Difference between revisions of "Fibroblastic/myofibroblastic tumours"
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*[[ | *[[Dermatofibrosarcoma protuberans]] (DFSP) - t(17;22) COLA1/PDGFB. | ||
*Congenital-infantile fibrosarcoma - t(12;15) ETV6/NTRK3. | *[[Congenital-infantile fibrosarcoma]] - t(12;15) ETV6/NTRK3. | ||
*[[Solitary fibrous tumour]]. | *[[Solitary fibrous tumour]]. | ||
*[[Synovial sarcoma]] - t(X;18) SYT/SSX. | *[[Synovial sarcoma]] - t(X;18) SYT/SSX. | ||
Revision as of 16:15, 20 August 2012
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 colli.
- Inclusion body fibromatosis.
- Fibroma of tendon sheath.
- Calcifying aponeurotic fibroma.
- Angiomyofibroblastoma.
- 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-grade 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 - predominant - key feature.[6]
- 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.[7]
Images:
IHC
Features - dependent on site:
- SMA +ve.[8]
- Vimentin +ve.
Variable staining with:
Negative:[8]
- S100, CD117, CD68.
Molecular
- ALK rearrangements.[6]
Proliferative fasciitis
General
- Benign.
- May mimic a sarcoma.[10]
Clinical:
- Solid subcutaneous nodule.
- Rapid growth.
- May be painful.
Gross
- Classically upper and lower extremities.[10]
- Poorly demarcated.
Microscopic
Features:[11]
- 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 fasciitis - several images (virginia.edu).
- Proliferative fasciitis (surgicalpathologyatlas.com).
Proliferative myositis
General
- Benign.
- Possible arise from pericytes.[13]
Microscopic
- Large ganglion-like cells.
- Cells have single prominent nucleolus.
- Spindle cells.
- +/-Binucleation.
- Mitotic activity.
- No atypical mitoses.
Image:
IHC
Features:[13]
- Vimentin +ve.
- SMA +ve.
- Desmin +ve/-ve.
Others:[13]
- Factor XIIIa -ve.
- S100 -ve.
- CAM5.2 -ve.
- NSE -ve.
Elastofibroma
General
Gross
Features:
- Yellow-white, moderate demarcation to surrounding tissue.[17]
Microscopic
Features:
- Thick bundles of collagen.
- Elastin fibres.
Image:
Nodular fasciitis
- Not to be confused with necrotizing 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.
- 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.
DDx:[22]
- Myxoid DFSP.
- Cellular dermatofibroma.
- Desmoid-type fibromatosis.
Images:
- www:
- 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).[23]
Desmoid-type fibromatosis
General
- Benign.
- One of many fibromatoses.
- Locally aggressive.[24]
- May be seen in the context of familial adenomatous polyposis.
Microscopic
- "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:
- Hypertrophic scar-like lesion; see also dermal scar.
Images:
- Desmoid tumour (surgicalpathologyatlas.com).
- Desmoid tumour (cheapmedicinechest.com).[27]
- Desmoid tumour (radiographics.rsna.org).
- Desmoid tumour (WC).
IHC
Features:[25]
- Beta-catenin +ve - important.
- SMA +ve ~50% of lesions.
Low-grade myofibroblastic sarcoma
General
- Rare ~ 100 cases in the literature.
- Usu. oral cavity or extremities.[28]
Microscopic
Features:
- Spindle cells in the storiform pattern[28] or in fasicles.
- Rare mitoses.
Images:
DDx:
- Atypical leiomyoma.
- GIST.
- Leiomyosarcoma.
IHC
Lipofibromatosis
- AKA infantile subcutaneous fibromatosis.
General
- Childhood.
Microscopic
Features:[25]
- Fibroblastic cells surrounding adipocytes.
Image:
IHC
Features:[25]
- CD34 +ve.
- BCL2 +ve.
- S100 +ve.
- CD99 +ve.
- Actin +ve.
- EMA +ve.
Solitary fibrous tumour
- Abbreviated SFT.
General
- Grouped with hemangiopericytoma in the WHO classification - as it is thought to be the same tumour.[25]
- May be benign or malignant; more commonly benign.[29][30]
- May be associated with hypoglycemia.
- Known as Doege-Potter syndrome.[31]
Gross
- Classically arise from the pleura - see solitary fibrous tumour of the pleura.
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:[25]
- Necrosis.
- Mitoses >4/10 HPF -- definition suffers from HPFitis.
- Increased cellularity.
- Marked nuclear atypia.
- Infiltrative margin.
Images:
- WC:
- www:
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 (?).[25]
- 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.[32]
- Oncogenic osteomalacia - assoc. with hemangiopericytoma.[32]
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.[33]
Location
- Usually extremities - femur or proximal tibial.[32]
Microscopic
Features:[33]
- Hypervascular lesion - key diagnostic feature.[34]
- 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.
