Cutaneous sarcomas (overview)

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Cutaneous sarcoma; Cutaneous sarcoma, cutaneous soft tissue sarcoma; Malignant connective tissue tumors; Sarcoma of the skin; Sarcomas; Skin Sarcomas; Soft tissue sarcoma of the skin

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DefinitionThis section has been translated automatically.

Heterogeneous group of malignant mesenchymal tumours originating in the cutis or subcutis (see also under benign cutaneous soft tissue tumours - soft tissue tumours, cutaneous).

Primary sarcomas of the skin are rare. In principle, however, all deep soft tissue sarcomas can also occur in the skin with varying incidence.

Sarcomas are classified according to their respective tissue of origin or their similarity to existing tissue types (sarcomas of fat, muscle, connective tissue, blood vessels and nerves) and their degree of differentiation.

ClassificationThis section has been translated automatically.

According to the WHO classification of 1994 about 15 clinically significant subgroups of sarcomas are described which may be of different dermatological relevance.

EtiopathogenesisThis section has been translated automatically.

The etiopathogenesis of sarcomas is manifold. Their molecular biology is equally diverse. It is important to distinguish between 2 molecular subentities:
  • Tumours with complex genomic aberrations with detectability of various chromosomal "gains and losses". These are often associated with p53 gene mutations (genome guard function). These include:
    • Leiomyosarcoma
    • Atypical fibroxanthoma
    • Angiosarcoma
  • Tumors with simple genomic aberrations:
    • Here the product of such a translocation is a functional fusion gene. In the typical case, the "new" gene product is a new transcription factor due to the fusion of the regulatory sections (promoter) of a gene A and the DNA binding domain of a transcription factor gene. This puts the complex function of a transcription factor under completely incorrect control. The example of the DFSP ( Dermatofibrosarcoma protuberans) is a good illustration of this: chromosomal translocation mutations ("ring chromosomes") are created by a fusion of chromosome regions 17q22 and 22q13. The fused COL1A1-PDGFß gene is removed from the other genes as a "ring chromosome". As a result, under the influence of transcription factors, excessive amounts of functional growth factor PDGF-beta are formed, which drive the tumor to grow autocrine via PDGF receptor stimulation. This dysfunction is highly characteristic of DFSP (detection in >90%) and is also the key to molecularly targeted therapy with tyrosine kinase inhibitors of the imatinib type (Gleevec).
    • Besides DFSP, this group includes:
      • Clear Cell Sarcoma
      • Myxoid round cell liposarcoma

In addition to the definition of the fine tissue subtype, the tumour is graded. A distinction is made between:
  • Well differentiated less aggressive
  • tumours
  • and
  • poorly differentiated, very aggressive tumours.
Grading of soft tissue sarcomas (according to UICC, 1997)
  • GX Degree of differentiation cannot be determinedG1
  • Well differentiated Low degree of malignancyG2
  • Moderately differentiated Medium degree of malignancyG3
  • Poorly differentiated High degree of malignancyG4
  • Undifferentiated High degree of malignancyThe
system of the UICC (UnionInternationale Contre Le Cancer) is usually used for the classification of stages. In addition to the size of the primary tumor, the infestation of regional lymph nodes and the presence of distant metastases, the histological grade of the tumor is also taken into account.Staging of the primary tumourTX Primary
  • tumour cannot be assessedT0
  • No evidence of primary tumourT1
  • Tumour maximum 5 cm in its largest extensionga
    • ) superficialb
    • ) deepT2
  • Tumour more than 5 cm in its largest extensionga
    • ) superficialb
    • ) deepStadia grouping
for soft tissue sarcomas (according to UICC 1997)
  • IA (G1 or G2;
  • Tumor < 5 cm superficial or deep localized; no lymph node or distant metastases)IB
  • (G1 or G2; tumor > 5 cm superficial localized;
  • no lymph node or distant metastases)
  • IIA ( G1 or G2; tumour > 5 cm superficially or deeply localised; no lymph node or distant metastases)IIB
  • ( G3 or G4; tumour > 5 cm superficially or deeply localised; no lymph node or distant metastases)IIC (G3
  • or G4;
  • Tumor > 5 cm superficially localized; no lymph node or distant metastases)III
  • ( G3 or G4; tumor > 5 cm deep localized; no lymph node or distant metastases)IV ( Any
grade; any size; lymph node metastases or distant metastases)

ManifestationThis section has been translated automatically.

