desmoplastic small round cell tumor

同

DSRCT

WordNet

1. wind around; move along a circular course; "round the bend"
2. become round, plump, or shapely; "The young woman is fleshing out" (同)flesh out, fill out
3. make round; "round the edges" (同)round_out, round_off
4. the usual activities in your day; "the doctor made his rounds" (同)daily round
5. a charge of ammunition for a single shot (同)unit of ammunition, one shot
6. (often plural) a series of professional calls (usually in a set order); "the doctor goes on his rounds first thing every morning"; "the postmans rounds"; "we enjoyed our round of the local bars"
7. a serving to each of a group (usually alcoholic); "he ordered a second round" (同)round of drinks
8. a partsong in which voices follow each other; one voice starts and others join in one after another until all are singing different parts of the song at the same time; "they enjoyed singing rounds" (同)troll
9. a cut of beef between the rump and the lower leg
10. an outburst of applause; "there was a round of applause"
11. the course along which communications spread; "the story is going the rounds in Washington"
12. from beginning to end; throughout; "It rains all year round on Skye"; "frigid weather the year around" (同)around
13. having a circular shape (同)circular
14. pronounce with rounded lips (同)labialize, labialise
15. (mathematics) expressed to the nearest integer, ten, hundred, or thousand; "in round numbers"
16. slight or limited; especially in degree or intensity or scope; "a series of death struggles with small time in between"
17. a garment size for a small person
18. the slender part of the back
19. limited or below average in number or quantity or magnitude or extent; "a little dining room"; "a little house"; "a small car"; "a little (or small) group" (同)little
20. have fine or very small constituent particles; "a small misty rain"
21. on a small scale; "think small"
22. small room in which a monk or nun lives (同)cubicle
23. a device that delivers an electric current as the result of a chemical reaction (同)electric cell
24. a room where a prisoner is kept (同)jail cell, prison cell
25. (biology) the basic structural and functional unit of all organisms; they may exist as independent units of life (as in monads) or may form colonies or tissues as in higher plants and animals
26. any small compartment; "the cells of a honeycomb"
27. a small unit serving as part of or as the nucleus of a larger political movement (同)cadre
28. (mathematics) a miscalculation that results from rounding off numbers to a convenient number of decimals; "the error in the calculation was attributable to rounding"; "taxes are rounded off to the nearest dollar but the rounding error is surprisingly small" (同)rounding error
29. an abnormal new mass of tissue that serves no purpose (同)tumour, neoplasm

PrepTutorEJDIC

1. …『を[ぐるっと]回って』,を一周して / …『を曲がって』 / …『の周囲に』,を取り巻いて / …『のあちこちに』,の辺りに / …『ころ』 / (ぐるりと)回って,巡って / 周囲が(に) / あちこち[に],方々[に];行き渡って / 回り道して / (ある場所へ)回って / 『球状の』,丸い / (タイヤ・輪のように)円形の,丸い / (断面の丸い)円筒(円錐)形の / (肩・筆跡などが)丸みのある,丸々とした / 《名詞の前にのみ用いて》(数量が)完全の,ちょうどの / 『丸い物』;円,輪;球,円筒形 / (中心・軸をまわる)『回転』,(天体の)回転運動 / 《しばしば複数形で》(職務上などの)巡回,見回り / 《単数形で》(行い・でき事の一つの)連続;繰り返り;(…が)ひとしきり続くこと《+『of』+『名』》 / (ボクシングの一勝負の中の)『ラウンド』,回,(ゴルフデ全部のホールを回る)ラウンド,(カードゲームの)一回り / 輪唱 / (銃砲の)1回の射撃,一斉射撃 / (射撃用弾薬の)1回分 / (牛のもも肉の)丸切り,(パンの)丸切り《+『of』+『名』》 / 輪舞,円舞 / …‘を'『丸くする』,‘に'丸みをつける / …‘を'『回る』,曲がる / 〈端数をもつ数字〉‘を'端数を切り捨てて(概数で)表す《+『down』+『名』+『to』+『名』》 / …‘を'完成する,仕上げる / …‘を'くちびるを丸くして発音する / 『丸くなる』 / 回る,曲る / 完成する
2. (大きさが)『小さい』,小形の;(量が)『少ない』,わずかな / 『取るに足りない』,ささいな(trivial) / 《名詞の前にのみ用いて》(仕事・活動などが)『小規模の』,ささやかな / 心が狭い,利己的な / (音・声が)弱い,小さい / (文字が)小型の,小文字の / 《the~》小さいもの;(…の)細い部分《+『of』+『名』》 / 《複数形で》《英》(衣類・ハンカチなどの)小物,小間物 / 小さく,細かく / (声などが)低く,弱く / 小規模に,こぢんまりと
3. (刑務所の)『独房』;(修道院の)小さい独居室 / (ミツバチの)みつ房,巣穴 / 小さい部屋 / 『細胞』 / 電池 / 花粉室 / (共産党などの)細胞

