Fibroblastic/myofibroblastic tumours: Difference between revisions

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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>
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>
*Large polygonal (ganglion-like) and/or spindled cells with:
*Large polygonal (ganglion-like) and/or spindled cells with:
**Vesicular (clear) nuclei.
**[[Vesicular nuclei|Vesicular (clear) nuclei]].
**Prominent nucleoli.
**Prominent nucleoli.
*+/-Binucleation.
*+/-Binucleation.
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==Elastofibroma==
==Elastofibroma==
===General===
{{Main|Elastofibroma}}
*Benign.
*Classically, subscapular in elderly women.<ref>URL: [http://emedicine.medscape.com/article/1057113-overview http://emedicine.medscape.com/article/1057113-overview]. Accessed on: 26 October 2011.</ref><ref>{{Cite journal  | last1 = Ben Hassouna | first1 = J. | last2 = Hamdi | first2 = N. | last3 = Ben Bachouche | first3 = W. | last4 = Bouzid | first4 = T. | last5 = Dhiab | first5 = T. | last6 = Rahal | first6 = K. | title = Elastofibroma dorsi. | journal = Orthop Traumatol Surg Res | volume = 96 | issue = 6 | pages = 717-20 | month = Oct | year = 2010 | doi = 10.1016/j.otsr.2010.03.019 | PMID = 20708994 }}</ref>
 
===Gross===
Features:
*Yellow-white, moderate demarcation to surrounding tissue.<ref name=Ref_AoGP592>{{Ref AoGP|592}}</ref>
 
DDx - shoulder lesions:
*[[Desmoplastic fibroblastoma]].
*[[Pleomorphic lipoma]].
 
===Microscopic===
Features:
*Thick bundles of collagen.
*Elastin fibres.
 
Image:
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20080802163630229 Elastofibroma (surgicalpathologyatlas.com)].


==Nodular fasciitis==
==Nodular fasciitis==
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===Molecular===
===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>
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).
*t(12;15)(p13;q25).
**Gene fusion ETV6-NTRK3.
**Gene fusion ETV6-[[NTRK3]].
***Same translocation in [[mesoblastic nephroma]].
***Same translocation in [[mesoblastic nephroma]].


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==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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*EMA -ve.
*EMA -ve.
*S100 -ve.
*S100 -ve.
===Images===
<gallery>
Image:Neuropathology_case_VI_02.jpg | Anaplastic hemangiopericytoma, low mag. (WC/jensflorian)
Image:Neuropathology_case_VI_03.jpg | Anaplastic hemangiopericytoma, intermed mag. (WC/jensflorian)
Image:Neuropathology_case_VI_04.jpg | Anaplastic hemangiopericytoma, high mag. (WC/jensflorian)
Image:Neuropathology_case_VI_01.jpg | Anaplastic hemangiopericytoma, [[STAT6]] immunostaining. (WC/jensflorian)
</gallery>


=Malignant=
=Malignant=
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*Should '''not''' be confused with ''[[myxofibrosarcoma]]''.
*Should '''not''' be confused with ''[[myxofibrosarcoma]]''.
*Abbreviated ''LGFMS''.
*Abbreviated ''LGFMS''.
===General===
{{Main|Low-grade fibromyxoid sarcoma}}
*Deep soft tissue.
*Related to ''hyalinizing spindle cell tumour with giant collagen rosettes''.<ref name=pmid20864787>{{Cite journal  | last1 = Ramaswamy | first1 = AS. | last2 = Chatura | first2 = KR. | title = Hyalinizing spindle cell tumor with giant rosettes. | journal = Ann Saudi Med | volume = 31 | issue = 1 | pages = 83-6 | month =  | year =  | doi =  | PMID = 20864787 | PMC = 3101732 }}</ref>
 
===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>
*[[Myxoid 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.
 
DDx:
*[[Leiomyoma]].
*[[Myxoid liposarcoma]].
*[[Desmoplastic fibroblastoma]].
*[[Intramuscular myxoma]].
 
Images:
*www:
**[http://www.springerimages.com/Images/MedicineAndPublicHealth/1-10.1007_s00428-009-0776-0-7 LGFMS (springerimages.com)].
**[http://www.uscap.org/site~/iap2006/slides18-2v.htm LGFMS (uscap.org)].
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Low-grade_fibromyxoid_sarcoma_-_low_mag.jpg LGFMS - low mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Low-grade_fibromyxoid_sarcoma_-_intermed_mag.jpg LGFMS - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Low-grade_fibromyxoid_sarcoma_-2-_high_mag.jpg LGFMS - high mag. (WC)].
 
===IHC===
Features:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
*EMA +ve.
*CD99 +ve.
*BCL2 +ve.
 
Others:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
*SMA -ve.
*S100 -ve.
*Desmin -ve.
 
===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>
*t(11;16).<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>


==Adult fibrosarcoma==
==Adult fibrosarcoma==
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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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*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>
*[[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>
*[[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>
===General===
{{Main|Myxofibrosarcoma}}
*Malignant.
*Usually older people, superficial (skin/dermis) and extremities (arm, legs).<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref><ref name=pmid17197925/>
*Prognosis better than [[pleomorphic undifferentiated sarcoma]].
**Metastatic potential inversely related to component/portion that is myxoid.<ref name=pmid192434>{{Cite journal  | last1 = Weiss | first1 = SW. | last2 = Enzinger | first2 = FM. | title = Myxoid variant of malignant fibrous histiocytoma. | journal = Cancer | volume = 39 | issue = 4 | pages = 1672-85 | month = Apr | year = 1977 | doi =  | PMID = 192434 }}</ref>
 
===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/>
*[[Myxoma]].
 
Image:
*[http://www.sarcomaimages.com/sub.php?v=sample-case&p=myxofibrosarcoma-myxoid-mfh Myxofibrosarcoma (sarcomaimages.com)].
 
===IHC===
*Vimentin +ve -- otherwise non-distinctive.<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref>


=See also=
=See also=
Retrieved from "https://librepathology.org/wiki/Fibroblastic/myofibroblastic_tumours"