Occurrence in all age groups; more frequent occurrence is in childhood and adolescence and from the 45th to the 55th year of life.

LocalizationThis section has been translated automatically.

Soft tissue sarcomas occur in about 40% of cases on the legs, 15% each on the neck and arms, and 30% on the trunk, chest or abdomen. Only a smaller proportion of these patients become primarily dermatologically noticeable. The angiosarcomas (skin) have different preferred localisations (e.g. angiosarcoma of the head and facial skin), as do dermatofibrosarcoma protuberans (shoulder and back areas) or malignant fibrous histiocytoma (scalp).

Clinical featuresThis section has been translated automatically.

The initial symptoms of the various soft tissue sarcomas are usually painless swellings. In the case of angiosarcomas of the skin, angiomatous dyschromas and new formations (red spots, papules, plaques or nodules) are found. In about 10% of patients with deep-seated soft tissue sarcomas, metastases (often in the lungs) are already present at the time of diagnosis.

General therapyThis section has been translated automatically.

The therapy of soft tissue sarcomas is little standardized. The most important therapeutic measure is an R0 resection to be aimed at. This applies to the initial tumour, recurrences and metastasis. In the case of metastasized soft tissue sarcomas, long-term tumor-free phases can be achieved with surgery of the initial tumor and, if possible, of the metastases. Repeated operations in the event of recurrence of metastases are also possible in principle. Chemotherapy is performed in addition to the operation. This depends on the type of sarcoma from case to case (see e.g. Dermatofibrosarcoma protuberans or Kaposi's sarcoma). .

Progression/forecastThis section has been translated automatically.

The prognosis depends essentially on the type of sarcoma (e.g. dermatofibrosarcoma protuberans or angiosarcoma of the head and facial skin) and on the grade of the primary tumour. In the case of well differentiated tumours, 75% of patients have no relapse of their disease 5 years after completion of treatment, in moderately differentiated tumours it is only 50% and in poorly differentiated tumours 25%. Local recurrences at the site of the primary tumour occur in only 10 to 20% of patients. More frequent (40 to 60% of all patients) are metastases in other organs, mainly in the lung, less frequently in the bones or liver. Lymph node metastases are very unusual (except for synovial sarcoma and rhabdomyosarcoma).

Note(s)This section has been translated automatically.

  • In the present chapter all sarcomas which may be of diagnostic importance for the dermatologist are summarized under "Sarcomas, cutaneous". It is not meant by this term that these tumors have their starting point in the skin.
  • Relatively frequent sarcomas which the dermatologist should know in all details are:
    • dermatofibrosarcoma protuberans
    • Leiomyosarcoma
    • Atypical fibroxanthoma
    • "Actinic" angiosarcoma
    • Undifferentiated pleomorphic sarcoma in distinction to malignant melanoma
  • There is a certain risk that such highly malignant tumours will be misinterpreted as cutaneous sarcomas due to their clinical inhomogeneity and rarity (about 0.7% of malignancies in adults) and that a delayed start of therapy will lead to a worsening of the prognosis.

LiteratureThis section has been translated automatically.

  1. Darier F, Ferrand M (1924) Dermato-fibromes progressifs et récidivantes ou fibro-sarcomes de la peau Annales de dermatologie et de syphilographie (Paris) 5: 45-62
  2. Grossmann AH et al (2012) Classification, molecular characterization, and the significance of pten alteration in leiomyosarcoma. Sarcoma: PubMed PMID: 22448121
  3. Rao J et al (2003) Cutaneous angiosarcoma as a delayed complication of radiation therapy for carcinoma of the breast. J Am Acad Dermatol 49: 532-538
  4. Schöfer H, Brockmeyer N (2002) German guideline: Kaposi's sarcoma.
  5. Ugurel S et al (2008) Short guideline - dermatofibrosarcoma protuberans. JDDG 6 (Suppl1) S19-S20
  6. Vogt T et al (2012) Malignant connective tissue tumor sarcomas. Act Dermatol 38: 248-264

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer