LiposarcomaC49.-
Synonym(s)
Cutaneous liposarcoma; Myxoid liposarcoma; Pleomorphic liposarcoma
DefinitionThis section has been translated automatically.
Malignant soft tissue tumor located in the skin and subcutis which is an extremely rare rarity in dermal or dermal/subcutaneous localization. Liposarcomas of the deep soft tissues of the extremities can be diagnosed via the Integument.
ClassificationThis section has been translated automatically.
Depending on the degree of cell atypes are distinguished:
- Atypical lipomatous tumor/highly differentiated liposarcoma (sclerosing or inflammatory) (40-45% of cases)
- Progression into a dedifferentiated liposarcoma (10% of cases)
- Myxoid/round cell liposarcoma (15 - 20% of cases)
- Pleomorphic liposarcoma (5% of cases)
- Spindle cell liposarcoma
EtiopathogenesisThis section has been translated automatically.
- Common to all highly differentiated liposarcomas is a ring chromosome or alterations of chromosome 12.
- In the group of myxoid or round cell liposarcomas, characteristic chromosomal aberrations are reciprocal translocations which lead to new gene fusions. Translocation between chromosomes 12 and 16; t (12; 16) (q13; p11). Affected are the CHOP gene (C/EBP-homologous protein) on chromosome 12q13 and the TLS gene (translocated in liposarcoma)/FUS (fusion) on chromosome 16. The fusion protein (TLS/FUS-CHOP fusion transcripts) resulting from t (12; 16) (q13; p11) acts as a transcription factor and thus plays a role in the oncogenesis of these liposarcoma subtypes.
- No uniform chromosomal aberrations have been described for the pleomorphic liposarcoma.
ManifestationThis section has been translated automatically.
Between the fourth and sixth decade. The myxoid liposarcoma as the most frequent of all liposarcomas (about 40-50%) is more likely to occur in younger men.
LocalizationThis section has been translated automatically.
Localization mainly in the area of the extremities, but also on the capillitium. The most frequent localisations are located beyond the dermatological diagnostic area (e.g. retroperitoneal space, mediastinum).
Clinical featuresThis section has been translated automatically.
The highly differentiated liposarcomas present themselves as circumscribed, coarsely lobed, coarse knots that can be easily distinguished from their surroundings. The subcutaneously located tumours initially show an inconspicuous surface, later also exophytic growth.
HistologyThis section has been translated automatically.
Typical for all forms are atypical cells in combination with lipoblasts. The cell nucleus is deformed by fat vacuoles in a seal-ring-like to star-shaped manner. This results in bizarre, hyperchromatic nuclear formations. Occasionally, micropseudocysts also develop. The vascular richness with a plexiform vascular pattern is striking.
Immunohistology: Reactivity to S100.
4 types of highly differentiated liposarcomas are distinguished:
- Lipoma-like highly differentiated liposarcoma
- Sclerosed highly differentiated liposarcoma
- Inflammatory highly differentiated liposarcoma
- Spindle cell highly differentiated liposarcoma.
Differential diagnosisThis section has been translated automatically.
Lipoma; silicone granuloma in the vicinity of silicone implants of the breast.
TherapyThis section has been translated automatically.
Complete resection of the entire tumor area under histological border control is crucial for the prognosis. In case of non-curative resection radiotherapy (60 Gy in 30 sessions). Chemotherapy (e.g. doxorubicin) for incomplete resection, recurrences or the presence of metastases. Relatively good results have been described for this. Cooperation with oncologists, radiotherapists.
Progression/forecastThis section has been translated automatically.
The prognosis for large liposarcomas localized in deep soft tissue is very unfavorable and often characterized by early metastasis in liver and lungs. The 5-year survival rate for myxoid liposarcomas is 90%, that of pleomorphic liposarcomas 20%.
The rare, purely dermal tumours have a favourable prognosis.
AftercareThis section has been translated automatically.
Close-meshed follow-up checks over 10 years.
LiteratureThis section has been translated automatically.
- Behranwala KA et al (2003) Buttock soft tissue sarcoma: clinical features, treatment, and prognosis. Ann Surg Oncol 10: 961-971
- Irie T et al (2003) Radiologically and histologically mixed liposarcoma: a report of two biphasic cases. Jpn J Clin Oncol 33: 482-485
- Koea JB et al (2003) Histopathologic type: an independent prognostic factor in primary soft tissue sarcoma of the extremity? Ann Surg Oncol 10: 432-440
- Kohlmeyer S et al (2017) Cutaneous sarcomas. J Dtsch Dermatol Ges 15: 630-647
- Nagashima T et al (2003) A case of renal capsular liposarcoma. Hinyokika Kiyo 49: 527-529
- Newlands SD et al (2003) Mixed myxoid/round cell liposarcoma of the scalp. At J Otolaryngol 24: 121-127
- Val-Bernal JF et al (2003) Primary purely intradermal pleomorphic liposarcoma. J Cutan catheter 30: 516-520
- Wilk M et al (2018) Mesenchymal and neuronal tumors. In: Plewig G et al (ed.) Braun-Falco`s Dermatology, Venerology and Allergology. Springer publishing house SS 1887-1919
- Zelger B (2003) Tumours of the nerves, muscles, cartilage, bones and fatty tissue of the skin. In: Kerl H et al (eds.) Histopathology of the skin. Springer Verlag, Berlin Heidelberg New York, S. 848-851