Successful treatment with doxorubicin and ifosfamide for mediastinal malignant peripheral nerve sheath tumor with loss of H3K27me3 expression
| Autor: | Daisuke Gomi, Nodoka Sekiguchi, Yoshiko Tsukahara, Takesumi Ozawa, Takashi Kobayashi, Kenji Sano, Noriko Seno, Tomonobu Koizumi, Hidehiro Matsushita, Toshirou Fukushima, Keiko Mamiya |
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| Rok vydání: | 2017 |
| Předmět: |
Pulmonary and Respiratory Medicine
Neurofibromatosis type I Thorax Pathology medicine.medical_specialty Ifosfamide business.industry Mediastinum Mediastinal tumor Malignant peripheral nerve sheath tumor General Medicine medicine.disease 03 medical and health sciences 0302 clinical medicine medicine.anatomical_structure Oncology 030220 oncology & carcinogenesis medicine 030212 general & internal medicine Differential diagnosis business medicine.drug Rare disease |
| Zdroj: | Thoracic Cancer. 8:720-723 |
| ISSN: | 1759-7706 |
| DOI: | 10.1111/1759-7714.12498 |
| Popis: | Malignant peripheral nerve sheath tumor (MPNST) in the thorax is an extremely rare disease, and half of all cases of MPNST are associated with neurofibromatosis type I. Sporadic intrathoracic MPNST is difficult to diagnose and treat. Because of the rarity of intrathoracic MPNST, the optimal method of diagnosis and the efficacy of chemotherapy are unknown. Herein, we present a case of inoperable mediastinal MPNST, in which the diagnosis was immunohistochemically made by the loss of H3K27me3 expression in a transbronchial needle biopsy specimen. The patient showed a good response to doxorubicin plus ifosfamide chemotherapy. The present case highlights that MPNST should be included in the differential diagnosis of non-posterior mediastinum thoracic lesions, and that appropriate diagnosis and treatment for intrathoracic MPNST should be considered in patients with a thoracic mass. |
| Databáze: | OpenAIRE |
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