Difference between revisions of "Fibroblastic/myofibroblastic tumours"
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*[[AKA]] ''desmoid tumour''. | *[[AKA]] ''desmoid tumour''. | ||
*[[AKA]] ''desmoid fibromatosis''. | *[[AKA]] ''desmoid fibromatosis''. | ||
{{Main|Desmoid-type fibromatosis}} | |||
==Lipofibromatosis== | ==Lipofibromatosis== |
Revision as of 00:08, 9 November 2013
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.
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 fasciitis - several images (virginia.edu).
- Proliferative fasciitis (surgicalpathologyatlas.com).
Proliferative myositis
General
- Benign.
- Possible arise from pericytes.[6]
Microscopic
- 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
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
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
- AKA collagenous fibroma.[12]
- Not to be confused with desmoplastic fibroma.
General
- Benign lesion.
Epidemiology:
Gross
- Classically found in the shoulder region.
DDx - shoulder region:
Microscopic
- 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]
- Beta-catenin -ve.[16]
- +ve in desmoid-type fibromatosis.
- Desmin -ve.
- S-100 -ve.
- CD34 -ve.
- MSA +ve (focal).
- alpha-SMA +ve (focal).
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
General
- Benign.
- Often < 2 years old; however, may be seen in adults.
- Not common.[19]
Gross
- Classically (oral) locations: tongue, buccal mucosa, lip.[19]
Microscopic
Features:[19]
- Spindle cells with:
- Tapering nuclei.
- Eosinophilic cytoplasm.
Note:
- Features overlap with myopericytoma.[20]
DDx:
IHC
Features:[19]
- Vimentin +ve.
- SMA +ve.
- S-100 -ve,
Cellular angiofibroma
General
- Rare.
- Benign.
- Probably related to spindle cell lipoma and mammary-type myofibroblastoma.[21]
- Predominantly female.
Gross
Features:[21]
- Superficial.
- Well-circumscribed.
Classic location:
- Vulva.[21]
Microscopic
Features:[21]
- Spindle cell lesion.
- Many small-to-medium blood vessls.
IHC
Features:[21]
- CD34 ~50% of cases.
- SMA ~41% of cases.
- CD99 -ve.
- EMA -ve.
Occasionally metastasizing
Inflammatory myofibroblastic tumour
- AKA inflammatory pseudotumour, AKA inflammatory fibrosarcoma,[22] AKA plasma cell granuloma.[23][24]
General
- Mostly benign.
- Children & young adults.
- Classically located in mesentery of ileocolic region or small bowel.[22]
Microscopic
Features:[22]
- Inflammation:
- Plasma cells - predominant - key feature.[25]
- 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.[26]
Images:
IHC
Features - dependent on site:
- SMA +ve.[27]
- Vimentin +ve.
Variable staining with:
Negative:[27]
- S100, CD117, CD68.
Molecular
- ALK rearrangements.[25]
Low-grade myofibroblastic sarcoma
General
- Rare ~ 100 cases in the literature.
- Usu. oral cavity or extremities.[29]
Microscopic
Features:
- Spindle cells in the storiform pattern[29] or in fasicles.
- Rare mitoses.
Images:
DDx:
- Atypical leiomyoma.
- GIST.
- Leiomyosarcoma.
IHC
Congenital-infantile fibrosarcoma
- Should not be confused with adult fibrosarcoma.
General
- Locally aggressive.
Microscopic
Features:[30]
- Spindle cell lesion.
Molecular
Characteristic translocation:[31]
- t(12;15)(p13;q25).
- Gene fusion ETV6-NTRK3.
- Same translocation in mesoblastic nephroma.
- Gene fusion ETV6-NTRK3.
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.[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.
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.[36]
Microscopic
Features:[37]
- Myxoid stroma alternating with fibrogenic areas - key feature.
- Low cellularity.
- Spindle cells.
- +/-Rosette of collagen with central hyaline core.[38]
Notes:
- Few/absent mitoses.
DDx:
Images:
- www:
- WC:
IHC
Features:[38]
- EMA +ve.
- CD99 +ve.
- BCL2 +ve.
Others:[38]
- SMA -ve.
- S100 -ve.
- Desmin -ve.
Molecular pathology
Adult fibrosarcoma
- AKA fibrosarcoma.
- Should not be confused with infantile fibrosarcoma.
General
- Malignant.
- Older adults.
- Locations: head & neck, extremities.
Microscopic
Feature:[38]
- 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:[38]
- Vimentin.
- SMA.
Myxofibrosarcoma
- Should not be confused with low-grade fibromyxoid sarcoma.[40]
- AKA myxoid malignant fibrous histiocytoma or myxoid MFH.[41]
General
- Malignant.
