Difference between revisions of "Fibroblastic/myofibroblastic tumours"

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(subdivide into non-malignant and malignant section, move 'calcifying fibrous tumour' up)
(rearr)
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=Non-malignant=
=Non-malignant=
==Inflammatory myofibroblastic tumour==
*[[AKA]] inflammatory pseudotumour, AKA inflammatory fibrosarcoma,<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref> AKA plasma cell granuloma.<ref>URL: [http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung]. Accessed on: 27 November 2011.</ref><ref name=pmid21772725>{{Cite journal  | last1 = Manohar | first1 = B. | last2 = Bhuvaneshwari | first2 = S. | title = Plasma cell granuloma of gingiva. | journal = J Indian Soc Periodontol | volume = 15 | issue = 1 | pages = 64-6 | month = Jan | year = 2011 | doi = 10.4103/0972-124X.82275 | PMID = 21772725 }}</ref>
===General===
*Mostly benign.
*Children & young adults.
*Classically located in mesentery of ileocolic region or small bowel.<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
===Microscopic===
Features:<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
*Inflammation:
**[[Plasma cells]] - predominant - '''key feature'''.<ref name=pmid21297584>{{Cite journal  | last1 = Saab | first1 = ST. | last2 = Hornick | first2 = JL. | last3 = Fletcher | first3 = CD. | last4 = Olson | first4 = SJ. | last5 = Coffin | first5 = CM. | title = IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link? | journal = Mod Pathol | volume = 24 | issue = 4 | pages = 606-12 | month = Apr | year = 2011 | doi = 10.1038/modpathol.2010.226 | PMID = 21297584 }}</ref>
**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.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2]. Accessed on: 10 May 2011.</ref>
Images:
*[http://commons.wikimedia.org/wiki/File:Inflammatory_myofibroblastic_tumour_-_high_mag.jpg IMT - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Inflammatory_myofibroblastic_tumour_-_very_high_mag.jpg IMT - very high mag. (WC)].
===IHC===
Features - dependent on site:
*SMA +ve.<ref name=pmid20350216 >{{Cite journal  | last1 = Shi | first1 = H. | last2 = Li | first2 = Y. | last3 = Wei | first3 = L. | last4 = Sun | first4 = L. | title = Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases. | journal = Pathology | volume = 42 | issue = 3 | pages = 235-41 | month = Apr | year = 2010 | doi = 10.3109/00313021003631312 | PMID = 20350216 }}
</ref>
*Vimentin +ve.
Variable staining with:
*CD34, AE1/AE3, calretin.<ref name=pmid20216379>{{Cite journal  | last1 = Miyamoto | first1 = H. | last2 = Montgomery | first2 = EA. | last3 = Epstein | first3 = JI. | title = Paratesticular fibrous pseudotumor: a morphologic and immunohistochemical study of 13 cases. | journal = Am J Surg Pathol | volume = 34 | issue = 4 | pages = 569-74 | month = Apr | year = 2010 | doi = 10.1097/PAS.0b013e3181d438cb | PMID = 20216379 }}</ref><ref name=pmid20350216>{{Cite journal  | last1 = Shi | first1 = H. | last2 = Li | first2 = Y. | last3 = Wei | first3 = L. | last4 = Sun | first4 = L. | title = Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases. | journal = Pathology | volume = 42 | issue = 3 | pages = 235-41 | month = Apr | year = 2010 | doi = 10.3109/00313021003631312 | PMID = 20350216 }}</ref>
Negative:<ref name=pmid20350216/>
*S100, CD117, CD68.
===Molecular===
*ALK rearrangements.<ref name=pmid21297584/>
==Proliferative fasciitis==
==Proliferative fasciitis==
===General===
===General===
Line 315: Line 267:
*Actin +ve.
*Actin +ve.
*EMA +ve.
*EMA +ve.
==Desmoplastic fibroblastoma==
*AKA ''collagenous fibroma''.<ref name=pmid18271804>{{Cite journal  | last1 = Watanabe | first1 = H. | last2 = Ishida | first2 = Y. | last3 = Nagashima | first3 = K. | last4 = Makino | first4 = T. | last5 = Norisugi | first5 = O. | last6 = Shimizu | first6 = T. | title = Desmoplastic fibroblastoma (collagenous fibroma). | journal = J Dermatol | volume = 35 | issue = 2 | pages = 93-7 | month = Feb | year = 2008 | doi = 10.1111/j.1346-8138.2008.00421.x | PMID = 18271804 }}</ref>
*'''Not''' to be confused with ''[[desmoplastic fibroma]]''.
===General===
*Benign lesion.
*Classically found in shoulder region.
Epidemiology:
*May be on the lip.
===Microscopic===
Features:<ref>URL: [http://www.dermatologyoutlines.com/dermskintumornonmelanocytic.html#collagenousfibroma http://www.dermatologyoutlines.com/dermskintumornonmelanocytic.html#collagenousfibroma]. Accessed on: 19 March</ref><ref name=Ref_Sternberg4_161>{{Ref Sternberg4|161}}</ref>
*Acellular stroma with abundant collagen.
Notes:
*'''No''' nuclear atypia.
===IHC===
*Beta-catenin -ve.<ref name=pmid18544056>{{Cite journal  | last1 = Takahara | first1 = M. | last2 = Ichikawa | first2 = R. | last3 = Oda | first3 = Y. | last4 = Uchi | first4 = H. | last5 = Takeuchi | first5 = S. | last6 = Moroi | first6 = Y. | last7 = Kiryu | first7 = H. | last8 = Furue | first8 = M. | title = Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis. | journal = J Cutan Pathol | volume = 35 Suppl 1 | issue =  | pages = 70-3 | month = Oct | year = 2008 | doi = 10.1111/j.1600-0560.2007.00964.x | PMID = 18544056 }}
