PMID- 36701777
OWN - NLM
STAT- MEDLINE
DCOM- 20230130
LR  - 20230202
IS  - 1512-0112 (Print)
IS  - 1512-0112 (Linking)
IP  - 332
DP  - 2022 Nov
TI  - RETROPERITONEAL PERIVASCULAR EPITHELIOID CELL NEOPLASM (PECOMA) RESPONSE TO MTOR 
      KINASE INHIBITION. A CASE REPORT WITH LITERATURE REVIEW.
PG  - 56-59
AB  - Perivascular epithelioid cell tumors (PEComas) are a very rare group of neoplasms 
      and were first reported in 1996. These tumors represent a family of mesenchymal 
      neoplasms, related through activation of the mammalian target of rapamycin (mTOR) 
      inhibitor signaling pathway. The objective of this case report is to demonstrate 
      significant regression of the tumor after neoadjuvant treatment with an oral mTOR 
      inhibitor, following surgical removal of the mass to avoid a multiorgan 
      resection. We present a case of a 27-year-old female with retroperitoneal PEComa 
      and evaluated the tumor with MRI and integrated 18F-FDG-PET/CT scans at 
      presentation and serially during treatment with everolimus. After 6 months of 
      treatment with everolimus the tumor showed a substantial size reduction. 
      Therefore, a multiorgan resection could be omitted. The patient has not 
      demonstrated any disease recurrence after nearly 2 years of follow-up. PEComas 
      are tumors with unpredictable behavior. Our report indicates that treatment of 
      PEComas with everolimus may achieve a significant clinical response. As indicated 
      by our case and past reports, mTOR inhibitors may be one of the best treatment 
      options for this disease.
FAU - Damirov, F
AU  - Damirov F
AD  - Department of Surgery, Division of Surgical Oncology and Thoracic Surgery, 
      University Medical Center Mannheim, Mannheim Medical Faculty, University of 
      Heidelberg, Mannheim, Germany.
FAU - Menge, F
AU  - Menge F
AD  - Department of Surgery, Division of Surgical Oncology and Thoracic Surgery, 
      University Medical Center Mannheim, Mannheim Medical Faculty, University of 
      Heidelberg, Mannheim, Germany.
FAU - Hohenberger, P
AU  - Hohenberger P
AD  - Department of Surgery, Division of Surgical Oncology and Thoracic Surgery, 
      University Medical Center Mannheim, Mannheim Medical Faculty, University of 
      Heidelberg, Mannheim, Germany.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - Georgia (Republic)
TA  - Georgian Med News
JT  - Georgian medical news
JID - 101218222
RN  - 9HW64Q8G6G (Everolimus)
RN  - EC 2.7.11.1 (TOR Serine-Threonine Kinases)
RN  - EC 2.7.1.1 (MTOR protein, human)
SB  - IM
MH  - Female
MH  - Humans
MH  - Adult
MH  - *Everolimus/therapeutic use
MH  - Positron Emission Tomography Computed Tomography
MH  - Neoplasm Recurrence, Local
MH  - *Perivascular Epithelioid Cell Neoplasms/diagnostic imaging/drug therapy
MH  - TOR Serine-Threonine Kinases/therapeutic use
EDAT- 2023/01/27 06:00
MHDA- 2023/01/31 06:00
CRDT- 2023/01/26 16:52
PHST- 2023/01/26 16:52 [entrez]
PHST- 2023/01/27 06:00 [pubmed]
PHST- 2023/01/31 06:00 [medline]
PST - ppublish
SO  - Georgian Med News. 2022 Nov;(332):56-59.