Original article results of multimodal treatment for. Desmoplastic small round cell tumor dsrct is a very rare mesenchymal tumor that mainly affects teenagers and young adults with a mean age at diagnosis around 2025 years. Treatment for desmoplastic small round cell tumors typically involves a combination of treatments, such as surgery, chemotherapy and radiation therapy. Desmoplastic small round cell tumors are a type of soft tissue sarcoma. Despite aggressive therapy, median survival ranges from. Desmoplastic small round cell tumors dsrct memorial sloan.
Desmoplastic small round cell tumor dsrct is a rare and highly aggressive mesenchymal tumor that was first described as a separate identity in 1989 by gerald and rosai. In the current report, a 14yearold male patient was admitted to the hospital for evaluation of abdominal distension, and abdominal pain. Pdf desmoplastic small roundcell tumor of the pancreas. An external file that holds a picture, illustration, etc. The majority of patients 90 percent are caucasian males between the ages of 10 and 30.
Our surgeons are leaders in procedures that not only completely remove tumors but also make sure. Desmoplastic small round cell tumour, dsrct, variously desmoplastic malignant small cell epithelial neuroectodermal tumour of the peritoneum intraabdominal with divergent differentiation coexpressing mesenchymal type intermediate filaments with multiphenotypic differentiation of childhood. It was first described by gerald and rosai in 1989 and formally named in 1991 1, 2. Also necrosis, cystic degeneration, glandular arrangements, signet ringlike cells, pseudorosette formations, rhabdoid cells, extensive areas of predominantly spindle cell morphology, carcinoidlike differentiation, adenoid cysticlike configuration. Primary desmoplastic small round cell tumor of bone. The genomics of desmoplastic small round cell tumor reveals.
Alkylator and anthracyclines based regimens are widely used as therapy and an initial response is common. Desmoplastic small round cell tumor dsrct is a high. Results of multimodal treatment for desmoplastic small round cell tumors dave r. It is known to involve the formation of fusion protein of ewings sarcoma genes and wilms tumor genes, and. Desmoplastic small round cell tumors dsrct are aggressive tumors that typically begin in the abdomen or pelvis. We retrospectively analysed 41 patients treated at or referred to two regional referral centres in. Nests and cords of cells surrounded by desmoplastic. A 14yearold girl presented with intermittent epigastric pain.
The desmoplastic small round cell tumor dsrct is a recently recognized type of primitive sarcoma defined by a predilection for young males, aggressive clinical behavior, widespread abdominal. Patients typically present with symptoms of abdominal sarcomatosis. Morphologically, the neoplasm consisted of small round cells of primitive appearance with a diffuse growth pattern replacing marrow space and eroding bone. Clinical characteristics and outcomes of abdominal desmoplastic.
Mayo clinic doctors and scientists are studying new ways to diagnose and treat desmoplastic small round cell tumors. Th e patient had a poor general condition and did not opt for further treatment. The radiology of desmoplastic small round cell tumour. 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. We used wholeexome sequencing to interrogate six consecutive pretreated dsrcts whose gene expression was. 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. Pdf background to evaluate optimal therapy and potential risk factors. Diagnosis is usually based on the tumors histological and immunohistochemical features. Results of multimodal treatment for desmoplastic small round cell tumor 9660 int j clin exp med 2015.
Desmoplastic small round cell tumor dsrct is a highly malignant small round cell tumor. Dsrct usually occurs in white adolescent and young men between the ages of ten and 30. Desmoplastic small round cell tumors dsrct memorial. The tumor has a specific genetic abnormality that helps confirm the diagnosis. Abdominal desmoplastic small round cell tumor without. Desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen. Desmoplasia originates from the ancient greek desmos, knot, bond and plasis, formation. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. It is usually used in the description of desmoplastic small round cell tumors neoplasia is the medical term used for both benign and malignant tumors, or any abnormal, excessive, uncoordinated, and autonomous cellular or tissue growth.
