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Spindle cell liposarcoma

MedGen UID:
266156
•Concept ID:
C1275275
•
Neoplastic Process
Synonyms: Atypical Spindle Cell Lipoma; Atypical Spindle Cell/Pleomorphic Lipomatous Tumor; spindle cell liposarcoma
SNOMED CT: Spindle cell liposarcoma (404073009)
 
Monarch Initiative: MONDO:0003596

Definition

A benign adipocytic neoplasm, characterized by ill-defined tumor margins and the presence of variable proportions of mild to moderately atypical spindle cells, adipocytes, lipoblasts, pleomorphic cells, multinucleated giant cells, and a myxoid or collagenous extracellular matrix. It has a low tendency for local recurrence if incompletely excised. Unlike conventional atypical lipomatous tumors, there is no risk for dedifferentiation. (WHO 2020) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSpindle cell liposarcoma

Professional guidelines

PubMed

Undifferentiated and dedifferentiated soft tissue neoplasms: Immunohistochemical surrogates for differential diagnosis.
Thway K, Fisher C
Semin Diagn Pathol 2021 Nov;38(6):170-186. Epub 2021 Sep 29 doi: 10.1053/j.semdp.2021.09.005. PMID: 34602314
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
From head and neck lipoma to liposarcoma: a wide spectrum of differential diagnoses and their therapeutic implications.
Barisella M, Giannini L, Piazza C
Curr Opin Otolaryngol Head Neck Surg 2020 Apr;28(2):136-143. doi: 10.1097/MOO.0000000000000608. PMID: 32011399

Recent clinical studies

Etiology

Novel CTNND2-TERT fusion in a spindle cell liposarcoma.
Occidental M, Shen G, Feng X, Zhu K, Kelly K, Nie Q, Reddi HV, Lakiotaki E, Viniou NA, Korkolopoulou P, Linos K, Jour G
Genes Chromosomes Cancer 2020 Sep;59(9):544-548. Epub 2020 May 6 doi: 10.1002/gcc.22856. PMID: 32352179
Fibrosarcoma-like lipomatous neoplasm: a reappraisal of so-called spindle cell liposarcoma defining a unique lipomatous tumor unrelated to other liposarcomas.
Deyrup AT, Chibon F, Guillou L, Lagarde P, Coindre JM, Weiss SW
Am J Surg Pathol 2013 Sep;37(9):1373-8. doi: 10.1097/PAS.0b013e31829562ed. PMID: 23887155
Liposarcoma: new entities and evolving concepts.
Dei Tos AP
Ann Diagn Pathol 2000 Aug;4(4):252-66. doi: 10.1053/adpa.2000.8133. PMID: 10982304
Lipomatous tumours of soft tissues: an update.
Mentzel T, Fletcher CD
Virchows Arch 1995;427(4):353-63. doi: 10.1007/BF00199383. PMID: 8548119
Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.
Dei Tos AP, Mentzel T, Newman PL, Fletcher CD
Am J Surg Pathol 1994 Sep;18(9):913-21. doi: 10.1097/00000478-199409000-00006. PMID: 8067512

Diagnosis

Well-differentiated Spindle Cell Liposarcoma of the Larynx: A Rare Case Report and Review of Literature.
Liao X, Fulmer S, Zhang D
In Vivo 2021 Sep-Oct;35(5):2779-2783. doi: 10.21873/invivo.12563. PMID: 34410968Free PMC Article
Spindle cell liposarcoma with a TRIO-TERT fusion transcript.
Suster DI, Deshpande V, Chebib I, Taylor MS, Mullen J, Bredella MA, Nielsen GP
Virchows Arch 2019 Sep;475(3):391-394. Epub 2019 Feb 22 doi: 10.1007/s00428-019-02545-5. PMID: 30793229
Recently characterized soft tissue tumors that bring biologic insight.
Fletcher CD
Mod Pathol 2014 Jan;27 Suppl 1:S98-112. doi: 10.1038/modpathol.2013.172. PMID: 24384856
Well-differentiated liposarcoma (atypical lipomatous tumors).
Laurino L, Furlanetto A, Orvieto E, Dei Tos AP
Semin Diagn Pathol 2001 Nov;18(4):258-62. PMID: 11757865
Liposarcoma: new entities and evolving concepts.
Dei Tos AP
Ann Diagn Pathol 2000 Aug;4(4):252-66. doi: 10.1053/adpa.2000.8133. PMID: 10982304

Therapy

Liposarcoma metastatic to the orbit.
Tehrani AH, Heegaard S, Prause JU, Fledelius HC, Daugaard S
Eur J Ophthalmol 2003 Jan-Feb;13(1):108-12. doi: 10.1177/112067210301300121. PMID: 12635688

Prognosis

Recently characterized soft tissue tumors that bring biologic insight.
Fletcher CD
Mod Pathol 2014 Jan;27 Suppl 1:S98-112. doi: 10.1038/modpathol.2013.172. PMID: 24384856
Primary retroperitoneal spindle cell liposarcoma: pathological and immunohistochemical findings.
Shioi Y, Hasegawa T, Otsuka K, Fujisawa K, Itabashi T, Kimura T, Wakabayashi G, Mue Y, Uesugi N, Sugai T
Pathol Int 2010 Jun;60(6):472-6. doi: 10.1111/j.1440-1827.2010.02541.x. PMID: 20518903
Spindle cell liposarcoma of the face: case report and literature review.
Agostini T, Catelani C, Acocella A, Franchi A, Bertolai R, Sacco R, Lazzeri D, Shokrollahi K
Br J Dermatol 2010 Sep;163(3):638-40. Epub 2010 May 8 doi: 10.1111/j.1365-2133.2010.09847.x. PMID: 20456346
Liposarcoma: new entities and evolving concepts.
Dei Tos AP
Ann Diagn Pathol 2000 Aug;4(4):252-66. doi: 10.1053/adpa.2000.8133. PMID: 10982304
Lipoleiomyosarcoma of the mediastinum.
Gómez-Román JJ, Val-Bernal JF
Pathology 1997 Nov;29(4):428-30. doi: 10.1080/00313029700169475. PMID: 9423230

Clinical prediction guides

Recently characterized soft tissue tumors that bring biologic insight.
Fletcher CD
Mod Pathol 2014 Jan;27 Suppl 1:S98-112. doi: 10.1038/modpathol.2013.172. PMID: 24384856
Monosomy 7 and absence of 12q amplification in two cases of spindle cell liposarcomas.
Italiano A, Chambonniere ML, Attias R, Chibon F, Coindre JM, Pedeutour F
Cancer Genet Cytogenet 2008 Jul 15;184(2):99-104. doi: 10.1016/j.cancergencyto.2008.04.004. PMID: 18617058
Liposarcoma: new entities and evolving concepts.
Dei Tos AP
Ann Diagn Pathol 2000 Aug;4(4):252-66. doi: 10.1053/adpa.2000.8133. PMID: 10982304
Multiple spindle cell lipomas: a report of 7 familial and 11 nonfamilial cases.
Fanburg-Smith JC, Devaney KO, Miettinen M, Weiss SW
Am J Surg Pathol 1998 Jan;22(1):40-8. doi: 10.1097/00000478-199801000-00005. PMID: 9422314
Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases.
Dei Tos AP, Mentzel T, Newman PL, Fletcher CD
Am J Surg Pathol 1994 Sep;18(9):913-21. doi: 10.1097/00000478-199409000-00006. PMID: 8067512

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