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/29 21:58:58」(JST)

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Desmoplastic small-round-cell tumor
Micrograph of a desmoplastic small round cell tumor, showing the characteristic desmoplastic stroma and angulated nests of small round cells. H&E stain.
Classification and external resources
Specialty	Oncology
ICD-O	8806/3

Display of small round blue cells characteristic of desmoplastic small-round-cell tumor.

Cell exhibiting blue oval and round shapes of desmoplastic small-round blue cell tumor

Desmoplastic small-round-cell tumor is an aggressive and rare cancer that primarily occurs as masses in the abdomen. 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. Reported sites of metatastic spread include the liver, lungs, lymph nodes, brain, skull, and bones.

The tumor is classified as a soft tissue sarcoma. It is considered a childhood cancer that predominantly strikes boys and young adults. The disease rarely occurs in females, but when it does the tumors can be mistaken for ovarian cancer.

In dogs, mast cell tumors are the most frequent round cell tumor.

Causes

There are no known risk factors that have been identified specific to the disease. The tumor appears to arise from the primitive cells of childhood, and is considered a childhood cancer.

Research has indicated that there is a chimeric relationship between desmoplastic small-round-cell tumor (DSRCT) and Wilms' tumor and Ewing's sarcoma. Together with neuroblastoma and non-Hodgkin's lymphoma, they form the small cell tumors.

DSRCT is associated with a unique chromosomal translocation (t11;22)(p13:q12) resulting in an EWS/WT1 transcript that is diagnostic of this tumor. This transcript codes for a protein that acts as a transcriptional activator that fails to suppress tumor growth.

The EWS/WT1 translocation product targets ENT4. ENT4 is also known as PMAT.

Symptoms

There are few early warning signs that a patient has a DSRCT. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by physicians. The abdominal masses can grow to enormous size before being noticed by the patient. The tumors can be felt as hard, round masses by palpating the abdomen.

First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and/or cachexia.

Other reported symptoms include unknown lumps, thyroid conditions, hormonal conditions, blood clotting, kidney or urological problems, testicle, breast, uterine, vaginal, or ovarian masses

Differential Diagnoses

Because this is a rare tumor, not many family physicians or oncologists are familiar with this disease. DSRCT in young patients can be mistaken for other abdominal tumors including rhabdomyosarcoma, neuroblastoma, and mesenteric carcinoid. In older patients DSRCT can resemble lymphoma, peritoneal mesothelioma, and peritoneal carcinomatosis. In males DSRCT may be mistaken for germ cell or testicular cancer while in females DSRCT can be mistaken for Ovarian cancer. DSRCT shares characteristics with other small-round blue cell cancers including Ewing's sarcoma, acute leukemia, small cell mesothelioma, neuroblastoma, primitive neuroectodermal tumor, rhabdomyosarcoma, and Wilms' tumor.

Pathology

The entity was first described by pathologists William L. Gerald and Juan Rosai in 1989. Pathology reveals well circumscribed solid tumor nodules within a dense desmoplastic stroma. Often areas of central necrosis are present. Tumor cells have hyperchromatic nuclei with increased nuclear/cytoplasmic ratio.

