Difference between revisions of "Fibroblastic/myofibroblastic tumours"

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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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*[[AKA]] ''desmoid tumour''.
*[[AKA]] ''desmoid tumour''.
*[[AKA]] ''desmoid fibromatosis''.
*[[AKA]] ''desmoid fibromatosis''.
===General===
{{Main|Desmoid-type fibromatosis}}
*Benign.
*One of many ''[[fibromatoses]]''.
*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]].
 
===Gross===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Location:
**Abdominal wall, proximal extremities - classic for adolescents and women.
**Head and neck - classic for children.
*Circumscribed mass.
*May be quite large (>10 cm).
 
===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>
*"Sweeping fascicles"/bundles.
*Spindle cells with:
**Small slender nuclei.
**Solid dark eosinophilic cytoplasm.
*+/-Mitoses - may be abundant.
*Long thin-walled vessels - parallel to one another - '''important feature'''.
 
DDx:
*[[Hypertrophic scar]]-like lesion, see ''[[dermal scar]]''.
*[[Gastrointestinal stromal tumour]]<ref name=pmid23020601>{{Cite journal  | last1 = Huss | first1 = S. | last2 = Nehles | first2 = J. | last3 = Binot | first3 = E. | last4 = Wardelmann | first4 = E. | last5 = Mittler | first5 = J. | last6 = Kleine | first6 = MA. | last7 = Künstlinger | first7 = H. | last8 = Hartmann | first8 = W. | last9 = Hohenberger | first9 = P. | title = β-catenin (CTNNB1) mutations and clinicopathological features of mesenteric desmoid-type fibromatosis. | journal = Histopathology | volume = 62 | issue = 2 | pages = 294-304 | month = Jan | year = 2013 | doi = 10.1111/j.1365-2559.2012.04355.x | PMID = 23020601 }}</ref>
- reported in abdominal wall.<ref name=pmid15257404>{{Cite journal  | last1 = Thalheimer | first1 = A. | last2 = Meyer | first2 = D. | last3 = Gattenlöhner | first3 = S. | last4 = Timmermann | first4 = W. | last5 = Thiede | first5 = A. | title = [Gastrointestinal stromal tumor of the abdominal wall. An unusual localization of a rare tumor]. | journal = Chirurg | volume = 75 | issue = 7 | pages = 708-12 | month = Jul | year = 2004 | doi = 10.1007/s00104-003-0696-5 | PMID = 15257404 }}</ref>
*[[Retroperitoneal fibrosis]] - no beta-catenin staining.<ref name=pmid23020601/>
*Other [[fibromatoses]].
*[[Nodular fasciitis]] - esp. with [[RBC extravasation]].
 
====Images====
<gallery>
Image:DesmoidFibromatosis.JPG | Desmoid tumour. (WC)
</gallery>
www:
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196 Desmoid tumour (surgicalpathologyatlas.com)].
*[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>
*[http://radiographics.rsna.org/content/29/7/2143/F28.expansion.html Desmoid tumour (radiographics.rsna.org)].
*[http://www.ncbi.nlm.nih.gov.qe2a-proxy.mun.ca/pmc/articles/PMC3700980/figure/f3-ol-05-06-1976/ Desmoid-type fibromatosis (nih.gov)].<ref name=pmid23833679>{{Cite journal  | last1 = Ma | first1 = JH. | last2 = Ma | first2 = ZH. | last3 = Dong | first3 = XF. | last4 = Yin | first4 = H. | last5 = Zhao | first5 = YF. | title = Abdominal wall desmoid tumors: A case report. | journal = Oncol Lett | volume = 5 | issue = 6 | pages = 1976-1978 | month = Jun | year = 2013 | doi = 10.3892/ol.2013.1297 | PMID = 23833679 }}</ref>
 
===IHC===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Beta-catenin +ve (nuclear<ref name=pmid23020601/>) - '''important'''.
*SMA +ve ~50% of lesions.
 
Others:
*CD117 -ve.


==Lipofibromatosis==
==Lipofibromatosis==
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==Myofibroma==
==Myofibroma==
===General===
{{Main|Myofibroma}}
*Benign.
*Often < 2 years old; however, may be seen in adults.
*Not common.<ref name=pmid20038894>{{Cite journal  | last1 = Brasileiro | first1 = BF. | last2 = Martins-Filho | first2 = PR. | last3 = Piva | first3 = MR. | last4 = da Silva | first4 = LC. | last5 = Nonaka | first5 = CF. | last6 = Miguel | first6 = MC. | title = Myofibroma of the oral cavity. A rare spindle cell neoplasm. | journal = Med Oral Patol Oral Cir Bucal | volume = 15 | issue = 4 | pages = e596-600 | month = Jul | year = 2010 | doi =  | PMID = 20038894 | url= http://www.medicinaoral.com/pubmed/medoralv15_i4_p596.pdf }}</ref>
 
===Gross===
*Classically (oral) locations: tongue, buccal mucosa, lip.<ref name=pmid20038894/>
 
===Microscopic===
Features:<ref name=pmid20038894/>
*Spindle cells with:
**Tapering nuclei.
**Eosinophilic cytoplasm.
 
Note:
*Features overlap with [[myopericytoma]].<ref name=pmid16394283>{{cite journal |author=Dray MS, McCarthy SW, Palmer AA, ''et al.'' |title=Myopericytoma: a unifying term for a spectrum of tumours that show overlapping features with myofibroma. A review of 14 cases |journal=J. Clin. Pathol. |volume=59 |issue=1 |pages=67–73 |year=2006 |month=January |pmid=16394283 |pmc=1860256 |doi=10.1136/jcp.2005.028704 |url= }}</ref>
 
DDx:
*[[Neurofibroma]].
*[[Leiomyoma]].
 
===IHC===
Features:<ref name=pmid20038894/>
*Vimentin +ve.
*SMA +ve.
*S-100 -ve,


==Cellular angiofibroma==
==Cellular angiofibroma==
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==Inflammatory myofibroblastic tumour==
==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>
*[[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>
===General===
{{Main|Inflammatory myofibroblastic tumour}}
*Mostly benign.
*Children & young adults.
*Classically located in mesentery of ileocolic region or small bowel.<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
 
===Microscopic===
Features:<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
*Inflammation:
**[[Plasma cells]] - predominant - '''key feature'''.<ref name=pmid21297584>{{Cite journal  | last1 = Saab | first1 = ST. | last2 = Hornick | first2 = JL. | last3 = Fletcher | first3 = CD. | last4 = Olson | first4 = SJ. | last5 = Coffin | first5 = CM. | title = IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link? | journal = Mod Pathol | volume = 24 | issue = 4 | pages = 606-12 | month = Apr | year = 2011 | doi = 10.1038/modpathol.2010.226 | PMID = 21297584 }}</ref>
**Lymphocytes.
**Eosinophils.
*Spindle cells without atypia.
*+/-Fasciular architecture.
*Mitoses -- though none atypical.
*+/-Necrosis.
*+/-Hemorrhage.
*Calcifications.
 
DDx:
*[[Calcifying fibrous pseudotumour]] (has [[psammoma body|psammomatous calcifications]]).
*[[Inflammatory fibroid tumour]].
*[[Nodular fasciitis]].
 
Notes:
*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>
 
Images:
*[http://commons.wikimedia.org/wiki/File:Inflammatory_myofibroblastic_tumour_-_high_mag.jpg IMT - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Inflammatory_myofibroblastic_tumour_-_very_high_mag.jpg IMT - very high mag. (WC)].
 
