Difference between revisions of "Fibroblastic/myofibroblastic tumours"

Jump to navigation Jump to search

Fibroblastic/myofibroblastic tumours (view source)

Revision as of 18:58, 24 March 2019

12,480 bytes removed ,  18:58, 24 March 2019
split out
(split out)
 
(39 intermediate revisions by 3 users not shown)
Line 67: Line 67:
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.
Line 111: Line 111:


==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>
 
===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==
:'''''Not''' to be confused with [[necrotizing fasciitis]]''.
{{Main|Nodular fasciitis}}
===General===
*Benign.
*All age groups.
*Associated with trauma.
 
===Microscopic===
Features:<ref name=Ref_WMSP606>{{Ref WMSP|606}}</ref><ref>{{cite journal |author=de Feraudy S, Fletcher CD |title=Intradermal nodular fasciitis: a rare lesion analyzed in a series of 24 cases |journal=Am. J. Surg. Pathol. |volume=34 |issue=9 |pages=1377–81 |year=2010 |month=September |pmid=20716998 |doi=10.1097/PAS.0b013e3181ed7374 |url=}}</ref>
*Usu. well-circumscribed.
*Clusters of (non-pleomorphic) spindle cells.
*Inflammation (lymphocytes).
*Microcysts in cellular regions - uncommon - ''discriminatory''.
*Mitoses - common.
*[[Extravasated RBC]]s.
 
The BD feature list:<ref>Dickson, B. 26 April 2011.</ref><ref>URL: [http://anvita.info/wiki/Nodular_Fasciitis http://anvita.info/wiki/Nodular_Fasciitis]. Accessed on: 11 November 2011.</ref>
*Tissue culture-like/CNS-like morphology.
*Thick (keloid-like) collagen bundles - '''key feature'''.
*[[Extravasated RBCs]].
*Inflammation.
*+/-[[Giant cell]]s.
 
Notes:
*No significant nuclear atypia.
*No atypical mitoses.
*May be cellular.
 
DDx:<ref>URL: [http://www.mckeedermpath.com/SPOT%20DIAGNOSIS%20CASE%20268.html http://www.mckeedermpath.com/SPOT%20DIAGNOSIS%20CASE%20268.html]. Accessed on: 11 November 2011.</ref>
*Myxoid [[DFSP]].
*Cellular [[dermatofibroma]].
*[[Desmoid-type fibromatosis]].
 
Images:
*www:
**[http://www.humpath.com/nodular-fasciitis NF (humpath.com)].
*[[WC]]:
**Nephron:
***[http://commons.wikimedia.org/wiki/File:Nodular_fasciitis_-_intermed_mag.jpg NF - case 1 - intermed. mag. (WC)].
***[http://commons.wikimedia.org/wiki/File:Nodular_fasciitis_-_high_mag.jpg NF - case 1 - high mag. (WC)].
***[http://commons.wikimedia.org/wiki/File:Nodular_fasciitis_-2-_intermed_mag.jpg NF - case 2 - intermed. mag. (WC)].
***[http://commons.wikimedia.org/wiki/File:Nodular_fasciitis_-2-_high_mag.jpg NF - case 2 - high mag. (WC)].
**KGH:
***[http://en.wikipedia.org/wiki/File:Nodular_fasciitis_%281%29.JPG NF - low mag. (WC)].
***[http://en.wikipedia.org/wiki/File:Nodular_fasciitis_%282%29.JPG NF - high mag. (WC)].
 
===IHC===
Routine spindle cell panel:
*CD34 -ve.
*Desmin -ve.
*SMA -ve.
*S100 -ve.
*AE1/AE3 -ve.
 
Others:
*H-caldesmon -ve.
*EMA -ve.
*Vimentin +ve.
 
===Molecular===
*Evolving - case reports.
**t(15;15)(q13;q25).<ref name=pmid12606136>{{cite journal |author=Velagaleti GV, Tapper JK, Panova NE, Miettinen M, Gatalica Z |title=Cytogenetic findings in a case of nodular fasciitis of subclavicular region |journal=Cancer Genet. Cytogenet. |volume=141 |issue=2 |pages=160–3 |year=2003 |month=March |pmid=12606136 |doi= |url=}}</ref>


==Desmoid-type fibromatosis==
==Desmoid-type fibromatosis==
*[[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.
*Spindle cells with:
**Small slender nuclei.
**Solid dark eosinophilic cytoplasm.
*+/-Mitoses - may be abundant.
*Long thin-walled vessels - parallel to one another - '''important feature'''.
 
Notes:
*[[Hypertrophic scar]]-like lesion; see also ''[[dermal scar]]''.
 
Images:
*[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://commons.wikimedia.org/wiki/File:DesmoidFibromatosis.JPG Desmoid tumour (WC)].
 
===IHC===
Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Beta-catenin +ve - '''important'''.
*SMA +ve ~50% of lesions.


==Lipofibromatosis==
==Lipofibromatosis==
Line 257: Line 156:
===Gross===
===Gross===
*Classically found in the shoulder region.
*Classically found in the shoulder region.
DDx - shoulder region:
*[[Desmoplastic fibroblastoma]].
*[[Elastofibroma]].


