title: Impressive Response to Dose-Dense Chemotherapy in a Patient with NUT Midline Carcinoma.
abstract: BACKGROUND
  NUT midline carcinoma (NMC) is a rare, highly lethal malignancy that results from a chromosome translocation and mostly arises in the midline organs. To date, no treatment has been established. Most patients receive combinations of chemotherapy regimens and radiation, and occasionally subsequent resection; nevertheless, patients have an average survival hardly exceeding 7 months.
  
  
  METHODS
  A 21-year-old patient was admitted to our division with a large mediastinal mass with lung nodules, multiple vertebral metastases, and massive nodal involvement. In a few days, the patient developed a superior vena cava syndrome and an acute respiratory failure. Due to the rapid course of the disease, based on preliminary histology of poorly differentiated carcinoma, a dose-dense biweekly chemotherapy with paclitaxel, ifosfamide, and cisplatin was started. In the meantime, the diagnosis of NMC was confirmed. A surprising clinical benefit was obtained after the first cycle of chemotherapy, and after 6 cycles a PET-CT scan showed a very good response. At this point, radiotherapy was started but the disease progressed outside of the radiation field. The patient entered into a compassionate use protocol with Romidepsin, but a PET/CT scan after the first course showed disease progression with peritoneal and retroperitoneal carcinosis. A treatment with Pemetrexed was then started but the patient eventually died with rapid progressive disease.
  
  
  CONCLUSIONS
  Our case history adds some interesting findings to available knowledge: NMC can be chemosensitive and radiosensitive. This opens the possibility to study more aggressive treatments, including high-dose consolidation chemotherapy and to evaluate the role of biological agents as maintenance treatments.
authors.affiliation: Division of Oncology, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Michela
authors.initials: M
authors.lastname: Maur
authors.affiliation: Division of Oncology, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Toss
authors.affiliation: Division of Oncology, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Dominici
authors.affiliation: Division of Oncology, S. Anna Hospital, Ferrara, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Frassoldati
authors.affiliation: Division of Respiratory Diseases, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Corradini
authors.affiliation: Division of Diagnostic Medicine, Clinic and Public Health, University Hospital of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Maiorana
authors.affiliation: Division of Oncology, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Fontana
authors.affiliation: Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy.
authors.forename: Pierfranco
authors.initials: P
authors.lastname: Conte
mesh_terms: D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage; D001706:Biopsy; D002294:Carcinoma, Squamous Cell / Q000175:diagnosis / Q000188:drug therapy*; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D006801:Humans; D008297:Male; D008479:Mediastinal Neoplasms / Q000175:diagnosis / Q000188:drug therapy*; D017239:Paclitaxel / Q000008:administration & dosage*; D049268:Positron-Emission Tomography; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: D000972:Antineoplastic Agents, Phytogenic; D017239:Paclitaxel