===IHC===
Features - dependent on site:
*SMA +ve.<ref name=pmid20350216 >{{Cite journal  | last1 = Shi | first1 = H. | last2 = Li | first2 = Y. | last3 = Wei | first3 = L. | last4 = Sun | first4 = L. | title = Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases. | journal = Pathology | volume = 42 | issue = 3 | pages = 235-41 | month = Apr | year = 2010 | doi = 10.3109/00313021003631312 | PMID = 20350216 }}
</ref>
*Vimentin +ve.
 
Variable staining with:
*CD34, AE1/AE3, calretin.<ref name=pmid20216379>{{Cite journal  | last1 = Miyamoto | first1 = H. | last2 = Montgomery | first2 = EA. | last3 = Epstein | first3 = JI. | title = Paratesticular fibrous pseudotumor: a morphologic and immunohistochemical study of 13 cases. | journal = Am J Surg Pathol | volume = 34 | issue = 4 | pages = 569-74 | month = Apr | year = 2010 | doi = 10.1097/PAS.0b013e3181d438cb | PMID = 20216379 }}</ref><ref name=pmid20350216>{{Cite journal  | last1 = Shi | first1 = H. | last2 = Li | first2 = Y. | last3 = Wei | first3 = L. | last4 = Sun | first4 = L. | title = Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases. | journal = Pathology | volume = 42 | issue = 3 | pages = 235-41 | month = Apr | year = 2010 | doi = 10.3109/00313021003631312 | PMID = 20350216 }}</ref>
 
Negative:<ref name=pmid20350216/>
*S100, CD117, CD68.
 
===Molecular===
*ALK rearrangements.<ref name=pmid21297584/>


==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]].


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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.


Line 547: Line 398:
*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=

Latest revision as of 18:58, 24 March 2019

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

List of tumours

Benign

WHO classification:[1]

Locally aggressive

WHO classification:[1]

Occasionally metastasizing

WHO classification:[2]

Malignant

WHO classification:[2]

Non-malignant

Proliferative fasciitis

General

  • Benign.
  • May mimic a sarcoma.[3]

Clinical:

  • Solid subcutaneous nodule.
  • Rapid growth.
  • May be painful.

Gross

  • Classically upper and lower extremities.[3]
  • Poorly demarcated.

Microscopic

Features:[4]

  • Large polygonal (ganglion-like) and/or spindled cells with:
  • +/-Binucleation.
  • Loose myxoid stroma.
  • Frequent typical mitoses.
    • No atypical mitoses.

DDx:

Images:

Proliferative myositis

General

  • Benign.
  • Possible arise from pericytes.[6]

Microscopic

Features:[6][7]

  • Large ganglion-like cells.
    • Cells have single prominent nucleolus.
  • Spindle cells.
  • +/-Binucleation.
  • Mitotic activity.
    • No atypical mitoses.

Image:

IHC

Features:[6]

  • Vimentin +ve.
  • SMA +ve.
  • Desmin +ve/-ve.

Others:[6]

  • Factor XIIIa -ve.
  • S100 -ve.
  • CAM5.2 -ve.
  • NSE -ve.

Elastofibroma

Main article: Elastofibroma

Nodular fasciitis

Main article: Nodular fasciitis

Desmoid-type fibromatosis

  • AKA desmoid tumour.
  • AKA desmoid fibromatosis.
Main article: Desmoid-type fibromatosis

Lipofibromatosis

  • AKA infantile subcutaneous fibromatosis.

General

  • Childhood.

Microscopic

Features:[8]

  • Fibroblastic cells surrounding adipocytes.

Image:

IHC

Features:[8]

  • CD34 +ve.
  • BCL2 +ve.
  • S100 +ve.
  • CD99 +ve.
  • Actin +ve.
  • EMA +ve.

Desmoplastic fibroblastoma

General

  • Benign lesion.

Epidemiology:

  • May be on the lip.
  • Male:female ~= 5:1.[10]
  • Age - typically 40s & 50s.[10]

Gross

  • Classically found in the shoulder region.

DDx - shoulder region:

Microscopic

Features:[11][12]

  • Spindle cells or stellate cells without nuclear atypia.
  • Acellular stroma with abundant collagen - key feature.
  • +/-Myxoid areas.
  • +/-Rare mitoses.

DDx:[11]

Images:

IHC

Features:[11]

Molecular

  • llq12 breakpoint described as being characteristic -- possibly the FOSL1 gene.[14]

Calcifying fibrous tumour

General

  • Rare.
  • Benign.

Microscopic

Features:[15]

  • Submucosal circumscribed fibrocollagenous nodule.
  • Psammomatous calcifications.
  • Focal plasma cells at the periphery.

Myofibroma

Main article: Myofibroma

Cellular angiofibroma

General

Gross

Features:[16]

  • Superficial.
  • Well-circumscribed.

Classic location:

Microscopic

Features:[16]

  • Spindle cell lesion.
  • Many small-to-medium blood vessls.

IHC

Features:[16]

  • CD34 ~50% of cases.
  • SMA ~41% of cases.
  • CD99 -ve.
  • EMA -ve.

Occasionally metastasizing

Inflammatory myofibroblastic tumour

  • AKA inflammatory pseudotumour, AKA inflammatory fibrosarcoma,[17] AKA plasma cell granuloma.[18][19]
Main article: Inflammatory myofibroblastic tumour

Low-grade myofibroblastic sarcoma

General

  • Rare ~ 100 cases in the literature.
  • Usu. oral cavity or extremities.[20]

Microscopic

Features:

  • Spindle cells in the storiform pattern[20] or in fasicles.
  • Rare mitoses.

Images:

DDx:

IHC

  • SMA +ve.
  • CD34 -ve.
  • CD117 -ve.[20]
  • H-caldesmon -ve.[20]


Congenital-infantile fibrosarcoma

Should not be confused with adult fibrosarcoma.

General

  • Locally aggressive.

Microscopic

Features:[21]

  • Spindle cell lesion.

Molecular

Characteristic translocation:[22]

Solitary fibrous tumour

Main article: Solitary fibrous tumour

Hemangiopericytoma

General

  • 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.[24]
  • Hematologic spread most common - to lungs.[25]
  • Oncogenic osteomalacia - assoc. with hemangiopericytoma.[25]
  • WHO grade II hemangiopericytoma (ICD-O: 9150/1), WHO grade III anaplastic hemangiopericytoma (ICD-O: 9150/3)

Presentation

  • 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

  • Intramedullary lytic mass.
  • May be well-circumscribed.
  • +/-Periosteal reaction.
  • +/-Sclerotic border.

May be worked-up with angiography to distinguish from a vascular malformation.[26]

Location

  • Usually extremities - femur or proximal tibial.[25]

Microscopic

Features:[26]

  • Hypervascular lesion - key diagnostic feature.[27]
    • Abundant thin-walled branching small vessels of variable size.
      • May be described as "staghorn vessels" or "antler-like" vasculature.
      • Cells may "onion-skin" around thin blood vessels.
  • Spindle or ovoid shaped cells in nests or sheets.

DDx:

IHC

Features:[8][27]

  • Vimentin +ve (usually).
  • Desmin -ve (typical).
  • Factor VIII -ve (marks endothelium).
  • CD34 +ve.
    • CD34 usu. -ve in synovial sarcoma.
  • CD31 -ve (marks benign endothelium).
  • vWF (von Willebrand factor) -ve.

May be in the DDx for meningioma:[28]

  • EMA -ve.
  • S100 -ve.

