Difference between revisions of "Fibroblastic/myofibroblastic tumours"

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This article covers '''fibroblastic/myofibroblastic tumours'''.  These tumours fit into the larger category of [[soft tissue lesions]].
This article covers '''fibroblastic/myofibroblastic tumours'''.  These tumours fit into the larger category of [[soft tissue lesions]].


==List of tumours==
=List of tumours=


===Benign===
===Benign===
WHO classification:<ref name=Ref_WMSP601>{{Ref WMSP|601}}</ref>
WHO classification:<ref name=Ref_WMSP601>{{Ref WMSP|601}}</ref>
*[[Nodular fasciitis]].
*[[Nodular fasciitis]].
*Proliferative fasciitis.
*[[Proliferative fasciitis]].
*Proliferative myositis.
*[[Proliferative myositis]].
*[[Myositis ossificans]].
*[[Myositis ossificans]].
*Ischemic fasciitis.
*Ischemic fasciitis.
*Elastofibroma.
*[[Elastofibroma]].
*Myofibroma.
*[[Myofibroma]].
*Fibromatosis coli.
*Fibromatosis colli. <!-- NOT 'coli' -->
*Inclusion body fibromatosis.
*Inclusion body fibromatosis.
*Fibroma of tendon sheath.
*Fibroma of tendon sheath.
*Calcifying aponeurotic fibroma.
*Calcifying aponeurotic fibroma.
*Angiomyofibroblstoma.
*[[Angiomyofibroblastoma]].
*Cellular angiofibroma.
*[[Cellular angiofibroma]].
*Nuchal-type fibroma.
*Nuchal-type fibroma.
*Gardner fibroma.
*Gardner fibroma.
Line 25: Line 25:
*Fibrous hamartoma of infancy.
*Fibrous hamartoma of infancy.
*Juvenile hyaline fibromatosis.
*Juvenile hyaline fibromatosis.
*Desmoplastic fibroblastoma.
*[[Desmoplastic fibroblastoma]].
*Mammary-type myofibroblastoma.
*Mammary-type myofibroblastoma.


Line 32: Line 32:
*Superfical fibromatosis.
*Superfical fibromatosis.
*[[Desmoid-type fibromatosis]].
*[[Desmoid-type fibromatosis]].
*Lipofibromatosis.
*[[Lipofibromatosis]].


===Occasionally metastasizing===
===Occasionally metastasizing===
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*[[Solitary fibrous tumour]].
*[[Solitary fibrous tumour]].
*[[Inflammatory myofibroblastic tumour]].
*[[Inflammatory myofibroblastic tumour]].
*Low-grade myofibroblastic sarcoma.
*[[Low-grade myofibroblastic sarcoma]].
*Myxoinflammatory fibroblastic sarcoma.
*Myxoinflammatory fibroblastic sarcoma.
*[[Infantile fibrosarcoma]].
*[[Infantile fibrosarcoma]].
Line 49: Line 49:
*Sclerosing epithelioid fibrosarcoma.
*Sclerosing epithelioid fibrosarcoma.


==Inflammatory myofibroblastic tumour==
=Non-malignant=
*[[AKA]] inflammatory pseudotumour, AKA inflammatory fibrosarcoma.<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
==Proliferative fasciitis==
===General===
===General===
*Mostly benign.
*Benign.
*Children & young adults.
*May mimic a sarcoma.<ref name=pmid1058047>{{Cite journal  | last1 = Chung | first1 = EB. | last2 = Enzinger | first2 = FM. | title = Proliferative fasciitis. | journal = Cancer | volume = 36 | issue = 4 | pages = 1450-8 | month = Oct | year = 1975 | doi =  | PMID = 1058047 }}</ref>
*Classically located in mesentery of ileocolic region or small bowel.<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
 
Clinical:
*Solid subcutaneous nodule.
*Rapid growth.
*May be painful.
 
===Gross===
*Classically upper and lower extremities.<ref name=pmid1058047/>
*Poorly demarcated.


===Microscopic===
===Microscopic===
Features:<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
Features:<ref name=pmid1566969>{{Cite journal  | last1 = Meis | first1 = JM. | last2 = Enzinger | first2 = FM. | title = Proliferative fasciitis and myositis of childhood. | journal = Am J Surg Pathol | volume = 16 | issue = 4 | pages = 364-72 | month = Apr | year = 1992 | doi =  | PMID = 1566969 }}</ref>
*Inflammation:
*Large polygonal (ganglion-like) and/or spindled cells with:
**Plasma cells.
**[[Vesicular nuclei|Vesicular (clear) nuclei]].
**Lymphocytes.
**Prominent nucleoli.
**Eosinophils.
*+/-Binucleation.
*Spindle cells without atypia.
*Loose myxoid stroma.
*+/-Fasciular architecture.
*Frequent typical mitoses.
*Mitoses -- though none atypical.
**'''No''' atypical mitoses.
*+/-Necrosis.
*+/-Hemorrhage.
*Calcifications.


