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| =Fibroblastic/myofibroblastic tumours= | | =Fibroblastic/myofibroblastic tumours= |
| | {{Main|Fibroblastic/myofibroblastic tumours}} |
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| ==Inflammatory myofibroblastic tumour==
| | This is a very large and important group of soft tissue lesions. It is covered in a separate article. |
| *[[AKA]] inflammatory pseudotumour, AKA inflammatory fibrosarcoma.<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
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| ===General===
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| *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.
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| **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 psammomatous calcifications).
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| *Inflammatory fibroid tumour.
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| *Nodular fasciitis.
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| Notes:
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| *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>
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| ==Nodular fasciitis==
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| ===General===
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| *Benign.
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| *All age groups.
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| *Associated with trauma.
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| ===Microscopic===
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| 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>
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| *Usu. well-circumscribed.
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| *Clusters of (non-pleomorphic) spindle cells.
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| *Inflammation (lymphocytes).
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| *Microcysts in cellular regions - uncommon - ''discriminatory''.
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| *Mitoses - common.
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| *Extravasated [[RBC]]s.
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| The BD feature list:<ref>BD. 26 April 2011.</ref>
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| *Tissue culture-like/CNS-like morphology.
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| *Thick (keloid-like) collagen bundles - '''key feature'''.
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| *Extravasated [[RBC]]s.
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| *Inflammation.
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| *+/-Giant cells.
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| Notes:
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| *No significant nuclear atypia.
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| *No atypical mitoses.
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| *May be cellular.
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| Images:
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| *[http://en.wikipedia.org/wiki/File:Nodular_fasciitis_%281%29.JPG NF - low mag. (WC)].
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| *[http://en.wikipedia.org/wiki/File:Nodular_fasciitis_%282%29.JPG NF - high mag. (WC)].
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| ===IHC===
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| Routine spindle cell panel:
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| *CD34 -ve.
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| *Desmin -ve..
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| *SMA -ve.
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| *S100 -ve.
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| *AE1/AE3 -ve.
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| Others:
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| *H-caldesmon -ve.
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| *EMA -ve.
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| *Vimentin +ve.
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| ===Molecular===
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| *Evolving - case reports.
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| **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>
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| ==Desmoid-type fibromatosis==
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| *[[AKA]] desmoid tumour.
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| ===General===
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| *Benign.
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| *Locally aggressive.<ref>URL: [http://www.dtrf.org/dtrf_aboutdesmoids.htm http://www.dtrf.org/dtrf_aboutdesmoids.htm]. Accessed on: 15 April 2011.</ref>
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| *May be seen in the context of [[familial adenomatous polyposis]].
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
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| *Abundant fibroblasts.
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| **Arranged in bundles or fascicles.
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| *+/-Collagen.
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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.
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| *SMA +ve ~50% of lesions.
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| ==Proliferative fasciitis==
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| *Need to write something here.
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| ==Solitary fibrous tumour==
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| ===General===
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| *Grouped with ''hemangiopericytoma'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
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| *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>
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| ===Microscopic===
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| Features:
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| *Well-circumscribed.
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| *Fibroblast-like cells (spindle cells).
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| *Hemangiopericytoma-like area (staghorn vessels) - not seen on image.
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| *Keloid-like collagen bundles.
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| Images:
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| *[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_low_mag.jpg SFT - low mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_intermed_mag.jpg SFT - intermed. mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Solitary_fibrous_tumour_high_mag.jpg SFT - high mag. (WC)].
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| ===IHC===
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| *CD34 ~90% +ve.
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| *CD99 ~70% +ve.
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| *BCL2 ~50% +ve.
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| ==Hemangiopericytoma==
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| ===General===
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| *Grouped with ''solitary fibrous tumour'' in the WHO classification; possibly the same tumour (?).<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref>
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| *Arises from the ''pericyte'', a connective tissue cell of small vessels that is thought to be involved in flow regulation.
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| *Hematologic spread most common - to lungs.<ref>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
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| *Oncogenic osteomalacia - assoc. with hemangiopericytoma.<ref>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
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| ===Presentation===
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| *Usually painless mass, slow enlargement.