Images

  • Anaplastic hemangiopericytoma, low mag. (WC/jensflorian)

  • Anaplastic hemangiopericytoma, intermed mag. (WC/jensflorian)

  • Anaplastic hemangiopericytoma, high mag. (WC/jensflorian)

  • Anaplastic hemangiopericytoma, STAT6 immunostaining. (WC/jensflorian)

Malignant

Low-grade fibromyxoid sarcoma

  • AKA hyalinizing spindle cell tumour.
  • Should not be confused with myxofibrosarcoma.
  • Abbreviated LGFMS.
Main article: Low-grade fibromyxoid sarcoma

Adult fibrosarcoma

General

  • Malignant.
  • Older adults.
  • Locations: head & neck, extremities.

Microscopic

Feature:[29]

DDx (herring bone):

  • MPNST.
  • Synovial sarcoma.
  • Fibrosarcoma.

DDx:

Images:

IHC

Features:[29]

  • Vimentin.
  • SMA.

Myxofibrosarcoma

Main article: Myxofibrosarcoma

See also

References

  1. ↑ 1.0 1.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 601. ISBN 978-0781765275.
  2. ↑ 2.0 2.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 602. ISBN 978-0781765275.
  3. ↑ 3.0 3.1 Chung, EB.; Enzinger, FM. (Oct 1975). "Proliferative fasciitis.". Cancer 36 (4): 1450-8. PMID 1058047.
  4. ↑ Meis, JM.; Enzinger, FM. (Apr 1992). "Proliferative fasciitis and myositis of childhood.". Am J Surg Pathol 16 (4): 364-72. PMID 1566969.
  5. ↑ Gleason, BC.; Hornick, JL. (Apr 2008). "Inflammatory myofibroblastic tumours: where are we now?". J Clin Pathol 61 (4): 428-37. doi:10.1136/jcp.2007.049387. PMID 17938159.
  6. ↑ 6.0 6.1 6.2 6.3 el-Jabbour, JN.; Bennett, MH.; Burke, MM.; Lessells, A.; O'Halloran, A. (Jul 1991). "Proliferative myositis. An immunohistochemical and ultrastructural study.". Am J Surg Pathol 15 (7): 654-9. PMID 2058761.
  7. ↑ Lundgren, L.; Kindblom, LG.; Willems, J.; Falkmer, U.; Angervall, L. (May 1992). "Proliferative myositis and fasciitis. A light and electron microscopic, cytologic, DNA-cytometric and immunohistochemical study.". APMIS 100 (5): 437-48. PMID 1586481.
  8. ↑ 8.0 8.1 8.2 8.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 609. ISBN 978-0781765275.
  9. ↑ Watanabe, H.; Ishida, Y.; Nagashima, K.; Makino, T.; Norisugi, O.; Shimizu, T. (Feb 2008). "Desmoplastic fibroblastoma (collagenous fibroma).". J Dermatol 35 (2): 93-7. doi:10.1111/j.1346-8138.2008.00421.x. PMID 18271804.
  10. ↑ 10.0 10.1 10.2 Walker, KR.; Bui-Mansfield, LT.; Gering, SA.; Ranlett, RD. (Dec 2004). "Collagenous fibroma (desmoplastic fibroblastoma) of the shoulder.". AJR Am J Roentgenol 183 (6): 1766. PMID 15547225.
  11. ↑ 11.0 11.1 11.2 Miettinen, M.; Fetsch, JF. (Jul 1998). "Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts.". Hum Pathol 29 (7): 676-82. PMID 9670823.
  12. ↑ Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 161. ISBN 978-0781740517.
  13. ↑ Takahara, M.; Ichikawa, R.; Oda, Y.; Uchi, H.; Takeuchi, S.; Moroi, Y.; Kiryu, H.; Furue, M. (Oct 2008). "Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis.". J Cutan Pathol 35 Suppl 1: 70-3. doi:10.1111/j.1600-0560.2007.00964.x. PMID 18544056.