DDx:
- Other vascular tumours.
- Vascular malformations.
- Synovial sarcoma.
- Dermatofibroma. (???)
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:[35]
- EMA -ve.
- S100 -ve.
Desmoplastic fibroblastoma
- AKA collagenous fibroma.[36]
- Not to be confused with desmoplastic fibroma.
General
- Benign lesion.
- Classically found in shoulder region.
Epidemiology:
- May be on the lip.
Microscopic
- Acellular stroma with abundant collagen.
Notes:
- No nuclear atypia.
IHC
- Beta-catenin -ve.[39]
- +ve in desmoid-type fibromatosis.
Low-grade fibromyxoid sarcoma
- AKA hyalinizing spindle cell tumour.
- Should not be confused with myxofibrosarcoma.
- Abbreviated LGFMS.
General
- Deep soft tissue.
Microscopic
Features:[40]
- Myxoid stroma alternating with fibrogenic areas - key feature.
- Low cellularity.
- Spindle cells.
- +/-Rosette of collagen with central hyaline core.[41]
Notes:
- Few/absent mitoses.
DDx:
Images:
IHC
Features:[41]
- EMA +ve.
- CD99 +ve.
- BCL2 +ve.
Others:[41]
- SMA -ve.
- S100 -ve.
- Desmin -ve.
Molecular pathology
Calcifying fibrous tumour
General
- Rare.
- Benign.
Microscopic
Features:[43]
- Submucosal circumscribed fibrocollagenous nodule.
- Psammomatous calcifications.
- Focal plasma cells at the periphery.
Adult fibrosarcoma
- AKA fibrosarcoma.
- Should not be confused with infantile fibrosarcoma.
General
- Malignant.
- Older adults.
- Locations: head & neck, extremities.
Microscopic
Feature:[41]
- Spindle cell lesion.
- Herring bone pattern - key feature.
- Mitoses.
DDx (herring bone):
- MPNST.
- Synovial sarcoma.
- Fibrosarcoma.
DDx:
- Dermatofibrosarcoma protuberans (DFSP) - t(17;22) COLA1/PDGFB.
- Congenital-infantile fibrosarcoma - t(12;15) ETV6/NTRK3.
- Solitary fibrous tumour.
- Synovial sarcoma - t(X;18) SYT/SSX.
- MPNST.
Images:
IHC
Features:[41]
- Vimentin.
- SMA.
Congenital-infantile fibrosarcoma
- Should not be confused with adult fibrosarcoma.
General
- Locally aggressive.
Microscopic
Features:[44]
- Spindle cell lesion.
Molecular
Characteristic translocation:[45]
- t(12;15)(p13;q25).
- Gene fusion ETV6-NTRK3.
- Same translocation in mesoblastic nephroma.
- Gene fusion ETV6-NTRK3.
Myxofibrosarcoma
- Should not be confused with low-grade fibromyxoid sarcoma.[46]
General
Microscopic
Features:[47]
- Discontinuous fibrous septae.
- Myxoid background.
- Variable cellularity and nuclear pleomorphism.
- Spindle cells or epithelioid cells.[48]
- Curvilinear vessels.[48]
DDx:
- Myxoid liposarcoma.
- Low-grade fibromyxoid sarcoma - alternating fibrous and myxoid areas.[46]
Image:
IHC
- Vimentin +ve -- otherwise non-distinctive.[47]
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.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung. Accessed on: 27 November 2011.
- ↑ Manohar, B.; Bhuvaneshwari, S. (Jan 2011). "Plasma cell granuloma of gingiva.". J Indian Soc Periodontol 15 (1): 64-6. doi:10.4103/0972-124X.82275. PMID 21772725.
- ↑ 6.0 6.1 Saab, ST.; Hornick, JL.; Fletcher, CD.; Olson, SJ.; Coffin, CM. (Apr 2011). "IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link?". Mod Pathol 24 (4): 606-12. doi:10.1038/modpathol.2010.226. PMID 21297584.
- ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2. Accessed on: 10 May 2011.
- ↑ 8.0 8.1 8.2 Shi, H.; Li, Y.; Wei, L.; Sun, L. (Apr 2010). "Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases.". Pathology 42 (3): 235-41. doi:10.3109/00313021003631312. PMID 20350216.
Cite error: Invalid
<ref>tag; name "pmid20350216" defined multiple times with different content - ↑ Miyamoto, H.; Montgomery, EA.; Epstein, JI. (Apr 2010). "Paratesticular fibrous pseudotumor: a morphologic and immunohistochemical study of 13 cases.". Am J Surg Pathol 34 (4): 569-74. doi:10.1097/PAS.0b013e3181d438cb. PMID 20216379.
