RhabdomyosarcomaC49.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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HistoryThis section has been translated automatically.

Weber, 1854

DefinitionThis section has been translated automatically.

Rare malignant tumour occurring mainly in the urogenital tract and in the deep soft tissue of the head and neck region (orbit). The rhabdomyosarcoma is one of the most frequent childhood sarcomas (20%). However, it does not play a significant role in dermatological practice.

ClassificationThis section has been translated automatically.

  • Embryonic rhabdomyosarcoma (most common subtype)
  • Alveolar rhabdomyosarcoma (Predominantly located on the extremities)
  • Pleomorphic rhabdomyosarcoma (mainly located in the retroperitoneal space and on the head and neck); predominantly in men in the 6th-7th decade of life.
  • Spindle cell rhabdomyosarcoma (in children: here mainly paratesticular and in adults: here soft tissue of the head and neck).

Clinical featuresThis section has been translated automatically.

Soft or rough, moderately sharply definable tumor masses in the deep soft tissue. The skin remains smooth on the surface.

HistologyThis section has been translated automatically.

Small cell round cell sarcomas with rhabdomyoblasts characterized by an eosinophilic, fibrillar cytoplasm. Immunohistologically, the cells are reactive for myogenin, the markers MYF4, MYODI, desmin, caldesmon, actin). Pleomorphic forms are characterized by bizarre polygonal, round to spindle cells.

Molecularly, embryonic rhabdomyosarcomas show genetic alterations at 11p15.5. Alveolar rhabdomyosarcomas show fusions of PAX3/7-FOXO1, among others. Spindle cell rhabdomyosarcomas show 8q13 rearrangements.

Differential diagnosisThis section has been translated automatically.

Leiomyosarcoma.

TherapyThis section has been translated automatically.

If possible excision, radiotherapy and polychemotherapy if necessary.

Progression/forecastThis section has been translated automatically.

Awkward. The prognosis is significantly improved by combined operative and radiochemotherapy. However, PX3/7-FOXO1 fusion positive alveolar and pleomorphic variants remain poor (Wilk M et al. 2018).

LiteratureThis section has been translated automatically.

  1. Bröcker EB et al (1992) Rhabdomyosarcoma: a differential diagnosis of cutaneous tumours in childhood. dermatologist 43: 590-593
  2. Ferrari A et al (2003) Rhabdomyosarcoma in infants younger than one year old: a report from the Italian Cooperative Group. Cancer 97: 2597-2604
  3. Leuschner I et al (1999) Pathology of childhood and adolescent rhabdomyosarcoma. Pathologist 20: 87-97
  4. Little DJ (2002) Adult rhabdomyosarcoma: outcome following multimodality treatment. Cancer 95: 377-388
  5. McDowell HP (2003) Update on childhood rhabdomyosarcoma. Arch Dis Child 88: 354-357
  6. Stout AP (1946) Rhabdomyosarcoma of skeletal muscles. Ann Surg 123: 447-472
  7. Weber CO (1854) Anatomical examination of a hypertrophic tongue with remarks on the new formation of transversely striated muscle fibers. Virchow Arch Pathol Anat 7: 115
  8. 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

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer