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. 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. 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. 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. 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. Desmoplastic small round cell tumor in the abdomen and.
An organoid growth pattern is also potentially apparent figs. Patients with localized disease have a 6070% chance of being disease free at 5. Desmoplastic small round cell tumor dsrct is a rare but highly aggressive neoplasm that afflicts mainly adolescents and young adults. 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. Biopsies are usually small and limited, resulting in considerable. 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. It is characterized by a distinct immunohistochemical profile and a recurrent, specific, chromosomal translocation. Cytologic features of smallcell carcinoma on thinprep. Desmoplastic small round cell tumor distinction between dsrct and small cell mesothelioma dsrct ewsr1wt1 gene fusion. Epithelial markernegative desmoplastic small round cell. It is usually found in the abdomen belly, but it can also occur in other parts of the body. In an undifferentiated tumor, showing a small round blue cell morphology, using the mnemonic. Modern diagnosis of small cell malignancies of children surgical. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue.
Desmoplastic small round cell tumor dsrct is a type of cancer. Small round blue cell tumors of the sinonasal tract. 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. Small blue round cell tumour definition of small blue. 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. 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. Differential diagnosis of paediatric small round and blue cell tumors an update ivo leuschner kiel paediatric tumor registry dept. Desmoplastic smallroundcell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Small blue round cell tumor an overview sciencedirect.
Desmoplastic small round cell tumors dsrct are a type of softtissue sarcoma. Small blue round cell tumor journal of the american. Round cell tumors of bone are a divergent group of neoplasms that largely constitute ewing sarcomaprimitive neuroectodermal tumor, small cell osteosarcoma, langerhans cell his. A molecular hallmark of dsrct is the ewswt1 reciprocal translocation. It is an aggressive and often misdiagnosed neoplasm of children and young adult. Differential diagnosis of paediatric small round and blue. Small round cells have diameters up to approximately three times that of a small mature lymphocyte. Metastatic small round blue cell tumors in children and young women have been the subject of multiple case reports and small studies. Desmoplastic small round cell tumor genetic and rare. Abdominopelvic or chest ct findings had no significant correlation with the outcome of patients. Dsrct causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known. In histopathology, a smallblueroundcell tumour abbreviated sbrct, also known as a.
The tumor shows positive immunostaining with antibodies to cytokeratin, vimentin, and desmin, a pattern unique to desmoplastic small round cell tumor. Smallblueroundcell tumor an overview sciencedirect. Pathology outlines 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.
As nucleus stains blue and cytoplasm pink, these tumors impart a distinct blue appearance. Tumors of the lung, discuss the differential diagnosis of. Small round blue cell tumors lymphoma neuroblastoma rhabdomyosarcoma ewing sarcomaprimitive neuroectodermal tumor pnet desomplastic small round cell tumor poorly differententiated synovial sarcoma. Molding was present in medulloblastoma and merkel cell carcinoma. The small round blue cell tumors of the sinonasal area. The cytoplasm of these cells is pale blue and contains variable numbers of punchedout vacuoles.
It belongs to a family of socalled small round blue tumor cells that comprise its histological mimics, such as rhabdomyosarcoma, nephroblastoma wilms tumor. In histopathology, a small blue cell tumor or small round blue cell tumors refer to tumors comprised of blue cell with very scant cytoplasm. The first case of a dsrct was recorded in 1989, and about 200 cases have been diagnosed since then. Pediatric neoplasia, diagnosis, small round blue cell tumors. Desmoplastic small round cell tumors dsrct md anderson. The following is a case presentation of an 18yearold male with a large softtissue mass of. Desmoplastic small round cell tumor is a rare malignant neoplasm. This criterion was absent in desmoplastic small round cell tumor, ewings sarcoma, immature teratoma, lymphoma, neuroblastoma, rhabdomyosarcoma, and reactive lymph nodes. Bone marrow infiltration in small blue cell tumours. 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. Small blue round cell tumors of childhood rarely present in the foot or ankle. 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.
It primarily affects children and young adults and is more common in males. Malignant small round cell tumors pubmed central pmc. 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. They also share many demographic, radiographic and clinical similarities. Smallblueroundcell tumor an overview sciencedirect topics. An update on recent molecular genetic advances shi wei, md, phd and gene p. In an undifferentiated tumor, showing a small round blue cell.
Discussion dsrct belongs to the family of small round blue cell tumors commonly found in the pediatric population. In histopathology, a small blueroundcell tumour abbreviated sbrct, also known as a. 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. Despite aggressive therapy, median survival ranges from. Blue cell tumor definition of blue cell tumor by medical. Small round blue cell tumors lymphoma tumor board november 6, 2015. Fewer than 200 cases have been reported in the literature, and only a few have included a description of radiologic findings 16. This group of neoplasms is characterized by small, round, relatively undifferentiated cells.
Desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen. The most common location is the peritoneal cavity, although tumors arising from other sites have been described. Small round blue cell tumors radiology reference article. Small round blue cell tumours dr johannes schmid and assoc prof frank gaillard et al. 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. Small round cell tumors of bone are a heterogeneous group of neoplasms with overlapping clinical, radiologic, morphologic, and immunohistochemical features. One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Small round blue cell tumors of the sinonasal tract lester dr. 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. A number of tumors share similar histology, composed of relatively uniform primitive small round blue cells. Case report open access primary desmoplastic small. These cells at low magnification resemble mature lymphocytes which also posses scant cytoplasm. Pdf the small round blue cell tumors of the sinonasal area.