ABSTRACT
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.
Subject(s)
Glottis/surgery , Hemangioendothelioma/genetics , Hemangioendothelioma/therapy , Kasabach-Merritt Syndrome/genetics , Kasabach-Merritt Syndrome/therapy , Laryngomalacia/genetics , Laryngomalacia/therapy , Laryngoplasty , Laser Therapy , Sarcoma, Kaposi/genetics , Sarcoma, Kaposi/therapy , Sirolimus/therapeutic use , TOR Serine-Threonine Kinases/genetics , Combined Modality Therapy , Hemangioendothelioma/diagnosis , Humans , Infant , Kasabach-Merritt Syndrome/diagnosis , Kasabach-Merritt Syndrome/surgery , Laryngomalacia/diagnosis , Male , Sarcoma, Kaposi/diagnosisABSTRACT
Although the glottis is amenable to chemotherapy, currently most lesions from stage I laryngeal dysplasia up to carcinoma in situ are excised. This literature review presents selected molecular biological aspects especially in relation to dysplasia of the larynx and its therapy, as well as currently preferred biomarkers for chemotherapeutic prevention of laryngeal dysplasia.
