Difference between revisions of "Fibroblastic/myofibroblastic tumours"

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==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==

Revision as of 02:56, 29 September 2013

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:
    • Vesicular (clear) nuclei.
    • Prominent nucleoli.
  • +/-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

General

  • Benign.
  • Classically, subscapular in elderly women.[8][9]

Gross

Features:

  • Yellow-white, moderate demarcation to surrounding tissue.[10]

DDx - shoulder lesions:

Microscopic

Features:

  • Thick bundles of collagen.
  • Elastin fibres.

Image:

Nodular fasciitis

Desmoid-type fibromatosis

  • AKA desmoid tumour.
  • AKA desmoid fibromatosis.

General

Microscopic

Features:[12][13]

  • "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:

Images:

IHC

Features:[12]

  • Beta-catenin +ve - important.
  • SMA +ve ~50% of lesions.

Lipofibromatosis

  • AKA infantile subcutaneous fibromatosis.

General

  • Childhood.

Microscopic

Features:[12]

  • Fibroblastic cells surrounding adipocytes.

Image:

IHC

Features:[12]

  • 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.[16]
  • Age - typically 40s & 50s.[16]

Gross

  • Classically found in the shoulder region.

DDx - shoulder region:

Microscopic

Features:[17][18]

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

DDx:[17]

Images:

IHC

Features:[17]

Molecular

  • llq12 breakpoint described as being characteristic -- possibly the FOSL1 gene.[20]

Calcifying fibrous tumour

General

  • Rare.
  • Benign.

Microscopic

Features:[21]

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

Myofibroma

General

  • Benign.
  • Often < 2 years old; however, may be seen in adults.
  • Not common.[22]

Gross

  • Classically (oral) locations: tongue, buccal mucosa, lip.[22]

Microscopic

Features:[22]

  • Spindle cells with:
    • Tapering nuclei.
    • Eosinophilic cytoplasm.

Note:

DDx:

IHC

Features:[22]

  • Vimentin +ve.
  • SMA +ve.
  • S-100 -ve,

Cellular angiofibroma

General

Gross

Features:[24]

  • Superficial.
  • Well-circumscribed.

Classic location:

Microscopic

Features:[24]

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

IHC

Features:[24]

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

Occasionally metastasizing

Inflammatory myofibroblastic tumour

  • AKA inflammatory pseudotumour, AKA inflammatory fibrosarcoma,[25] AKA plasma cell granuloma.[26][27]

General

  • Mostly benign.
  • Children & young adults.
  • Classically located in mesentery of ileocolic region or small bowel.[25]

Microscopic

Features:[25]

  • Inflammation:
  • Spindle cells without atypia.
  • +/-Fasciular architecture.
  • Mitoses -- though none atypical.
  • +/-Necrosis.
  • +/-Hemorrhage.
  • Calcifications.

DDx:

Notes:

  • Some consider this a wastebasket diagnosis... for benign appearing spindle cell lesions.[29]

Images:

IHC

Features - dependent on site:

  • SMA +ve.[30]
  • Vimentin +ve.

Variable staining with:

Negative:[30]

  • S100, CD117, CD68.

Molecular

  • ALK rearrangements.[28]

Low-grade myofibroblastic sarcoma

General

  • Rare ~ 100 cases in the literature.
  • Usu. oral cavity or extremities.[32]

Microscopic

Features:

  • Spindle cells in the storiform pattern[32] or in fasicles.
  • Rare mitoses.

Images:

DDx:

IHC

  • SMA +ve.
  • CD34 -ve.
  • CD117 -ve.[32]
  • H-caldesmon -ve.[32]


Congenital-infantile fibrosarcoma

Should not be confused with adult fibrosarcoma.

General

  • Locally aggressive.

Microscopic

Features:[33]

  • Spindle cell lesion.

Molecular

Characteristic translocation:[34]

Solitary fibrous tumour

  • Abbreviated SFT.

General

  • Grouped with hemangiopericytoma in the WHO classification - as it is thought to be the same tumour.[12]
  • May be benign or malignant; more commonly benign.[35][36]
  • May be associated with hypoglycemia.
    • Known as Doege-Potter syndrome.[37]

Gross

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:[12]

  • Necrosis.
  • Mitoses >4/10 HPF -- definition suffers from HPFitis.
  • Increased cellularity.
  • Marked nuclear atypia.
  • Infiltrative margin.

Images:

IHC

  • CD34 ~90% +ve.
  • CD99 ~70% +ve.
  • BCL2 ~50% +ve.

Hemangiopericytoma

General

  • Grouped with solitary fibrous tumour in the WHO classification; possibly the same tumour (?).[12]
  • Arises from the pericyte, a connective tissue cell of small vessels that is thought to be involved in flow regulation.
  • Hematologic spread most common - to lungs.[38]
  • Oncogenic osteomalacia - assoc. with hemangiopericytoma.[38]

Presentation

  • Usually painless mass, slow enlargement.

Radiology

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

May be worked-up with angiography to distinguish from a vascular malformation.[39]

Location

  • Usually extremities - femur or proximal tibial.[38]

Microscopic

Features:[39]

  • Hypervascular lesion - key diagnostic feature.[40]
    • 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:[12][40]

  • 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:[41]

  • EMA -ve.
  • S100 -ve.

Malignant

Low-grade fibromyxoid sarcoma

  • AKA hyalinizing spindle cell tumour.
  • Should not be confused with myxofibrosarcoma.
  • Abbreviated LGFMS.

General

  • Deep soft tissue.
  • Related to hyalinizing spindle cell tumour with giant collagen rosettes.[42]

Microscopic

Features:[43]

  • Myxoid stroma alternating with fibrogenic areas - key feature.
  • Low cellularity.
  • Spindle cells.
  • +/-Rosette of collagen with central hyaline core.[44]

Notes:

  • Few/absent mitoses.

DDx:

Images:

IHC

Features:[44]

  • EMA +ve.
  • CD99 +ve.
  • BCL2 +ve.

Others:[44]

  • SMA -ve.
  • S100 -ve.
  • Desmin -ve.

Molecular pathology

Adult fibrosarcoma

General

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

Microscopic

Feature:[44]

  • Spindle cell lesion.
  • Herring bone pattern - key feature.
  • Mitoses.

DDx (herring bone):

  • MPNST.
  • Synovial sarcoma.
  • Fibrosarcoma.

DDx:

Images:

IHC

Features:[44]

  • Vimentin.
  • SMA.

Myxofibrosarcoma

General

Microscopic

Features:[48]

  • Discontinuous fibrous septae.
  • Myxoid background.
  • Variable cellularity and nuclear pleomorphism.
  • Spindle cells or epithelioid cells.[49]
  • Curvilinear vessels.[49]

DDx:

Image:

IHC

  • Vimentin +ve -- otherwise non-distinctive.[48]

See also

References

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  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.
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