On immunohistochemistry, these cells have trilinear coexpression including the epithelial marker cytokeratin, the mesenchymal markers desmin and vimentin, and the neuronal marker neuron-specific enolase. Thus, although initially thought to be of mesothelial origin due to sites of presentation, it is now hypothesized to arise from a progenitor cell with multiphenotypic differentiation.

Treatment

DSRCT is frequently misdiagnosed. Adult patients should always be referred to a sarcoma specialist. This is an aggressive, rare, fast spreading tumor and both pediatric and adult patients should be treated at a sarcoma center.

There is no standard protocol for the disease; however, recent journals and studies have reported that some patients respond to high dose (P6 Protocol) chemotherapy, maintenance chemotherapy, debulking operation, cytoreductive surgery, and radiation therapy. Other treatment options include: hematopoietic stem cell transplantation, intensity-modulated radiation Therapy, radiofrequency ablation, stereotactic body radiation therapy, intraperitoneal hyperthermic chemoperfusion, and clinical trials.

Prognosis

The prognosis for DSRCT remains poor. The 5-year survival rate of DRSCT is only approximately 15%. Prognosis depends upon the stage of the cancer. Because the disease can be misdiagnosed or remain undetected, tumors frequently grow large within the abdomen and metastasize or seed to other parts of the body.

There is no known organ or area of origin. DSRCT can metastasize through lymph nodes or the blood stream. Sites of metastasis include the spleen, diaphragm, liver, large and small intestine, lungs, central nervous system, bones, uterus, bladder, genitals, abdominal cavity, and the brain.

A multi-modality approach of high dose chemotherapy, aggressive surgical resection, radiation, and stem cell rescue improves survival for some patients. Reports have indicated that patients will initially respond to first line chemotherapy and treatment but that relapse is common.

Some patients in remission or with inoperable tumor seem to benefit from long term low dose chemotherapy, turning DSRCT into a chronic disease.

Research

The Stehlin Foundation currently offers DSRCT patients the opportunity to send samples of their tumors free of charge for testing. Research scientists are growing the samples on nude mice and testing various chemical agents to find which are most effective against the individual's tumor.

Patients with advanced DSRCT may qualify to participate in clinical trials that are researching new drugs to treat the disease.

Alternative names

This disease is also known as: desmoplastic small round blue cell tumor; intraabdominal desmoplastic small round blue cell tumor; desmoplastic small cell tumor; desmoplastic cancer; desmoplastic sarcoma; DSRCT.

There is no connection to peritoneal mesothelioma which is another disease sometimes described as desmoplastic.

References

External links

• humpath #2002 (Pathology images)
• Desmoplastic Small Round Cell Tumor in the Sarcoma Learning Center
• Desmoplastic Small Round Cell Tumor
• Desmoplastic Small Round Blue Cell Tumor

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 軟部組織肉腫の臨床所見、病理組織学的検査、診断的評価、および病期分類 clinical presentation histopathology diagnostic evaluation and staging of soft tissue sarcoma
• 2. 転移性軟部組織肉腫の全身治療 systemic treatment of metastatic soft tissue sarcoma
• 3. 軟部および骨肉腫の病原因子 pathogenetic factors in soft tissue and bone sarcomas
• 4. ユーイング肉腫ファミリーの腫瘍の疫学、病理、および分子遺伝学 epidemiology pathology and molecular genetics of the ewing sarcoma family of tumors
• 5. 精巣腫瘍の解剖学および病理 anatomy and pathology of testicular tumors

English Journal

• Whole Abdominopelvic Intensity-Modulated Radiation Therapy for Desmoplastic Small Round Cell Tumor after Surgery.

