Desmoplastic small round cell tumor (DSRCT) is rare and a recently described, poorly differentiated malignant tumor and it usually presents with widespread intra-abdominal involvement. We report a case of DSRCT arising form the mediastinum which was treated with multimodality anticancer therapy in 15 year-old girl.
Adolescent ; Desmoplastic Small Round Cell Tumor* ; Female ; Humans ; Mediastinum* ; Pathology

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive neoplasm. The cytological diagnosis of this tumor has only been reported in a few cases. In most of these cases, the diagnosis was made using fine-needle aspiration cytology. Most DSRCTs resemble disseminated carcinomatoses in their clinical manifestation as well as cytomorphologically, even in young-adult patients. These authors report a case of using peritoneal-washing and pleural-effusion ThinPrep cytology to diagnose DSRCT, with extensive glandular differentiation and mucin vacuoles. We found that fibrillary stromal fragment, clinical setting, and adjunctive immunocytochemical staining were most helpful for avoiding misdiagnosis.
Biopsy, Fine-Needle ; Carcinoma ; Desmin ; Desmoplastic Small Round Cell Tumor ; Diagnostic Errors ; Humans ; Mucins ; Vacuoles

Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm of young adults and it is characterized by polyphenotypic differentiation. We experienced a case of abdominal DSRCT that occurred in a 19-year-old female who presented with painful swelling of her right forearm. The tumor was cytokeratin-negative and it exhibited some tumor cells with intranuclear inclusions. Molecular demonstration of EWS-WT1 fusion transcripts is particularly useful to confirm the diagnosis of DSRCT without epithelial differentiation. We report here on a case of cytokeratin-negative DSRCT that showed an unusual feature of intranuclear inclusions.
Desmoplastic Small Round Cell Tumor ; Female ; Forearm ; Humans ; Intranuclear Inclusion Bodies ; Keratins ; Young Adult

Peritoneal desmoplastic small round cell tumor is a very rare malignant neoplasm and has specific clinical features; It is predominant in children and young males and has a well-demarcated large intra-abdominal tumor, which has not been associated with a primary visceral organ, with diffusely scattered multiple small tumors and rarely involves ovaries. It is a very aggressive and fast growing tumor along the peritoneal surfaces of the abdomen and pelvis. It has a typical histologic features and a specific immunohistochemical staining pattern. There is no definite treatment. It responses to surgery and chemotherapy at early period of therapy but relapses soon and rapidly progresses and then causes the death. We have experienced a peritoneal desmoplastic small round cell tumor which involved both ovaries, so we report this case with a brief review of literature.
Abdomen ; Child ; Desmoplastic Small Round Cell Tumor* ; Drug Therapy ; Female ; Humans ; Male ; Ovary* ; Pelvis ; Recurrence

Desmoplastic small round cell tumor is a rare and aggressive tumor that affects young males. It is usually an abdominopelvic malignancy that demonstrates distinct histological appearances and a unique cytogenetic profile. There have been many different approaches to the treatment of desmoplastic small round cell tumor, but unfortunately it remains incurable and has poor long-term survival rates. However, with an aggressive approach to the treatment using multiple modalities, some temporary benefit to survival can be achieved. There has not yet been a case in which treatment has led to a curative outcome. Recently, we also experienced a case of very poor outcome of 31-year-old female with small round cell tumor in peritoneum. Here, we report the case with a brief review of literatures.
Adult ; Cytogenetics ; Desmoplastic Small Round Cell Tumor ; Female ; Humans ; Male ; Peritoneum ; Survival Rate

PURPOSE: To review MRI in order to compare Gd-DTPA enhanced images before and after preoperative intraarterialchemotherapy for osteosarcoma. MATERIALS AND METHODS: In sixteen patients with pathologically-proven osteosarcomawho underwent intra-arterial chemotherapy, MR images were obtained before and after this treatment. The degree[grade 0(low) to IV(high)] and pattern (homogenous, inhomogenous) of changes in contrast enhancement and tumorsize were evaluated. RESULTS: Enhancement changed from Grade III to I in eight patients and from Grade III to IIin five. In three, the enhancement pattern changed from homogenous to inhomogenous; in ten, the inhomogenouspattern did not change; and in three, there was a change from thick, irregular to thin, smooth-rimmed enhancement.In all patients except one, the intraosseous tumor component was not changed. The extraosseous component becamelarger in three patients, smaller in two, and showed no size change in eleven. CONCLUSION: In osteosarcoma, MRimaging after Gd-enhancement following intra-arterial chemotherapy showed decresased enhancement, and increasedinhomogeneity and smooth rim enhancement. Neither the intra-nor extraosseous tumor component showed size changewith time.
Desmoplastic Small Round Cell Tumor* ; Drug Therapy ; Female* ; Gadolinium DTPA ; Humans ; Magnetic Resonance Imaging ; Osteosarcoma

Peritoneal desmoplastic small round cell tumor is a very rare and highly aggressive neoplasm that predominantly strikes male children and young adults. It frequently presents as a large abdominal mass with widespread peritoneal involvement at diagnosis. We report one case of desmoplastic small round cell tumors in the pelvis in a old aged woman with a brief review of literature.
Aged ; Child ; Desmoplastic Small Round Cell Tumor ; Female ; Humans ; Male ; Pelvis ; Strikes, Employee ; Young Adult

Intraabdominal desmopl small round cell tumor is a rare neoplasm predominant in adolescent or young adult male. The tumor is characterized by widespread growht along the peritoneal surfaces of the abdomen and pelvis,typical histologic features, and a specific immunohistochemical staining pattern. We report the clinical,radiologic, and pathologc findings of intraabdominal desmoplastic small round cell tumor.
Abdomen ; Adolescent ; Desmoplastic Small Round Cell Tumor* ; Humans ; Male ; Young Adult

Desmoplastic small round cell tumor (DSRCT) is a rare and highly malignant mesenchymal tumor found in the abdominal cavity. It mainly affects young male patients. We report a case of DSRCT that occurred in the abdominal cavity of a 50-year-old man. The tumor was characterized by small round tumor cells with irregular nests in the prominent desmoplastic stroma. The tumor cells showed immunoreactivity for epithelial membrane antigen, desmin, vimentin, and neuron specific enolase.
Abdominal Cavity ; Desmin ; Desmoplastic Small Round Cell Tumor* ; Humans ; Male ; Middle Aged ; Mucin-1 ; Phosphopyruvate Hydratase ; Vimentin

A desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm that occurs predominantly in children and young men. It presents as a large mass inside the abdomen, particularly within the pelvis, and may be accompanied by extensive tumor implants throughout the peritoneum. Microscopically, it typically appears as nests of small undifferentiated cells within a desmoplastic stroma. A DSRCT shows a special immunohistochemical staining pattern, expressing epithelial, neural, and muscle markers. A DSRCT is associated with a specific chromosomal translocation, t (11;22) (p13;q12), resulting in a chimeric EWS/WT1 transcript that is helpful for diagnosing this tumor. We experienced a case of DSRCT in a 19-year-old man who had been diagnosed with Down's syndrome.
Abdomen ; Child ; Desmoplastic Small Round Cell Tumor ; Down Syndrome ; Humans ; Male ; Muscles ; Pelvis ; Peritoneum ; Translocation, Genetic ; Young Adult