Fibroblastic/myofibroblastic tumours

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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:
  • +/-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

  • 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.[27]
  • Hematologic spread most common - to lungs.[28]
  • Oncogenic osteomalacia - assoc. with hemangiopericytoma.[28]
  • WHO grade II hemangiopericytoma (ICD-O: 9150/1), WHO grade III anaplastic hemangiopericytoma (ICD-O: 9150/3)

Presentation

  • 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

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

May be worked-up with angiography to distinguish from a vascular malformation.[29]

Location

  • Usually extremities - femur or proximal tibial.[28]

Microscopic

Features:[29]

  • Hypervascular lesion - key diagnostic feature.[30]
    • 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][30]

  • 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:[31]

  • EMA -ve.
  • S100 -ve.

Images

Malignant

Low-grade fibromyxoid sarcoma

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

Adult fibrosarcoma

General

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

Microscopic

Feature:[32]

DDx (herring bone):

  • MPNST.
  • Synovial sarcoma.
  • Fibrosarcoma.

DDx:

Images:

IHC

Features:[32]

  • Vimentin.
  • SMA.

Myxofibrosarcoma

See also

References

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