Sarcoma low-malignant fibromyxoidesC49.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

cute malignant fibromyxoidal sarcoma; Evans' tumor; Low-grade fibromyxoid sarcoma

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HistoryThis section has been translated automatically.

Evans 1993

DefinitionThis section has been translated automatically.

Low malignant, fibroblastically differentiated new formation, which can occur as a blurred, nodular 2.0-26 cm large tumor mass in the deep soft tissue, but also dermal-subcutaneously. Postoperatively, the tumor tends to recur, which can occur even after years of latency.

Occurrence/EpidemiologyThis section has been translated automatically.

The incidence was 0.18/1 million people according to a Danish study. m:f=4:6

ManifestationThis section has been translated automatically.

Children and more often adolescents and younger adults.

LocalizationThis section has been translated automatically.

Subfascial on extremities/trunk (90%), rarely head and neck. Dermal-subcutaneous regions are rarely included.

HistologyThis section has been translated automatically.

Tumor nodes consisting of EMA-, MUC4-, and also alpha-smooth muscle actin positive spindle tumor cells are characterized by alternating arc-shaped fascicles with myxoid sections. Tumour cells with cytological atypia, also epithelioid aspects and mitoses. A traslocation t(7;16)(q33;p11) leading to a fusion gene (FUS-CREB3L2) can be detected.

TherapyThis section has been translated automatically.

Excision with sufficient safety distance.

Progression/forecastThis section has been translated automatically.

Metastasis risk is about 10%.

LiteratureThis section has been translated automatically.

  1. Evans HL (2993) Low-grade fibromyxoid sarcoma. A report of 12 cases. Am J Surg Pathol 19: 595-600
  2. Lee JH et al (2016) Low-Grade Fibromyxoid Sarcoma of the Malar Area. Arch Plast Surgery 43:110-112.
  3. Mazari PM et al (2015) Cytogenetically confirmed low-grade fibromyxoid sarcoma arising from the tibia. Hum Pathol. 48:56-59.
  4. Moon A et al (2015) Myxoid dermatofibroma on a great toe: a case report. Int J Clin Exp Pathol. 8: 7605-7509.+
  5. Shibata S et al (2016) Radiation-induced low-grade fibromyxoid sarcoma of the chest wall nine years subsequent to radiotherapy for breast carcinoma: A case report. Oncol Lett. 11: 2520-2524.
  6. Soma S et al (2015) Low Grade Fibromyxoid Sarcoma of the Palate: A Case Report. J Clin Diagn Res 9:XD01-XD02.
  7. Zou MX et al. (2016) Infiltrative low-grade fibromyxoid sarcoma of the thoracic spine. Spine J doi: 10.1016/j.spinee.2016.01.201.

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer