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Impressive Response to Dose-Dense Chemotherapy in a Patient with NUT Midline Carcinoma.

Maur M, Toss A, Dominici M, Frassoldati A, Corradini P, Maiorana A, Fontana A, Conte P - Am J Case Rep (2015)

Bottom Line: In a few days, the patient developed a superior vena cava syndrome and an acute respiratory failure.In the meantime, the diagnosis of NMC was confirmed.Our case history adds some interesting findings to available knowledge: NMC can be chemosensitive and radiosensitive.

View Article: PubMed Central - PubMed

Affiliation: Division of Oncology, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia, Modena, Italy.

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.

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.

No MeSH data available.


Related in: MedlinePlus

The chest CT scan performed after the first course of Romidepsin showed disease progression to the pleural ring and in the subcutaneous areas, and nodal involvement.
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f5-amjcaserep-16-424: The chest CT scan performed after the first course of Romidepsin showed disease progression to the pleural ring and in the subcutaneous areas, and nodal involvement.

Mentions: Because of the rapid progression, based on pre-clinical data and after approval by the Local Ethics Committee, the patient stopped radiotherapy and entered into a compassionate use protocol with Romidepsin, a potent histone deacetylase (HDAC) inhibitor. The Local Ethics Committee approved the compassionate use also on the basis of some preliminary encouraging data obtained with another HDAC inhibitor (Vorinostat) (French C., personal communication at that time and now on ref. [15]). Therefore, the patient received weekly intravenous doses of Romidepsin (14 mg/mq) for 3 doses. No significant toxicities were observed, but a PET/CT scan after the first course showed disease progression to the pleural ring, in the subcutaneous areas, nodal involvement, peritoneal and retroperitoneal carcinosis, and new bone lesions (Figure 5). On the basis of the pattern of progression suggestive for mesothelioma-like behavior, an empirically based treatment with Pemetrexed (500 mg/mq every 3 weeks) was started. Unfortunately, the disease further progressed very rapidly and the patient eventually died after 1 course of Pemetrexed.


Impressive Response to Dose-Dense Chemotherapy in a Patient with NUT Midline Carcinoma.

Maur M, Toss A, Dominici M, Frassoldati A, Corradini P, Maiorana A, Fontana A, Conte P - Am J Case Rep (2015)

The chest CT scan performed after the first course of Romidepsin showed disease progression to the pleural ring and in the subcutaneous areas, and nodal involvement.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4492486&req=5

f5-amjcaserep-16-424: The chest CT scan performed after the first course of Romidepsin showed disease progression to the pleural ring and in the subcutaneous areas, and nodal involvement.
Mentions: Because of the rapid progression, based on pre-clinical data and after approval by the Local Ethics Committee, the patient stopped radiotherapy and entered into a compassionate use protocol with Romidepsin, a potent histone deacetylase (HDAC) inhibitor. The Local Ethics Committee approved the compassionate use also on the basis of some preliminary encouraging data obtained with another HDAC inhibitor (Vorinostat) (French C., personal communication at that time and now on ref. [15]). Therefore, the patient received weekly intravenous doses of Romidepsin (14 mg/mq) for 3 doses. No significant toxicities were observed, but a PET/CT scan after the first course showed disease progression to the pleural ring, in the subcutaneous areas, nodal involvement, peritoneal and retroperitoneal carcinosis, and new bone lesions (Figure 5). On the basis of the pattern of progression suggestive for mesothelioma-like behavior, an empirically based treatment with Pemetrexed (500 mg/mq every 3 weeks) was started. Unfortunately, the disease further progressed very rapidly and the patient eventually died after 1 course of Pemetrexed.

Bottom Line: In a few days, the patient developed a superior vena cava syndrome and an acute respiratory failure.In the meantime, the diagnosis of NMC was confirmed.Our case history adds some interesting findings to available knowledge: NMC can be chemosensitive and radiosensitive.

View Article: PubMed Central - PubMed

Affiliation: Division of Oncology, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia, Modena, Italy.

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.

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.

No MeSH data available.


Related in: MedlinePlus