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Mediastinal desmoplastic small round cell tumor
Dacheng Jin1, Meng Chen1, Bing Wang1,2
1The first department of thoracic Surgery, Gansu Provincial Hospital.
Rationale:
Desmoplastic small round cell tumor (DSRCT) is a rare distinct tumor with a high-grade malignancy.
Patient Concerns:
A 51-year-old male visited a local hospital in April 2016 complaining of shortness of breath, chest tightness and pain, and exhibited significant swelling in both sides of the chest.
Diagnoses:
CT demonstrated thoracic symmetry and no abnormalities were observed in the soft tissues of the ribs and the chest wall. A general observation of CT-guided puncture biopsy revealed 2 stripes of gray and grayish-white puncture tissues of 0.5 and 1 cm in length, respectively, and 0.1 cm in diameter. These results preliminarily suggested a (mediastinum) malignant small round cell tumor.
Intervention:
Given the progression of the disease, the chemotherapy regimen, consisting of ifosfamide and etoposide, was altered during the course and radiotherapy (total of 70 Gy of mediastinal Y field radiation) was conducted.
Outcomes:
The patient and his family declined further treatment. Through follow-up, the total survival period was determined as 17 months.
Lessons:
DSRCT is a rare interstitial malignant tumor. Effective cytoreduction combined with comprehensive therapies could achieve partial remission or prolong the survival of patients.
