A 13-year-old boy was admitted to the hospital with 1-month history of neck pain and a 2-week history of bilateral hip joint pain accompanied by low fever. Positron emission tomography-computed tomography (PET-CT) revealed the presence of a malignant tumor in the left kidney with metastases to the left renal hilum, retroperitoneum, para-aortic lymph nodes, and multiple bone sites throughout the body. Given that the patient\'s left kidney capsule was intact and the boundary with surrounding tissues was clear, left nephrectomy was performed. Postoperative pathological diagnosis showed desmoplastic small round cell tumor (DSRCT) of the left kidney. CAV-VIP alternating chemotherapy was given 20 days after the first stage surgery. After the end of the 6th cycle, the patient underwent surgery again. The tumor in front of the aorta and postcava, the greater omentum, the retroperitoneal lymph nodes and the hepatic hilum lymph nodes, and the visible tumors in the abdomen were removed. CAV-VIP alternating chemotherapy was continued after the second stage surgery. At the end of the 4th cycle of post operation chemotherapy, radiotherapy was started. An abdominal CT scan conducted 11 months after second-stage surgery did not reveal any recurrence of abdominal tumors; however bone metastases persisted. The patient is currently receiving oral targeted therapy with anlotinib while ongoing follow-up continues.

Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy predominantly affecting adolescents and young adults. We report a case of multifocal DSRCT in an 11-year-old male who presented with complaints of unilateral forehead swelling, proptosis, and ophthalmoplegia for four months along with abdominal pain and dysphagia for six months. A whole-body computed tomography revealed widespread lesions in the skull, orbit, thorax, and abdomen with local infiltration. Ultrasound-guided biopsy of the forehead lump was performed. Based on histopathological and immunohistochemical investigations, it was diagnosed to be a DSRCT with multifocal presentation. The patient underwent chemo-radiation but unfortunately succumbed to neutropenic sepsis and renal failure. DSRCT is a very rare, highly aggressive malignancy with an extremely poor prognosis. Orbital presentations are even rarer, with less than 10 such cases currently described in English medical literature.

UNASSIGNED: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, mesenchymal malignancy of a separate clinicopathological entity. It has a predilection for young men, with no evidence of any ethnic predilection. The current diagnostic gold standard for DSRCT includes histopathologic, immunohistochemical, and cytogenetic studies in order to confirm the variable phenotypic expression and characteristic chromosomal translocation.
UNASSIGNED: A 65-year-old man presented with a sensation of an abdominal mass and a presentation of an incomplete bowel obstruction. Initial lab tests were in the normal range except for carbohydrate antigen. Contrast-enhanced CT showed that a large, mass-confounding density was occupied in the omentum majus area of the middle and lower abdominal wall. A 3D reconstruction of the images was performed to clarify the relationship between the tumor and the colon and was confirmed by a colonoscopy. After surgery, immunohistochemistry and fluorescence in situ hybridization (FISH) revealed EWSR1-WT1 gene rearrangement at 22q12, confirming the diagnosis of desmoplastic small round cell tumor.
UNASSIGNED: Being different from the predilection of DSRCT for young men, the patient in our case is a 65-year-old man with a huge mass involving the transverse colon and the bladder.

Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor that occurs mainly in the retroperitoneum of children and young adults. In its prototypical form, DSCRT displays characteristic morphology with nested primitive small round cells in a desmoplastic stroma and a distinctive immunophenotype with polyphenotypic differentiation. However, DSCRT can also exhibit a broader clinical, histologic and immunohistochemical spectrum and, therefore, cause diagnostic difficulties. Given that DSCRT is an aggressive and nearly universally fatal disease, making the correct diagnosis is critically important. Herein, we report three patients with DSRCT and unusual clinical, morphologic or immunohistochemical characteristics, in order to highlight its remarkable diversity and increase awareness of this unusual, distinctive neoplasm.