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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.
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| *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>
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| | |
| ===Gross===
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| Features:
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| *Yellow-white, moderate demarcation to surrounding tissue.<ref name=Ref_AoGP592>{{Ref AoGP|592}}</ref>
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| | |
| DDx - shoulder lesions:
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| *[[Desmoplastic fibroblastoma]].
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| *[[Pleomorphic lipoma]].
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| | |
| ===Microscopic===
| |
| Features:
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| *Thick bundles of collagen.
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| *Elastin fibres.
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| | |
| Image:
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| *[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20080802163630229 Elastofibroma (surgicalpathologyatlas.com)].
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|
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| ==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]].
| |
| | |
| ===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.
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| *Spindle cells with:
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| **Small slender nuclei.
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| **Solid dark eosinophilic cytoplasm.
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| *+/-Mitoses - may be abundant.
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| *Long thin-walled vessels - parallel to one another - '''important feature'''.
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| Notes:
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| *[[Hypertrophic scar]]-like lesion; see also ''[[dermal scar]]''.
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| | |
| DDx:
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| *[[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>
| |
| *[[Retroperitoneal fibrosis]] - no beta-catenin staining.<ref name=pmid23020601/>
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| *Other [[fibromatoses]].
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| | |
| ====Images====
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| <gallery>
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| Image:DesmoidFibromatosis.JPG | Desmoid tumour. (WC)
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| </gallery>
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| www:
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| *[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196 Desmoid tumour (surgicalpathologyatlas.com)].
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| *[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>
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| *[http://radiographics.rsna.org/content/29/7/2143/F28.expansion.html Desmoid tumour (radiographics.rsna.org)].
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| *[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>
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| | |
| ===IHC===
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| Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
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| *Beta-catenin +ve (nuclear<ref name=pmid23020601/>) - '''important'''.
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| *SMA +ve ~50% of lesions.
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| Others:
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| *CD117 -ve.
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|
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|
| ==Lipofibromatosis== | | ==Lipofibromatosis== |
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| ==Myofibroma== | | ==Myofibroma== |
| ===General===
| | {{Main|Myofibroma}} |
| *Benign.
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| *Often < 2 years old; however, may be seen in adults.
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| *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>
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| ===Gross===
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| *Classically (oral) locations: tongue, buccal mucosa, lip.<ref name=pmid20038894/>
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| ===Microscopic===
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| Features:<ref name=pmid20038894/>
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| *Spindle cells with:
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| **Tapering nuclei.
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| **Eosinophilic cytoplasm.
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| Note:
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| *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>
| |
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| DDx:
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| *[[Neurofibroma]].
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| *[[Leiomyoma]].
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| | |
| ===IHC===
| |
| Features:<ref name=pmid20038894/>
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| *Vimentin +ve.
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| *SMA +ve.
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| *S-100 -ve,
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|
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|
| ==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.
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| *Children & young adults.
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| *Classically located in mesentery of ileocolic region or small bowel.<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
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| | |
| ===Microscopic===
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| Features:<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
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| *Inflammation:
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| **[[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.
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| **Eosinophils.
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| *Spindle cells without atypia.
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| *+/-Fasciular architecture.
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| *Mitoses -- though none atypical.
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| *+/-Necrosis.
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| *+/-Hemorrhage.
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| *Calcifications.
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| | |
| DDx:
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| *[[Calcifying fibrous pseudotumour]] (has [[psammoma body|psammomatous calcifications]]).
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| *[[Inflammatory fibroid tumour]].
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| *[[Nodular fasciitis]].
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| | |
| 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)].
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| *[http://commons.wikimedia.org/wiki/File:Inflammatory_myofibroblastic_tumour_-_very_high_mag.jpg IMT - very high mag. (WC)].
| |
| | |
| ===IHC===
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| Features - dependent on site:
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| *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>
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| *Vimentin +ve.
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| Variable staining with:
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| *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/>
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| *S100, CD117, CD68.
| |
| | |
| ===Molecular===
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| *ALK rearrangements.<ref name=pmid21297584/>
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|
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| ==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.
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| *Spindle cells.
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| *+/-Rosette of collagen with central hyaline core.<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
| |
| | |
| Notes:
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| *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>
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| *EMA +ve.
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| *CD99 +ve.
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| *BCL2 +ve.
| |
| | |
| Others:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
| |
| *SMA -ve.
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| *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>
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| *t(11;16).<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
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| ==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.
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| *Usually older people, superficial (skin/dermis) and extremities (arm, legs).<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref><ref name=pmid17197925/>
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| *Prognosis better than [[pleomorphic undifferentiated sarcoma]].
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| **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>
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref>
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| *Discontinuous fibrous septae.
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| *Myxoid background.
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| *Variable cellularity and nuclear pleomorphism.
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| *Spindle cells or epithelioid cells.<ref name=pmid17197925/>
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| *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>
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| DDx:
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| *[[Liposarcoma|Myxoid liposarcoma]].
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| *[[Low-grade fibromyxoid sarcoma]] - alternating fibrous and myxoid areas.<ref name=pmid8650138/>
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| *[[Myxoma]].
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| Image:
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| *[http://www.sarcomaimages.com/sub.php?v=sample-case&p=myxofibrosarcoma-myxoid-mfh Myxofibrosarcoma (sarcomaimages.com)].
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| ===IHC===
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| *Vimentin +ve -- otherwise non-distinctive.<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref>
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| =See also= | | =See also= |