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| ==Liposarcoma== | | ==Liposarcoma== |
| ===General===
| | {{Main|Liposarcoma}} |
| *Most common malignant sarcoma in the retroperitoneum.
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| *Not all (large) retroperitoneal adipocytic tumours are liposarcomas.
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| Notes:
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| *Retroperitoneal sarcomas: #1: liposarcoma, #2: [[pleomorphic undifferentiated sarcoma]], #3: leiomyosarcoma, #4: [[MPNST]].
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| **Extremely rare in retroperitoneum: [[synovial sarcoma]].
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| ===Microscopic===
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| Features:
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| *Lipoblasts - '''key feature'''.
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| **Large sharply demarcated vacuole.
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| **Nucleus:
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| ***Hyperchromatic (dark staining) nucleus.
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| ***Eccentric location.
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| ***Nuclear indentation.
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| *Chicken wire-like vascular.
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| *+/-[[Myxoid]] background.
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| *Cell size variation.
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| DDx:
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| *[[Angiolipoma]].
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| *[[Pleomorphic undifferentiated sarcoma]].
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| Images:
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| *[http://commons.wikimedia.org/wiki/File:Myxoid_liposarcoma_%2806%29.JPG Myxoid liposarcoma (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Myxoid_liposarcoma_%2805%29.JPG Myxoid liposarcoma (WC)].
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| *[http://www.john-libbey-eurotext.fr/e-docs/00/04/09/14/texte_alt_jleejd00046_gr5.jpg Lipoblasts (john-libbey-eurotext.fr)].
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| *[http://commons.wikimedia.org/wiki/File:Dedifferentiated_liposarcoma_-_cropped_-_very_high_mag.jpg Dediff. liposarcoma - lipoblasts - very high mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Dedifferentiated_liposarcoma_-_intermed_mag.jpg Dediff. liposarcoma - shows dediff. component - intermed. mag. (WC)].
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| *[http://path.upmc.edu/cases/case302.html Poorly diff. liposarcoma - several images (upmc.edu)].
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| ====Subtypes====
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| Main subtypes:<ref name=Ref_WMSP_601>{{Ref WMSP|601}}</ref>
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| *Dedifferentiated liposarcoma.
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| *Myxoid liposarcoma.
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| **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 }}
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| </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.
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| *Mixed-type liposarcoma.
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| *Pleomorphic liposarcoma.
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| *Liposarcoma not otherwise specified (NOS).
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| Additional reported type:
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| *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>
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| =====Myxoid liposarcoma=====
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| *[[AKA]] ''myxoid/round cell liposarcoma''.
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| *[[AKA]] ''round cell liposarcoma''.
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| ======Gross======
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| 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>
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| *90% in lower limb.
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| *81% deep.
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| ======Microscopic======
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| Features:
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| *Chickenwire-type blood vessels.
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| *Clear spaces.
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| *Adipocytes - may be rare.
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| DDx:
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| *[[Low-grade fibromyxoid sarcoma]].
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| Images:
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| *[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>
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| *[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>
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| *[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)].
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| ======Molecular======
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| Typically has a [[translocations|translocation]]:
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| *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>
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| *May have the translocation usually seen in [[clear cell sarcoma]]:
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| *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>
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| =====Dedifferentiated liposarcoma=====
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| *Has an undifferentiated component that, if seen alone, would be diagnosed as [[pleomorphic undifferentiated sarcoma]].
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| *The diagnosis depends on the presence of the differentiated component of the tumour, i.e. the presence of lipoblasts.
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| ===IHC===
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| *IHC is of limited value.
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| *S-100 +ve ~1/3 of the time.
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| *Reticulin ???.
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| =See also= | | =See also= |
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
Lipoblastoma
General
- Rare paediatric tumour.[4]
Usual presentation:[4]
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
Main article:
Liposarcoma
See also
References
- ↑ 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.
- ↑ URL: http://journals.lww.com/amjdermatopathology/Citation/2004/12000/Original_Observation_to_Rediscovery__Nuclear.9.aspx. Accessed on: 18 April 2011.
- ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970574-5. Accessed on: 18 April 2011.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 7.0 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.
- ↑ URL: http://www.webpathology.com/image.asp?n=2&Case=435. Accessed on: 3 October 2011.
- ↑ 9.0 9.1 URL: http://surgpathcriteria.stanford.edu/softfat/spindle_cell_lipoma/. Accessed on: 4 December 2010.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.