Dsrct usually occurs in white adolescent and young men between the ages of ten and 30. A molecular hallmark of dsrct is the ewswt1 reciprocal translocation. To the editor we read with great attention the article by tun et al 1 describing a case of advanced desmoplastic small round cell tumor dsrct and discussing its optimal management. 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. I was an ambitious, driven doctor just two years away from finishing my training to become an elderly medicine consultant in the uk. New hope unlimited has devoted years in antitumor protocol research for desmoplastic small round cell tumor treatment. Keratin and desmin positive neoplasm composed of nests of small cells with surrounding desmoplastic stroma. Desmoplastic small round cell tumors of the pleura. Since dsrct is complex and seldom seen, no standard treatment exists. Desmoplastic small round blue cell tumors dsrcts originate from a cell with multilineage potential.
This type of cancer usually begins in the abdomen or pelvis, and does not respond well to treatment. Multimodal treatment combining chemotherapy, surgery and radiotherapy is the best option. Desmoplastic small round cell tumor genetic and rare. Current research is limited by the scarcity of this disease. Dsrct is a rare and highly aggressive tumour that usually occurs in males during adolescence and early adulthood. Although we agree with the authors1 on the fact that data are scarce on the clinical management of advanced dsrct, we. 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. It tends to have a predilection for adolescent males with an annual incidence of about 0. Of the patients who develop this cancer, 90 percent are male, and 85 percent are caucasian. A case of longterm survival of metastatic desmoplastic small round. A 14yearold girl presented with intermittent epigastric pain.
The desmoplastic small round cell tumor dsrct is an extremely rare tumor that mainly affects adolescents and mostly involves the abdominal and pelvic peritoneum. Desmoplastic smallroundcell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Connective tissues include fat, muscles, tendons, lymph and blood vessels, and nerves. Dsrct is a rare and very aggressive form of soft tissue sarcoma that primarily affects young males. Pathology outlines desmoplastic small round cell tumor. Epidemiology they are usually seen in young adolescents and has male predominance with a mean survival of 23 years. Desmoplastic small round cell tumor dsrct is a highly aggressive and rare mesenchymal tumor, around 200450 cases have been so described so far 1,2 despite aggressive therapy, median survival ranges from 17 to 25 month 2,8 a 5. Pdf desmoplastic small round blue cell tumors dsrcts originate from a cell. 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. Desmoplastic small round cell tumor is a rare malignant neoplasm that usually occurs in adolescents and young adult males and typically presents as a large, often multinodular, intraabdominal mass.
Desmoplastic small round cell tumors dsrct md anderson. 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. Desmoplastic small round cell tumor dsrct is a rare dis. Background desmoplastic small round cell tumor dsrct is a rare disease affecting predominantly children and young adults and for which the benefit of hyperthermic intraperitoneal chemotherapy hipec after complete cytoreductive surgery ccrs remains unknown. Desmoplastic small round cell tumor dsrct is a soft tissue sarcoma of mesenchymal cell origin, presenting with multiple intraabdominal tumors. Dsrct belongs to the family of small round blue cell tumours, which includes primitive neuroectodermal tumour. Desmoplastic small round cell tumor dsrct is a type of cancer.
It is characterized by a distinct immunohistochemical profile and a recurrent, specific, chromosomal translocation. Desmoplastic small round cell tumor of the pleura is a rare malignancy, with only a few cases reported in the scientific literature. Jan 30, 2018 desmoplastic small round cell tumor dsrct is a rare sarcoma that primarily affects adolescents and young adults. Prognosis is uncertain in such an aggressive neoplasm as chemotherapy ct or radiotherapy rt shows various outcomes. Scribd is the worlds largest social reading and publishing site. The desmoplastic small round cell tumor dsrct is a rare, aggressive, and malignant tumor, first described by gerald in 1989. Round cell tumors final lymphoma metastasis free 30. Desmoplastic small round cell tumor dsrct is an extremely infrequent, aggressive soft tissue tumor it is regarded as a kind of highgrade malignancy that spreads and infiltrates into the surrounding tissues and organs. We conducted a phase 2 clinical trial utilizing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy crshipec with cisplatin for dsrct and pediatrictype abdominal sarcomas. Abdominal desmoplastic small round cell tumor without. Methods to identify patients with dsrct without extraperitoneal metastases epm who underwent ccrs between 1991 and 2015, a.
The most common symptoms include pain or a mass in the abdomen. Desmoplastic small round cell tumor dsrct is a rare, biologically aggressive, multifocal primary peritoneal sarcoma that predominantly affects white adolescent boys and young adult men. Desmoplastic small round cell tumor in the abdomen and pelvis. We report the case of a 26yearold woman who suffered from dyspepsia and abdominal pain for 2 months. It primarily affects children and young adults and is more common in males. It usually happens in the tissue that lines the inside of the belly. Potential therapeutic genomic alterations in desmoplastic. To the editor we read with great attention the article by tun et al1 describing a case of advanced desmoplastic small round cell tumor dsrct and discussing its optimal management. Round cell tumors final free download as powerpoint presentation.