• Pinnix CC, Fontanilla HP, Hayes-Jordan A, Subbiah V, Bilton SD, Chang EL, Grosshans DR, McAleer MF, Sulman EP, Woo SY, Anderson P, Green HL, Mahajan A.SourceDepartment of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
• International journal of radiation oncology, biology, physics.Int J Radiat Oncol Biol Phys.2011 Nov 19. [Epub ahead of print]
• PURPOSE: Desmoplastic small round cell tumor (DSCRT) is an uncommon pediatric tumor with a poor prognosis. Aggressive multimodality therapy is the current treatment approach; however. treatment toxicity is of concern. We report our results with whole abdominopelvic intensity-modulated radiation ther
• PMID 22104361

• WT1 expression in the female genital tract.

• Bárcena C, Oliva E.SourcePathology Department, Massachusetts General Hospital, Boston, USA.
• Advances in anatomic pathology.Adv Anat Pathol.2011 Nov;18(6):454-65.
• The Wilms tumor gene 1 (WT1) has been reported in normal tissues and many neoplasms of the female genital tract. This review discusses WT1 expression in the female genital tract and its potential utility in the differential diagnosis of neoplasms that occur at this location. WT1 is of value in the d
• PMID 21993272

Japanese Journal

• P15-05 desmoplastic small round cell tumor (DSRCT)に対する温熱化学療法を併用したcytoreductive surgery(ポスターセッション15 腫瘍2)

• 北河 徳彦,大浜 用克,新開 真人,武 浩志,薄井 佳子,小坂 太一郎,湊 雅嗣,大澤 絵都子,浜之上 聡,気賀澤 寿人,田中 水緒,田中 祐吉
• 日本小児外科学会雑誌 48(3), 558, 2012-05-01
• NAID 110009582134

• E-17)管腔形成の目立つdesmoplastic small round cell tumorの一例(E 骨軟部腫瘍,2010年度小児腫瘍症例検討会)

• 井上 健,久保 勇記,福島 裕子,小林 庸次
• 小児がん : 小児悪性腫瘍研究会記録 48(1), 56, 2011-02-25
• NAID 110008608604

• PO-039 Desmoplastic small round cell tumorの1例(腫瘍(肝・胚細胞腫瘍・直腸),ポスターセッション,病気の子供達に笑顔 小児外科に夢そして革新を,第47回 日本小児外科学会学術集会)

• 正畠 和典,中村 哲郎,東 孝,大野 耕一,山田 弘人,林 宏昭
• 日本小児外科学会雑誌 46(3), 618, 2010-05-20
• NAID 110008016089

Related Links

• Desmoplastic Small Round Cell Tumor

Desmoplastic Small Round Cell Tumor is an aggressive cancer that usually occurs in multiple masses in the abdomen and affects the lymph nodes and lining of the pelvis and abdomen. The most common symptoms are abdominal ...

• Desmoplastic small round cell tumorとは - goo Wikipedia ...

Desmoplastic small round cell tumor is classified as a soft tissue sarcoma. It is an aggressive and rare tumor that primarily occurs as masses in the abdomen. [1] Other areas affected may include the lymph nodes, the lining of the abdomen ...

Related Pictures

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★リンクテーブル★

リンク元	「DSRCT」「線維形成性小円形細胞腫瘍」
関連記事	「round」「small」「tumor」「desmoplastic」「cell」

「DSRCT」

　　[★]

同

desmoplastic small round cell tumor

「線維形成性小円形細胞腫瘍」

　　[★]

英

desmoplastic small round cell tumor

「round」

　　[★]

• n.

• 回転、ラウンド、一巡、回診

• adj.

• 円形の

関

circular、convolution、revolution、revolve、roll、rolling、rotate、rotation、rotatory、turn、turning

　 　 　 　 　 　 　 　 　 　 　 　 　 　 　 　

「small」

　　[★]

• adj.

• 小さい

関

little、petit、tiny

　 　 　 　 　 　 　

「tumor」

　　[★]

• n.

• 腫瘍、腫瘤、腫脹

　

「desmoplastic」

　　[★]

• 線維形成性の

関

fibrogenic

「cell」

　　[★] 細胞