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*Calcifying aponeurotic fibroma. | *Calcifying aponeurotic fibroma. | ||
*[[Angiomyofibroblastoma]]. | *[[Angiomyofibroblastoma]]. | ||
*Cellular angiofibroma. | *[[Cellular angiofibroma]]. | ||
*Nuchal-type fibroma. | *Nuchal-type fibroma. | ||
*Gardner fibroma. | *Gardner fibroma. | ||
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*Fibrous hamartoma of infancy. | *Fibrous hamartoma of infancy. | ||
*Juvenile hyaline fibromatosis. | *Juvenile hyaline fibromatosis. | ||
*Desmoplastic fibroblastoma. | *[[Desmoplastic fibroblastoma]]. | ||
*Mammary-type myofibroblastoma. | *Mammary-type myofibroblastoma. | ||
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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== | ||
{{Main|Elastofibroma}} | |||
==Nodular fasciitis== | ==Nodular fasciitis== | ||
{{Main|Nodular fasciitis}} | |||
==Desmoid-type fibromatosis== | ==Desmoid-type fibromatosis== | ||
*[[AKA]] ''desmoid tumour''. | *[[AKA]] ''desmoid tumour''. | ||
*[[AKA]] ''desmoid fibromatosis''. | *[[AKA]] ''desmoid fibromatosis''. | ||
{{Main|Desmoid-type fibromatosis}} | |||
==Lipofibromatosis== | ==Lipofibromatosis== | ||
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===General=== | ===General=== | ||
*Benign lesion. | *Benign lesion. | ||
Epidemiology: | Epidemiology: | ||
*May be on the lip. | *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]]. | |||
===Microscopic=== | ===Microscopic=== | ||
Features:<ref | Features:<ref name=pmid9670823/><ref name=Ref_Sternberg4_161>{{Ref Sternberg4|161}}</ref> | ||
*Acellular stroma with abundant collagen. | *Spindle cells ''or'' stellate cells without nuclear atypia. | ||
*Acellular stroma with abundant collagen - '''key feature'''. | |||
*+/-Myxoid areas. | |||
*+/-Rare mitoses. | |||
DDx:<ref name=pmid9670823/> | |||
*[[Fibromatosis]]. | |||
*[[Low-grade fibromyxoid sarcoma]]. | |||
Images: | |||
* | *[http://www.webpathology.com/image.asp?case=458&n=1 Desmoplastic fibroblastoma (webpathology.com)]. | ||
*[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=== | ||
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 }} | *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> | </ref> | ||
**+ve in [[desmoid-type fibromatosis]]. | **+ve in [[desmoid-type fibromatosis]]. | ||
*Desmin -ve. | |||
*S-100 -ve. | |||
*CD34 -ve. | |||
*MSA +ve (focal). | |||
*alpha-SMA +ve (focal). | |||
===Molecular=== | |||
*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> | |||
==Calcifying fibrous tumour== | ==Calcifying fibrous tumour== | ||
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*Focal plasma cells at the periphery. | *Focal plasma cells at the periphery. | ||
= | ==Myofibroma== | ||
{{Main|Myofibroma}} | |||
==Cellular angiofibroma== | |||
===General=== | ===General=== | ||
* | *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= | 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 | Features:<ref name=pmid20852591/> | ||
*CD34 ~50% of cases. | |||
*SMA ~41% of cases. | |||
* | *CD99 -ve. | ||
*EMA -ve. | |||
=Occasionally metastasizing= | |||
* | ==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}} | |||
==Low-grade myofibroblastic sarcoma== | ==Low-grade myofibroblastic sarcoma== | ||
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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]]. | ||
==Solitary fibrous tumour== | ==Solitary fibrous tumour== | ||
{{Main|Solitary fibrous tumour}} | |||
==Hemangiopericytoma== | ==Hemangiopericytoma== | ||
===General=== | ===General=== | ||
*Grouped with ''solitary fibrous tumour'' in the WHO classification; | *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> | ||
* | |||
*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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*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''. | ||
{{Main|Low-grade fibromyxoid sarcoma}} | |||
==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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==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> | ||
*[[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> | |||
* | {{Main|Myxofibrosarcoma}} | ||
=== | |||
=See also= | =See also= |
edits