Difference between revisions of "Adipocytic tumours"

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*Lipomatosis.
*Lipomatosis.
*Lipomatosis of nerve.
*Lipomatosis of nerve.
*Lipoblastoma.
*[[Lipoblastoma]].
*Extrarenal [[angiomyolipoma]].
*Extrarenal [[angiomyolipoma]].
*Extra-adrenal [[myelolipoma]].
*Extra-adrenal [[myelolipoma]].
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===IHC===
===IHC===
*S100 +ve.
*[[S-100]] +ve.


==Lipoma==
==Lipoblastoma==
===General===
===General===
*Benign.
*Rare paediatric tumour.<ref name=pmid20472310>{{Cite journal  | last1 = Pham | first1 = NS. | last2 = Poirier | first2 = B. | last3 = Fuller | first3 = SC. | last4 = Dublin | first4 = AB. | last5 = Tollefson | first5 = TT. | title = Pediatric lipoblastoma in the head and neck: a systematic review of 48 reported cases. | journal = Int J Pediatr Otorhinolaryngol | volume = 74 | issue = 7 | pages = 723-8 | month = Jul | year = 2010 | doi = 10.1016/j.ijporl.2010.04.010 | PMID = 20472310 }}</ref>
*Several variants exist.
**Angiolipoma - one of the (classically) [[painful skin lesions]].


Usual presentation:<ref name=pmid20472310/>
*Painless neck mass.
===Microscopic===
===Microscopic===
Features:
Features:
*Collection of mature adipocytes.
*Nests of cells in the dermis with abundant pale cytoplasm - vaguely resemble adipocytes.
**Variation of size may be seen -- should prompt a search for lipoblasts.<ref name=pmid8548119>{{Cite journal  | last1 = Mentzel | first1 = T. | last2 = Fletcher | first2 = CD. | title = Lipomatous tumours of soft tissues: an update. | journal = Virchows Arch | volume = 427 | issue = 4 | pages = 353-63 | month =  | year = 1995 | doi =  | PMID = 8548119 }}</ref>
**Smaller than mature adipocytes.


DDx:
DDx:
*[[Liposarcoma]].
*[[Myxoid liposarcoma]].<ref name=pmid21212605>{{Cite journal  | last1 = Nagano | first1 = A. | last2 = Ohno | first2 = T. | last3 = Nishimoto | first3 = Y. | last4 = Hirose | first4 = Y. | last5 = Miyake | first5 = S. | last6 = Shimizu | first6 = K. | title = Lipoblastoma mimicking myxoid liposarcoma: a clinical report and literature review. | journal = Tohoku J Exp Med | volume = 223 | issue = 1 | pages = 75-8 | month =  | year = 2011 | doi =  | PMID = 21212605 }}</ref>
*Benign adipose tissue.
 
Notes:
*Microscopically ''not'' definitely distinguishable from mature clump of fat.
**The lesion must be labeled ''lipoma'' (by the clinican) to be signed-out as such.


Images:
Images:
*[http://circ.ahajournals.org/content/113/21/e778/F5.expansion.html Lipoma - cardiac (ahajournals.org)].<ref name=pmid16735681>{{Cite journal  | last1 = Friedberg | first1 = MK. | last2 = Chang | first2 = IL. | last3 = Silverman | first3 = NH. | last4 = Ramamoorthy | first4 = C. | last5 = Chan | first5 = FP. | title = Images in cardiovascular medicine. Near sudden death from cardiac lipoma in an adolescent. | journal = Circulation | volume = 113 | issue = 21 | pages = e778-9 | month = May | year = 2006 | doi = 10.1161/CIRCULATIONAHA.105.589630 | PMID = 16735681 | url = http://circ.ahajournals.org/content/113/21/e778.full }}</ref>
*[http://www.sarcomaimages.com/index.php?v=Lipoblastoma Lipoblastoma (sarcomaimages.com)].


====Variants====
==Lipoblastoma like tumor==
=====Angiolipoma=====
Microscopic:
*Numerous blood vessels present.
 
DDx:
*[[Liposarcoma]].
=====Myolipoma=====
Microscopic:
*Muscle present.


