We report a case of a 26-year-old male who was diagnosed with metastatic desmoplastic small round cell tumor initially treated with systemic chemotherapy followed by tumor debulking and hyperthermic intra-peritoneal chemotherapy. The patient was in complete remission by clinical and imaging criteria for 11 months, until he developed bi-lobar hepatic disease, which was successfully treated with selective internal radiation therapy by Yttrium-90. The patient demonstrated liver-specific complete response on follow-up imaging obtained 18 months after the procedure.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8041655 | PMC |
http://dx.doi.org/10.1016/j.radcr.2021.02.066 | DOI Listing |
Cardiovasc Pathol
January 2025
Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine.. Electronic address:
A rare autopsy case of malignant transcription factor E3 (TFE3)-rearranged perivascular epithelioid cell tumor (PEComa)-like neoplasm is presented. An 84-year-old woman manifested multiple cerebral infarctions and repetitive embolic events in the supra mesenchymal artery (SMA), and the presence of a mobile mass in the heart's left ventricle was also revealed. Tumoral lesions were also found in a pelvic space and a right pleural cavity, and a biopsy was performed from one of the disseminated tumor masses in the right pleura.
View Article and Find Full Text PDFPediatr Dev Pathol
January 2025
Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, St. Louis, MO, USA.
A desmoplastic small round cell tumor (DSRCT) presented in a 13-year-old female with an acute abdomen due to torsion of a fallopian tube cyst. She was found to have an incidental 2 cm pedunculated, solid, and multicystic mass attached to the pelvic floor on laparoscopy. The neoplasm had a variably myxoid and spindle cell pattern with nests and cords of small cells, forming pseudocysts, and true cysts lined by ciliated epithelium which were PAX-8+ and ER+/PR+.
View Article and Find Full Text PDFClin Nucl Med
November 2024
From the Department of Nuclear Medicine, the First Medical Centre, Chinese PLA General Hospital, Beijing, China.
Desmoplastic small round cell tumor (DSRCT) as a subtype of sarcoma is rare and aggressive. We present the case of a 23-year-old man with a histologically confirmed DSRCT who underwent 18F-PSMA PET/CT before prostate biopsy and surgical resection of the tumor. It is well known that PSMA PET has a high accuracy for detecting clinically significant prostate adenocarcinoma and is useful in guiding prostate biopsy.
View Article and Find Full Text PDFClin Nucl Med
February 2025
From the Department of Nuclear Medicine, the First Medical Centre, Chinese PLA General Hospital, Beijing, China.
Desmoplastic small round cell tumor (DSRCT) as a subtype of sarcoma is rare and aggressive. We present the case of a 23-year-old man with a histologically confirmed DSRCT who underwent 18F-PSMA PET/CT before prostate biopsy and surgical resection of the tumor. It is well known that PSMA PET has a high accuracy for detecting clinically significant prostate adenocarcinoma and is useful in guiding prostate biopsy.
View Article and Find Full Text PDFAm J Case Rep
December 2024
Department of Molecular Medicine and Surgery, Center for Molecular Medicine, Karolinska Institute, Stockholm, Sweden.
BACKGROUND Limb-girdle muscular dystrophy recessive 1 (LGMDR1) is an autosomal recessive degenerative muscle disorder characterized by progressive muscular weakness caused by pathogenic variants in the CAPN3 gene. Desmoplastic small round cell tumors (DSRCT) are ultra-rare and aggressive soft tissue sarcomas usually in the abdominal cavity, molecularly characterized by the presence of a EWSR1::WT1 fusion transcript. Mouse models of muscular dystrophy, including LGMDR1, present an increased risk of soft tissue sarcomas.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!