Fibroblastic/myofibroblastic tumours

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This article covers fibroblastic/myofibroblastic tumours. These tumours fit into the larger category of soft tissue lesions.

Inflammatory myofibroblastic tumour

  • AKA inflammatory pseudotumour, AKA inflammatory fibrosarcoma.[1]

General

  • Mostly benign.
  • Children & young adults.
  • Classically located in mesentery of ileocolic region or small bowel.[1]

Microscopic

Features:[1]

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

DDx:

  • Calcifying fibrous pseudotumour (has psammomatous calcifications).
  • Inflammatory fibroid tumour.
  • Nodular fasciitis.

Notes:

  • Some consider this a wastebasket diagnosis... for benign appearing spindle cell lesions.[2]

Nodular fasciitis

General

  • Benign.
  • All age groups.
  • Associated with trauma.

Microscopic

Features:[3][4]

  • Usu. well-circumscribed.
  • Clusters of (non-pleomorphic) spindle cells.
  • Inflammation (lymphocytes).
  • Microcysts in cellular regions - uncommon - discriminatory.
  • Mitoses - common.
  • Extravasated RBCs.

The BD feature list:[5]

  • Tissue culture-like/CNS-like morphology.
  • Thick (keloid-like) collagen bundles - key feature.
  • Extravasated RBCs.
  • Inflammation.
  • +/-Giant cells.

Notes:

  • No significant nuclear atypia.
  • No atypical mitoses.
  • May be cellular.

Images:

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).[6]

Desmoid-type fibromatosis

  • AKA desmoid tumour.

General

Microscopic

Features:[8]

  • Abundant fibroblasts.
    • Arranged in bundles or fascicles.
  • +/-Collagen.

IHC

Features:[8]

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

Proliferative fasciitis

  • Need to write something here.

Solitary fibrous tumour

General

  • Grouped with hemangiopericytoma in the WHO classification; possibly the same tumour (?).[8]
  • May be benign or malignant; more commonly benign.[9][10]

Microscopic

Features:

  • Well-circumscribed.
  • Fibroblast-like cells (spindle cells).
  • Hemangiopericytoma-like area (staghorn vessels) - not seen on image.
  • Keloid-like collagen bundles.

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 (?).[8]
  • 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.[11]
  • Oncogenic osteomalacia - assoc. with hemangiopericytoma.[12]

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.[13]

Location

  • Usually extremities - femur or prox. tibial.[14]

Histology

Features:[15]

  • Hypervascular lesion - key diagnostic feature.[16]
    • 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.

IHC

Features:[8][16]

  • 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:[17]

  • EMA -ve.
  • S100 -ve.

DDx

  • Other vascular tumours.
  • Vascular malformations.
  • Synovial sarcoma.

Desmoplastic fibroblastoma

  • AKA collagenous fibroma.[18]
  • Benign lesion.
  • Classically found in shoulder region.

IHC

  • Beta-catenin -ve.[19]
    • Significance ???

Low-grade fibromyxoid sarcoma

  • AKA hyalinizing spindle cell tumour.

General

  • Deep soft tissue.

Microscopic

Features:[20]

  • Myoid stroma - key feature.
  • Low cellularity.
  • Spindle cells.

Notes:

  • Few/absent mitoses.

Molecular pathology

t(7;16)(q33;p11)[21]

See also

References

  1. ↑ 1.0 1.1 1.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 610. ISBN 978-0781765275.
  2. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2. Accessed on: 10 May 2011.
  3. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 606. ISBN 978-0781765275.
  4. ↑ de Feraudy S, Fletcher CD (September 2010). "Intradermal nodular fasciitis: a rare lesion analyzed in a series of 24 cases". Am. J. Surg. Pathol. 34 (9): 1377–81. doi:10.1097/PAS.0b013e3181ed7374. PMID 20716998.
  5. ↑ BD. 26 April 2011.
  6. ↑ Velagaleti GV, Tapper JK, Panova NE, Miettinen M, Gatalica Z (March 2003). "Cytogenetic findings in a case of nodular fasciitis of subclavicular region". Cancer Genet. Cytogenet. 141 (2): 160–3. PMID 12606136.
  7. ↑ URL: http://www.dtrf.org/dtrf_aboutdesmoids.htm. Accessed on: 15 April 2011.
  8. ↑ 8.0 8.1 8.2 8.3 8.4 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 609. ISBN 978-0781765275.
  9. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9. Accessed on: 25 June 2010.
  10. ↑ URL: http://wjso.com/content/6/1/86. Accessed on: 25 June 2010.
  11. ↑ URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
  12. ↑ URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
  13. ↑ URL: http://emedicine.medscape.com/article/1255879-diagnosis. Accessed on: 2 May 2010.
  14. ↑ URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
  15. ↑ URL: http://emedicine.medscape.com/article/1255879-diagnosis. Accessed on: 2 May 2010.
  16. ↑ 16.0 16.1 Enzinger & Weiss's Soft Tissue Tumors. 4th Ed. PP.1007-13. ISBN 0-323-01200-0.
  17. ↑ Croul, SE. 8 November 2010.
  18. ↑ Watanabe, H.; Ishida, Y.; Nagashima, K.; Makino, T.; Norisugi, O.; Shimizu, T. (Feb 2008). "Desmoplastic fibroblastoma (collagenous fibroma).". J Dermatol 35 (2): 93-7. doi:10.1111/j.1346-8138.2008.00421.x. PMID 18271804.
  19. ↑ Takahara, M.; Ichikawa, R.; Oda, Y.; Uchi, H.; Takeuchi, S.; Moroi, Y.; Kiryu, H.; Furue, M. (Oct 2008). "Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis.". J Cutan Pathol 35 Suppl 1: 70-3. doi:10.1111/j.1600-0560.2007.00964.x. PMID 18544056.
  20. ↑ Vernon SE, Bejarano PA (September 2006). "Low-grade fibromyxoid sarcoma: a brief review". Arch. Pathol. Lab. Med. 130 (9): 1358–60. PMID 16948525.
  21. ↑ Panagopoulos I, Storlazzi CT, Fletcher CD, et al. (July 2004). "The chimeric FUS/CREB3l2 gene is specific for low-grade fibromyxoid sarcoma". Genes Chromosomes Cancer 40 (3): 218–28. doi:10.1002/gcc.20037. PMID 15139001.
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