DDx:
DDx:
*[[Calcifying fibrous pseudotumour]] (has psammomatous calcifications).
*[[Inflammatory myofibroblastic tumour]].<ref name=pmid17938159>{{Cite journal  | last1 = Gleason | first1 = BC. | last2 = Hornick | first2 = JL. | title = Inflammatory myofibroblastic tumours: where are we now? | journal = J Clin Pathol | volume = 61 | issue = 4 | pages = 428-37 | month = Apr | year = 2008 | doi = 10.1136/jcp.2007.049387 | PMID = 17938159 }}</ref>
*[[Inflammatory fibroid tumour]].
 
*[[Nodular fasciitis]].
Images:
*[http://www.medicine.virginia.edu/clinical/departments/pathology/Case%20Studies/known-pathology-cases/95_4-page Proliferative fasciitis - several images (virginia.edu)].
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090721114221299 Proliferative fasciitis (surgicalpathologyatlas.com)].
 
==Proliferative myositis==
===General===
*Benign.
*Possible arise from pericytes.<ref name=pmid2058761/>
 
===Microscopic===
Features:<ref name=pmid2058761>{{Cite journal  | last1 = el-Jabbour | first1 = JN. | last2 = Bennett | first2 = MH. | last3 = Burke | first3 = MM. | last4 = Lessells | first4 = A. | last5 = O'Halloran | first5 = A. | title = Proliferative myositis. An immunohistochemical and ultrastructural study. | journal = Am J Surg Pathol | volume = 15 | issue = 7 | pages = 654-9 | month = Jul | year = 1991 | doi =  | PMID = 2058761 }}</ref><ref name=pmid1586481>{{Cite journal  | last1 = Lundgren | first1 = L. | last2 = Kindblom | first2 = LG. | last3 = Willems | first3 = J. | last4 = Falkmer | first4 = U. | last5 = Angervall | first5 = L. | title = Proliferative myositis and fasciitis. A light and electron microscopic, cytologic, DNA-cytometric and immunohistochemical study. | journal = APMIS | volume = 100 | issue = 5 | pages = 437-48 | month = May | year = 1992 | doi =  | PMID = 1586481 }}</ref>
*Large ganglion-like cells.
**Cells have single prominent nucleolus.
*Spindle cells.
*+/-Binucleation.
*Mitotic activity.
**No atypical mitoses.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Proliferative_myositis_%28HE%29.jpg Proliferative myositis (WC)].
 
===IHC===
Features:<ref name=pmid2058761/>
*Vimentin +ve.
*SMA +ve.
*Desmin +ve/-ve.
 
Others:<ref name=pmid2058761/>
*Factor XIIIa -ve.
*S100 -ve.
*CAM5.2 -ve.
*NSE -ve.


Notes:
==Elastofibroma==
*Some consider this a wastebasket diagnosis... for benign appearing spindle cell lesions.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2]. Accessed on: 10 May 2011.</ref>
{{Main|Elastofibroma}}


==Nodular fasciitis==
==Nodular fasciitis==
{{Main|Nodular fasciitis}}
==Desmoid-type fibromatosis==
*[[AKA]] ''desmoid tumour''.
*[[AKA]] ''desmoid fibromatosis''.
{{Main|Desmoid-type fibromatosis}}
==Lipofibromatosis==
*[[AKA]] infantile subcutaneous fibromatosis.
===General===
===General===
*Benign.
*Childhood.
*All age groups.
*Associated with trauma.


===Microscopic===
===Microscopic===
Features:<ref name=Ref_WMSP606>{{Ref WMSP|606}}</ref><ref>{{cite journal |author=de Feraudy S, Fletcher CD |title=Intradermal nodular fasciitis: a rare lesion analyzed in a series of 24 cases |journal=Am. J. Surg. Pathol. |volume=34 |issue=9 |pages=1377–81 |year=2010 |month=September |pmid=20716998 |doi=10.1097/PAS.0b013e3181ed7374 |url=}}</ref>
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Usu. well-circumscribed.
*Fibroblastic cells surrounding adipocytes.
*Clusters of (non-pleomorphic) spindle cells.
 
*Inflammation (lymphocytes).
Image:
*Microcysts in cellular regions - uncommon - ''discriminatory''.
*[http://cases.edinburgh-dermatopathology.org.uk/#2.0 Lipofibromatosis (edinburgh-dermatopathology.org.uk)].
*Mitoses - common.
 
