Angiosarcomas are uncommon malignant tumours arising from the cells that normally make up the walls of the blood or lymphatic vessels.1
Angiosarcomas may occur in the liver, breast, spleen, bone or heart, but are particularly found in skin and soft tissue.2
The upper forehead and scalp have insidious cutaneous involvements.3
Angiosarcoma of the brain, either primary or secondary, is very scantily reported in the medical literature. Metastasis to the brain is rare in comparison to metastasis to lungs, lymph nodes, bones, liver adrenal glands, spleen and pleura.4
The tumour is biologically aggressive, locally invasive and has a high recurrence rate. