fibrosarcoma

線維肉腫

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/09/02 16:21:02」(JST)

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Fibrosarcoma
Micrograph of a tumour with the herringbone pattern as may be seen in fibrosarcoma. H&E stain.
Classification and external resources
Specialty	oncology
ICD-10	C49 (ILDS C49.M42)
ICD-O	M8810/3
MeSH	D005354

Fibrosarcoma (fibroblastic sarcoma) is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. It is usually found in males aged 30 to 40 . It originates in fibrous tissues of the bone and invades long or flat bones such as femur, tibia, and mandible. It also involves periosteum and overlying muscle.

Pathology

The tumor may present different degrees of differentiation: low grade (differentiated), intermediate malignancy and high malignancy (anaplastic). Depending on this differentiation, tumour cells may resemble mature fibroblasts (spindle-shaped), secreting collagen, with rare mitoses. These cells are arranged in short fascicles which split and merge, giving the appearance of "fish bone" known as a herringbone pattern. Poorly differentiated tumors consist in more atypical cells, pleomorphic, giant cells, multinucleated, numerous atypical mitoses and reduced collagen production. Presence of immature blood vessels (sarcomatous vessels lacking endothelial cells) favors the bloodstream metastasizing. There are many tumors in the differential diagnosis, including spindle cell melanoma, spindle cell squamous cell carcinoma, synovial sarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor and biphenotypic sinonasal sarcoma.

Clinical presentation in humans

Adult-type

Individuals presenting with fibrosarcoma are usually adults aged thirty to fifty five years, often presenting with pain. In adults, males have a higher incidence for fibrosarcoma than females.

Infantile-type

In infants, fibrosarcoma (often termed congenital infantile fibrosarcoma) is usually congenital. Infants presenting with this fibrosarcoma usually do so in the first two years of their life. Cytogenetically, congenital infantile fibrosarcoma is characterized by the majority of cases having a translocation between chromosomes 12 and 15 (notated as t(12;15)(p13;q25)) that results in formation of the fusion gene, ETV6-NTRK3, plus individual cases exhibiting trisomy for chromosomes 8, 11, 17, or 20. The histology, association with the ETV6-NRTK3 fusion gene as well as certain chromosome trisomies, and the distribution of markers for cell type (i.e. cyclin D1 and Beta-catenin) within this tumor are similar to those found in the cellular form of mesoblastic nephroma. Indeed, mesoblastic nephroma and congenital infantile sarcoma appear to be the same disease with the exception that mesoblastic lymphoma originates in the kidney whereas congenital infantile sarcoma originates in non-renal tissues.

Ancillary testing

Ancillary testing for fibrosarcoma includes IHC, where vimentin is positive, cytokeratin and S100 are negative, and actin is variable.

Fibrosarcoma in dogs and cats

Cat after 4th Fibrosarcoma OP

Fibrosarcoma occurs most frequently in the mouth in dogs . The tumor is locally invasive, and often recurs following surgery . Radiation therapy and chemotherapy are also used in treatment. Fibrosarcoma is also a rare bone tumor in dogs.

In cats, fibrosarcoma occurs on the skin. It is also the most common vaccine-associated sarcoma. In 2014, Merial launched Oncept IL-2 in Europe for the management of such feline fibrosarcomas.

See also

• Benign fibrous histiocytoma
• Dermatofibrosarcoma protuberans
• Fibrous connective tissue
• Fibroma
• Malignant fibrous histiocytoma
• Neurofibrosarcoma

External links

• Fibrosarcoma of Bone: Review of a Rare Primary Malignancy of Bone
• Atlas of Pathology

UpToDate Contents

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• 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. 新生児および幼児における皮膚結節 skin nodules in newborns and infants
• 3. 隆起性皮膚線維肉腫：疫学、病因、臨床症状、診断、および病期分類 dermatofibrosarcoma protuberans epidemiology pathogenesis clinical presentation diagnosis and staging
• 4. 隆起性皮膚線維肉腫：治療 dermatofibrosarcoma protuberans treatment
• 5. 四肢および胸壁の原発性軟部組織肉腫の局所治療 local treatment for primary soft tissue sarcoma of the extremities and chest wall

English Journal

• Applications of calcium electroporation to effective apoptosis induction in fibrosarcoma cells and stimulation of normal muscle cells.