==Pleomorphic lipoma==
[[File: 5 22974716644038 sl 1.png|Lipoblastoma like tumor]]
===General===
[[File: 5 22974716644038 sl 2.png|Lipoblastoma like tumor]]
*Rare.
[[File: 5 22974716644038 sl 3.png|Lipoblastoma like tumor]]
*May mimic a malignancy.<ref name=pmid15510605>{{Cite journal  | last1 = Persichetti | first1 = P. | last2 = Di Lella | first2 = F. | last3 = Marangi | first3 = GF. | last4 = Cagli | first4 = B. | last5 = Simone | first5 = P. | last6 = Tenna | first6 = S. | last7 = Rabitri | first7 = C. | last8 = Cassandro | first8 = R. | last9 = Esposito | first9 = V. | title = Pleomorphic lipoma: a definite histopathological entity. | journal = Anticancer Res | volume = 24 | issue = 5B | pages = 3157-9 | month =  | year =  | doi =  | PMID = 15510605 }}</ref>
[[File: 5 22974716644038 sl 4.png|Lipoblastoma like tumor]]
*Classically shoulder and neck region in adults.<ref>URL: [http://www.webpathology.com/image.asp?n=2&Case=435 http://www.webpathology.com/image.asp?n=2&Case=435]. Accessed on: 3 October 2011.</ref>
*Male > female.<ref name=pmid18181679>{{Cite journal  | last1 = Syed | first1 = S. | last2 = Martin | first2 = AM. | last3 = Haupt | first3 = H. | last4 = Podolski | first4 = V. | last5 = Brooks | first5 = JJ. | title = Frequent detection of androgen receptors in spindle cell lipomas: an explanation for this lesion's male predominance? | journal = Arch Pathol Lab Med | volume = 132 | issue = 1 | pages = 81-3 | month = Jan | year = 2008 | doi = 10.1043/1543-2165(2008)132[81:FDOARI]2.0.CO;2 | PMID = 18181679 }}</ref>


===Microscopic===
Features:
*Multinucleated cells - "floret cells" - '''key feature'''.
**Solid eosinophilic cytoplasm.
**Peripheral nuclei - impart a knobby border to the cells.
*Fibrous septa.


Notes:
Lipoblastoma like tumor in 26 yo woman, tumor of pelvis. A. Lobulated appearance at low power. B. Cytologically bland lipoblasts with a myxoid stroma. C. Numerous thin-walled branching blood vessels. D. Loose collagenous stroma. The morphologic features, combined with molecular findings of negative DDI T3 gene rearrangement and lack of MDM2 amplification permit the diagnosis.
*May overlap with ''spindle cell lipoma''.<ref name=spc_stanford>URL: [http://surgpathcriteria.stanford.edu/softfat/spindle_cell_lipoma/ http://surgpathcriteria.stanford.edu/softfat/spindle_cell_lipoma/]. Accessed on: 4 December 2010.</ref>
*Floret cell - may look similar to [[Touton giant cell]]s.


Images:
==Lipoma==
*[http://www.webpathology.com/image.asp?case=435&n=1 Pleomorphic lipoma - low mag. (webpathology.com)].
{{Main|Lipoma}}
*[http://www.webpathology.com/image.asp?n=2&Case=435 Pleomorphic lipoma - high mag. (webpathology.com)].


DDx:
==Pleomorphic lipoma==
*[[Liposarcoma]].
{{Main|Pleomorphic lipoma}}
*Ancient [[neurofibroma]].
*Ancient [[schwannoma]].
 
===IHC===
*AR +ve - ~95% in men, ~85% in women.<ref name=pmid18181679>{{Cite journal  | last1 = Syed | first1 = S. | last2 = Martin | first2 = AM. | last3 = Haupt | first3 = H. | last4 = Podolski | first4 = V. | last5 = Brooks | first5 = JJ. | title = Frequent detection of androgen receptors in spindle cell lipomas: an explanation for this lesion's male predominance? | journal = Arch Pathol Lab Med | volume = 132 | issue = 1 | pages = 81-3 | month = Jan | year = 2008 | doi = 10.1043/1543-2165(2008)132[81:FDOARI]2.0.CO;2 | PMID = 18181679 }}</ref>


