Key result
In a 51-year-old male with a rare primary mediastinal desmoplastic small round cell tumor, treatment with chemotherapy and radiotherapy resulted in a total survival period of 17 months.
Why the study?
Desmoplastic small round cell tumor is a rare distinct tumor with high-grade malignancy.
Case Report (n=1)
Primary mediastinal desmoplastic small round cell tumor is an extremely rare, highly malignant tumor where comprehensive therapy including chemotherapy and radiotherapy may achieve partial remission and prolong survival.
May support chemoradiation for survival in ultra-rare mediastinal DSRCT; hypothesis-generating and should not change practice.
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.
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Jin et al. (2020) conducted a case report in Mediastinal desmoplastic small round cell tumor (n=1). Chemotherapy and radiotherapy was evaluated on Total survival period. In a 51-year-old male with a rare primary mediastinal desmoplastic small round cell tumor, treatment with chemotherapy and radiotherapy resulted in a total survival period of 17 months.
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