  14. ↑ Macchia, G.; Trombetta, D.; Möller, E.; Mertens, F.; Storlazzi, CT.; Debiec-Rychter, M.; Sciot, R.; Nord, KH. (May 2012). "FOSL1 as a candidate target gene for 11q12 rearrangements in desmoplastic fibroblastoma.". Lab Invest 92 (5): 735-43. doi:10.1038/labinvest.2012.46. PMID 22411068.
  15. ↑ Attila T, Chen D, Gardiner GW, Ptak TW, Marcon NE (July 2006). "Gastric calcifying fibrous tumor". Can. J. Gastroenterol. 20 (7): 487–9. PMC 2659917. PMID 16858502. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659917/.
  16. ↑ 16.0 16.1 16.2 16.3 16.4 Flucke, U.; van Krieken, JH.; Mentzel, T. (Jan 2011). "Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma.". Mod Pathol 24 (1): 82-9. doi:10.1038/modpathol.2010.170. PMID 20852591.
  17. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 610. ISBN 978-0781765275.
  18. ↑ URL: http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung. Accessed on: 27 November 2011.
  19. ↑ Manohar, B.; Bhuvaneshwari, S. (Jan 2011). "Plasma cell granuloma of gingiva.". J Indian Soc Periodontol 15 (1): 64-6. doi:10.4103/0972-124X.82275. PMID 21772725.
  20. ↑ 20.0 20.1 20.2 20.3 Miyazawa, M.; Naritaka, Y.; Miyaki, A.; Asaka, S.; Isohata, N.; Yamaguchi, K.; Murayama, M.; Shimakawa, T. et al. (Sep 2011). "A low-grade myofibroblastic sarcoma in the abdominal cavity.". Anticancer Res 31 (9): 2989-94.
  21. ↑ Corsi, A.; Boldrini, R.; Bosman, C. (Oct 1994). "Congenital-infantile fibrosarcoma: study of two cases and review of the literature.". Tumori 80 (5): 392-400. PMID 7839472.
  22. ↑ Sheng, WQ.; Hisaoka, M.; Okamoto, S.; Tanaka, A.; Meis-Kindblom, JM.; Kindblom, LG.; Ishida, T.; Nojima, T. et al. (Mar 2001). "Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues.". Am J Clin Pathol 115 (3): 348-55. doi:10.1309/3H24-E7T7-V37G-AKKQ. PMID 11242790.
  23. ↑ Schweizer, L.; Koelsche, C.; Sahm, F.; Piro, RM.; Capper, D.; Reuss, DE.; Pusch, S.; Habel, A. et al. (May 2013). "Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein.". Acta Neuropathol 125 (5): 651-8. doi:10.1007/s00401-013-1117-6. PMID 23575898.
  24. ↑ Gengler, C.; Guillou, L. (Jan 2006). "Solitary fibrous tumour and haemangiopericytoma: evolution of a concept.". Histopathology 48 (1): 63-74. doi:10.1111/j.1365-2559.2005.02290.x. PMID 16359538.
  25. ↑ 25.0 25.1 25.2 URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
  26. ↑ 26.0 26.1 URL: http://emedicine.medscape.com/article/1255879-diagnosis. Accessed on: 2 May 2010.
  27. ↑ 27.0 27.1 Enzinger & Weiss's Soft Tissue Tumors. 4th Ed. PP.1007-13. ISBN 0-323-01200-0.
  28. ↑ Croul, SE. 8 November 2010.
  29. ↑ 29.0 29.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 611. ISBN 978-0781765275.
  30. ↑ Mentzel, T.; Katenkamp, D.; Fletcher, CD. (Mar 1996). "[Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course].". Pathologe 17 (2): 116-21. PMID 8650138.
  31. ↑ Fujimura, T.; Okuyama, R.; Terui, T.; Okuno, K.; Masu, A.; Masu, T.; Chiba, S.; Kunii, T. et al. (Aug 2005). "Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases.". J Cutan Pathol 32 (7): 512-5. doi:10.1111/j.0303-6987.2005.00368.x. PMID 16008697.
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