Difference between revisions of "Adipocytic tumours"

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==Liposarcoma==
==Liposarcoma==
===General===
{{Main|Liposarcoma}}
*Most common malignant sarcoma in the retroperitoneum.
*Not all (large) retroperitoneal adipocytic tumours are liposarcomas.
 
Notes:
*Retroperitoneal sarcomas: #1: liposarcoma, #2: [[pleomorphic undifferentiated sarcoma]], #3: leiomyosarcoma, #4: [[MPNST]].
**Extremely rare in retroperitoneum: [[synovial sarcoma]].
 
===Microscopic===
Features:
*Lipoblasts - '''key feature'''.
**Large sharply demarcated vacuole.
**Nucleus:
***Hyperchromatic (dark staining) nucleus.
***Eccentric location.
***Nuclear indentation.
*Chicken wire-like vascular.
*+/-[[Myxoid]] background.
*Cell size variation.
 
DDx:
*[[Angiolipoma]].
*[[Pleomorphic undifferentiated sarcoma]].
 
Images:
*[http://commons.wikimedia.org/wiki/File:Myxoid_liposarcoma_%2806%29.JPG Myxoid liposarcoma (WC)].
*[http://commons.wikimedia.org/wiki/File:Myxoid_liposarcoma_%2805%29.JPG Myxoid liposarcoma (WC)].
*[http://www.john-libbey-eurotext.fr/e-docs/00/04/09/14/texte_alt_jleejd00046_gr5.jpg Lipoblasts (john-libbey-eurotext.fr)].
*[http://commons.wikimedia.org/wiki/File:Dedifferentiated_liposarcoma_-_cropped_-_very_high_mag.jpg Dediff. liposarcoma - lipoblasts - very high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Dedifferentiated_liposarcoma_-_intermed_mag.jpg Dediff. liposarcoma - shows dediff. component - intermed. mag. (WC)].
*[http://path.upmc.edu/cases/case302.html Poorly diff. liposarcoma - several images (upmc.edu)].
 
====Subtypes====
Main subtypes:<ref name=Ref_WMSP_601>{{Ref WMSP|601}}</ref>
*Dedifferentiated liposarcoma.
*Myxoid liposarcoma.
**Round cell liposarcoma - a subtype of myxoid liposarcoma<ref name=pmid8554106>{{Cite journal  | last1 = Smith | first1 = TA. | last2 = Easley | first2 = KA. | last3 = Goldblum | first3 = JR. | title = Myxoid/round cell liposarcoma of the extremities. A clinicopathologic study of 29 cases with particular attention to extent of round cell liposarcoma. | journal = Am J Surg Pathol | volume = 20 | issue = 2 | pages = 171-80 | month = Feb | year = 1996 | doi =  | PMID = 8554106 }}
</ref> that has a worse prognosis;<ref name=pmid21253554>{{Cite journal  | last1 = Conyers | first1 = R. | last2 = Young | first2 = S. | last3 = Thomas | first3 = DM. | title = Liposarcoma: molecular genetics and therapeutics. | journal = Sarcoma | volume = 2011 | issue =  | pages = 483154 | month =  | year = 2011 | doi = 10.1155/2011/483154 | PMID = 21253554 }}</ref> characterized by regions of high cellularity.
*Mixed-type liposarcoma.
*Pleomorphic liposarcoma.
*Liposarcoma not otherwise specified (NOS).
 
Additional reported type:
*Spindle cell liposarcoma.<ref name=pmid8067512>{{Cite journal  | last1 = Dei Tos | first1 = AP. | last2 = Mentzel | first2 = T. | last3 = Newman | first3 = PL. | last4 = Fletcher | first4 = CD. | title = Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases. | journal = Am J Surg Pathol | volume = 18 | issue = 9 | pages = 913-21 | month = Sep | year = 1994 | doi =  | PMID = 8067512 }}</ref>
=====Myxoid liposarcoma=====
*[[AKA]] ''myxoid/round cell liposarcoma''.
*[[AKA]] ''round cell liposarcoma''.
 
======Gross======
Location:<ref name=pmid22052112>{{Cite journal  | last1 = Moreau | first1 = LC. | last2 = Turcotte | first2 = R. | last3 = Ferguson | first3 = P. | last4 = Wunder | first4 = J. | last5 = Clarkson | first5 = P. | last6 = Masri | first6 = B. | last7 = Isler | first7 = M. | last8 = Dion | first8 = N. | last9 = Werier | first9 = J. | title = Myxoid\Round Cell Liposarcoma (MRCLS) Revisited: An Analysis of 418 Primarily Managed Cases. | journal = Ann Surg Oncol | volume = 19 | issue = 4 | pages = 1081-1088 | month = Apr | year = 2012 | doi = 10.1245/s10434-011-2127-z | PMID = 22052112 }}</ref>
*90% in lower limb.
*81% deep.
 
======Microscopic======
Features:
*Chickenwire-type blood vessels.
*Clear spaces.
*Adipocytes - may be rare.
 
DDx:
*[[Low-grade fibromyxoid sarcoma]].
 