===Microscopic===
===Microscopic===
Line 299: Line 202:


==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==
Line 356: Line 234:
==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==
Line 436: Line 271:


===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==
*Abbreviated ''SFT''.
{{Main|Solitary fibrous tumour}}
===General===
*Grouped with ''hemangiopericytoma'' in the WHO classification - as it is thought to be the same tumour.<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*May be benign ''or'' malignant; more commonly benign.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9]. Accessed on: 25 June 2010.</ref><ref>URL: [http://wjso.com/content/6/1/86 http://wjso.com/content/6/1/86]. Accessed on: 25 June 2010.</ref>
*May be associated with hypoglycemia.
**Known as ''Doege-Potter syndrome''.<ref name=pmid1474302>{{Cite journal  | last1 = Roy | first1 = TM. | last2 = Burns | first2 = MV. | last3 = Overly | first3 = DJ. | last4 = Curd | first4 = BT. | title = Solitary fibrous tumor of the pleura with hypoglycemia: the Doege-Potter syndrome. | journal = J Ky Med Assoc | volume = 90 | issue = 11 | pages = 557-60 | month = Nov | year = 1992 | doi =  | PMID = 1474302 }}</ref>
 
===Gross===
*Classically arise from the pleura - see ''[[solitary fibrous tumour of the pleura]]''.
 
===Microscopic===
Features - benign:
*Spindle cells in a patternless pattern.
*Hemangiopericytoma-like area ([[staghorn vessels]]).
*Keloid-like collagen bundles - '''key feature'''.
*Usually well-circumscribed.
 
Criteria for malignancy:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
*Necrosis.
*Mitoses >4/10 HPF -- definition suffers from [[HPFitis]].
*Increased cellularity.
*Marked nuclear atypia.
*Infiltrative margin.
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_low_mag.jpg Benign SFT - low mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_intermed_mag.jpg Benign SFT - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_high_mag.jpg Benign SFT - high mag. (WC)].
*www:
**[http://path.upmc.edu/cases/case272.html SFT of the brain - several images (upmc.edu)].
 
===IHC===
*CD34 ~90% +ve.
*CD99 ~70% +ve.
*BCL2 ~50% +ve.


==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===
Line 508: Line 326:


DDx:
DDx:
*Other vascular tumours.
*Other [[vascular tumours]].
*Vascular malformations.
*[[Vascular malformations]].
*[[Synovial sarcoma]].
*[[Synovial sarcoma]].
*[[Dermatofibroma]]. (???)
*[[Dermatofibroma]]. (???)
Line 526: Line 344:
*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=
Line 532: Line 358:
*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]].
 
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==
Line 586: Line 372:
Feature:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
Feature:<ref name=Ref_WMSP611>{{Ref WMSP|611}}</ref>
*Spindle cell lesion.
*Spindle cell lesion.
*Herring bone pattern - '''key feature'''.
*[[Herring bone pattern]] - '''key feature'''.
*Mitoses.
*Mitoses.


Line 611: Line 397:
==Myxofibrosarcoma==
==Myxofibrosarcoma==
*Should '''not''' be confused with ''[[low-grade fibromyxoid sarcoma]]''.<ref name=pmid8650138>{{Cite journal  | last1 = Mentzel | first1 = T. | last2 = Katenkamp | first2 = D. | last3 = Fletcher | first3 = CD. | title = [Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course]. | journal = Pathologe | volume = 17 | issue = 2 | pages = 116-21 | month = Mar | year = 1996 | doi =  | PMID = 8650138 }}</ref>
*Should '''not''' be confused with ''[[low-grade fibromyxoid sarcoma]]''.<ref name=pmid8650138>{{Cite journal  | last1 = Mentzel | first1 = T. | last2 = Katenkamp | first2 = D. | last3 = Fletcher | first3 = CD. | title = [Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course]. | journal = Pathologe | volume = 17 | issue = 2 | pages = 116-21 | month = Mar | year = 1996 | doi =  | PMID = 8650138 }}</ref>
===General===
*[[AKA]] ''myxoid malignant fibrous histiocytoma'' or ''myxoid MFH''.<ref>{{Cite journal  | last1 = Fujimura | first1 = T. | last2 = Okuyama | first2 = R. | last3 = Terui | first3 = T. | last4 = Okuno | first4 = K. | last5 = Masu | first5 = A. | last6 = Masu | first6 = T. | last7 = Chiba | first7 = S. | last8 = Kunii | first8 = T. | last9 = Tagami | first9 = H. | title = Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases. | journal = J Cutan Pathol | volume = 32 | issue = 7 | pages = 512-5 | month = Aug | year = 2005 | doi = 10.1111/j.0303-6987.2005.00368.x | PMID = 16008697 }}</ref>
*Malignant.
{{Main|Myxofibrosarcoma}}
*Usu. older people, superficial (skin/dermis) and extremities (arm, legs).<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref><ref name=pmid17197925/>
 
===Microscopic===
Features:<ref name=Ref_WMSP612-3>{{Ref WMSP|612-3}}</ref>
*Discontinuous fibrous septae.
*Myxoid background.
*Variable cellularity and nuclear pleomorphism.
*Spindle cells or epithelioid cells.<ref name=pmid17197925/>
*Curvilinear vessels.<ref name=pmid17197925>{{Cite journal  | last1 = Nascimento | first1 = AF. | last2 = Bertoni | first2 = F. | last3 = Fletcher | first3 = CD. | title = Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases. | journal = Am J Surg Pathol | volume = 31 | issue = 1 | pages = 99-105 | month = Jan | year = 2007 | doi = 10.1097/01.pas.0000213379.94547.e7 | PMID = 17197925 }}</ref>
 
DDx:
*[[Liposarcoma|Myxoid liposarcoma]].
*[[Low-grade fibromyxoid sarcoma]] - alternating fibrous and myxoid areas.<ref name=pmid8650138/>
 
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=
Michael
48,933

edits

Retrieved from "https://librepathology.org/wiki/Special:MobileDiff/20182...49970"

Navigation menu