- Usually older people, superficial (skin/dermis) and extremities (arm, legs).[42][43]
- Prognosis better than pleomorphic undifferentiated sarcoma.
- Metastatic potential inversely related to component/portion that is myxoid.[44]
Microscopic
Features:[42]
- Discontinuous fibrous septae.
- Myxoid background.
- Variable cellularity and nuclear pleomorphism.
- Spindle cells or epithelioid cells.[43]
- Curvilinear vessels.[43]
DDx:
- Myxoid liposarcoma.
- Low-grade fibromyxoid sarcoma - alternating fibrous and myxoid areas.[40]
- Myxoma.
Image:
IHC
- Vimentin +ve -- otherwise non-distinctive.[42]
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 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.
- ↑ 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.
- ↑ 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.
- ↑ 11.0 11.1 11.2 11.3 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.
- ↑ 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.
- ↑ 13.0 13.1 13.2 Walker, KR.; Bui-Mansfield, LT.; Gering, SA.; Ranlett, RD. (Dec 2004). "Collagenous fibroma (desmoplastic fibroblastoma) of the shoulder.". AJR Am J Roentgenol 183 (6): 1766. PMID 15547225.
- ↑ 14.0 14.1 14.2 Miettinen, M.; Fetsch, JF. (Jul 1998). "Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts.". Hum Pathol 29 (7): 676-82. PMID 9670823.
- ↑ 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.
- ↑ Macchia, G.; Trombetta, D.; Möller, E.; Mertens, F.; Storlazzi, CT.; Debiec-Rychter, M.; Sciot, R.; Nord, KH. (May 2012). "FOSL1 as a candidate target gene for 11q12 rearrangements in desmoplastic fibroblastoma.". Lab Invest 92 (5): 735-43. doi:10.1038/labinvest.2012.46. PMID 22411068.
- ↑ 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/.
- ↑ 19.0 19.1 19.2 19.3 Brasileiro, BF.; Martins-Filho, PR.; Piva, MR.; da Silva, LC.; Nonaka, CF.; Miguel, MC. (Jul 2010). "Myofibroma of the oral cavity. A rare spindle cell neoplasm.". Med Oral Patol Oral Cir Bucal 15 (4): e596-600. PMID 20038894. http://www.medicinaoral.com/pubmed/medoralv15_i4_p596.pdf.
- ↑ Dray MS, McCarthy SW, Palmer AA, et al. (January 2006). "Myopericytoma: a unifying term for a spectrum of tumours that show overlapping features with myofibroma. A review of 14 cases". J. Clin. Pathol. 59 (1): 67–73. doi:10.1136/jcp.2005.028704. PMC 1860256. PMID 16394283. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860256/.
- ↑ 21.0 21.1 21.2 21.3 21.4 Flucke, U.; van Krieken, JH.; Mentzel, T. (Jan 2011). "Cellular angiofibroma: analysis of 25 cases emphasizing its relationship to spindle cell lipoma and mammary-type myofibroblastoma.". Mod Pathol 24 (1): 82-9. doi:10.1038/modpathol.2010.170. PMID 20852591.
- ↑ 22.0 22.1 22.2 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.
- ↑ 25.0 25.1 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.
- ↑ 27.0 27.1 27.2 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.
- ↑ 29.0 29.1 29.2 29.3 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.
- ↑ 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.
- ↑ 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.
- ↑ Ramaswamy, AS.; Chatura, KR.. "Hyalinizing spindle cell tumor with giant rosettes.". Ann Saudi Med 31 (1): 83-6. PMC 3101732. PMID 20864787. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3101732/.
- ↑ Vernon SE, Bejarano PA (September 2006). "Low-grade fibromyxoid sarcoma: a brief review". Arch. Pathol. Lab. Med. 130 (9): 1358–60. PMID 16948525.
- ↑ 38.0 38.1 38.2 38.3 38.4 38.5 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.
- ↑ 40.0 40.1 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.
- ↑ Fujimura, T.; Okuyama, R.; Terui, T.; Okuno, K.; Masu, A.; Masu, T.; Chiba, S.; Kunii, T. et al. (Aug 2005). "Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases.". J Cutan Pathol 32 (7): 512-5. doi:10.1111/j.0303-6987.2005.00368.x. PMID 16008697.
- ↑ 42.0 42.1 42.2 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.
- ↑ 43.0 43.1 43.2 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.
- ↑ Weiss, SW.; Enzinger, FM. (Apr 1977). "Myxoid variant of malignant fibrous histiocytoma.". Cancer 39 (4): 1672-85. PMID 192434.