*Extravasated [[RBC]]s.
===IHC===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*CD34 +ve.
*BCL2 +ve.
*S100 +ve.
*CD99 +ve.
*Actin +ve.
*EMA +ve.
 
==Desmoplastic fibroblastoma==
*AKA ''collagenous fibroma''.<ref name=pmid18271804>{{Cite journal | last1 = Watanabe | first1 = H. | last2 = Ishida | first2 = Y. | last3 = Nagashima | first3 = K. | last4 = Makino | first4 = T. | last5 = Norisugi | first5 = O. | last6 = Shimizu | first6 = T. | title = Desmoplastic fibroblastoma (collagenous fibroma). | journal = J Dermatol | volume = 35 | issue = 2 | pages = 93-7 | month = Feb | year = 2008 | doi = 10.1111/j.1346-8138.2008.00421.x | PMID = 18271804 }}</ref>
*'''Not''' to be confused with ''[[desmoplastic fibroma]]''.
===General===
*Benign lesion.
 
Epidemiology:
*May be on the lip.
*Male:female ~= 5:1.<ref name=pmid15547225/>
*Age - typically 40s & 50s.<ref name=pmid15547225/>
 
===Gross===
*Classically found in the shoulder region.
 
DDx - shoulder region:
*[[Desmoplastic fibroblastoma]].
*[[Elastofibroma]].


The BD feature list:<ref>BD. 26 April 2011.</ref>
===Microscopic===
*Tissue culture-like/CNS-like morphology.
Features:<ref name=pmid9670823/><ref name=Ref_Sternberg4_161>{{Ref Sternberg4|161}}</ref>
*Thick (keloid-like) collagen bundles - '''key feature'''.
*Spindle cells ''or'' stellate cells without nuclear atypia.
*Extravasated [[RBC]]s.
*Acellular stroma with abundant collagen - '''key feature'''.
*Inflammation.
*+/-Myxoid areas.
*+/-Giant cells.
*+/-Rare mitoses.


Notes:
DDx:<ref name=pmid9670823/>
*No significant nuclear atypia.
*[[Fibromatosis]].
*No atypical mitoses.
*[[Low-grade fibromyxoid sarcoma]].
*May be cellular.


Images:
Images:
*[http://en.wikipedia.org/wiki/File:Nodular_fasciitis_%281%29.JPG NF - low mag. (WC)].
*[http://www.webpathology.com/image.asp?case=458&n=1 Desmoplastic fibroblastoma (webpathology.com)].
*[http://en.wikipedia.org/wiki/File:Nodular_fasciitis_%282%29.JPG NF - high mag. (WC)].
*[http://www.ajronline.org/content/183/6/1766/F3.expansion Desmoplastic fibroblastoma (ajronline.org)].<ref name=pmid15547225>{{Cite journal  | last1 = Walker | first1 = KR. | last2 = Bui-Mansfield | first2 = LT. | last3 = Gering | first3 = SA. | last4 = Ranlett | first4 = RD. | title = Collagenous fibroma (desmoplastic fibroblastoma) of the shoulder. | journal = AJR Am J Roentgenol | volume = 183 | issue = 6 | pages = 1766 | month = Dec | year = 2004 | doi =  | PMID = 15547225 }}</ref>


===IHC===
===IHC===
Routine spindle cell panel:
Features:<ref name=pmid9670823>{{Cite journal  | last1 = Miettinen | first1 = M. | last2 = Fetsch | first2 = JF. | title = Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts. | journal = Hum Pathol | volume = 29 | issue = 7 | pages = 676-82 | month = Jul | year = 1998 | doi =  | PMID = 9670823 }}</ref>
*Beta-catenin -ve.<ref name=pmid18544056>{{Cite journal  | last1 = Takahara | first1 = M. | last2 = Ichikawa | first2 = R. | last3 = Oda | first3 = Y. | last4 = Uchi | first4 = H. | last5 = Takeuchi | first5 = S. | last6 = Moroi | first6 = Y. | last7 = Kiryu | first7 = H. | last8 = Furue | first8 = M. | title = Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis. | journal = J Cutan Pathol | volume = 35 Suppl 1 | issue =  | pages = 70-3 | month = Oct | year = 2008 | doi = 10.1111/j.1600-0560.2007.00964.x | PMID = 18544056 }}
</ref>
**+ve in [[desmoid-type fibromatosis]].
*Desmin -ve.
*S-100 -ve.
*CD34 -ve.
*CD34 -ve.
*Desmin -ve..
*MSA +ve (focal).
*SMA -ve.
*alpha-SMA +ve (focal).
*S100 -ve.
*AE1/AE3 -ve.
 