• Zielichowska A1, Daczewska M1, Saczko J2, Michel O2, Kulbacka J3.
• Bioelectrochemistry (Amsterdam, Netherlands).Bioelectrochemistry.2016 Jun;109:70-8. doi: 10.1016/j.bioelechem.2016.01.005. Epub 2016 Jan 27.
• The electroporation (EP) supports various types of anticancer therapies by the selective transport of cytostatics. Increase in intracellular calcium level by EP may be a new approach to fibrosarcoma treatment. Calcium is one of the most important factors of cell proliferation, differentiation and ce
• PMID 26874618

• Squaraines bearing halogenated moieties as anticancer photosensitizers: Synthesis, characterization and biological evaluation.

• Serpe L1, Ellena S2, Barbero N3, Foglietta F1, Prandini F1, Gallo MP4, Levi R4, Barolo C2, Canaparo R1, Visentin S5.
• European journal of medicinal chemistry.Eur J Med Chem.2016 May 4;113:187-97. doi: 10.1016/j.ejmech.2016.02.035. Epub 2016 Feb 20.
• We report the synthesis and characterization of a series of symmetrical indolenine-based squaraine dyes along with the evaluation of their singlet oxygen generation efficiency. The photodynamic activity of these new photosensitizers has been evaluated on a human tumor fibrosarcoma (HT-1080) cell lin
• PMID 26942626

• Potential of activatable FAP-targeting immunoliposomes in intraoperative imaging of spontaneous metastases.

• Tansi FL1, Rüger R2, Böhm C3, Kontermann RE4, Teichgraeber UK3, Fahr A5, Hilger I6.
• Biomaterials.Biomaterials.2016 May;88:70-82. doi: 10.1016/j.biomaterials.2016.02.028. Epub 2016 Feb 23.
• Despite intensive research and medical advances met, metastatic disease remains the most common cause of death in cancer patients. This results from late diagnosis, poor therapeutic response and undetected micrometastases and tumor margins during surgery. One approach to overcome these challenges in
• PMID 26945457

Japanese Journal

• Caffeine activates tumor suppressor PTEN in sarcoma cells

• Miwa Shinji,Sugimoto Naotoshi,Shirai Toshiharu,Hayashi Katsuhiro,Nishida Hideji,Ohnari Issei,Takeuchi Akihiko,Yachie Akihiro,Tsuchiya Hiroyuki
• International Journal of Oncology 39(2), 465-472, 2011-04
• … We found that caffeine induced increased intracellular cAMP levels, PTEN activation and Akt inactivation, which together prevented proliferation of human osteosarcoma cells (MG63) and fibrosarcoma cells (HT1080). …
• NAID 120003239459

• 下顎骨に発生したエナメル上皮線維肉腫の1例

• 美馬 孝至,鏡内 肇,横田 祐介 [他]
• 日本口腔外科学会雑誌 57(2), 78-82, 2011-02
• NAID 40018730611

Related Links

• Fibrosarcoma: Background, History of the Procedure ...

Fibrosarcoma is a tumor of mesenchymal cell origin that is composed of malignant fibroblasts in a collagen background. It can occur as a soft-tissue mass or as a primary or secondary bone tumor.

• Fibrosarcoma 線維肉腫 - PukiWiki

乳児型線維肉腫 Fibrosarcoma, Infantile/congenital Variant (IFS) † IFSは組織学的に成人型線維肉腫と同じものであるが, ほとんど転移を起こさず, 線維腫症と類似した自然経過をしめすなど全く異なる臨床経過をとる。IFSは形態的にも遺伝子的 ...

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fibrosarcoma, brosarcoma

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線維形成肉腫 fibroplastisches Sarkom

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