</ref>
**+ve in [[desmoid-type fibromatosis]].
==Calcifying fibrous tumour==
===General===
*Rare.
*Benign.
===Microscopic===
Features:<ref name=pmid16858502>{{cite journal |author=Attila T, Chen D, Gardiner GW, Ptak TW, Marcon NE |title=Gastric calcifying fibrous tumor |journal=Can. J. Gastroenterol. |volume=20 |issue=7 |pages=487–9 |year=2006 |month=July |pmid=16858502 |pmc=2659917 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659917/}}</ref>
*Submucosal circumscribed fibrocollagenous nodule.
*Psammomatous calcifications.
*Focal plasma cells at the periphery.
=Occasionally metastasizing=
==Inflammatory myofibroblastic tumour==
*[[AKA]] inflammatory pseudotumour, AKA inflammatory fibrosarcoma,<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref> AKA plasma cell granuloma.<ref>URL: [http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung]. Accessed on: 27 November 2011.</ref><ref name=pmid21772725>{{Cite journal  | last1 = Manohar | first1 = B. | last2 = Bhuvaneshwari | first2 = S. | title = Plasma cell granuloma of gingiva. | journal = J Indian Soc Periodontol | volume = 15 | issue = 1 | pages = 64-6 | month = Jan | year = 2011 | doi = 10.4103/0972-124X.82275 | PMID = 21772725 }}</ref>
===General===
*Mostly benign.
*Children & young adults.
*Classically located in mesentery of ileocolic region or small bowel.<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
===Microscopic===
Features:<ref name=Ref_WMSP610>{{Ref WMSP|610}}</ref>
*Inflammation:
**[[Plasma cells]] - predominant - '''key feature'''.<ref name=pmid21297584>{{Cite journal  | last1 = Saab | first1 = ST. | last2 = Hornick | first2 = JL. | last3 = Fletcher | first3 = CD. | last4 = Olson | first4 = SJ. | last5 = Coffin | first5 = CM. | title = IgG4 plasma cells in inflammatory myofibroblastic tumor: inflammatory marker or pathogenic link? | journal = Mod Pathol | volume = 24 | issue = 4 | pages = 606-12 | month = Apr | year = 2011 | doi = 10.1038/modpathol.2010.226 | PMID = 21297584 }}</ref>
**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.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2]. Accessed on: 10 May 2011.</ref>
Images:
*[http://commons.wikimedia.org/wiki/File:Inflammatory_myofibroblastic_tumour_-_high_mag.jpg IMT - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Inflammatory_myofibroblastic_tumour_-_very_high_mag.jpg IMT - very high mag. (WC)].
===IHC===
Features - dependent on site:
*SMA +ve.<ref name=pmid20350216 >{{Cite journal  | last1 = Shi | first1 = H. | last2 = Li | first2 = Y. | last3 = Wei | first3 = L. | last4 = Sun | first4 = L. | title = Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases. | journal = Pathology | volume = 42 | issue = 3 | pages = 235-41 | month = Apr | year = 2010 | doi = 10.3109/00313021003631312 | PMID = 20350216 }}
</ref>
*Vimentin +ve.
Variable staining with:
*CD34, AE1/AE3, calretin.<ref name=pmid20216379>{{Cite journal  | last1 = Miyamoto | first1 = H. | last2 = Montgomery | first2 = EA. | last3 = Epstein | first3 = JI. | title = Paratesticular fibrous pseudotumor: a morphologic and immunohistochemical study of 13 cases. | journal = Am J Surg Pathol | volume = 34 | issue = 4 | pages = 569-74 | month = Apr | year = 2010 | doi = 10.1097/PAS.0b013e3181d438cb | PMID = 20216379 }}</ref><ref name=pmid20350216>{{Cite journal  | last1 = Shi | first1 = H. | last2 = Li | first2 = Y. | last3 = Wei | first3 = L. | last4 = Sun | first4 = L. | title = Primary colorectal inflammatory myofibroblastic tumour: a clinicopathological and immunohistochemical study of seven cases. | journal = Pathology | volume = 42 | issue = 3 | pages = 235-41 | month = Apr | year = 2010 | doi = 10.3109/00313021003631312 | PMID = 20350216 }}</ref>
Negative:<ref name=pmid20350216/>
*S100, CD117, CD68.
===Molecular===
*ALK rearrangements.<ref name=pmid21297584/>
==Congenital-infantile fibrosarcoma==
:Should not be confused with ''[[adult fibrosarcoma]]''.
===General===
*Locally aggressive.
===Microscopic===
Features:<ref name=pmid7839472>{{Cite journal  | last1 = Corsi | first1 = A. | last2 = Boldrini | first2 = R. | last3 = Bosman | first3 = C. | title = Congenital-infantile fibrosarcoma: study of two cases and review of the literature. | journal = Tumori | volume = 80 | issue = 5 | pages = 392-400 | month = Oct | year = 1994 | doi =  | PMID = 7839472 }}</ref>
*Spindle cell lesion.
===Molecular===
Characteristic [[translocation]]:<ref>{{Cite journal  | last1 = Sheng | first1 = WQ. | last2 = Hisaoka | first2 = M. | last3 = Okamoto | first3 = S. | last4 = Tanaka | first4 = A. | last5 = Meis-Kindblom | first5 = JM. | last6 = Kindblom | first6 = LG. | last7 = Ishida | first7 = T. | last8 = Nojima | first8 = T. | last9 = Hashimoto | first9 = H. | title = Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. | journal = Am J Clin Pathol | volume = 115 | issue = 3 | pages = 348-55 | month = Mar | year = 2001 | doi = 10.1309/3H24-E7T7-V37G-AKKQ | PMID = 11242790 }}</ref>
*t(12;15)(p13;q25).
**Gene fusion ETV6-NTRK3.
***Same translocation in [[mesoblastic nephroma]].