Others:
*H-caldesmon -ve.
*EMA -ve.
*Vimentin +ve.


===Molecular===
===Molecular===
*Evolving - case reports.
*llq12 breakpoint described as being characteristic -- possibly the ''FOSL1 gene''.<ref name=pmid22411068>{{Cite journal | last1 = Macchia | first1 = G. | last2 = Trombetta | first2 = D. | last3 = Möller | first3 = E. | last4 = Mertens | first4 = F. | last5 = Storlazzi | first5 = CT. | last6 = Debiec-Rychter | first6 = M. | last7 = Sciot | first7 = R. | last8 = Nord | first8 = KH. | title = FOSL1 as a candidate target gene for 11q12 rearrangements in desmoplastic fibroblastoma. | journal = Lab Invest | volume = 92 | issue = 5 | pages = 735-43 | month = May | year = 2012 | doi = 10.1038/labinvest.2012.46 | PMID = 22411068 }}</ref>
**t(15;15)(q13;q25).<ref name=pmid12606136>{{cite journal |author=Velagaleti GV, Tapper JK, Panova NE, Miettinen M, Gatalica Z |title=Cytogenetic findings in a case of nodular fasciitis of subclavicular region |journal=Cancer Genet. Cytogenet. |volume=141 |issue=2 |pages=160–3 |year=2003 |month=March |pmid=12606136 |doi= |url=}}</ref>


==Desmoid-type fibromatosis==
==Calcifying fibrous tumour==
*[[AKA]] desmoid tumour.
===General===
===General===
*Rare.
*Benign.
*Benign.
*Locally aggressive.<ref>URL: [http://www.dtrf.org/dtrf_aboutdesmoids.htm http://www.dtrf.org/dtrf_aboutdesmoids.htm]. Accessed on: 15 April 2011.</ref>
*May be seen in the context of [[familial adenomatous polyposis]].


===Microscopic===
===Microscopic===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref>URL: [http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196 http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196]. Accessed on: 4 October 2011.</ref>
Features:<ref name=pmid16858502>{{cite journal |author=Attila T, Chen D, Gardiner GW, Ptak TW, Marcon NE |title=Gastric calcifying fibrous tumor |journal=Can. J. Gastroenterol. |volume=20 |issue=7 |pages=487–9 |year=2006 |month=July |pmid=16858502 |pmc=2659917 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659917/}}</ref>
*"Sweeping fascicles"/bundles.
*Submucosal circumscribed fibrocollagenous nodule.
*Spindle cells with:
*Psammomatous calcifications.
**Small slender nuclei.
*Focal plasma cells at the periphery.
**Solid dark eosinophilic cytoplasm.
*+/-Mitoses - may be abundant.
*Long thin-walled vessels - parallel to one another - '''important feature'''.


Notes:
==Myofibroma==
*Hypertrophic scar-like lesion; see also ''[[dermal scar]]''.
{{Main|Myofibroma}}


Images:
==Cellular angiofibroma==
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196 Desmoid tumour (surgicalpathologyatlas.com)].
===General===
*[http://www.cheapmedicinechest.com/wp-content/uploads/2010/10/Figure-4.-Desmoid-tumor-with-fibroblastic-proliferation.png Desmoid tumour (cheapmedicinechest.com)].<ref>URL: [http://www.cheapmedicinechest.com/abdominal-pain-and-colonic-obstruction-from-an-intra-abdominal-desmoid-tumor.html http://www.cheapmedicinechest.com/abdominal-pain-and-colonic-obstruction-from-an-intra-abdominal-desmoid-tumor.html]. Accessed on: 4 October 2011.</ref>
*Rare.
*Benign.
*Probably related to [[spindle cell lipoma]] and [[mammary-type myofibroblastoma]].<ref name=pmid20852591>{{Cite journal  | last1 = Flucke | first1 = U. | last2 = van Krieken | first2 = JH. | last3 = Mentzel | first3 = T. | title = Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma. | journal = Mod Pathol | volume = 24 | issue = 1 | pages = 82-9 | month = Jan | year = 2011 | doi = 10.1038/modpathol.2010.170 | PMID = 20852591 }}</ref>
*Predominantly female.
 
===Gross===
Features:<ref name=pmid20852591/>
*Superficial.
*Well-circumscribed.
 
Classic location:
*Vulva.<ref name=pmid20852591/>
 
===Microscopic===
Features:<ref name=pmid20852591/>
*Spindle cell lesion.
*Many small-to-medium blood vessls.


