Difference between revisions of "Fibroblastic/myofibroblastic tumours"

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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==

Revision as of 22:46, 15 November 2014

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.

Lipofibromatosis

  • AKA infantile subcutaneous fibromatosis.

General

  • Childhood.

Microscopic

Features:[11]

  • Fibroblastic cells surrounding adipocytes.

Image:

IHC

Features:[11]

  • 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.[13]
  • Age - typically 40s & 50s.[13]

Gross

  • Classically found in the shoulder region.

DDx - shoulder region:

Microscopic

Features:[14][15]

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

DDx:[14]

Images:

IHC

Features:[14]

Molecular

  • llq12 breakpoint described as being characteristic -- possibly the FOSL1 gene.[17]

Calcifying fibrous tumour

General

  • Rare.
  • Benign.

Microscopic

Features:[18]

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

Myofibroma

Cellular angiofibroma

General

Gross

Features:[19]

  • Superficial.
  • Well-circumscribed.

Classic location:

Microscopic

Features:[19]

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

IHC

Features:[19]

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

Occasionally metastasizing

Inflammatory myofibroblastic tumour

  • AKA inflammatory pseudotumour, AKA inflammatory fibrosarcoma,[20] AKA plasma cell granuloma.[21][22]

Low-grade myofibroblastic sarcoma

General

  • Rare ~ 100 cases in the literature.
  • Usu. oral cavity or extremities.[23]

Microscopic

Features:

  • Spindle cells in the storiform pattern[23] or in fasicles.
  • Rare mitoses.

Images:

DDx:

IHC

  • SMA +ve.
  • CD34 -ve.
  • CD117 -ve.[23]
  • H-caldesmon -ve.[23]


Congenital-infantile fibrosarcoma

Should not be confused with adult fibrosarcoma.

General

  • Locally aggressive.

Microscopic

Features:[24]

  • Spindle cell lesion.

Molecular

Characteristic translocation:[25]

Solitary fibrous tumour

Hemangiopericytoma

General

  • Grouped with solitary fibrous tumour in the WHO classification; possibly the same tumour (?).[11]
  • 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.[26]
  • Oncogenic osteomalacia - assoc. with hemangiopericytoma.[26]

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.[27]

Location

  • Usually extremities - femur or proximal tibial.[26]

Microscopic

Features:[27]

  • Hypervascular lesion - key diagnostic feature.[28]
    • 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:[11][28]

  • 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:[29]

  • 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.[30]

Microscopic

Features:[31]

  • Myxoid stroma alternating with fibrogenic areas - key feature.
  • Low cellularity.
  • Spindle cells.
  • +/-Rosette of collagen with central hyaline core.[32]

Notes:

  • Few/absent mitoses.

DDx:

Images:

IHC

Features:[32]

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

Others:[32]

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

Molecular pathology

Adult fibrosarcoma

General

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

Microscopic

Feature:[32]

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

DDx (herring bone):

  • MPNST.
  • Synovial sarcoma.
  • Fibrosarcoma.

DDx:

Images:

IHC

Features:[32]

  • Vimentin.
  • SMA.

Myxofibrosarcoma

General

Microscopic

Features:[36]

  • Discontinuous fibrous septae.
  • Myxoid background.
  • Variable cellularity and nuclear pleomorphism.
  • Spindle cells or epithelioid cells.[37]
  • Curvilinear vessels.[37]

DDx:

Image:

IHC

  • Vimentin +ve -- otherwise non-distinctive.[36]

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.
  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. URL: http://emedicine.medscape.com/article/1057113-overview. Accessed on: 26 October 2011.
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