Images:
*[http://pubs.rsna.org/doi/full/10.1148/radiographics.20.4.g00jl021007 Myxoid liposarcoma (rsna.org)].<ref name=pmid10903690>{{Cite journal  | last1 = Sung | first1 = MS. | last2 = Kang | first2 = HS. | last3 = Suh | first3 = JS. | last4 = Lee | first4 = JH. | last5 = Park | first5 = JM. | last6 = Kim | first6 = JY. | last7 = Lee | first7 = HG. | title = Myxoid liposarcoma: appearance at MR imaging with histologic correlation. | journal = Radiographics | volume = 20 | issue = 4 | pages = 1007-19 | month =  | year =  | doi = 10.1148/radiographics.20.4.g00jl021007 | PMID = 10903690 }}</ref>
*[http://pubs.rsna.org/doi/figure/10.1148/rg.255055106 Myxoid liposarcoma - among other things (rsna.org)].<ref name=pmid16160117>{{Cite journal  | last1 = Murphey | first1 = MD. | last2 = Arcara | first2 = LK. | last3 = Fanburg-Smith | first3 = J. | title = From the archives of the AFIP: imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation. | journal = Radiographics | volume = 25 | issue = 5 | pages = 1371-95 | month =  | year =  | doi = 10.1148/rg.255055106 | PMID = 16160117 }}</ref>
*[http://www.cap.org/apps/cap.portal?_nfpb=true&cntvwrPtlt_actionOverride=%2Fportlets%2FcontentViewer%2Fshow&_windowLabel=cntvwrPtlt&cntvwrPtlt{actionForm.contentReference}=cap_foundation%2FcaseOfMonth%2Ffeb13%2Ffeb_2013_cotm_diagnosis.html&_state=maximized&_pageLabel=cntvwr Myxoid liposarcoma (cap.org)].
 
======Molecular======
Typically has a [[translocations|translocation]]:
*t(12;16)(q13;p11) TLS-CHOP.<ref name=pmid7805034>{{Cite journal  | last1 = Knight | first1 = JC. | last2 = Renwick | first2 = PJ. | last3 = Dal Cin | first3 = P. | last4 = Van den Berghe | first4 = H. | last5 = Fletcher | first5 = CD. | title = Translocation t(12;16)(q13;p11) in myxoid liposarcoma and round cell liposarcoma: molecular and cytogenetic analysis. | journal = Cancer Res | volume = 55 | issue = 1 | pages = 24-7 | month = Jan | year = 1995 | doi =  | PMID = 7805034 }}</ref>
*May have the translocation usually seen in [[clear cell sarcoma]]:
*t(12;22)(q13;q12) EWS-ATF1.<ref name=pmid21115923>{{Cite journal  | last1 = Suzuki | first1 = K. | last2 = Matsui | first2 = Y. | last3 = Endo | first3 = K. | last4 = Kubo | first4 = T. | last5 = Hasegawa | first5 = T. | last6 = Kimura | first6 = T. | last7 = Ohtani | first7 = O. | last8 = Yasui | first8 = N. | title = Myxoid liposarcoma with EWS-CHOP type 1 fusion gene. | journal = Anticancer Res | volume = 30 | issue = 11 | pages = 4679-83 | month = Nov | year = 2010 | doi =  | PMID = 21115923 }}</ref>
 
=====Dedifferentiated liposarcoma=====
*Has an undifferentiated component that, if seen alone, would be diagnosed as [[pleomorphic undifferentiated sarcoma]].
*The diagnosis depends on the presence of the differentiated component of the tumour, i.e. the presence of lipoblasts.
 
===IHC===
*IHC is of limited value.
 
*S-100 +ve ~1/3 of the time.
*Reticulin ???.


=See also=
=See also=

Revision as of 00:29, 6 January 2014

Adipocytic tumours fall into the grouping soft tissue lesions and includes things that are very common (e.g. lipoma) and everything from benign to malignant.

Overview

This grouping includes a number of tumours, which can be divided based on their behaviour into benign, intermediate and malignant.

Benign

Benign adipocytic tumours:[1]

Intermediate

Intermediate adipocytic tumours:[1]

  • Atypical lipomatous tumour.

Malignant

Malignant adipocytic tumours:[1]

  • Dedifferentiated liposarcoma.
  • Myxoid liposarcoma.
  • Pleomorphic liposarcoma.
  • Mixed-type liposarcoma.
  • Liposarcoma NOS.

Detail section

Normal mature fat

Microscopic

Features:

  • Adipocytes of approximately equal size.
  • Not vascular.
  • No nuclear hyperchromasia.

Notes:

  • May have nuclear pseudoinclusions (Lockhern cell).[2]
    • There is some suggestion this is not benign.[3]

IHC

  • S100 +ve.

Lipoblastoma

General

  • Rare paediatric tumour.[4]

Usual presentation:[4]

  • Painless neck mass.

Microscopic

Features:

  • Nests of cells in the dermis with abundant pale cytoplasm - vaguely resemble adipocytes.
    • Smaller than mature adipocytes.