===IHC===
===IHC===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
Features:<ref name=pmid20852591/>
*Beta-catenin +ve - '''important'''.
*CD34 ~50% of cases.
*SMA +ve ~50% of lesions.
*SMA ~41% of cases.
*CD99 -ve.
*EMA -ve.


==Proliferative fasciitis==
=Occasionally metastasizing=
*Need to write something here.
==Inflammatory myofibroblastic tumour==
*[[AKA]] inflammatory pseudotumour, AKA inflammatory fibrosarcoma,<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref> AKA plasma cell granuloma.<ref>URL: [http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung]. Accessed on: 27 November 2011.</ref><ref name=pmid21772725>{{Cite journal  | last1 = Manohar | first1 = B. | last2 = Bhuvaneshwari | first2 = S. | title = Plasma cell granuloma of gingiva. | journal = J Indian Soc Periodontol | volume = 15 | issue = 1 | pages = 64-6 | month = Jan | year = 2011 | doi = 10.4103/0972-124X.82275 | PMID = 21772725 }}</ref>
{{Main|Inflammatory myofibroblastic tumour}}


==Solitary fibrous tumour==
==Low-grade myofibroblastic sarcoma==
*Abbreviated ''SFT''.
===General===
===General===
*Grouped with ''hemangiopericytoma'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Rare ~ 100 cases in the literature.
*May be benign ''or'' malignant; more commonly benign.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9]. Accessed on: 25 June 2010.</ref><ref>URL: [http://wjso.com/content/6/1/86 http://wjso.com/content/6/1/86]. Accessed on: 25 June 2010.</ref>
*Usu. oral cavity or extremities.<ref name=pmid21868549/>  


===Microscopic===
===Microscopic===
Features:
Features:
*Well-circumscribed.
*Spindle cells in the storiform pattern<ref name=pmid21868549/> ''or'' in fasicles.
*Fibroblast-like cells (spindle cells).
*Rare mitoses.
*Hemangiopericytoma-like area ([[staghorn vessels]]).
*Keloid-like collagen bundles - '''key feature'''.


Images:
Images:
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_low_mag.jpg SFT - low mag. (WC)].
*[http://path.upmc.edu/cases/case673.html Low-grade myofibroblastic sarcoma - several images (upmc.edu)].
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_intermed_mag.jpg SFT - intermed. mag. (WC)].
 
*[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_high_mag.jpg SFT - high mag. (WC)].
DDx:
*Atypical [[leiomyoma]].
*[[GIST]].
*[[Leiomyosarcoma]].


===IHC===
===IHC===
*CD34 ~90% +ve.
*SMA +ve.
*CD99 ~70% +ve.
*CD34 -ve.
*BCL2 ~50% +ve.
*CD117 -ve.<ref name=pmid21868549/>
*H-caldesmon -ve.<ref name=pmid21868549>{{Cite journal  | last1 = Miyazawa | first1 = M. | last2 = Naritaka | first2 = Y. | last3 = Miyaki | first3 = A. | last4 = Asaka | first4 = S. | last5 = Isohata | first5 = N. | last6 = Yamaguchi | first6 = K. | last7 = Murayama | first7 = M. | last8 = Shimakawa | first8 = T. | last9 = Katsube | first9 = T. | title = A low-grade myofibroblastic sarcoma in the abdominal cavity. | journal = Anticancer Res | volume = 31 | issue = 9 | pages = 2989-94 | month = Sep | year = 2011 | doi =  | PMID =  }}</ref>
 
 
==Congenital-infantile fibrosarcoma==
:Should not be confused with ''[[adult fibrosarcoma]]''.
===General===
*Locally aggressive.
 
===Microscopic===
Features:<ref name=pmid7839472>{{Cite journal  | last1 = Corsi | first1 = A. | last2 = Boldrini | first2 = R. | last3 = Bosman | first3 = C. | title = Congenital-infantile fibrosarcoma: study of two cases and review of the literature. | journal = Tumori | volume = 80 | issue = 5 | pages = 392-400 | month = Oct | year = 1994 | doi =  | PMID = 7839472 }}</ref>
*Spindle cell lesion.
 
===Molecular===
Characteristic [[translocation]]:<ref name=pmid11242790 >{{Cite journal  | last1 = Sheng | first1 = WQ. | last2 = Hisaoka | first2 = M. | last3 = Okamoto | first3 = S. | last4 = Tanaka | first4 = A. | last5 = Meis-Kindblom | first5 = JM. | last6 = Kindblom | first6 = LG. | last7 = Ishida | first7 = T. | last8 = Nojima | first8 = T. | last9 = Hashimoto | first9 = H. | title = Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. | journal = Am J Clin Pathol | volume = 115 | issue = 3 | pages = 348-55 | month = Mar | year = 2001 | doi = 10.1309/3H24-E7T7-V37G-AKKQ | PMID = 11242790 }}</ref>
*t(12;15)(p13;q25).
**Gene fusion ETV6-[[NTRK3]].
***Same translocation in [[mesoblastic nephroma]].
 
