Title: Fibrosarcoma

{{Short description|Malignant tumors composed of fibrous tissue}}
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| caption         = [[Micrograph]] of a tumour with the [[herringbone pattern]] as may be seen in fibrosarcoma. [[H&amp;E stain]].
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'''Fibrosarcoma''' (fibroblastic sarcoma) is a [[malignant]] [[mesenchymal]] [[tumour]] derived from fibrous connective tissue and characterized by the presence of immature [[cell growth|proliferating]] [[fibroblasts]] or undifferentiated [[anaplastic]] spindle cells in a [[storiform pattern]]. Fibrosarcomas mainly arise in people between the ages of 25 and 79,&lt;ref&gt;{{Cite web|url=https://www.encyclopedia.com/medicine/diseases-and-conditions/pathology/fibrosarcoma|title=fibrosarcoma|website=encyclopedia.com|language=en|access-date=2021-10-30}}&lt;/ref&gt; but may also occur in infants. It originates in fibrous tissues of the bone and invades long or flat bones such as the femur, tibia, and mandible. It also involves the periosteum and overlying muscle.

==Presentation==

===Adult-type===
Individuals presenting with fibrosarcoma are usually adults thirty to fifty-five years old, often presenting with pain. Among adults, fibrosarcomas develop equally in men and women. &lt;ref&gt;{{Cite web|url=https://www.encyclopedia.com/medicine/diseases-and-conditions/pathology/fibrosarcoma|title=Fibrosarcoma|website=encyclopedia.com|language=en|access-date=2021-10-30}}&lt;/ref&gt; 

===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 [[Chromosomal translocation|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.&lt;ref name=&quot;pmid23938179&quot;&gt;{{cite journal | vauthors = Walther C, Nilsson J, von Steyern FV, Wiebe T, Bauer HC, Nord KH, Gisselsson D, Domanski HA, Mandahl N, Mertens F | title = Cytogenetic and single nucleotide polymorphism array findings in soft tissue tumors in infants | journal = Cancer Genetics | volume = 206 | issue = 7–8 | pages = 299–303 | year = 2013 | pmid = 23938179 | doi = 10.1016/j.cancergen.2013.06.004 }}&lt;/ref&gt; 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.&lt;ref name=&quot;pmid27020209&quot;&gt;{{cite journal | vauthors = El Demellawy D, Cundiff CA, Nasr A, Ozolek JA, Elawabdeh N, Caltharp SA, Masoudian P, Sullivan KJ, de Nanassy J, Shehata BM | title = Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement | journal = Pathology | volume = 48 | issue = 1 | pages = 47–50 | year = 2016 | pmid = 27020209 | doi = 10.1016/j.pathol.2015.11.007 }}&lt;/ref&gt;&lt;ref name=&quot;pmid25295083&quot;&gt;{{cite journal | vauthors = Wang ZP, Li K, Dong KR, Xiao XM, Zheng S | title = Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature | journal = Oncology Letters | volume = 8 | issue = 5 | pages = 2007–2011 | year = 2014 | pmid = 25295083 | pmc = 4186628 | doi = 10.3892/ol.2014.2489 }}&lt;/ref&gt;&lt;ref name=&quot;pmid26206116&quot;&gt;{{cite journal | vauthors = Ud Din N, Minhas K, Shamim MS, Mushtaq N, Fadoo Z | title = Congenital (infantile) fibrosarcoma of the scalp: a case series and review of literature | journal = Child's Nervous System | volume = 31 | issue = 11 | pages = 2145–9 | year = 2015 | pmid = 26206116 | doi = 10.1007/s00381-015-2824-1 | s2cid = 25198570 }}&lt;/ref&gt;

== Pathology ==
The tumor may present different degrees of [[differentiation (cellular)|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 &quot;fish bone&quot; 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|biphenotypic sinonasal sarcoma]].{{cn|date=October 2020}}

== Diagnosis ==
Ancillary testing for fibrosarcoma includes [[Immunohistochemistry|IHC]], where [[vimentin]] is positive, [[cytokeratin]] and [[S-100 protein|S100]] are negative, and [[actin]] is variable.{{cn|date=October 2020}}

