Round cell tumors of bone are a divergent group of neoplasms that largely constitute ewing sarcomaprimitive neuroectodermal tumor, small cell osteosarcoma, langerhans cell his. The cytoplasm of these cells is pale blue and contains variable numbers of punchedout vacuoles. Small round blue cell tumors of the sinonasal tract lester dr. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Fewer than 200 cases have been reported in the literature, and only a few have included a description of radiologic findings 16. It primarily affects children and young adults and is more common in males. Desmoplastic small round cell tumors dsrct are a type of softtissue sarcoma. A molecular hallmark of dsrct is the ewswt1 reciprocal translocation. In histopathology, a smallblueroundcell tumour abbreviated sbrct, also known as a. Small round blue cell tumors radiology reference article.
Pathology outlines desmoplastic small round cell tumor. Case report open access primary desmoplastic small round cell tumor of the duodenum qi liu, nan liu and dexing chen abstract the desmoplastic small round cell tumor dsrct is an extremely rare tumor that mainly affects adolescents and mostly involves the abdominal and pelvic peritoneum. These ewinglike sarcomas also show polyphenotypia, aggressiveness, and drug resistance similar to desmoplastic small round cell tumor. Small blue round cell tumor journal of the american. Small round cells have diameters up to approximately three times that of a small mature lymphocyte.
The following is a case presentation of an 18yearold male with a large softtissue mass of. Desmoplastic small round cell tumor in the abdomen and. A number of tumours share similar histology, composed of relatively uniform primitive small round blue cells. Cytologic features of smallcell carcinoma on thinprep. Differential diagnosis of paediatric small round and blue cell tumors an update ivo leuschner kiel paediatric tumor registry dept. Tumors of the lung, discuss the differential diagnosis of. The spectrum of small round blue cell neoplasms is broad, but within the sinonasal tract snt, tumours of the dispersed neuroendocrine cell system narrow to olfactory neuroblastoma onb, pituitary adenoma, melanoma, and neuroendocrine carcinoma. Desmoplastic small round cell tumor dsrct is a rare but highly aggressive neoplasm that afflicts mainly adolescents and young adults. Small round blue cell tumor srbct or a small round cell tumour srct is a group of malignant neoplasms which are seen more often in children 020 yearsold than in adults. Pdf the small round blue cell tumors of the sinonasal area. Small blue cells, bone marrow infiltration, staging introduction the small round blue cell tumors are a group of neoplasms occurring usually in children and adolescents and are characterized by small round relatively undifferentiated cells. Small round blue cell tumors lymphoma tumor board november 6, 2015.
Desmoplastic small round cell tumor distinction between dsrct and small cell mesothelioma dsrct ewsr1wt1 gene fusion. It is formed by small, round cancer cells surrounded by scarlike tissue and is often found in the tissue peritoneum that lines the inside of the abdomen and pelvis. In an undifferentiated tumor, showing a small round blue cell. Dsrct causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known. Malignant small round cell tumors msrct is a term used for tumors composed of malignant round cells that are slightly larger or double the size of red blood cells in airdried smears. Smallblueroundcell tumor an overview sciencedirect. Desmoplastic small round cell tumor dsrct is a type of cancer. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. Bone marrow infiltration in small blue cell tumours.
Small blue round cell tumor an overview sciencedirect. The diagnostic classification of small round blue cell tumors of the sinonasal area to include diverse malignancies of epithelial, hematolymphoid, neuroectodermal, and mesenchymal origin is challenging to the surgical pathologist using conventional histopathologic approaches because the cytomorphologic features are often overlapping or indistinctive. Desmoplastic small round cell tumor is a rare malignant neoplasm. Small round blue cell tumors of the sinonasal tract. Other small round blue cell tumors, including rhabdomyosarcoma, ewing. The small round blue cell tumors of the sinonasal area. The tumor shows positive immunostaining with antibodies to cytokeratin, vimentin, and desmin, a pattern unique to desmoplastic small round cell tumor. Of note, rarely one may see small round cell tumors with the polyphenotypia and desmoplasia of desmoplastic small round cell tumor but containing an ewsfli1 fusion. Small round blue cell tumours dr johannes schmid and assoc prof frank gaillard et al. Case report open access primary desmoplastic small. Epithelial markernegative desmoplastic small round cell.