==Solitary fibrous tumour==
{{Main|Solitary fibrous tumour}}


==Hemangiopericytoma==
==Hemangiopericytoma==
===General===
===General===
*Grouped with ''solitary fibrous tumour'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Grouped with ''[[solitary fibrous tumour]]'' in the WHO classification; share same genetic NAB2-STAT6 fusion.<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref>{{Cite journal  | last1 = Schweizer | first1 = L. | last2 = Koelsche | first2 = C. | last3 = Sahm | first3 = F. | last4 = Piro | first4 = RM. | last5 = Capper | first5 = D. | last6 = Reuss | first6 = DE. | last7 = Pusch | first7 = S. | last8 = Habel | first8 = A. | last9 = Meyer | first9 = J. | title = Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. | journal = Acta Neuropathol | volume = 125 | issue = 5 | pages = 651-8 | month = May | year = 2013 | doi = 10.1007/s00401-013-1117-6 | PMID = 23575898 }}</ref>
*Arises from the ''pericyte'', a connective tissue cell of small vessels that is thought to be involved in flow regulation.
 
*Thought to arise from the ''pericyte'', a connective tissue cell of small vessels that is thought to be involved in flow regulation while others consider a fibroblastic nature.<ref>{{Cite journal  | last1 = Gengler | first1 = C. | last2 = Guillou | first2 = L. | title = Solitary fibrous tumour and haemangiopericytoma: evolution of a concept. | journal = Histopathology | volume = 48 | issue = 1 | pages = 63-74 | month = Jan | year = 2006 | doi = 10.1111/j.1365-2559.2005.02290.x | PMID = 16359538 }}</ref>
*Hematologic spread most common - to lungs.<ref name=emed1255879ov>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
*Hematologic spread most common - to lungs.<ref name=emed1255879ov>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
*Oncogenic osteomalacia - assoc. with hemangiopericytoma.<ref name=emed1255879ov>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
*[[Oncogenic osteomalacia]] - assoc. with hemangiopericytoma.<ref name=emed1255879ov>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
*WHO grade II hemangiopericytoma (ICD-O: 9150/1), WHO grade III anaplastic hemangiopericytoma (ICD-O: 9150/3)


====Presentation====
====Presentation====
*Usually painless mass, slow enlargement.
*Usually painless mass, slow enlargement.
*May profusely bleed during resection.
*May invade bone.
====Histology====
*high cellular density.
*indistinct cell borders.
*random tumor cell orientation.
*little fibrosis.
*plenty reticulin.
*vascular with slit-like channels ("staghorn-like vessels").
====IHC====
* Vimentin +ve.
* CD34 +ve (often patchy, used to differentiate from SFT).
* [[STAT6]] nuclear +ve.
* EMA +/-ve.


===Radiology===
===Radiology===
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DDx:
DDx:
*Other vascular tumours.
*Other [[vascular tumours]].
*Vascular malformations.
*[[Vascular malformations]].
*[[Synovial sarcoma]].
*[[Synovial sarcoma]].
*[[Dermatofibroma]]. (???)
*[[Dermatofibroma]]. (???)
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*S100 -ve.
*S100 -ve.


==Desmoplastic fibroblastoma==
===Images===
*AKA ''collagenous fibroma''.<ref name=pmid18271804>{{Cite journal  | last1 = Watanabe | first1 = H. | last2 = Ishida | first2 = Y. | last3 = Nagashima | first3 = K. | last4 = Makino | first4 = T. | last5 = Norisugi | first5 = O. | last6 = Shimizu | first6 = T. | title = Desmoplastic fibroblastoma (collagenous fibroma). | journal = J Dermatol | volume = 35 | issue = 2 | pages = 93-7 | month = Feb | year = 2008 | doi = 10.1111/j.1346-8138.2008.00421.x | PMID = 18271804 }}</ref>
<gallery>
*'''Not''' to be confused with ''[[desmoplastic fibroma]]''.
Image:Neuropathology_case_VI_02.jpg | Anaplastic hemangiopericytoma, low mag. (WC/jensflorian)
===General===
Image:Neuropathology_case_VI_03.jpg | Anaplastic hemangiopericytoma, intermed mag. (WC/jensflorian)
*Benign lesion.
Image:Neuropathology_case_VI_04.jpg | Anaplastic hemangiopericytoma, high mag. (WC/jensflorian)
*Classically found in shoulder region.
Image:Neuropathology_case_VI_01.jpg | Anaplastic hemangiopericytoma, [[STAT6]] immunostaining. (WC/jensflorian)
 
</gallery>
Epidemiology:
*May be on the lip.
 