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| ====Radiology====
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| *Intramedullary lytic mass.
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| *May be well-circumscribed.
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| *+/-Periosteal reaction.
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| *+/-Sclerotic border.
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| May be worked-up with angiography to distinguish from a [[vascular malformation]].<ref>URL: [http://emedicine.medscape.com/article/1255879-diagnosis http://emedicine.medscape.com/article/1255879-diagnosis]. Accessed on: 2 May 2010.</ref>
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| ===Location===
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| *Usually extremities - femur or prox. tibial.<ref>URL: [http://emedicine.medscape.com/article/1255879-overview http://emedicine.medscape.com/article/1255879-overview]. Accessed on: 2 May 2010.</ref>
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| ===Histology===
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| Features:<ref>URL: [http://emedicine.medscape.com/article/1255879-diagnosis http://emedicine.medscape.com/article/1255879-diagnosis]. Accessed on: 2 May 2010.</ref>
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| *Hypervascular lesion - '''key diagnostic feature'''.<ref name=enzinger>Enzinger & Weiss's Soft Tissue Tumors. 4th Ed. PP.1007-13. ISBN 0-323-01200-0.</ref>
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| **Abundant thin-walled branching small vessels of variable size.
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| ***May be described as "staghorn vessels" or "antler-like" vasculature.
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| ***Cells may "onion-skin" around thin blood vessels.
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| *Spindle or ovoid shaped cells in nests or sheets.
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| ===IHC===
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| Features:<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref name=enzinger/>
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| *Vimentin +ve (usually).
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| *Desmin -ve (typical).
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| *Factor VIII -ve (marks endothelium).
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| *CD34 +ve.
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| **CD34 usu. -ve in synovial sarcoma.
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| *CD31 -ve (marks benign endothelium).
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| *vWF (von Willebrand factor) -ve.
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| May be in the DDx for [[meningioma]]:<ref>Croul, SE. 8 November 2010.</ref>
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| *EMA -ve.
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| *S100 -ve.
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| ===DDx===
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| *Other vascular tumours.
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| *Vascular malformations.
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| *Synovial sarcoma.
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| ==Desmoplastic fibroblastoma==
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| *AKA ''collagenous fibroma''.<ref name=pmid18271804>{{Cite journal | last1 = Watanabe | first1 = H. | last2 = Ishida | first2 = Y. | last3 = Nagashima | first3 = K. | last4 = Makino | first4 = T. | last5 = Norisugi | first5 = O. | last6 = Shimizu | first6 = T. | title = Desmoplastic fibroblastoma (collagenous fibroma). | journal = J Dermatol | volume = 35 | issue = 2 | pages = 93-7 | month = Feb | year = 2008 | doi = 10.1111/j.1346-8138.2008.00421.x | PMID = 18271804 }}</ref>
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| *Benign lesion.
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| *Classically found in shoulder region.
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| ===IHC===
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| *Beta-catenin -ve.<ref name=pmid18544056>{{Cite journal | last1 = Takahara | first1 = M. | last2 = Ichikawa | first2 = R. | last3 = Oda | first3 = Y. | last4 = Uchi | first4 = H. | last5 = Takeuchi | first5 = S. | last6 = Moroi | first6 = Y. | last7 = Kiryu | first7 = H. | last8 = Furue | first8 = M. | title = Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis. | journal = J Cutan Pathol | volume = 35 Suppl 1 | issue = | pages = 70-3 | month = Oct | year = 2008 | doi = 10.1111/j.1600-0560.2007.00964.x | PMID = 18544056 }}
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| </ref>
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| **Significance ???
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| ==Low-grade fibromyxoid sarcoma==
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| *[[AKA]] ''hyalinizing spindle cell tumour''.
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| ===General===
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| *Deep soft tissue.
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| ===Microscopic===
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| 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>
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| *Myoid stroma - '''key feature'''.
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| *Low cellularity.
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| *Spindle cells.
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| Notes:
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| *Few/absent mitoses.
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| ===Molecular pathology===
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| 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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| =Perivascular tumours= | | =Perivascular tumours= |