They also share many demographic, radiographic and clinical similarities. Ewing sarcoma and dsrct are treated similarly due to similar oncogene activation pathways, and dsrct has been represented in very limited numbers in sarcoma studies. These cells at low magnification resemble mature lymphocytes which also posses scant cytoplasm. Desmoplastic smallroundcell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Abdominopelvic or chest ct findings had no significant correlation with the outcome of patients. In histopathology, a small blue cell tumor or small round blue cell tumors refer to tumors comprised of blue cell with very scant cytoplasm. An organoid growth pattern is also potentially apparent figs. Molding was present in medulloblastoma and merkel cell carcinoma. In histopathology, a small blue round cell tumour abbreviated sbrct, also known as a small round blue cell tumor srbct or a small round cell tumour srct, is any one of a group of malignant neoplasms that have a characteristic appearance under the microscope, i.
In an undifferentiated tumor, showing a small round blue cell morphology, using the mnemonic. Small blue round cell tumour definition of small blue. Malignant small round cell tumors small blue cell malignancies prototypically are composed of cells with round to oval nuclei, scant cytoplasm, and often extensive cellular dissociation. As nucleus stains blue and cytoplasm pink, these tumors impart a distinct blue appearance. One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Small blue round cell tumors of childhood rarely present in the foot or ankle. Patients with localized disease have a 6070% chance of being disease free at 5. Small round blue cell tumors lymphoma neuroblastoma rhabdomyosarcoma ewing sarcomaprimitive neuroectodermal tumor pnet desomplastic small round cell tumor poorly differententiated synovial sarcoma. Pdf round cell tumors as the name suggest are comprised round cells with increased nuclearcytoplasmic ratio. Small round cell tumors of bone are a heterogeneous group of neoplasms with overlapping clinical, radiologic, morphologic, and immunohistochemical features.
Desmoplastic small round cell tumor genetic and rare. Small round blue cell blue tumor srbct tumors characterized by small, round, relatively undifferentiated cells cytology is an excellent tool for the diagnosis of these tumors. A number of tumors share similar histology, composed of relatively uniform primitive small round blue cells. It is an aggressive and often misdiagnosed neoplasm of children and young adult. The most common location is the peritoneal cavity, although tumors arising from other sites have been described. Desmoplastic small round cell tumors dsrct md anderson. Even though questions remain regarding cell of origin and histogenesis, cytogenetic and molecular genetic studies have enhanced our understanding in bringing together some of these tumors. Pediatric neoplasia, diagnosis, small round blue cell tumors. Malignant small round cell tumors request pdf researchgate. Modern diagnosis of small cell malignancies of children surgical.
Blue cell tumor definition of blue cell tumor by medical. Discussion dsrct belongs to the family of small round blue cell tumors commonly found in the pediatric population. Desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen. Despite aggressive therapy, median survival ranges from. Malignant small round cell tumors pubmed central pmc. Metastatic small round blue cell tumors in children and young women have been the subject of multiple case reports and small studies. The first case of a dsrct was recorded in 1989, and about 200 cases have been diagnosed since then. A generic term for tumours almost invariably malignant characterised by sheets of relatively homogenous small cells with scant cytoplasm and prominent blue nuclei, which cannot be reliably differentiated by light microscopy and require ancillary studiesin particular, immunohistochemistryto identify cell. It is characterized by a distinct immunohistochemical profile and a recurrent, specific, chromosomal translocation.
In histopathology, a small blueroundcell tumour abbreviated sbrct, also known as a. It belongs to a family of socalled small round blue tumor cells that comprise its histological mimics, such as rhabdomyosarcoma, nephroblastoma wilms tumor. This group of neoplasms is characterized by small, round, relatively undifferentiated cells. An update on recent molecular genetic advances shi wei, md, phd and gene p. This criterion was absent in desmoplastic small round cell tumor, ewings sarcoma, immature teratoma, lymphoma, neuroblastoma, rhabdomyosarcoma, and reactive lymph nodes. Biopsies are usually small and limited, resulting in considerable. Small blue cell tumors of the pleura small cell mesothelioma desmoplastic small round cell tumor metastatic small cell carcinoma lymphoma cllsll intrapleural thymoma ewing family of tumors histology. Differential diagnosis of paediatric small round and blue. Small round blue cell tumor with cicdux4translocation antonescu, 20 cd99.
Smallblueroundcell tumor an overview sciencedirect topics. It is usually found in the abdomen belly, but it can also occur in other parts of the body. Display of small round blue cells characteristic of desmoplastic small round cell tumour. Desmoplastic small round blue cell tumors dsrcts originate from a cell with multilineage potential. The differential diagnosis of sinonasal small round blue cell tumors and tumors with neuroendocrine differentiation at this site is broad and complex 6, 21,25,34,36,43,47,59, including.
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