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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.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.

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 a few weeks after the end of radiotherapy on the initial mediastinal tumour area documented disease progression with right pleural effusion while the initial mediastinal mass was still decreasing in size.
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f4-amjcaserep-16-424: The chest CT scan performed a few weeks after the end of radiotherapy on the initial mediastinal tumour area documented disease progression with right pleural effusion while the initial mediastinal mass was still decreasing in size.

Mentions: Thirty-five days after the last cycle of chemotherapy, the patient started a radiation treatment targeting the initial mediastinal tumour area with a dose of 2 Gy/daily. Unfortunately, after 18 days and a total dose administered of 28 Gy, the patient was admitted to the hospital with worsening chest pain and dyspnea. A new CT scan documented disease progression mainly outside of the radiation field with right pleural effusion, peritoneal and retroperitoneal involvement, increase of bone lesions, and multiple subcutaneous nodules, but the initial mediastinal mass was still decreasing in size (Figure 4). At that time, considering that the pleural effusion was unilateral and the disease was progressing in several sites, we considered the fluid as a malignant pleural effusion, thus a diagnostic thoracentesis was not performed.


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 a few weeks after the end of radiotherapy on the initial mediastinal tumour area documented disease progression with right pleural effusion while the initial mediastinal mass was still decreasing in size.
© Copyright Policy
Related In: Results  -  Collection

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

f4-amjcaserep-16-424: The chest CT scan performed a few weeks after the end of radiotherapy on the initial mediastinal tumour area documented disease progression with right pleural effusion while the initial mediastinal mass was still decreasing in size.
Mentions: Thirty-five days after the last cycle of chemotherapy, the patient started a radiation treatment targeting the initial mediastinal tumour area with a dose of 2 Gy/daily. Unfortunately, after 18 days and a total dose administered of 28 Gy, the patient was admitted to the hospital with worsening chest pain and dyspnea. A new CT scan documented disease progression mainly outside of the radiation field with right pleural effusion, peritoneal and retroperitoneal involvement, increase of bone lesions, and multiple subcutaneous nodules, but the initial mediastinal mass was still decreasing in size (Figure 4). At that time, considering that the pleural effusion was unilateral and the disease was progressing in several sites, we considered the fluid as a malignant pleural effusion, thus a diagnostic thoracentesis was not performed.

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.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.

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