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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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVSpindle cell liposarcoma

Disease
- Neoplasm
  - Neoplasm by Special Category
    - Benign neoplasm
      - Benign connective and soft tissue neoplasm
        Benign lipomatous neoplasm
        Spindle 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: 33879215 Free 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

See all (16)

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

See all (7)

Diagnosis

Primary spindle cell sarcoma of the seminal vesicle: A case report and literature review.

Song Y, Ning H, Yao Z, Wu H, Shao J, Fan M, Lu J
Andrologia 2022 May;54(4):e14363. Epub 2022 Jan 5 doi: 10.1111/and.14363. PMID: 34984692

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: 34410968 Free 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

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

See all (22)

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

See all (1)

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

See all (10)

Clinical prediction guides

The Value of H3K27me3 Immunohistochemistry in Differentiating Malignant Peripheral Nerve Sheath Tumour with Its Histologic Mimickers.

Mustapar N, Zawawi MSF, Tuan Sharif SE
Asian Pac J Cancer Prev 2020 Mar 1;21(3):699-705. doi: 10.31557/APJCP.2020.21.3.699. PMID: 32212796 Free PMC Article

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

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

See all (6)