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Cutaneous undifferentiated pleomorphic sarcoma

MedGen UID:
224813
•Concept ID:
C1275254
•
Neoplastic Process
Synonyms: cutaneous malignant fibrous histiocytoma; Cutaneous Unclassified Pleomorphic Sarcoma; cutaneous unclassified pleomorphic sarcoma; Cutaneous Unclassified Pleomorphic Sarcoma (Formerly Cutaneous "Malignant Fibrous Histiocytoma"); cutaneous unclassified pleomorphic sarcoma (formerly cutaneous "malignant fibrous histiocytoma"); Cutaneous Unclassified Pleomorphic Sarcoma (Formerly Cutaneous "MFH"); cutaneous unclassified pleomorphic sarcoma (formerly cutaneous "MFH"); cutaneous unclassified pleomorphic sarcoma (formerly cutaneous); Cutaneous Undifferentiated Pleomorphic Sarcoma; cutaneous undifferentiated pleomorphic sarcoma; malignant cutaneous fibrous histiocytoma; Malignant fibrous histiocytoma of skin; malignant fibrous histiocytoma of skin; malignant fibrous histiocytoma of the skin; malignant skin fibrous histiocytoma; Pleomorphic Dermal Sarcoma; Skin Undifferentiated Pleomorphic Sarcoma; undifferentiated pleomorphic sarcoma of zone of skin; zone of skin undifferentiated pleomorphic sarcoma
SNOMED CT: Malignant fibrous histiocytoma of skin (404014008)
 
Monarch Initiative: MONDO:0002141

Definition

An undifferentiated pleomorphic sarcoma arising from the skin. It is characterized by the presence of spindle cells in a storiform pattern and histiocytes with abundant cytoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCutaneous undifferentiated pleomorphic sarcoma

Professional guidelines

PubMed

Diagnosis and differential diagnosis of dermatofibrosarcoma protuberans: Utility of high-resolution dynamic contrast-enhanced (DCE) MRI.
Yu Q, Zhu Y, Huang R, Li Y, Song L, Zhang X, Tang M, Gu Q, Li P, Zhou N, Li Y
Skin Res Technol 2022 Sep;28(5):651-663. Epub 2022 May 31 doi: 10.1111/srt.13164. PMID: 35639715Free PMC Article
Prognostic factors, treatment, and survival in cutaneous pleomorphic sarcoma.
Ibanez MA, Rismiller K, Knackstedt T
J Am Acad Dermatol 2020 Aug;83(2):388-396. Epub 2018 Nov 8 doi: 10.1016/j.jaad.2018.08.054. PMID: 30414918
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719

Recent clinical studies

Etiology

Cutaneous sarcomas.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457
Kutane Sarkome.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-649. doi: 10.1111/ddg.13249_g. PMID: 28591446

Diagnosis

Patterns of infiltration and local recurrences of various types of cutaneous sarcomas following three-dimensional histology.
Welsch K, Breuninger H, Metzler G, Sickinger F, Eberle FC, Häfner HM, Schnabl SM
J Dtsch Dermatol Ges 2018 Dec;16(12):1434-1442. doi: 10.1111/ddg.13708. PMID: 30537312
Cutaneous sarcomas.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457
Kutane Sarkome.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-649. doi: 10.1111/ddg.13249_g. PMID: 28591446

Prognosis

Surgical excision margins for fibrohistiocytic tumors, including atypical fibroxanthoma and undifferentiated pleomorphic sarcoma: A probability model based on a systematic review.
Jibbe A, Worley B, Miller CH, Alam M
J Am Acad Dermatol 2022 Oct;87(4):833-840. Epub 2021 Sep 26 doi: 10.1016/j.jaad.2021.09.036. PMID: 34587553
Cutaneous sarcomas.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457
Kutane Sarkome.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-649. doi: 10.1111/ddg.13249_g. PMID: 28591446

Clinical prediction guides

Surgical excision margins for fibrohistiocytic tumors, including atypical fibroxanthoma and undifferentiated pleomorphic sarcoma: A probability model based on a systematic review.
Jibbe A, Worley B, Miller CH, Alam M
J Am Acad Dermatol 2022 Oct;87(4):833-840. Epub 2021 Sep 26 doi: 10.1016/j.jaad.2021.09.036. PMID: 34587553

Recent systematic reviews

Surgical excision margins for fibrohistiocytic tumors, including atypical fibroxanthoma and undifferentiated pleomorphic sarcoma: A probability model based on a systematic review.
Jibbe A, Worley B, Miller CH, Alam M
J Am Acad Dermatol 2022 Oct;87(4):833-840. Epub 2021 Sep 26 doi: 10.1016/j.jaad.2021.09.036. PMID: 34587553

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