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dangerous to health; characterized by progressive and uncontrolled growth (especially of a tumor)
(of meat) full of sinews; especially impossible to chew (同)sinewy, stringy, unchewable

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悪意のある,敵意のある / (病気が)悪性の
繊維の,繊維状(質)の

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/10/25 19:30:47」(JST)

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Pleomorphic undifferentiated sarcoma (abbreviated PUS), also undifferentiated pleomorphic sarcoma and previously malignant fibrous histiocytoma (abbreviated MFH), is a type of soft tissue sarcoma.

It is considered a diagnosis of exclusion for sarcomas that cannot be more precisely categorized.^[1]^[2]

Epidemiology

PUS is regarded as the most common soft tissue sarcoma of late adult life. It rarely occurs in children.^[3] It occurs more often in Caucasians than in those of African or Asian descent and is a male-predominant disease, afflicting two males for every female.

Presentation

PUS occurs most commonly in the extremities and retroperitoneum, but has been reported in other sites. Metastasis occurs most frequently in the lungs (90%), bones (8%), and liver (1%).

In the extremities, it presents itself as a painless enlarging soft tissue mass.

Diagnosis

It can be detected by magnetic resonance imaging (MRI), but a biopsy is required for the definitive diagnosis. MRI findings typically show a well-circumscribed mass that is dark on T1-weighted images and bright on T2-weighted images. Central necrosis is often present and identifiable by imaging, especially in larger masses.

Pathology

Pleomorphic undifferentiated sarcomas are, by definition, undifferentiated, meaning (as the name implies) that they do not bear a resemblance to any normal tissue.

The histomorphology, otherwise, is characterized by high cellularity, marked nuclear pleomorphism, usually accompanied by abundant mitotic activity (including atypical mitoses), and a spindle cell morphology. Necrosis is common and characteristic of high grade lesions.

Treatment

Treatment consists of surgical excision (the extent of which ranges from tumor excision to limb amputation, depending on the tumor) and in almost all cases radiation. Radiation eliminates the need for limb amputation and there is level I evidence to show that it leads to equivalent rates of survival (Rosenberg et al. NCI Canada). Radiation may be delivered either pre-op or post-op depending on surgeon and multidisciplinary tumor board's recommendations. Radiation can be omitted for low grade, Stage I excised tumors with >1cm margin (NCCN). Chemotherapy remains controversial in MFH.

The usual site of metastatic disease is the lungs, and metastases should be resected if possible. Unresectable or inoperable lung metastasis may be treated with stereotactic body radiation therapy (SBRT) with excellent local control. However, neither surgery nor SBRT will prevent emergence of additional metastasis elsewhere in the lung. Therefore, role of chemotherapy needs to be further explored to address systemic metastasis.

Prognosis

Prognosis depends on the primary tumor grade (appearance under the microscope as judged by a pathologist), size, resectability (whether it can be completely removed surgically), and presence of metastases. The five-year survival ranges from 35 to 60%.

Human-human transmission

There is a case report of human-human transmission of cancer, in a surgeon who injured his hand while working on a PUS case.^[4]

References

^ Matushansky I, Charytonowicz E, Mills J, Siddiqi S, Hricik T, Cordon-Cardo C (August 2009). "MFH classification: differentiating undifferentiated pleomorphic sarcoma in the 21st Century". Expert Rev Anticancer Ther 9 (8): 1135–44. doi:10.1586/era.09.76. PMC 3000413. PMID 19671033.
^ "Neoplasia".
^ Alaggio R, Collini P, Randall RL, Barnette P, Million L, Coffin CM (2010). "Undifferentiated high-grade pleomorphic sarcomas in children: a clinicopathologic study of 10 cases and review of literature". Pediatr. Dev. Pathol. 13 (3): 209–17. doi:10.2350/09-07-0673-OA.1. PMID 20055602.
^ Gärtner, Hermine-Valeria; Christian Seidl; Christine Luckenbach; Georg Schumm; Erhard Seifried; Horst Ritter; Burkhard Bultmann (1996-11-14). "Genetic Analysis of a Sarcoma Accidentally Transplanted from a Patient to a Surgeon". N Engl J Med 335 (20): 1494–1497. doi:10.1056/NEJM199611143352004. PMID 8890100. Retrieved 2010-01-05.

External links

Malignant Fibrous Histiocytoma (MFH) - Sarcoma Learning Center

UpToDate Contents

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English Journal

Angiomatoid Fibrous Histiocytoma With Prominent Myxoid Stroma: A Case Report and Review of the Literature.

Justin Wong SB1, Wee A, Puhaindran ME, Pang B, Lee VK.
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PMID 25406850

A Clinical Investigation of Oral Sarcomas at Multi-institutions Over the Past 30 Years.

Sumida T1, Otawa N2, Kamata YU2, Yamada T3, Uchida K4, Nakano H5, Hamakawa H6, Yamamoto T7, Ueyama Y4, Mori Y5.
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BACKGROUND/AIM: Sarcoma of the oral cavity is rare accounting for around 1% of all malignant oral tumors. The purpose of this study was to find important prognostic factors for patients with oral sarcoma.PATIENTS AND METHODS: The study included 1,643 patients examined from April 1980 to March 2010 a
PMID 26168500

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Brewer JD1, Shanafelt TD2, Call TG2, Cerhan JR3, Roenigk RK1, Weaver AL4, Otley CC1.
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PMID 25772131

Japanese Journal

腹腔鏡下に摘出可能であった後腹膜悪性線維性組織球腫の1例

光成健輔,杠葉美樹,木原敏晴 [他]
西日本泌尿器科 = The Nishinihon journal of urology 76(1), 25-29, 2014-01
NAID 40019948937

症例報告陰茎原発悪性線維性組織球症/未分化多形細胞肉腫の1例

杉浦晋平,蓼沼知之,坂田綾子 [他]
日本泌尿器科学会雑誌 = The Japanese journal of urology 104(6), 706-711, 2013-11
NAID 40019885477

腎被膜に発生した悪性線維性組織球腫の1例

松下慎,岡田宜之,川村憲彦 [他],氏家剛,任幹夫,辻畑正雄
泌尿器科紀要 = Acta urologica Japonica 59(11), 733-736, 2013-11
… Pathological examination revealed the malignant fibrous histiocytoma (MFH) arising from the renal capsule, striform-pleomorphic type, low grade malignancy. …
NAID 120005350251

Related Pictures

myxoid type malignant fibrous histiocytoma - PukiWiki Histiocytoma, Malignant Fibrous; Malignant Fibrous Histiocytoma Angiomatoid fibrous histiocytoma - Wikipedia

★リンクテーブル★

リンク元	「悪性線維性組織球腫」「MFH」
関連記事	「fibrous」「malignant」

「悪性線維性組織球腫」

Pleomorphic undifferentiated sarcoma
	Micrograph of a pleomorphic undifferentiated sarcoma. H&E stain.
Classification and external resources
Specialty	Oncology
ICD-10	C49 (ILDS C49.M10)
DiseasesDB	31471
eMedicine	radio/420
MeSH	D051677