Hemangioendothelioma pseudomyogenes C49.-

Ubiquitous, mostly multifocal, malignant vascular tumor occurring mainly in the soft tissue and bone of the lower extremity, which also has a clinically less spectacular dermal variant.

First manifestation between the ages of 10 and 50.

Lower extremity; however, ubiquitous occurrence is possible.

Clinically, the sometimes painful, mostly multicentre, pseudomyogenic haemangioendothelioma shows no clinical characteristics. It is diagnosed as dermatofibroma, cyst or similar. Deeper flat indurations can also be impressive.

Fuzzily defined, dermal tumour clusters that can also infiltrate the subcutaneous fatty tissue. In addition to solid sections, myxoid sections are also impressive. There are several large tumour cells with rather uniform, oval vesicle-shaped cell nuclei and an eosinophilic cytoplasm. Few mitoses.

Immunohistologically the cells express pancytokeratins (AE1/AE3 - but not MNF116) as well as endothelial markers (ERG, CD31), furthermore alpha smooth muscle actin with different intensity. Cytogenetically, a translocation t(7;19)(q22;q13) is detectable.

In a larger collective (n=50), 1x lymph node metastasis and 1x distant metastasis were detected (quoted by Kutzer et al.)

1. Fan C et al (2015) Pseudomyogenic hemangioendothelioma/epithelioid sarcoma-like hemangioendothelioma of the lower limb: report of a rare case. Diagn Pathol 10:150.
2. Ide YH et al (2015) Penile pseudomyogenic hemangioendothelioma/epithelioid sarcoma-like hemangioendothelioma with a novel pattern of SERPINE1-FOSB fusion detected by RT-PCR--report of a case. Pathol Res Pract. 211:4145-20.
3. Jo VY et al (2014) WHO classification of soft tissue tumours: an update based on the 2013 (4th) edition. Pathology 46: 95-104.
4. Kutzner H et al (2016) Vascular tumors of the skin. In: Cerroni L et al Histopathology of the skin. Springer-Verlag Berlin Heidelberg New-York S. 854
5. Shah AR et al (2015) An aggressive case of pseudomyogenic haemangioendothelioma of bone with pathological fracture and rapidly progressive pulmonary metastatic disease: case report and review of the literature. Skeletal radiol 44:1381-1386.
6. Stuart LN et al (2013) Epithelioid sarcoma-like (pseudomyogenic) hemangioendothelioma, clinically mimicking dermatofibroma, diagnosed by skin biopsy in a 30-year-old man. J Cutan Pathol 40: 909-913.