Molecular testing for desmoplastic small roundcell tumor. Desmoplastic small round cell tumor dsrct is a rare malignancy most commonly originating in the peritoneum. Desmoplastic small round cell tumor is a rare tumor that was reported by gerald and rosai in 1989, and it is said to be related to primitive neuroectodermal tumor. If you have problems viewing pdf files, download the latest version of adobe reader. Cancer research is conducted in coordination with the mayo clinic cancer center. Desmoplastic smallroundcell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen.
Desmoplastic small round cell tumor dsrct is a rare and highly aggressive. Only a few hundred cases have been reported in worldwide literature. The cytology literature regarding dsrct, including aspirates and ascitic fluid, is limited. Immunophenotype of desmoplastic small round cell tumors as. Desmoplastic small round cell tumor dsrct is a type of cancer.
We report a rare case of desmoplastic small round cell tumor, which arose from the left lung. Desmoplastic small round cell tumors dsrct are a type of softtissue sarcoma. Desmoplastic small round blue cell tumor dsrct originates from a cell with multilineage potential. Pdf abstract desmoplastic small round cell tumor dsrct was. Ewings and dsrct are treated similarly due to similar oncogene activation pathways and dsrct has been represented in very limited numbers in sarcoma studies. Twentythree desmoplastic small round cell tumors were identified in the consultation file of one of the authors fgb and collected from the surgical pathology files of the authors institutions. Desmoplastic small round cell tumor dsrct is a rare, aggressive, and poorly investigated simple sarcoma with a low frequency of genetic deregulation other than an ewing sarcoma rna binding protein 1 ewsr1wilms tumor suppressor wt1 translocation. In medicine, desmoplasia is the growth of fibrous or connective tissue. Desmoplastic small round cell tumor dsrct is a rare and highly aggressive mesenchymal tumor that was first described as a separate identity in 1989 by gerald and rosai 1. I was an ambitious, driven doctor just two years away from finishing my training to become an elderly medicine consultant in the uk. Pdf desmoplastic small round cell tumor 20 years after its discovery.
Although the imaging characteristics of desmoplastic small round cell tumor have rarely been described with a renal primary tumor, several case reports describe multiple appearances. Jiang y, subbiah v, janku f, ludwig ja, naing a, benjamin rs, brown re, anderson p, kurzrock r. Apr 25, 2014 desmoplastic small round cell tumour dsrct is a rare sarcoma typically affecting young males and usually widely metastatic at presentation. Desmoplastic small round cell peditric ii tumor of the kidney. Desmoplastic small round cell tumor radiology reference. Results of multimodal treatment for desmoplastic small. Usually affecting young males and presenting as an abdominal mass, the tumor grows along serosal membranes with multiple nodules attached to the peritoneal surface 2. Case report solidpattern desmoplastic small round cell tumor of. Our surgeons are leaders in procedures that not only completely remove tumors but also make sure patients can use the affected body part as freely as possible. Multimodal treatment combining chemotherapy, surgery and radiotherapy is the best option. It is frequently prevalent in the abdominal and pelvic cavities of adolescent males with a. A new molecular variant of desmoplastic small round cell tumor.
Desmoplastic small round cell tumors are a rare and highly aggressive primary peritoneal malignancy. Desmoplastic small round cell tumour dsrct is a rare sarcoma typically affecting young males and usually widely metastatic at presentation. Results of multimodal treatment for desmoplastic small round. Pdf desmoplastic small round cell tumor 20 years after. It is formed by small, round cancer cells surrounded by scarlike tissue and is often found in the tissue peritoneum that lines the inside.
Pathology, genetics, and potential therapeutic strategies. Orbital desmoplastic small round cell tumor in an infant case. Epidemiology they are usually seen in young adolescents and has male predominance with a mean survival of 23 years. To the authors knowledge, findings in thinprep slides have not been described previously.
Sep 09, 2016 desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen. For language access assistance, contact the ncats public information officer. A molecular hallmark of dsrct is the ewswt1 reciprocal translocation. Desmoplastic small round cell tumor dsrct is a rare, biologically aggressive soft tissue neoplasm of uncertain differentiation, most often arising in the abdominal and pelvic cavities of adolescents and young adults with a striking male predominance. Desmoplastic small round cell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Antiangiogenic effects in patients with progressive. Desmoplastic small round cell tumor symptoms, diagnosis.
Desmoplastic small cell tumor surgical pathology criteria. Desmoplastic small round cell tumor dsrct is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults. Results of multimodal treatment for desmoplastic small round cell tumors 253. 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. It is also called desmoplastic reaction to emphasize that it is secondary to an insult. Desmoplastic small round cell tumor dsrct by fernanda arnaldez, md and david loeb, md, phd also available in chinese, japanese, french, italian and spanish. The most common symptoms include pain or a mass in the abdomen. Kate was 29 when she was diagnosed with desmoplastic small round cell tumor at the abdomen. Desmoplastic small round cell tumors dsrct treatment. Learn indepth information on molecular testing for desmoplastic small roundcell tumor, on why the laboratory test is performed, specimen collected, the significance of the results, and its use in diagnosing medical conditions. Desmoplastic small round cell tumor dsrct is an aggressive malignant neoplasm that occurs in adolescents and young adults. Jul 26, 2017 desmoplastic small round cell tumor dsrct is a rare, biologically aggressive soft tissue neoplasm of uncertain differentiation, most often arising in the abdominal and pelvic cavities of adolescents and young adults with a striking male predominance.
Desmoplastic small round cell tumor, also known as dsrct, is a very aggressive form of tumor that can be very life threatening. Desmoplastic small round blue cell tumors dsrcts originate from a cell with multilineage potential. We retrieved from the cytological files of hospital do cancer a. This type of cancer usually begins in the abdomen or pelvis, and does not respond well to treatment. Jan 10, 2014 desmoplastic small round cell tumor is a rare malignant tumor that has a poor prognosis. Orbital desmoplastic small round cell tumor in an infant.
As observed by both histologic and cytologic examinations, small. Treating desmoplastic small round cell tumors dsrcts usually starts with chemotherapy to shrink the tumor and prevent new ones from forming. Primary desmoplastic small round cell tumor of the. Pdf abstract desmoplastic small round cell tumor dsrct was proposed as a distinct disease entity by william l gerald and juan rosai in 1991. Desmoplastic small round cell tumor dsrct is an aggressive small round cell neoplasm which predominantly occurs intraabdominally in. Desmoplastic roundcelltumor was fi rst described as a pathological entity by gerald and rosai in 1989 1. Methods data of dsrct patients find, read and cite all the research you need on. Background desmoplastic small round cell tumour dsrct is a rare but highly aggressive soft tissue sarcoma that arises most commonly in the abdominopelvic cavity of males in. Immunohistochemical data on desmoplastic round cell tumors15. Cytology of desmoplastic small round cell tumor crapanzano.
Fewer than 500 cases have been reported in the literature 24. Pathology outlines desmoplastic small round cell tumor. This type of tumor is usually found in the lining of the abdomen, but is sometimes found in other parts of the body. Histologically, it is characterized by islands of uniform small round cells in prominent desmoplastic stroma, and it has a polyimmunophenotypic. Management of desmoplastic small round cell tumors in children and young adults. 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. A 25yearold man was found to have an abnormal shadow during a routine physical examination and was. All authors read and approved the final manuscript.
We report a case of desmoplastic small round cell tumor occurring in the right ilium of a yearold boy. The mayo clinic cancer center receives funding from the national cancer institute and is designated as a comprehensive cancer center recognition for an institutions scientific. Solid nests of roundoval cells surrounded by cellular desmoplastic stroma. Desmoplasia may occur around a neoplasm, causing dense fibrosis around the tumor, or scar tissue within the abdomen after abdominal surgery. Intraabdominal desmoplastic small round cell tumor. Desmoplastic small round cell tumor of the lung request pdf. Keratin and desmin positive neoplasm composed of nests of small cells with surrounding desmoplastic stroma.
To the editor we read with great attention the article by tun et al describing a case of advanced desmoplastic small round cell tumor dsrct. Optimal multimodal treatment for desmoplastic small round cell. It is usually found in the abdomen belly, but it can also occur in other parts of the body. All structured data from the file and property namespaces is available under the creative commons cc0 license. Despite multimodal treatment approaches, the prognosis for dsrct is extremely poor. Desmoplastic small round cell tumor genetic and rare. Nov 26, 20 desmoplastic small round cell tumour dsrct is a rare but frequently fatal sarcoma, and many of its characteristics still require further clarification. Desmoplastic small round cell tumors symptoms and causes. Pdf desmoplastic small round cell tumor 20 years after its. The desmoplastic small round cell tumor dsrct is a rare, aggressive, and malignant tumor, first described by gerald in 1989. Imaging examination revealed a high prevalence of multiple intraperitoneal and liver parenchymal cystic and solid tumors. Management of desmoplastic small roundcell tumors in children and young adults. Histologically, it is characterized by islands of uniform small round cells in prominent desmoplastic stroma, and it has a.
Primary desmoplastic small round cell tumor of the duodenum. Dsrct is a rare and highly aggressive tumour that usually occurs in males during adolescence and early adulthood. The desmoplastic small round cell tumor dsrct is an extremely rare tumor that mainly affects adolescents and mostly involves the abdominal and pelvic peritoneum. Orbital desmoplastic small round cell tumor in an infant amy huang, bs,a and nishita patel, md b auniversity of central florida college of medicine, orlando. Mar 29, 2012 desmoplastic small round cell tumor dsrct is a rare and highly aggressive mesenchymal tumor that was first described as a separate identity in 1989 by gerald and rosai 1. Desmoplastic small round cell tumors dsrct are a type of rare, soft tissue sarcoma. Differential diagnoses include all the small round cells tumors. Desmoplastic small round cell tumor dsrct is a rare and aggressive abdominal tumor typi cally arising in adolescent. A molecular hallmark of dscrt is the ewswt1 reciprocal translocation. Dsrct causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known. Then we perform surgery to remove the cancerous areas.
Desmoplastic small round cell tumour dsrct is a rare but frequently fatal sarcoma, and many of its characteristics still require further clarification. The genomics of desmoplastic small round cell tumor. Desmoplastic small round cell tumor is an aggressive malignancy with few longterm survivors. Although initial management still needs standardization, many centers will use multimodal treatment including intensive chemotherapy, extensive surgical resection followed by radiotherapy. Desmoplastic small round cell tumor dsrct is a rare and highly aggressive neoplasm described as a distinct clinicopathologic entity in 1989 by gerald and rosai 1. Desmoplastic small round cell tumor dsrct is a rare abdominal disease affecting predominantly children and young adults. The documents contained in this web site are presented for information purposes only. It mainly affects adolescents and mostly involves the abdominal or pelvic peritoneum. Novel secondary somatic mutations in ewings sarcoma and desmoplastic small round cell tumors. Desmoplastic small round cell tumors dsrct md anderson. Files are available under licenses specified on their description page. Solid nests of round oval cells surrounded by cellular desmoplastic stroma.
This is a highly malignant mesenchymal tumour, most often involving the. A malignant serosa related small round cell tumor with an epithelial growth pattern in a desmoplastic stroma first described by gerald and rosai in 1989 pediatr pathol 1989. The purpose of this poster is to describe the imaging features of desmoplastic small round cell tumour dsrct in the context of the clinical and demographic data. The mayo clinic cancer center receives funding from the national cancer institute and is designated as a comprehensive cancer center recognition. It primarily affects children and young adults and is more common in males. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree.
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