Desmoplastic small round cell tumors dsrct memorial. Mayo clinic doctors and scientists are studying new ways to diagnose and treat desmoplastic small round cell tumors. Desmoplastic small round cell tumors symptoms and causes. The peritoneal cavity is the most common location of the tumor with. Desmoplastic small round cell tumors dsrct memorial sloan. Desmoplastic small round cell tumor presenting as a neck. Surgery is almost always part of treatment for dsrct. Desmoplastic small round cell tumour dsrct is an uncommon malignant. The term round cell tumor describes a group of highly aggressive malignant tumors composed of relatively small and monotonous undifferentiated cells with increased nuclearcytoplasmic ratio. Desmoplastic small round cell tumour successfully treated with.
Intraabdominal desmoplastic small round cell tumor. Desmoplastic small round cell tumor of meckels diverticulum. Desmoplastic small round cell tumor dsrct is a rare peritoneal surface malignancy. Desmoplastic small round cell tumor dsrct by fernanda arnaldez, md and david loeb, md, phd also available in chinese, japanese, french, italian and spanish. Desmoplastic small round cell tumor dsrct is a rare sarcoma that primarily affects adolescents and young adults. 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. Chemotherapy, desmoplastic small round cell tumor, immunohistochemistry, radiotherapy. Durable responses are exceptionally rare so further systemic. Desmoplastic small round cell tumor is a rare tumor that was reported by gerald and rosai 1 in 1989, and it is said to be related to primitive neuroectodermal tumor. Diagnosis is usually based on the tumors histological and immunohistochemical features. It mainly affects adolescents and mostly involves the abdominal or pelvic peritoneum. It is known to involve the formation of fusion protein of ewings sarcoma genes and wilms tumor genes, and it has been gaining attention in recent years. Hayesjordan4,5 morani ac, bathala tk, surabhi vr, et al.
Desmoplastic small round cell tumors dsrct are aggressive tumors that typically begin in the abdomen or pelvis. Desmoplastic small round cell tumor dsrct is a rare abdominal disease affecting predominantly children and young adults. The most common site is the abdominal serosa, although other sites have been described. The mayo clinic cancer center receives funding from the national cancer institute and is designated as a comprehensive cancer center recognition. 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. The first case of a dsrct was recorded in 1989, and about 200 cases have been diagnosed since then. Overall only 2 patients 10% were alive and diseasefree at last followup. Only a few hundred cases have been reported in worldwide literature. 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. Trabectedin for desmoplastic small round cell tumours. Desmoplastic small round cell tumor dsrct is a rare but aggressive primitive malignant neoplasm that occurs mainly in adolescents and young adults. Pdf round cell tumors as the name suggest are comprised round cells with increased nuclearcytoplasmic ratio. 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. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue.
Dsrct causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known. After carefully evaluating your childs case, our team of experts will discuss a recommended course of treatment for dsrct. 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 tumor is a tumor composed of small round cells associated with marked desmoplasia and multiphenotypic differentiation. This cancer happens when abnormal cells form in the soft tissues. Dsrct mainly develops in adolescent and young adults with a strong male predominance. Desmoplastic small round cell tumour dsrct is a rare but frequently. It is formed by small, round cancer cells surrounded by scarlike tissue and is often found in the tissue peritoneum that lines the inside. Then, it was more elaborately described by gerald and rosai in 1989. 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. Kate was 29 when she was diagnosed with desmoplastic small round cell tumor at the abdomen.
Report of ct findings in 11 affected children and young adults objective. Desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen. Although we agree with the authors 1 on the fact that data are scarce on the clinical management of advanced dsrct, we would like to comment on several issues regarding the current knowledge on the. No standard therapy is currently available for patients with dsrct, and dsrct has a very poor prognosis, and its median survival range is from 17 to 25 months, with only a 29% actuarial 3year survival rate and a 5year survival rate of 18%. In this report, we describe a young female who presented with bilateral ovarian masses that mimicked the classic clinical picture of ovarian cancer. Mar 23, 2017 desmoplastic small round cell tumor dsrct is a soft tissue sarcoma of mesenchymal cell origin, presenting with multiple intraabdominal tumors. Desmoplastic small round cell tumor dsrct is a rare disease predominantly affecting adolescent and young adult males, which originates and spreads on peritoneal surfaces. Desmoplastic small round cell tumor dsrct is an aggressive malignant neoplasm that occurs in adolescents and young adults. Our objective was to evaluate the ct features of desmoplastic small round cell tumor dsrct of the abdomen and pelvis in pediatric and young adult patients. Desmoplastic small round cell tumor of meckels diverticulum a6yearoldboypresentedwith6monthhistoryofintermittent abdominal pain associated with episodic vomiting suggestive of subacute intestinal obstruction, which had worsened signi.
Therapeutic trial for patients with ewing sarcoma family of. Desmoplastic small round cell tumors dsrct are a very rare type of soft tissue sarcoma. Desmoplastic small round cell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Factors affecting overall survival os were assessed. Desmoplastic small round cell tumor dsrct presents as a rare separate clinical pathological entity, and pleural dsrct is very rare. Desmoplastic small round cell tumor of the middle ear. Here is an updated pdf on whats new with dsrct as provided by md anderson cancer center the facts. Desmoplastic small round cell tumors dsrct are a type of softtissue sarcoma. Histologic, immunohistochemical, and genetic features are discussed. Wt1 staining reliably differentiates desmoplastic small round cell tumor from ewing sarcomaprimitive neuroectodermal tumor. Desmoplastic small round cell tumor dsrct is a rare, aggressive, and malignant tumor primarily affecting young males. Solid nests of roundoval cells surrounded by cellular desmoplastic stroma 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.
The disease can affect any person at any age but is. Primary desmoplastic smallroundcell tumor of the ovary. Definition of desmoplastic small round cell tumor nci. Desmoplastic small round cell tumor dsrct is a rare, aggressive and. Kates sarcoma story desmoplastic small round cell tumor. It is an aggressive and often misdiagnosed neoplasm of children and young adult. Desmoplastic small round cell tumors dsrct national. Toomer a rare, aggressive cancer that usually affects young males and usually is located in the abdomen. Desmoplastic small round cell tumor dsrct is an aggressive, polyphenotypic, malignant tumor occurring predominantly in children and young adults, with a male predominance and an age range spanning the first through fifth decades. Clinical, cytologic, and immunocytochemical findings are described.
One patient was still diseasefree 20 months after treatment, although the rest. Desmoplastic small round cell tumor radiology reference. Desmoplastic small round cell tumors, or dsrct, are tumors that grow in the abdomen and pelvic area of the body. Desmoplastic small round cell tumor dsrct the brett. Despite multimodal treatment approaches, the prognosis for dsrct is extremely poor. Desmoplastic smallroundcell tumor is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Management of desmoplastic small round cell tumors in children and young adults.
Desmoplastic smallroundcell tumor dsrct is an extremely rare and highly aggressive malignancy that was first described in 1987 by sesterhenn et al. The majority of patients 90 percent are caucasian males between the ages of 10 and 30. 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. Nov 15, 2019 desmoplastic small round cell tumor dsrct is an extremely rare and highly aggressive malignancy. Nests and cords of cells surrounded by desmoplastic. It is of yet unclear origin, but it is assumed to be of a mesothelial origin based on its tendency for widespread metastasis in serosal linings. Desmoplastic small round cell tumors are diagnosed with a procedure to remove a sample of cells for testing.
Desmoplastic small round cell tumor dsrct is a rare and highly. This group of tumor includes entities such as peripheral neuroectodermal tumor, rhabdomyosarcoma, synovial sarcoma, nonhodgkins lymphoma, neuroblastoma, hepatoblastoma, wilms tumor, and desmoplastic small round cell tumor. Round cell tumors as the name suggest are comprised round cells with increased nuclearcytoplasmic ratio. Immunohistochemical expression of wt1 by desmoplastic small round cell tumor. It affects mainly children and young adults and has a. To remove the cells, you might need surgery or a needle can be passed through the skin. Fewer than 500 cases have been reported in the literature 24. It is usually found in the abdomen belly, but it can also occur in other parts of the body.
The abdomen and pelvis are the sites most likely to be involved, while dsrct of the pleura is even more rare. Sep 19, 20 therapeutic trial for patients with ewing sarcoma family of tumor and desmoplastic small round cell tumors the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Desmoplastic small round cell tumors diagnosis and. Desmoplastic small round cell tumor dsrct is a rare malignancy most commonly. Cancer research is conducted in coordination with the mayo clinic cancer center.
Case report solidpattern desmoplastic small round cell tumor of. Desmoplastic small round cell tumors alternative treatments. Desmoplastic small cell tumor surgical pathology criteria. The peritoneal cavity is the most common location of the tumor with multiple satellite nodules often noted at presentation. Desmoplastic small round cell tumor is a very rare malignancy. The prolonged progressionfree survival period 15 months. Alkylator and anthracyclines based regimens are widely used as therapy and an initial response is common. Desmoplastic small round cell tumors are a type of soft tissue sarcoma, which is a type of cancer that forms in the connective tissue of the body. The tumor has a specific genetic abnormality that helps confirm the diagnosis. Optimal multimodal treatment for desmoplastic small round. It is rarely isolated to one tumor implant in the abdomen and in most cases presents with dozens to hundreds of abdominal peritoneal tumors. Desmoplastic small round cell tumor is a rare malignant tumor that has a poor prognosis. Patients can present with many peritoneal implants. Desmoplastic small round cell tumor is a rare malignant neoplasm.
In the current report, a 14yearold male patient was admitted to the. Primary desmoplastic small round cell tumor of the duodenum. A rare form of cancer, a desmoplastic small round cell tumor is most likely to develop in adolescents and young adults. The organ or specific tissue type of origin is unknown. Management of desmoplastic small roundcell tumors in children and young adults. Dsrct most often begins in the tissue lining the inside of the abdominal cavity, which is also known as the peritoneum. Desmoplastic small round cell tumour dsrct is a rare tumour, usually arising in the abdominal cavity. This cancer usually affects children and young adults, and it is more common in males.
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