==Solitary fibrous tumour==
==Solitary fibrous tumour==
Line 401: Line 449:
*EMA -ve.
*EMA -ve.
*S100 -ve.
*S100 -ve.
==Desmoplastic fibroblastoma==
*AKA ''collagenous fibroma''.<ref name=pmid18271804>{{Cite journal  | last1 = Watanabe | first1 = H. | last2 = Ishida | first2 = Y. | last3 = Nagashima | first3 = K. | last4 = Makino | first4 = T. | last5 = Norisugi | first5 = O. | last6 = Shimizu | first6 = T. | title = Desmoplastic fibroblastoma (collagenous fibroma). | journal = J Dermatol | volume = 35 | issue = 2 | pages = 93-7 | month = Feb | year = 2008 | doi = 10.1111/j.1346-8138.2008.00421.x | PMID = 18271804 }}</ref>
*'''Not''' to be confused with ''[[desmoplastic fibroma]]''.
===General===
*Benign lesion.
*Classically found in shoulder region.
Epidemiology:
*May be on the lip.
===Microscopic===
Features:<ref>URL: [http://www.dermatologyoutlines.com/dermskintumornonmelanocytic.html#collagenousfibroma http://www.dermatologyoutlines.com/dermskintumornonmelanocytic.html#collagenousfibroma]. Accessed on: 19 March</ref><ref name=Ref_Sternberg4_161>{{Ref Sternberg4|161}}</ref>
*Acellular stroma with abundant collagen.
Notes:
*'''No''' nuclear atypia.
===IHC===
*Beta-catenin -ve.<ref name=pmid18544056>{{Cite journal  | last1 = Takahara | first1 = M. | last2 = Ichikawa | first2 = R. | last3 = Oda | first3 = Y. | last4 = Uchi | first4 = H. | last5 = Takeuchi | first5 = S. | last6 = Moroi | first6 = Y. | last7 = Kiryu | first7 = H. | last8 = Furue | first8 = M. | title = Desmoplastic fibroblastoma: a case presenting as a protruding nodule in the dermis. | journal = J Cutan Pathol | volume = 35 Suppl 1 | issue =  | pages = 70-3 | month = Oct | year = 2008 | doi = 10.1111/j.1600-0560.2007.00964.x | PMID = 18544056 }}
</ref>
**+ve in [[desmoid-type fibromatosis]].
==Calcifying fibrous tumour==
===General===
*Rare.
*Benign.
===Microscopic===
Features:<ref name=pmid16858502>{{cite journal |author=Attila T, Chen D, Gardiner GW, Ptak TW, Marcon NE |title=Gastric calcifying fibrous tumor |journal=Can. J. Gastroenterol. |volume=20 |issue=7 |pages=487–9 |year=2006 |month=July |pmid=16858502 |pmc=2659917 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659917/}}</ref>
*Submucosal circumscribed fibrocollagenous nodule.
*Psammomatous calcifications.
*Focal plasma cells at the periphery.


