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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 at the end of the dose-dense chemotherapy showed a very good partial response, with a reduction of the mediastinal bulk (2.6×1.8 cm).
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f3-amjcaserep-16-424: The chest CT scan performed at the end of the dose-dense chemotherapy showed a very good partial response, with a reduction of the mediastinal bulk (2.6×1.8 cm).

Mentions: Surprisingly, a relevant clinical benefit was obtained after the first cycle of chemotherapy, with a progressive disappearance of palpable nodes and an improvement of all symptoms. The patient received a total of 6 dose-dense treatments with an expected hematological toxicity. At the end of chemotherapy, a total-body PET-CT scan showed a very good partial response with a reduction of the mediastinal bulk (2.6×1.8 cm), a complete disappearance of lung lesions and pleural effusion, and a significant reduction of pathological nodes (Figure 3). The superior mediastinal residual disease had a moderate dyshomogeneous fluorodeoxyglucose uptake.


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 at the end of the dose-dense chemotherapy showed a very good partial response, with a reduction of the mediastinal bulk (2.6×1.8 cm).
© Copyright Policy
Related In: Results  -  Collection

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

f3-amjcaserep-16-424: The chest CT scan performed at the end of the dose-dense chemotherapy showed a very good partial response, with a reduction of the mediastinal bulk (2.6×1.8 cm).
Mentions: Surprisingly, a relevant clinical benefit was obtained after the first cycle of chemotherapy, with a progressive disappearance of palpable nodes and an improvement of all symptoms. The patient received a total of 6 dose-dense treatments with an expected hematological toxicity. At the end of chemotherapy, a total-body PET-CT scan showed a very good partial response with a reduction of the mediastinal bulk (2.6×1.8 cm), a complete disappearance of lung lesions and pleural effusion, and a significant reduction of pathological nodes (Figure 3). The superior mediastinal residual disease had a moderate dyshomogeneous fluorodeoxyglucose uptake.

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