Difference between revisions of "Fibroblastic/myofibroblastic tumours"
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This article covers '''fibroblastic/myofibroblastic tumours'''. These tumours fit into the larger category of [[soft tissue lesions]]. | This article covers '''fibroblastic/myofibroblastic tumours'''. These tumours fit into the larger category of [[soft tissue lesions]]. | ||
==List of tumours== | |||
===Benign=== | |||
WHO classification:<ref name=Ref_WMSP601>{{Ref WMSP|601}}</ref> | |||
*[[Nodular fasciitis]]. | |||
*Proliferative fasciitis. | |||
*Proliferative myositis. | |||
*[[Myositis ossificans]]. | |||
*Ischemic fasciitis. | |||
*Elastofibroma. | |||
*Myofibroma. | |||
*Fibromatosis coli. | |||
*Inclusion body fibromatosis. | |||
*Fibroma of tendon sheath. | |||
*Calcifying aponeurotic fibroma. | |||
*Angiomyofbiroblstoma. | |||
*Cellular angiofibroma. | |||
*Nuchal-type fibroma. | |||
*Gardner fibroma. | |||
*Calcifying fibrous tumour. | |||
*Giant cell angiofibroma. | |||
*Fibrous hamartoma of infancy. | |||
*Juvenile hyaline fibromatosis. | |||
*Desmoplastic fibroblastoma. | |||
*Mammary-type myofibroblastoma. | |||
===Locally aggressive=== | |||
WHO classification:<ref name=Ref_WMSP601>{{Ref WMSP|601}}</ref> | |||
*Superfical fibromatosis. | |||
*[[Desmoid-type fibromatosis]]. | |||
*Lipofibromatosis. | |||
===Occasionally metastasizing=== | |||
WHO classification:<ref name=Ref_WMSP602>{{Ref WMSP|602}}</ref> | |||
*[[Solitary fibrous tumour]]. | |||
*[[Inflammatory myofibroblastic tumour]]. | |||
*Low-grad myofibroblastic sarcoma. | |||
*Myxoinflammatory fibroblastic sarcoma. | |||
*Infantile fibrosarcoma. | |||
===Malignant=== | |||
WHO classification:<ref name=Ref_WMSP602>{{Ref WMSP|602}}</ref> | |||
*Adult fibrosarcoma. | |||
*Myxofibrosarcoma. | |||
*[[Low-grade fibromyxoid sarcoma]] (hyalinizing spindle cell tumour). | |||
*Sclerosing epithelioid fibrosarcoma. | |||
==Inflammatory myofibroblastic tumour== | ==Inflammatory myofibroblastic tumour== | ||
Revision as of 18:25, 18 May 2011
This article covers fibroblastic/myofibroblastic tumours. These tumours fit into the larger category of soft tissue lesions.
List of tumours
Benign
WHO classification:[1]
- Nodular fasciitis.
- Proliferative fasciitis.
- Proliferative myositis.
- Myositis ossificans.
- Ischemic fasciitis.
- Elastofibroma.
- Myofibroma.
- Fibromatosis coli.
- Inclusion body fibromatosis.
- Fibroma of tendon sheath.
- Calcifying aponeurotic fibroma.
- Angiomyofbiroblstoma.
- Cellular angiofibroma.
- Nuchal-type fibroma.
- Gardner fibroma.
- Calcifying fibrous tumour.
- Giant cell angiofibroma.
- Fibrous hamartoma of infancy.
- Juvenile hyaline fibromatosis.
- Desmoplastic fibroblastoma.
- Mammary-type myofibroblastoma.
Locally aggressive
WHO classification:[1]
- Superfical fibromatosis.
- Desmoid-type fibromatosis.
- Lipofibromatosis.
Occasionally metastasizing
WHO classification:[2]
- Solitary fibrous tumour.
- Inflammatory myofibroblastic tumour.
- Low-grad myofibroblastic sarcoma.
- Myxoinflammatory fibroblastic sarcoma.
- Infantile fibrosarcoma.
Malignant
WHO classification:[2]
- Adult fibrosarcoma.
- Myxofibrosarcoma.
- Low-grade fibromyxoid sarcoma (hyalinizing spindle cell tumour).
- Sclerosing epithelioid fibrosarcoma.
Inflammatory myofibroblastic tumour
General
- Mostly benign.
- Children & young adults.
- Classically located in mesentery of ileocolic region or small bowel.[3]
Microscopic
Features:[3]
- 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.[4]
Nodular fasciitis
General
- Benign.
- All age groups.
- Associated with trauma.
Microscopic
- 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:[7]
- 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).[8]
Desmoid-type fibromatosis
- AKA desmoid tumour.
General
- Benign.
- Locally aggressive.[9]
- May be seen in the context of familial adenomatous polyposis.
Microscopic
Features:[10]
- Abundant fibroblasts.
- Arranged in bundles or fascicles.
- +/-Collagen.
IHC
Features:[10]
- 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 (?).[10]
- May be benign or malignant; more commonly benign.[11][12]
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 (?).[10]
- 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.[13]
- Oncogenic osteomalacia - assoc. with hemangiopericytoma.[14]
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.[15]
Location
- Usually extremities - femur or prox. tibial.[16]
Histology
Features:[17]
- Hypervascular lesion - key diagnostic feature.[18]
- 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.
- Abundant thin-walled branching small vessels of variable size.
- Spindle or ovoid shaped cells in nests or sheets.
IHC
- 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:[19]
- EMA -ve.
- S100 -ve.
DDx
- Other vascular tumours.
- Vascular malformations.
- Synovial sarcoma.
Desmoplastic fibroblastoma
- AKA collagenous fibroma.[20]
- Benign lesion.
- Classically found in shoulder region.
IHC
- Beta-catenin -ve.[21]
- Significance ???
Low-grade fibromyxoid sarcoma
- AKA hyalinizing spindle cell tumour.
General
- Deep soft tissue.
Microscopic
Features:[22]
- Myoid stroma - key feature.
- Low cellularity.
- Spindle cells.
Notes:
- Few/absent mitoses.
Molecular pathology
- t(7;16)(q33;p11).[23]
See also
References
- ↑ 1.0 1.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 601. ISBN 978-0781765275.
- ↑ 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.
- ↑ 3.0 3.1 3.2 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.
- ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2. Accessed on: 10 May 2011.
- ↑ 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.
- ↑ 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.
- ↑ BD. 26 April 2011.
- ↑ 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.
- ↑ URL: http://www.dtrf.org/dtrf_aboutdesmoids.htm. Accessed on: 15 April 2011.
- ↑ 10.0 10.1 10.2 10.3 10.4 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.
- ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9. Accessed on: 25 June 2010.
- ↑ URL: http://wjso.com/content/6/1/86. Accessed on: 25 June 2010.
- ↑ URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
- ↑ URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
- ↑ URL: http://emedicine.medscape.com/article/1255879-diagnosis. Accessed on: 2 May 2010.
- ↑ URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
- ↑ URL: http://emedicine.medscape.com/article/1255879-diagnosis. Accessed on: 2 May 2010.
- ↑ 18.0 18.1 Enzinger & Weiss's Soft Tissue Tumors. 4th Ed. PP.1007-13. ISBN 0-323-01200-0.
- ↑ Croul, SE. 8 November 2010.
- ↑ 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.
- ↑ 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.
- ↑ Vernon SE, Bejarano PA (September 2006). "Low-grade fibromyxoid sarcoma: a brief review". Arch. Pathol. Lab. Med. 130 (9): 1358–60. PMID 16948525.
- ↑ 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.