==Spindle cell lipoma==
==Spindle cell lipoma==
===General===
{{Main|Spindle cell lipoma}}
*Rare.
*Predominantly men.<ref name=pmid15371618/>
 
Note:
*Spindle cell lipoma may immunohistochemically and histomorphologically overlap with ''mammary-type myofibroblastoma''<ref name=pmid11474286>{{Cite journal  | last1 = McMenamin | first1 = ME. | last2 = Fletcher | first2 = CD. | title = Mammary-type myofibroblastoma of soft tissue: a tumor closely related to spindle cell lipoma. | journal = Am J Surg Pathol | volume = 25 | issue = 8 | pages = 1022-9 | month = Aug | year = 2001 | doi =  | PMID = 11474286 }}</ref> - see: ''[[mammary myofibroblastoma]]''.
 
===Microscopic===
Features:<ref name=pmid15371618>{{Cite journal  | last1 = Murphey | first1 = MD. | last2 = Carroll | first2 = JF. | last3 = Flemming | first3 = DJ. | last4 = Pope | first4 = TL. | last5 = Gannon | first5 = FH. | last6 = Kransdorf | first6 = MJ. | title = From the archives of the AFIP: benign musculoskeletal lipomatous lesions. | journal = Radiographics | volume = 24 | issue = 5 | pages = 1433-66 | month =  | year =  | doi = 10.1148/rg.245045120 | PMID = 15371618 }}</ref>
*Aligned bland spindled cells adjacent to fat.
*Rope-like collagen bundles - '''key feature'''.
**May be described as "shreaded wheat".
*+/-[[Myxoid]] component.
*+/-[[staghorn vessels|Staghorn-like vessels]].
 
Notes:
*May overlap with ''[[pleomorphic lipoma]]''.<ref name=spc_stanford>URL: [http://surgpathcriteria.stanford.edu/softfat/spindle_cell_lipoma/ http://surgpathcriteria.stanford.edu/softfat/spindle_cell_lipoma/]. Accessed on: 4 December 2010.</ref>
 
DDx:
*[[Neurofibroma]].
 
Image:
*[http://www.sarcoma.org/pathology_review/round%20cell%20and%20soft%20tissue%20tumors/source/spindle_cell_lipoma3.html Spindle cell lipoma (sarcoma.org)].
 
===IHC===
*CD34 +ve.<ref name=pmid20559119>{{Cite journal  | last1 = Wood | first1 = L. | last2 = Fountaine | first2 = TJ. | last3 = Rosamilia | first3 = L. | last4 = Helm | first4 = KF. | last5 = Clarke | first5 = LE. | title = Cutaneous CD34+ spindle cell neoplasms: Histopathologic features distinguish spindle cell lipoma, solitary fibrous tumor, and dermatofibrosarcoma protuberans. | journal = Am J Dermatopathol | volume = 32 | issue = 8 | pages = 764-8 | month = Dec | year = 2010 | doi = 10.1097/DAD.0b013e3181d0c587 | PMID = 20559119 }}</ref>
*Desmin +ve.


==Hibernoma==
==Hibernoma==
===General===
{{Main|Hibernoma}}
*Consists of ''brown fat'' (present in the infants to generate heat).<ref name=Ref_WMSP605>{{Ref WMSP|605}}</ref>
*Benign.
*Usually asymptomatic.<ref name=pmid19131775>{{cite journal |author=Ahmed SA, Schuller I |title=Pediatric hibernoma: a case review |journal=J. Pediatr. Hematol. Oncol. |volume=30 |issue=12 |pages=900–1 |year=2008 |month=December |pmid=19131775 |doi=10.1097/MPH.0b013e318184e6dd |url=}}</ref>
 
Epidemiology:
*Young adults.
 
===Gross===
*Well-circumscribed.
*Lobulated and light-brown on sectioning.
 
===Microscopic===
Features:<ref name=pmid9537018>{{cite journal |author=Chen DY, Wang CM, Chan HL |title=Hibernoma. Case report and literature review |journal=Dermatol Surg |volume=24 |issue=3 |pages=393–5 |year=1998 |month=March |pmid=9537018 |doi= |url=}}</ref>
*Large polygonal/oval cells:
**Nucleus - central & small.<ref>[http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675(06)70271-6 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675(06)70271-6]</ref>
***Nucleoli typically prominent.<ref>[http://surgpathcriteria.stanford.edu/softfat/hibernoma/ http://surgpathcriteria.stanford.edu/softfat/hibernoma/]</ref>
**Cytoplasm - multivacuolated, oval, eosinophilic, granular.
 
