Desmoplastic small round cell tumors dsrct memorial. Dsrct is a rare and highly aggressive tumour that usually occurs in males during adolescence and early adulthood. 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. Current research is limited by the scarcity of this disease.
Desmoplastic small round cell tumor is a tumor composed of small round cells associated with marked desmoplasia and multiphenotypic differentiation. 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. Keratin and desmin positive neoplasm composed of nests of small cells with surrounding desmoplastic stroma. Dsrct is a rare and very aggressive form of soft tissue sarcoma that primarily affects young males. 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. 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. 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 tumour dsrct is a rare tumour, usually arising in the abdominal cavity. Desmoplastic small round cell tumors alternative treatments. 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 tumor dsrct by fernanda arnaldez, md and david loeb, md, phd also available in chinese, japanese, french, italian and spanish. In the current report, a 14yearold male patient was admitted to the. Then, it was more elaborately described by gerald and rosai in 1989.
Desmoplastic small round cell tumor dsrct is a rare disease predominantly affecting adolescent and young adult males, which originates and spreads on peritoneal surfaces. Scribd is the worlds largest social reading and publishing site. Definition of desmoplastic small round cell tumor nci. 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. Round cell tumors final free download as powerpoint presentation. 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. Clinical, cytologic, and immunocytochemical findings are described. Kates sarcoma story desmoplastic small round cell tumor. The tumor has a specific genetic abnormality that helps confirm the diagnosis. 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. Chemotherapy, desmoplastic small round cell tumor, immunohistochemistry, radiotherapy.
Desmoplastic small round cell tumors dsrct are aggressive tumors that typically begin in the abdomen or pelvis. Desmoplastic small round cell tumors dsrct memorial sloan. 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. Dsrct belongs to the family of small round blue cell tumours, which includes primitive neuroectodermal tumour. Histologic, immunohistochemical, and genetic features are discussed. Desmoplastic small round cell tumor of the pleura is a rare malignancy, with only a few cases reported in the scientific literature. Wt1 staining reliably differentiates desmoplastic small round cell tumor from ewing sarcomaprimitive neuroectodermal tumor. This cancer happens when abnormal cells form in the soft tissues.
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%. Desmoplastic small round cell tumor dsrct is a rare sarcoma that primarily affects adolescents and young adults. Apr 25, 2014 desmoplastic small round cell tumour dsrct is a rare sarcoma typically affecting young males and usually widely metastatic at presentation. Dsrct usually occurs in white adolescent and young men between the ages of ten and 30. Since dsrct is complex and seldom seen, no standard treatment exists. It is formed by small, round cancer cells surrounded by scarlike tissue and is often found in the tissue peritoneum that lines the inside. Desmoplastic small round cell tumor dsrct is a rare, aggressive, and malignant tumor primarily affecting young males. In this report, we describe a young female who presented with bilateral ovarian masses that mimicked the classic clinical picture of ovarian cancer. 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. 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. Overall only 2 patients 10% were alive and diseasefree at last followup.
Here is an updated pdf on whats new with dsrct as provided by md anderson cancer center the facts. Despite multimodal treatment approaches, the prognosis for dsrct is extremely poor. 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. Desmoplastic small round cell tumor dsrct is a rare dis. 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 tumour dsrct is an uncommon malignant.
Report of ct findings in 11 affected children and young adults objective. Desmoplastic small round cell tumors are a rare and highly aggressive primary peritoneal malignancy. Desmoplastic small round cell tumor dsrct is a rare peritoneal surface malignancy. Round cell tumors final lymphoma metastasis free 30. Of the patients who develop this cancer, 90 percent are male, and 85 percent are caucasian. Mayo clinic doctors and scientists are studying new ways to diagnose and treat desmoplastic small round cell tumors. Desmoplastic small round cell tumor is a rare malignant neoplasm. Pathology outlines desmoplastic small round cell tumor. The radiology of desmoplastic small round cell tumour. Durable responses are exceptionally rare so further systemic. Desmoplastic small round cell tumour dsrct is a rare but frequently. The organ or specific tissue type of origin is unknown. 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. It tends to have a predilection for adolescent males with an annual incidence of about 0.