===Microscopic===
Features:<ref>URL: [http://www.dermatologyoutlines.com/dermskintumornonmelanocytic.html#collagenousfibroma http://www.dermatologyoutlines.com/dermskintumornonmelanocytic.html#collagenousfibroma]. Accessed on: 19 March</ref><ref name=Ref_Sternberg4_161>{{Ref Sternberg4|161}}</ref>
*Acellular stroma with abundant collagen.
 
Notes:
*'''No''' nuclear atypia.
 
===IHC===
*Beta-catenin -ve.<ref name=pmid18544056>{{Cite journal  | last1 = Takahara | first1 = M. | last2 = Ichikawa | first2 = R. | last3 = Oda | first3 = Y. | last4 = Uchi | first4 = H. | last5 = Takeuchi | first5 = S. | last6 = Moroi | first6 = Y. | last7 = Kiryu | first7 = H. | last8 = Furue | first8 = M. | title = Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis. | journal = J Cutan Pathol | volume = 35 Suppl 1 | issue =  | pages = 70-3 | month = Oct | year = 2008 | doi = 10.1111/j.1600-0560.2007.00964.x | PMID = 18544056 }}
</ref>
**+ve in [[desmoid-type fibromatosis]].


=Malignant=
==Low-grade fibromyxoid sarcoma==
==Low-grade fibromyxoid sarcoma==
*[[AKA]] ''hyalinizing spindle cell tumour''.
*[[AKA]] ''hyalinizing spindle cell tumour''.
*Should '''not''' be confused with ''[[myxofibrosarcoma]]''.
*Should '''not''' be confused with ''[[myxofibrosarcoma]]''.
===General===
*Abbreviated ''LGFMS''.
*Deep soft tissue.
{{Main|Low-grade fibromyxoid sarcoma}}
 
===Microscopic===
Features:<ref>{{cite journal |author=Vernon SE, Bejarano PA |title=Low-grade fibromyxoid sarcoma: a brief review |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=9 |pages=1358–60 |year=2006 |month=September |pmid=16948525 |doi= |url=}}</ref>
*Myoid stroma alternating with fibrogenic areas - '''key feature'''.
*Low cellularity.
*Spindle cells.
*+/-Rosette of collagen with central hyaline core.<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
 
Notes:
*Few/absent mitoses.
 
===Molecular pathology===
*t(7;16)(q33;p11).<ref>{{cite journal |author=Panagopoulos I, Storlazzi CT, Fletcher CD, ''et al.'' |title=The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma |journal=Genes Chromosomes Cancer |volume=40 |issue=3 |pages=218–28 |year=2004 |month=July |pmid=15139001 |doi=10.1002/gcc.20037 |url=}}</ref>
 
==Calcifying fibrous tumour==
===General===
*Rare.
*Benign.
 
===Microscopic===
Features:<ref name=pmid16858502>{{cite journal |author=Attila T, Chen D, Gardiner GW, Ptak TW, Marcon NE |title=Gastric calcifying fibrous tumor |journal=Can. J. Gastroenterol. |volume=20 |issue=7 |pages=487–9 |year=2006 |month=July |pmid=16858502 |pmc=2659917 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659917/}}</ref>
*Submucosal circumscribed fibrocollagenous nodule.
*Psammomatous calcifications.
*Focal plasma cells at the periphery.


==Adult fibrosarcoma==
==Adult fibrosarcoma==
Line 291: Line 372:
Feature:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
Feature:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
*Spindle cell lesion.
*Spindle cell lesion.
*Herring bone pattern - '''key feature'''.
*[[Herring bone pattern]] - '''key feature'''.
*Mitoses.
*Mitoses.


