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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 first chest CT scan performed at the admission at the Division of Medical Oncology revealed a large (13.5×8 cm) right anterior upper mediastinal mass with extension into the right lobe.
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f1-amjcaserep-16-424: The first chest CT scan performed at the admission at the Division of Medical Oncology revealed a large (13.5×8 cm) right anterior upper mediastinal mass with extension into the right lobe.

Mentions: Following a rapid and progressive worsening of chest pain and the appearance of palpable supraclavicular and axillary lymph nodes, on day +7 from the first physical examination, the patient was admitted to our division. A total-body CT scan revealed a large (13.5×8 cm) right anterior upper mediastinal mass with extension into the right lobe, right lung nodules of less than 1.5 cm in size, multiple vertebral tumour infiltrates, and massive nodal involvement to the right supraclavicular, axillary, and mediastinal regions (Figure 1). Multiple focal areas of fluorodeoxyglucose uptake were documented in all the abnormal sites by positron emission tomography (PET). A core biopsy of a supraclavicular lymph node showed a poorly differentiated carcinoma with a high mitotic index.


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 first chest CT scan performed at the admission at the Division of Medical Oncology revealed a large (13.5×8 cm) right anterior upper mediastinal mass with extension into the right lobe.
© Copyright Policy
Related In: Results  -  Collection

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

f1-amjcaserep-16-424: The first chest CT scan performed at the admission at the Division of Medical Oncology revealed a large (13.5×8 cm) right anterior upper mediastinal mass with extension into the right lobe.
Mentions: Following a rapid and progressive worsening of chest pain and the appearance of palpable supraclavicular and axillary lymph nodes, on day +7 from the first physical examination, the patient was admitted to our division. A total-body CT scan revealed a large (13.5×8 cm) right anterior upper mediastinal mass with extension into the right lobe, right lung nodules of less than 1.5 cm in size, multiple vertebral tumour infiltrates, and massive nodal involvement to the right supraclavicular, axillary, and mediastinal regions (Figure 1). Multiple focal areas of fluorodeoxyglucose uptake were documented in all the abnormal sites by positron emission tomography (PET). A core biopsy of a supraclavicular lymph node showed a poorly differentiated carcinoma with a high mitotic index.

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