Impressive response to dose-dense chemotherapy in a patient with NUT midline carcinoma

Michela Maur, Angela Toss, Massimo Dominici, Antonio Frassoldati, Paolo Corradini, Antonio Maiorana, Annalisa Fontana, Pierfranco Conte

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: Rare disease 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. Case Report: 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.

Original languageEnglish
Article numberA093
Pages (from-to)424-429
Number of pages6
JournalAmerican Journal of Case Reports
Volume16
DOIs
Publication statusPublished - Jul 3 2015

Keywords

  • Antineoplastic agents
  • Carcinoma
  • Genetic
  • Mediastinal neoplasms
  • Translocation

ASJC Scopus subject areas

  • Medicine(all)

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