| Desmoplastic small round cell tumor (DSRCT) is a rare yet potentially lethal tumor that has a predilection for adolescent males. It typically appears in the abdominal cavity, or involves other serosal surfaces including the testis, ovary, pleura, lung, pericardium, parotid gland. Cases of uncommon nonserosal DSRCT have also been reported which involved the hand, scalp, posterior fossa, ethmoid sinus and orbit. Clinical manifestations and radiographic examination usually contribute to the diagnosis of DSRCT, but its unique pathological profile remains to be the gold standard. As observed by histologic examinations, well-defined nests of small round blue tumor cells separated by abundant desmoplastic stroma are the striking morphologic features of DSRCT. The typical immunohistochemical profile is characterized by coexpression of epithelial, mesenchymal and neural markers. Cytogenetically, this tumor harbors a specific translocation involving the Ewing sarcoma gene and Wilms' tumor gene at t(11;22) (p13;q12). The prognosis are poor, combined modality treatments including surgical debulking, radiotherapy, and chemotherapy offer some improvements in patients' survival. Surgical resection is currently accepted to be of primary importance for achieving prolonged disease-free survival. Radiotherapy and alkylator-based multi-agent chemotherapy improve the resection rate and reduce the recurrence and metastasis. New molecular targeting drugs, endocrine therapy and autologous stem cell transplantation are on trial, and needto be further verified. A 21 years old girl with a complaint of reduced vision of her right eye was refered to our department. Her computed tomography (CT) scans showed opacification of the ethmoid sinuses, involving sphenoid sinuses, frontal lobe and orbit。 Biopsy of the lymph node in left neck revealed DSRCT. After 2 cycles of Ifosfamide and Teniposide, the patient's vision recovered to nomal. |
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