Desmoplastic small round cell tumors dsrct national. New hope unlimited has devoted years in antitumor protocol research for desmoplastic small round cell tumor treatment. Desmoplastic small round cell tumor in the abdomen and pelvis. This type of cancer usually begins in the abdomen or pelvis, and does not respond well to treatment. We conducted a phase 2 clinical trial utilizing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy crshipec with cisplatin for dsrct and pediatrictype abdominal sarcomas.
Desmoplastic small round cell tumor dsrct is a rare, aggressive and. Epidemiology they are usually seen in young adolescents and has male predominance with a mean survival of 23 years. 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. Pdf desmoplastic small round blue cell tumors dsrcts originate from a cell. After carefully evaluating your childs case, our team of experts will discuss a recommended course of treatment for dsrct. Desmoplastic small round cell tumor of meckels diverticulum a6yearoldboypresentedwith6monthhistoryofintermittent abdominal pain associated with episodic vomiting suggestive of subacute intestinal obstruction, which had worsened signi. It primarily affects children and young adults and is more common in males. This cancer usually affects children and young adults, and it is more common in males. Desmoplastic small round cell tumor dsrct is a rare abdominal disease affecting predominantly children and young adults. Intraabdominal desmoplastic small round cell tumor.
Desmoplastic small round cell tumors diagnosis and. Desmoplastic small round cell tumor dsrct is a soft tissue sarcoma of mesenchymal cell origin, presenting with multiple intraabdominal tumors. 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. Potential therapeutic genomic alterations in desmoplastic. Desmoplastic small round cell tumors of the pleura. Desmoplastic small round cell tumor dsrct is an aggressive malignant neoplasm that occurs in adolescents and young adults. Kate was 29 when she was diagnosed with desmoplastic small round cell tumor at the abdomen. 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. 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 dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen. Dsrct causes multiple tumors to form in the abdomen and pelvis area, but exactly where it begins usually is not known.
Desmoplastic small round cell tumor dsrct the brett. Mar 23, 2017 desmoplastic small round cell tumor dsrct is a soft tissue sarcoma of mesenchymal cell origin, presenting with multiple intraabdominal tumors. Connective tissues include fat, muscles, tendons, lymph and blood vessels, and nerves. 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 small round cell tumor is a rare malignant tumor that has a poor prognosis. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. Desmoplastic small round cell tumor dsrct is a rare but aggressive primitive malignant neoplasm that occurs mainly in adolescents and young adults. Desmoplastic small round cell tumor dsrct presents as a rare separate clinical pathological entity, and pleural dsrct is very rare.
Management of desmoplastic small round cell tumors in children and young adults. The desmoplastic small round cell tumor dsrct is a rare, aggressive, and malignant tumor, first described by gerald in 1989. Desmoplastic small round cell tumor genetic and rare. We report the case of a 26yearold woman who suffered from dyspepsia and abdominal pain for 2 months. 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. Desmoplastic small round cell tumors dsrct are a very rare type of soft tissue sarcoma. 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.
Nov 15, 2019 desmoplastic small round cell tumor dsrct is an extremely rare and highly aggressive malignancy. Desmoplastic small round cell tumor presenting as a neck. 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 tumors dsrct md anderson. A molecular hallmark of dsrct is the ewswt1 reciprocal translocation. 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. 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.
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. Primary desmoplastic smallroundcell tumor of the ovary. Trabectedin for desmoplastic small round cell tumours. Only a few hundred cases have been reported in worldwide literature. Nests and cords of cells surrounded by desmoplastic. Desmoplastic small round cell tumor of meckels diverticulum. The prolonged progressionfree survival period 15 months. Alkylator and anthracyclines based regimens are widely used as therapy and an initial response is common. Despite aggressive therapy, median survival ranges from. 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. Although we agree with the authors1 on the fact that data are scarce on the clinical management of advanced dsrct, we. Methods to identify patients with dsrct without extraperitoneal metastases epm who underwent ccrs between 1991 and 2015, a. Management of desmoplastic small roundcell tumors in children and young adults.