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DDx:
DDx:
*[[DFSP]] - t(17;22).
*[[Dermatofibrosarcoma protuberans]] (DFSP) - t(17;22) COLA1/PDGFB.
*Congenital-infantile fibrosarcoma - t(12;15) ETV6/NTRK3.  
*[[Congenital-infantile fibrosarcoma]] - t(12;15) ETV6/NTRK3.  
*[[Solitary fibrous tumour]].
*[[Solitary fibrous tumour]].
*[[Synovial sarcoma]] - t(X;18) SYT/SSX.
*[[Synovial sarcoma]] - t(X;18) SYT/SSX.
Line 313: Line 394:
*Vimentin.
*Vimentin.
*SMA.
*SMA.
==Congenital-infantile fibrosarcoma==
:Should not be confused with ''[[adult fibrosarcoma]]''.
===General===
*Locally aggressive.
===Microscopic===
Features:<ref name=pmid7839472>{{Cite journal  | last1 = Corsi | first1 = A. | last2 = Boldrini | first2 = R. | last3 = Bosman | first3 = C. | title = Congenital-infantile fibrosarcoma: study of two cases and review of the literature. | journal = Tumori | volume = 80 | issue = 5 | pages = 392-400 | month = Oct | year = 1994 | doi =  | PMID = 7839472 }}</ref>
*Spindle cell lesion.
===Molecular===
Characteristic [[translocation]]:<ref>{{Cite journal  | last1 = Sheng | first1 = WQ. | last2 = Hisaoka | first2 = M. | last3 = Okamoto | first3 = S. | last4 = Tanaka | first4 = A. | last5 = Meis-Kindblom | first5 = JM. | last6 = Kindblom | first6 = LG. | last7 = Ishida | first7 = T. | last8 = Nojima | first8 = T. | last9 = Hashimoto | first9 = H. | title = Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. | journal = Am J Clin Pathol | volume = 115 | issue = 3 | pages = 348-55 | month = Mar | year = 2001 | doi = 10.1309/3H24-E7T7-V37G-AKKQ | PMID = 11242790 }}</ref>
*t(12;15)(p13;q25).
**Gene fusion ETV6-NTRK3.
***Same translocation in [[mesoblastic nephroma]].


==Myxofibrosarcoma==
==Myxofibrosarcoma==
*Should '''not''' be confused with ''[[low-grade fibromyxoid sarcoma]]''.<ref name=pmid8650138>{{Cite journal  | last1 = Mentzel | first1 = T. | last2 = Katenkamp | first2 = D. | last3 = Fletcher | first3 = CD. | title = [Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course]. | journal = Pathologe | volume = 17 | issue = 2 | pages = 116-21 | month = Mar | year = 1996 | doi =  | PMID = 8650138 }}</ref>
*Should '''not''' be confused with ''[[low-grade fibromyxoid sarcoma]]''.<ref name=pmid8650138>{{Cite journal  | last1 = Mentzel | first1 = T. | last2 = Katenkamp | first2 = D. | last3 = Fletcher | first3 = CD. | title = [Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course]. | journal = Pathologe | volume = 17 | issue = 2 | pages = 116-21 | month = Mar | year = 1996 | doi =  | PMID = 8650138 }}</ref>
===General===
*[[AKA]] ''myxoid malignant fibrous histiocytoma'' or ''myxoid MFH''.<ref>{{Cite journal  | last1 = Fujimura | first1 = T. | last2 = Okuyama | first2 = R. | last3 = Terui | first3 = T. | last4 = Okuno | first4 = K. | last5 = Masu | first5 = A. | last6 = Masu | first6 = T. | last7 = Chiba | first7 = S. | last8 = Kunii | first8 = T. | last9 = Tagami | first9 = H. | title = Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases. | journal = J Cutan Pathol | volume = 32 | issue = 7 | pages = 512-5 | month = Aug | year = 2005 | doi = 10.1111/j.0303-6987.2005.00368.x | PMID = 16008697 }}</ref>
*Malignant.
{{Main|Myxofibrosarcoma}}
*Usu. older people, superficial (skin/dermis) and extremities (arm, legs).<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref><ref name=pmid17197925/>
 
===Microscopic===
Features:<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref>
*Discontinuous fibrous septae.
*Myxoid background.
*Variable cellularity and nuclear pleomorphism.
*Spindle cells or epithelioid cells.<ref name=pmid17197925/>
*Curvilinear vessels.<ref name=pmid17197925>{{Cite journal  | last1 = Nascimento | first1 = AF. | last2 = Bertoni | first2 = F. | last3 = Fletcher | first3 = CD. | title = Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases. | journal = Am J Surg Pathol | volume = 31 | issue = 1 | pages = 99-105 | month = Jan | year = 2007 | doi = 10.1097/01.pas.0000213379.94547.e7 | PMID = 17197925 }}</ref>
 
DDx:
*[[Liposarcoma|Myxoid liposarcoma]].
*[[Low-grade fibromyxoid sarcoma]] - alternating fibrous and myxoid areas.<ref name=pmid8650138/>


==See also==
=See also=
*[[Soft tissue lesions]].
*[[Soft tissue lesions]].


==References==
=References=
{{Reflist|2}}
{{Reflist|2}}


[[Category:Soft tissue lesions]]
[[Category:Soft tissue lesions]]
Michael
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