DDx:
*Reaction to silicone implant.
 
Image:
*[http://en.wikipedia.org/wiki/File:Hibernoma1.jpg Hibernoma - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Hibernoma3.jpg Hibernoma - low mag. (WC)].


==Atypical lipomatous tumour==
==Atypical lipomatous tumour==
*[[AKA]] ''well-differentiated liposarcoma'', abbreviated ''WDLPS''.
*[[AKA]] ''well-differentiated liposarcoma'', abbreviated ''WDLPS''.<ref name=pmid24659226>{{Cite journal  | last1 = Creytens | first1 = D. | last2 = van Gorp | first2 = J. | last3 = Savola | first3 = S. | last4 = Ferdinande | first4 = L. | last5 = Mentzel | first5 = T. | last6 = Libbrecht | first6 = L. | title = Atypical spindle cell lipoma: a clinicopathologic, immunohistochemical, and molecular study emphasizing its relationship to classical spindle cell lipoma. | journal = Virchows Arch | volume = 465 | issue = 1 | pages = 97-108 | month = Jul | year = 2014 | doi = 10.1007/s00428-014-1568-8 | PMID = 24659226 }}</ref>
*Abbreviated ''ALT/WDLPS''.
*Abbreviated ''ALT/WDLPS''.


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==Liposarcoma==
==Liposarcoma==
{{Main|Liposarcoma}}
==Angiolipoma==
===General===
===General===
*Most common malignant sarcoma in the retroperitoneum.
*May be syndromic - typically [[autosomal recessive]].<ref name=pmid25671454>{{cite journal |authors=Garib G, Siegal GP, Andea AA |title=Autosomal-dominant familial angiolipomatosis |journal=Cutis |volume=95 |issue=1 |pages=E26–9 |date=January 2015 |pmid=25671454 |doi= |url=}}</ref>
*Not all (large) retroperitoneal adipocytic tumours are liposarcomas.
*Can be seen in the context of [[Birt–Hogg–Dubé syndrome]].<ref name=pmid8734663>{{cite journal |authors=Chung JY, Ramos-Caro FA, Beers B, Ford MJ, Flowers F |title=Multiple lipomas, angiolipomas, and parathyroid adenomas in a patient with Birt-Hogg-Dube syndrome |journal=Int J Dermatol |volume=35 |issue=5 |pages=365–7 |date=May 1996 |pmid=8734663 |doi=10.1111/j.1365-4362.1996.tb03642.x |url=}}</ref>
 
*[[Painful skin lesion]].
Notes:
*Retroperitoneal sarcomas: #1: liposarcoma, #2: [[pleomorphic undifferentiated sarcoma]], #3: leiomyosarcoma, #4: [[MPNST]].
**Extremely rare in retroperitoneum: [[synovial sarcoma]].


===Microscopic===
===Microscopic===
Features:
Features:
*Lipoblasts - '''key feature'''.
*Adipose tissue.
**Large sharply demarcated vacuole.
*Small blood vessels (clustered).
**Nucleus:
***Hyperchromatic (dark staining) nucleus.
***Eccentric location.
***Nuclear indentation.
*Chicken wire-like vascular.
*+/-[[Myxoid]] background.
*Cell size variation.


DDx:
DDx:
*[[Angiolipoma]].
*[[Liposarcoma]].
*[[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====
There are several 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).
 
=====Myxoid liposarcoma=====
*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>
 
Microscopic features:
*Chickenwire-type blood vessels.
 
=====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=
*[[Soft tissue lesions]].
*[[Soft tissue lesions]].
*[[Bone]].
*[[Bone]].
*[[Brown fat]].


=References=
=References=
{{Reflist|2}}
{{Reflist|2}}


[[Category:Adipocytic tumours]]
[[Category:Soft tissue lesions]]
[[Category:Soft tissue lesions]]
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