=Malignant=
=Malignant=
Line 510: Line 525:
*Vimentin.
*Vimentin.
*SMA.
*SMA.
==Congenital-infantile fibrosarcoma==
:Should not be confused with ''[[adult fibrosarcoma]]''.
===General===
*Locally aggressive.
===Microscopic===
Features:<ref name=pmid7839472>{{Cite journal  | last1 = Corsi | first1 = A. | last2 = Boldrini | first2 = R. | last3 = Bosman | first3 = C. | title = Congenital-infantile fibrosarcoma: study of two cases and review of the literature. | journal = Tumori | volume = 80 | issue = 5 | pages = 392-400 | month = Oct | year = 1994 | doi =  | PMID = 7839472 }}</ref>
*Spindle cell lesion.
===Molecular===
Characteristic [[translocation]]:<ref>{{Cite journal  | last1 = Sheng | first1 = WQ. | last2 = Hisaoka | first2 = M. | last3 = Okamoto | first3 = S. | last4 = Tanaka | first4 = A. | last5 = Meis-Kindblom | first5 = JM. | last6 = Kindblom | first6 = LG. | last7 = Ishida | first7 = T. | last8 = Nojima | first8 = T. | last9 = Hashimoto | first9 = H. | title = Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. | journal = Am J Clin Pathol | volume = 115 | issue = 3 | pages = 348-55 | month = Mar | year = 2001 | doi = 10.1309/3H24-E7T7-V37G-AKKQ | PMID = 11242790 }}</ref>
*t(12;15)(p13;q25).
**Gene fusion ETV6-NTRK3.
***Same translocation in [[mesoblastic nephroma]].


==Myxofibrosarcoma==
==Myxofibrosarcoma==

Revision as of 19:45, 10 September 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]

  • Fibrous hamartoma of infancy.
  • Juvenile hyaline fibromatosis.
  • Desmoplastic fibroblastoma.
  • Mammary-type myofibroblastoma.

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:
    • Vesicular (clear) nuclei.
    • Prominent nucleoli.
  • +/-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]

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

Features:[11][12]

  • 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:[13][14]

  • 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:[15]

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).[16]

Desmoid-type fibromatosis

  • AKA desmoid tumour.
  • AKA desmoid fibromatosis.