== In animals ==
[[File:Cat after Fibrosarcom op.jpg|thumb|right| [[Cat]] after fourth fibrosarcoma operation]]

===Dogs===

Fibrosarcoma occurs most frequently in the mouth in dogs.&lt;ref&gt;{{Cite web|url=http://vcahospitals.com/know-your-pet/oral-tumors-in-dogs-fibrosarcoma|title=Oral Tumors in Dogs - Fibrosarcomas|website=vca_corporate|language=en|access-date=2019-05-29}}&lt;/ref&gt; The tumor is locally invasive, and often recurs following surgery.&lt;ref&gt;{{Cite book|last=Fossum|first=Theresa Welch|title=Small Animal Surgery Textbook|publisher=Elsevier Health Sciences|year=2013|pages=414}}&lt;/ref&gt; [[Radiation therapy]] and [[chemotherapy]] are also used in treatment. Fibrosarcoma is also a rare bone tumor in dogs.&lt;ref name=&quot;Ettinger_1995&quot;&gt;{{cite book|author1=Ettinger, Stephen J. |author2=Feldman, Edward C. |title=Textbook of Veterinary Internal Medicine|edition=4th|publisher=W.B. Saunders Company|year=1995|isbn=0-7216-6795-3}}&lt;/ref&gt;

===Cats===

In cats, fibrosarcoma occurs on the skin. It is also the most common [[vaccine-associated sarcoma]].&lt;ref name=&quot;Ettinger_1995&quot; /&gt; In 2014, [[Merial]] launched Oncept IL-2 in Europe for the management of such feline fibrosarcomas.&lt;ref&gt;{{cite web|title=Merial Launches Oncept IL-2, The First Veterinary Immunotherapeutic Product In Europe For The Management Of Cancer In Pets|url=http://www.vetclick.com/news/merial-launches-oncept-il-2-the-first-veterinary-immunotherapeutic-product-in-europe-for-the-management-of-cancer-in-pets-p3213.php|website=www.vetclick.com/|access-date=February 23, 2016|date=February 23, 2016}}&lt;/ref&gt;

Bostock DE, et al. performed a study of cats that had fibrosarcomas excised and were followed for a minimum of three years, or until death. Two factors, tumor site and mitotic index, were found to be of prognostic significance, but tumor size, duration of growth, and histologic appearance were not. Following removal of fibrosarcomas from the flank in six cats, none died as a result of the tumor but 24 of 35 (70%) cats with fibrosarcoma in the skin of the head, back, or limbs were euthanized because of local recurrence, usually within nine months of surgery.&lt;ref&gt;{{Cite journal|last1=Bostock|first1=D. E.|last2=Dye|first2=M. T.|date=1979-10-01|title=Prognosis after surgical excision of fibrosarcomas in cats|journal=Journal of the American Veterinary Medical Association|volume=175|issue=7|pages=727–728|issn=0003-1488|pmid=528318}}&lt;/ref&gt;

== See also ==
* [[Benign fibrous histiocytoma]]
* [[Dermatofibrosarcoma protuberans]]
* [[Fibrous connective tissue]]
* [[Fibroma]]
* [[Malignant fibrous histiocytoma]]
* [[Neurofibrosarcoma]]

== References ==
{{Reflist}}

== External links ==
* [http://sarcomahelp.org/fibrosarcoma.html Fibrosarcoma of Bone: Review of a Rare Primary Malignancy of Bone] {{Webarchive|url=https://web.archive.org/web/20210309091148/http://sarcomahelp.org/fibrosarcoma.html |date=2021-03-09 }}
* [http://www.pathologyatlas.ro/fibrosarcoma.php Atlas of Pathology]

{{Medical resources
| DiseasesDB      =
| ICD10           = {{ICD10|C|49||c|49}} ([[ILDS]] C49.M42)
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| ICDO            = {{ICDO|8810|3}}
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| MeshID          = D005354
| SNOMED CT       = 53654007
| Orphanet        = 2030
}}
{{Soft tissue tumors and sarcomas}}
{{Osseous and chondromatous tumors}}

[[Category:Connective and soft tissue neoplasms]]
[[Category:Sarcoma]]
[[Category:Osseous and chondromatous neoplasia]]
[[Category:Cancer in cats]]
[[Category:Cancer in dogs]]