PMID- 24810750
OWN - NLM
STAT- MEDLINE
DCOM- 20150910
LR  - 20220309
IS  - 1439-3824 (Electronic)
IS  - 0300-8630 (Linking)
VI  - 226
IP  - 6-7
DP  - 2014 Nov
TI  - Laryngomalacia and complicated, life-threatening mTOR-positive Kaposiform 
      hemangioendothelioma cured by Supraglottoplasty and sirolimus.
PG  - 362-8
LID - 10.1055/s-0034-1372587 [doi]
AB  - The therapy of complicated Kaposiform hemangioendothelioma (KHE) is still 
      difficult. We present the first case of laryngomalacia with simultaneous 
      mammalian target of Rapamycin (mTOR)-positive KHE of the neck and thoracic inlet 
      and concurrent Kasabach-Meritt Phenomenon (KMP) in an 11-month-old boy suffering 
      life-threatening progress despite intravenous vincristine, corticosteroids, 
      propranolol and local interstitial laser-application. The laryngomalacia restored 
      after laser-supraglottoplasty. Successfully treatment of the prior fatal course 
      of the KHE with KMP was initiated not till adding the mTOR inhibitor sirolimus to 
      therapy. After 16 months single therapy of KHE with oral sirolimus the boy 
      presented free of symptoms with minimal residual disease and excellent functional 
      long-term results. Thus we stopped sirolimus therapy. The results are stable for 
      9 months without therapy. The special features including full report of 
      histopathologic findings of this utmost complicated case are demonstrated in 
      detail underlining the effectiveness of sirolimus for KHE.
CI  - (c) Georg Thieme Verlag KG Stuttgart . New York.
FAU - Schroeder, U
AU  - Schroeder U
AD  - ENT, University Hospital of Schleswig-Holstein, Luebeck, Germany.
FAU - Lauten, M
AU  - Lauten M
AD  - Department of Pediatric Hematology & Oncology, University Hospital of 
      Schleswig-Holstein, Luebeck, Germany.
FAU - Stichtenoth, G
AU  - Stichtenoth G
AD  - Department of Pediatric Hematology & Oncology, University Hospital of 
      Schleswig-Holstein, Luebeck, Germany.
FAU - Gebhard, M P E
AU  - Gebhard MP
AD  - Department of Pathology, University Hospital of Schleswig-Holstein, -Luebeck, 
      Germany.
FAU - Buchholz, M
AU  - Buchholz M
AD  - Department of Radiology, University Hospital of Schleswig-Holstein, -Luebeck, 
      Germany.
FAU - Kaiser, M M
AU  - Kaiser MM
AD  - Department of Pediatric Surgery, University Hospital of Schleswig-Holstein, 
      Luebeck, Germany.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20140508
PL  - Germany
TA  - Klin Padiatr
JT  - Klinische Padiatrie
JID - 0326144
RN  - EC 2.7.1.1 (MTOR protein, human)
RN  - EC 2.7.11.1 (TOR Serine-Threonine Kinases)
RN  - W36ZG6FT64 (Sirolimus)
RN  - Kaposiform Hemangioendothelioma
SB  - IM
MH  - Combined Modality Therapy
MH  - Glottis/*surgery
MH  - Hemangioendothelioma/diagnosis/*genetics/*therapy
MH  - Humans
MH  - Infant
MH  - Kasabach-Merritt Syndrome/diagnosis/*genetics/surgery/*therapy
MH  - Laryngomalacia/diagnosis/*genetics/*therapy
MH  - *Laryngoplasty
MH  - *Laser Therapy
MH  - Male
MH  - Sarcoma, Kaposi/diagnosis/*genetics/*therapy
MH  - Sirolimus/*therapeutic use
MH  - TOR Serine-Threonine Kinases/*genetics
EDAT- 2014/05/09 06:00
MHDA- 2015/09/12 06:00
CRDT- 2014/05/10 06:00
PHST- 2014/05/10 06:00 [entrez]
PHST- 2014/05/09 06:00 [pubmed]
PHST- 2015/09/12 06:00 [medline]
AID - 10.1055/s-0034-1372587 [doi]
PST - ppublish
SO  - Klin Padiatr. 2014 Nov;226(6-7):362-8. doi: 10.1055/s-0034-1372587. Epub 2014 May 
      8.