General

Microscopic

Features:[18][19]

  • "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:

Images:

IHC

Features:[18]

  • 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.[21]

Microscopic

Features:

  • Spindle cells in the storiform pattern[21] or in fasicles.
  • Rare mitoses.

Images:

DDx:

IHC

  • SMA +ve.
  • CD34 -ve.
  • CD117 -ve.[21]
  • H-caldesmon -ve.[21]

Lipofibromatosis

  • AKA infantile subcutaneous fibromatosis.

General

  • Childhood.

Microscopic

Features:[18]

  • Fibroblastic cells surrounding adipocytes.

Image:

IHC

Features:[18]

  • CD34 +ve.
  • BCL2 +ve.
  • S100 +ve.
  • CD99 +ve.
  • Actin +ve.
  • EMA +ve.

Desmoplastic fibroblastoma

General

  • Benign lesion.
  • Classically found in shoulder region.

Epidemiology:

  • May be on the lip.

Microscopic

Features:[23][24]

  • Acellular stroma with abundant collagen.

Notes:

  • No nuclear atypia.

IHC

Calcifying fibrous tumour

General

  • Rare.
  • Benign.

Microscopic

Features:[26]

  • Submucosal circumscribed fibrocollagenous nodule.
  • Psammomatous calcifications.
  • Focal plasma cells at the periphery.

Occasionally metastasizing

Inflammatory myofibroblastic tumour

  • AKA inflammatory pseudotumour, AKA inflammatory fibrosarcoma,[27] AKA plasma cell granuloma.[28][29]

General

  • Mostly benign.
  • Children & young adults.
  • Classically located in mesentery of ileocolic region or small bowel.[27]

Microscopic

Features:[27]

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

DDx:

Notes:

  • Some consider this a wastebasket diagnosis... for benign appearing spindle cell lesions.[31]

Images:

IHC

Features - dependent on site:

  • SMA +ve.[32]
  • Vimentin +ve.

Variable staining with:

Negative:[32]

  • S100, CD117, CD68.

Molecular

  • ALK rearrangements.[30]

Congenital-infantile fibrosarcoma

Should not be confused with adult fibrosarcoma.

General

  • Locally aggressive.

Microscopic

Features:[34]

  • Spindle cell lesion.

Molecular

Characteristic translocation:[35]

Solitary fibrous tumour

  • Abbreviated SFT.

General

  • Grouped with hemangiopericytoma in the WHO classification - as it is thought to be the same tumour.[18]
  • May be benign or malignant; more commonly benign.[36][37]
  • May be associated with hypoglycemia.
    • Known as Doege-Potter syndrome.[38]

Gross

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:[18]

  • Necrosis.
  • Mitoses >4/10 HPF -- definition suffers from HPFitis.
  • Increased cellularity.
  • Marked nuclear atypia.
  • Infiltrative margin.

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 (?).[18]
  • 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.[39]
  • Oncogenic osteomalacia - assoc. with hemangiopericytoma.[39]

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.[40]

Location

  • Usually extremities - femur or proximal tibial.[39]

Microscopic

Features:[40]

  • Hypervascular lesion - key diagnostic feature.[41]
    • 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:[18][41]

  • 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:[42]

  • 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.

Microscopic

Features:[43]

  • Myxoid stroma alternating with fibrogenic areas - key feature.
  • Low cellularity.
  • Spindle cells.
  • +/-Rosette of collagen with central hyaline core.[44]

Notes:

  • Few/absent mitoses.

DDx:

Images:

IHC

Features:[44]

  • EMA +ve.
  • CD99 +ve.
  • BCL2 +ve.

Others:[44]

  • SMA -ve.
  • S100 -ve.
  • Desmin -ve.

Molecular pathology

Adult fibrosarcoma

General

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

Microscopic

Feature:[44]

  • Spindle cell lesion.
  • Herring bone pattern - key feature.
  • Mitoses.

DDx (herring bone):

  • MPNST.
  • Synovial sarcoma.
  • Fibrosarcoma.

DDx:

Images:

IHC

Features:[44]

  • Vimentin.
  • SMA.