Primary desmoplastic small round cell tumor of the duodenum. Desmoplastic small round cell tumor dsrct is a type of cancer. Desmoplastic small round cell tumour successfully treated with. Primary desmoplastic small round cell tumor of the. The abdomen and pelvis are the sites most likely to be involved, while dsrct of the pleura is even more rare.
The majority of patients 90 percent are caucasian males between the ages of 10 and 30. Desmoplastic small round cell tumor is a very rare malignancy. Diagnosis is usually based on the tumors histological and immunohistochemical features. 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. A rare form of cancer, a desmoplastic small round cell tumor is most likely to develop in adolescents and young adults. To remove the cells, you might need surgery or a needle can be passed through the skin. Toomer a rare, aggressive cancer that usually affects young males and usually is located in the abdomen. Fewer than 500 cases have been reported in the literature 24. Optimal multimodal treatment for desmoplastic small round.
A 14yearold girl presented with intermittent epigastric pain. It affects mainly children and young adults and has a. It is usually found in the abdomen belly, but it can also occur in other parts of the body. It is an aggressive and often misdiagnosed neoplasm of children and young adult. Desmoplastic smallroundcell tumor dsrct is an extremely rare and highly aggressive malignancy that was first described in 1987 by sesterhenn et al. Desmoplastic smallroundcell tumor is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Desmoplastic smallroundcell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen. One patient was still diseasefree 20 months after treatment, although the rest. Sep 09, 2016 desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen.
Desmoplastic small round blue cell tumors dsrcts originate from a cell with multilineage potential. Desmoplastic small round cell tumor of the middle ear. Dsrct most often begins in the tissue lining the inside of the abdominal cavity, which is also known as the 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. The disease can affect any person at any age but is. Pdf round cell tumors as the name suggest are comprised round cells with increased nuclearcytoplasmic ratio.
Desmoplastic small round cell tumor radiology reference. 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. Abdominal desmoplastic small round cell tumor without. It usually happens in the tissue that lines the inside of the belly. Factors affecting overall survival os were assessed. The first case of a dsrct was recorded in 1989, and about 200 cases have been diagnosed since then. An unusual case study of a desmoplastic small round cell tumor presenting as a 3. The most common symptoms include pain or a mass in the abdomen. Hayesjordan4,5 morani ac, bathala tk, surabhi vr, et al. Cancer research is conducted in coordination with the mayo clinic cancer center. 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 tumors dsrct are a type of softtissue sarcoma.
Multimodal treatment combining chemotherapy, surgery and radiotherapy is the best option. The peritoneal cavity is the most common location of the tumor with multiple satellite nodules often noted at presentation. A case of longterm survival of metastatic desmoplastic small round. Desmoplastic small round cell tumor dsrct is a rare malignancy most commonly. Surgery is almost always part of treatment for dsrct. Case report solidpattern desmoplastic small round cell tumor of. I was an ambitious, driven doctor just two years away from finishing my training to become an elderly medicine consultant in the uk. Desmoplastic small round cell tumors dsrct are a type of rare, soft tissue sarcoma. Patients can present with many peritoneal implants. Desmoplastic small round cell tumor dsrct is a rare and highly. Round cell tumors as the name suggest are comprised round cells with increased nuclearcytoplasmic ratio. 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. Therapeutic trial for patients with ewing sarcoma family of.
Dsrct mainly develops in adolescent and young adults with a strong male predominance. Desmoplastic small round cell tumors are diagnosed with a procedure to remove a sample of cells for testing. It mainly affects adolescents and mostly involves the abdominal or pelvic peritoneum. The peritoneal cavity is the most common location of the tumor with. Desmoplastic small round cell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen. It is characterized by a distinct immunohistochemical profile and a recurrent, specific, chromosomal translocation.
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