- ↑ 10.0 10.1 Chung, EB.; Enzinger, FM. (Oct 1975). "Proliferative fasciitis.". Cancer 36 (4): 1450-8. PMID 1058047.
- ↑ Meis, JM.; Enzinger, FM. (Apr 1992). "Proliferative fasciitis and myositis of childhood.". Am J Surg Pathol 16 (4): 364-72. PMID 1566969.
- ↑ 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.
- ↑ 13.0 13.1 13.2 13.3 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.
- ↑ 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.
- ↑ URL: http://emedicine.medscape.com/article/1057113-overview. Accessed on: 26 October 2011.
- ↑ Ben Hassouna, J.; Hamdi, N.; Ben Bachouche, W.; Bouzid, T.; Dhiab, T.; Rahal, K. (Oct 2010). "Elastofibroma dorsi.". Orthop Traumatol Surg Res 96 (6): 717-20. doi:10.1016/j.otsr.2010.03.019. PMID 20708994.
- ↑ Rose, Alan G. (2008). Atlas of Gross Pathology with Histologic Correlation (1st ed.). Cambridge University Press. pp. 592. ISBN 978-0521868792.
- ↑ 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.
- ↑ URL: http://anvita.info/wiki/Nodular_Fasciitis. Accessed on: 11 November 2011.
- ↑ URL: http://www.mckeedermpath.com/SPOT%20DIAGNOSIS%20CASE%20268.html. Accessed on: 11 November 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.
- ↑ 25.0 25.1 25.2 25.3 25.4 25.5 25.6 25.7 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.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196. Accessed on: 4 October 2011.
- ↑ URL: http://www.cheapmedicinechest.com/abdominal-pain-and-colonic-obstruction-from-an-intra-abdominal-desmoid-tumor.html. Accessed on: 4 October 2011.
- ↑ 28.0 28.1 28.2 28.3 Miyazawa, M.; Naritaka, Y.; Miyaki, A.; Asaka, S.; Isohata, N.; Yamaguchi, K.; Murayama, M.; Shimakawa, T. et al. (Sep 2011). "A low-grade myofibroblastic sarcoma in the abdominal cavity.". Anticancer Res 31 (9): 2989-94.
- ↑ 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.
- ↑ Roy, TM.; Burns, MV.; Overly, DJ.; Curd, BT. (Nov 1992). "Solitary fibrous tumor of the pleura with hypoglycemia: the Doege-Potter syndrome.". J Ky Med Assoc 90 (11): 557-60. PMID 1474302.
- ↑ 32.0 32.1 32.2 URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
- ↑ 33.0 33.1 URL: http://emedicine.medscape.com/article/1255879-diagnosis. Accessed on: 2 May 2010.
- ↑ 34.0 34.1 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.
- ↑ URL: http://www.dermatologyoutlines.com/dermskintumornonmelanocytic.html#collagenousfibroma. Accessed on: 19 March
- ↑ Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 161. ISBN 978-0781740517.
- ↑ 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.
- ↑ 41.0 41.1 41.2 41.3 41.4 41.5 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 611. ISBN 978-0781765275.
- ↑ 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.
- ↑ Attila T, Chen D, Gardiner GW, Ptak TW, Marcon NE (July 2006). "Gastric calcifying fibrous tumor". Can. J. Gastroenterol. 20 (7): 487–9. PMC 2659917. PMID 16858502. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659917/.
- ↑ Corsi, A.; Boldrini, R.; Bosman, C. (Oct 1994). "Congenital-infantile fibrosarcoma: study of two cases and review of the literature.". Tumori 80 (5): 392-400. PMID 7839472.
- ↑ Sheng, WQ.; Hisaoka, M.; Okamoto, S.; Tanaka, A.; Meis-Kindblom, JM.; Kindblom, LG.; Ishida, T.; Nojima, T. et al. (Mar 2001). "Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues.". Am J Clin Pathol 115 (3): 348-55. doi:10.1309/3H24-E7T7-V37G-AKKQ. PMID 11242790.
- ↑ 46.0 46.1 Mentzel, T.; Katenkamp, D.; Fletcher, CD. (Mar 1996). "[Low malignancy myxofibrosarcoma versus low malignancy fibromyxoid sarcoma. Distinct entities with similar names but different clinical course].". Pathologe 17 (2): 116-21. PMID 8650138.
- ↑ 47.0 47.1 47.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 612-3. ISBN 978-0781765275.
- ↑ 48.0 48.1 48.2 Nascimento, AF.; Bertoni, F.; Fletcher, CD. (Jan 2007). "Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases.". Am J Surg Pathol 31 (1): 99-105. doi:10.1097/01.pas.0000213379.94547.e7. PMID 17197925.