumors such as the present case should also be noted, reinforcing the importance 
      of systemic imaging scrutiny in addition to early diagnosis and long-term 
      follow-up of MEN1 patients.
CI  - (c) 2024. The Author(s).
FAU - Ishida, Hiroto
AU  - Ishida H
AD  - Department of General Thoracic Surgery, Osaka University Graduate School of 
      Medicine, 2-2, Yamadaoka, Suita-City, Osaka, 565-0871, Japan.
FAU - Funaki, Soichiro
AU  - Funaki S
AUID- ORCID: 0000-0003-2386-4736
AD  - Department of General Thoracic Surgery, Osaka University Graduate School of 
      Medicine, 2-2, Yamadaoka, Suita-City, Osaka, 565-0871, Japan. 
      funaki@thoracic.med.osaka-u.ac.jp.
FAU - Taniguchi, Seiji
AU  - Taniguchi S
AD  - Department of Thoracic Surgery, Osaka Habikino Medical Center, 3-7-1, Habikino, 
      Habikino-City, Osaka, 583-8588, Japan.
FAU - Morii, Eiichi
AU  - Morii E
AD  - Department of Pathology, Osaka University Graduate School of Medicine, 2-2, 
      Yamadaoka, Suita-City, Osaka, 565-0871, Japan.
FAU - Shintani, Yasushi
AU  - Shintani Y
AD  - Department of General Thoracic Surgery, Osaka University Graduate School of 
      Medicine, 2-2, Yamadaoka, Suita-City, Osaka, 565-0871, Japan.
LA  - eng
PT  - Journal Article
DEP - 20240111
PL  - Germany
TA  - Surg Case Rep
JT  - Surgical case reports
JID - 101662125
PMC - PMC10781903
OTO - NOTNLM
OT  - Low-grade fibromyxoid sarcoma
OT  - Multiple endocrine neoplasia type 1
OT  - Thoracic tumor
COIS- The authors declare that they have no competing interests.
EDAT- 2024/01/11 00:42
MHDA- 2024/01/11 00:43
PMCR- 2024/01/11
CRDT- 2024/01/10 23:31
PHST- 2023/11/16 00:00 [received]
PHST- 2024/01/03 00:00 [accepted]
PHST- 2024/01/11 00:43 [medline]
PHST- 2024/01/11 00:42 [pubmed]
PHST- 2024/01/10 23:31 [entrez]
PHST- 2024/01/11 00:00 [pmc-release]
AID - 10.1186/s40792-024-01809-w [pii]
AID - 1809 [pii]
AID - 10.1186/s40792-024-01809-w [doi]
PST - epublish
SO  - Surg Case Rep. 2024 Jan 11;10(1):16. doi: 10.1186/s40792-024-01809-w.