Myxofibrosarcoma

General

  • Malignant.
  • Usu. older people, superficial (skin/dermis) and extremities (arm, legs).[47][48]

Microscopic

Features:[47]

  • Discontinuous fibrous septae.
  • Myxoid background.
  • Variable cellularity and nuclear pleomorphism.
  • Spindle cells or epithelioid cells.[48]
  • Curvilinear vessels.[48]

DDx:

Image:

IHC

  • Vimentin +ve -- otherwise non-distinctive.[47]

See also

References

  1. ↑ 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. ↑ 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. ↑ 3.0 3.1 Chung, EB.; Enzinger, FM. (Oct 1975). "Proliferative fasciitis.". Cancer 36 (4): 1450-8. PMID 1058047.
  4. ↑ Meis, JM.; Enzinger, FM. (Apr 1992). "Proliferative fasciitis and myositis of childhood.". Am J Surg Pathol 16 (4): 364-72. PMID 1566969.
  5. ↑ 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. ↑ 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.
  7. ↑ 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.
  8. ↑ URL: http://emedicine.medscape.com/article/1057113-overview. Accessed on: 26 October 2011.
  9. ↑ 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.
  10. ↑ Rose, Alan G. (2008). Atlas of Gross Pathology with Histologic Correlation (1st ed.). Cambridge University Press. pp. 592. ISBN 978-0521868792.
  11. ↑ 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.
  12. ↑ 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.
  13. ↑ BD. 26 April 2011.
  14. ↑ URL: http://anvita.info/wiki/Nodular_Fasciitis. Accessed on: 11 November 2011.
  15. ↑ URL: http://www.mckeedermpath.com/SPOT%20DIAGNOSIS%20CASE%20268.html. Accessed on: 11 November 2011.
  16. ↑ 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.
  17. ↑ URL: http://www.dtrf.org/dtrf_aboutdesmoids.htm. Accessed on: 15 April 2011.
  18. ↑ 18.0 18.1 18.2 18.3 18.4 18.5 18.6 18.7 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.
  19. ↑ URL: http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?f=0&sort=0&s=20090717111548196. Accessed on: 4 October 2011.
  20. ↑ URL: http://www.cheapmedicinechest.com/abdominal-pain-and-colonic-obstruction-from-an-intra-abdominal-desmoid-tumor.html. Accessed on: 4 October 2011.
  21. ↑ 21.0 21.1 21.2 21.3 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.
  22. ↑ 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.
  23. ↑ URL: http://www.dermatologyoutlines.com/dermskintumornonmelanocytic.html#collagenousfibroma. Accessed on: 19 March
  24. ↑ 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.
  25. ↑ 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.
  26. ↑ 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/.
  27. ↑ 27.0 27.1 27.2 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.
  28. ↑ URL: http://www.uptodate.com/contents/inflammatory-myofibroblastic-tumor-plasma-cell-granuloma-of-the-lung. Accessed on: 27 November 2011.
  29. ↑ 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.
  30. ↑ 30.0 30.1 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.
  31. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970283-2. Accessed on: 10 May 2011.
  32. ↑ 32.0 32.1 32.2 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
  33. ↑ 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.
  34. ↑ 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.
  35. ↑ 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.
  36. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9. Accessed on: 25 June 2010.
  37. ↑ URL: http://wjso.com/content/6/1/86. Accessed on: 25 June 2010.
  38. ↑ 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.
  39. ↑ 39.0 39.1 39.2 URL: http://emedicine.medscape.com/article/1255879-overview. Accessed on: 2 May 2010.
  40. ↑ 40.0 40.1 URL: http://emedicine.medscape.com/article/1255879-diagnosis. Accessed on: 2 May 2010.
  41. ↑ 41.0 41.1 Enzinger & Weiss's Soft Tissue Tumors. 4th Ed. PP.1007-13. ISBN 0-323-01200-0.
  42. ↑ Croul, SE. 8 November 2010.
  43. ↑ Vernon SE, Bejarano PA (September 2006). "Low-grade fibromyxoid sarcoma: a brief review". Arch. Pathol. Lab. Med. 130 (9): 1358–60. PMID 16948525.
  44. ↑ 44.0 44.1 44.2 44.3 44.4 44.5 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.
  45. ↑ 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.
  46. ↑ 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. ↑ 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. ↑ 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.
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