DDx:

Images:

Lipoma

Pleomorphic lipoma

General

  • Rare.
  • May mimic a malignancy.[6]
  • Male > female.[7]

Gross

  • Classically shoulder and neck region in adults.[8]

DDx - shoulder lesions:

Microscopic

Features:

  • Multinucleated cells - "floret cells" - key feature.
    • Solid eosinophilic cytoplasm.
    • Peripheral nuclei - impart a knobby border to the cells.
  • Fibrous septa.

Notes:

Images:

DDx:

IHC

  • AR +ve - ~95% in men, ~85% in women.[7]

Spindle cell lipoma

General

  • Rare.
  • Predominantly men.[10]

Note:

  • Spindle cell lipoma may immunohistochemically and histomorphologically overlap with mammary-type myofibroblastoma[11] - see: mammary myofibroblastoma.

Microscopic

Features:[10]

  • Aligned bland spindled cells adjacent to fat.
  • Rope-like collagen bundles - key feature.
    • May be described as "shreaded wheat".
  • +/-Myxoid component.
  • +/-Staghorn-like vessels.

Notes:

DDx:

Image

www:

IHC

  • CD34 +ve.[13]
  • Desmin +ve.
  • S100 -ve. (???)

Hibernoma

Atypical lipomatous tumour

  • AKA well-differentiated liposarcoma, abbreviated WDLPS.
  • Abbreviated ALT/WDLPS.

General

  • Atypical lipomatous tumour is a term used to save people with a (curable) peripheral liposarcoma from getting denied life insurance.

Microscopic

Features:[14]

  • Large adipocytes.
  • Atypical lipoblasts - focal, scattered:
    • Nuclear hyperchromasia.
    • +/-Multinucleated.

Liposarcoma

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. 601. ISBN 978-0781765275.
  2. URL: http://journals.lww.com/amjdermatopathology/Citation/2004/12000/Original_Observation_to_Rediscovery__Nuclear.9.aspx. Accessed on: 18 April 2011.
  3. URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970574-5. Accessed on: 18 April 2011.
  4. 4.0 4.1 Pham, NS.; Poirier, B.; Fuller, SC.; Dublin, AB.; Tollefson, TT. (Jul 2010). "Pediatric lipoblastoma in the head and neck: a systematic review of 48 reported cases.". Int J Pediatr Otorhinolaryngol 74 (7): 723-8. doi:10.1016/j.ijporl.2010.04.010. PMID 20472310.
  5. Nagano, A.; Ohno, T.; Nishimoto, Y.; Hirose, Y.; Miyake, S.; Shimizu, K. (2011). "Lipoblastoma mimicking myxoid liposarcoma: a clinical report and literature review.". Tohoku J Exp Med 223 (1): 75-8. PMID 21212605.
  6. Persichetti, P.; Di Lella, F.; Marangi, GF.; Cagli, B.; Simone, P.; Tenna, S.; Rabitri, C.; Cassandro, R. et al. "Pleomorphic lipoma: a definite histopathological entity.". Anticancer Res 24 (5B): 3157-9. PMID 15510605.
  7. 7.0 7.1 Syed, S.; Martin, AM.; Haupt, H.; Podolski, V.; Brooks, JJ. (Jan 2008). "Frequent detection of androgen receptors in spindle cell lipomas: an explanation for this lesion's male predominance?". Arch Pathol Lab Med 132 (1): 81-3. doi:10.1043/1543-2165(2008)132[81:FDOARI]2.0.CO;2. PMID 18181679.
  8. URL: http://www.webpathology.com/image.asp?n=2&Case=435. Accessed on: 3 October 2011.
  9. 9.0 9.1 URL: http://surgpathcriteria.stanford.edu/softfat/spindle_cell_lipoma/. Accessed on: 4 December 2010.
  10. 10.0 10.1 Murphey, MD.; Carroll, JF.; Flemming, DJ.; Pope, TL.; Gannon, FH.; Kransdorf, MJ.. "From the archives of the AFIP: benign musculoskeletal lipomatous lesions.". Radiographics 24 (5): 1433-66. doi:10.1148/rg.245045120. PMID 15371618.
  11. McMenamin, ME.; Fletcher, CD. (Aug 2001). "Mammary-type myofibroblastoma of soft tissue: a tumor closely related to spindle cell lipoma.". Am J Surg Pathol 25 (8): 1022-9. PMID 11474286.
  12. Dei Tos, AP.; Mentzel, T.; Newman, PL.; Fletcher, CD. (Sep 1994). "Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.". Am J Surg Pathol 18 (9): 913-21. PMID 8067512.
  13. Wood, L.; Fountaine, TJ.; Rosamilia, L.; Helm, KF.; Clarke, LE. (Dec 2010). "Cutaneous CD34+ spindle cell neoplasms: Histopathologic features distinguish spindle cell lipoma, solitary fibrous tumor, and dermatofibrosarcoma protuberans.". Am J Dermatopathol 32 (8): 764-8. doi:10.1097/DAD.0b013e3181d0c587. PMID 20559119.
  14. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 605. ISBN 978-0781765275.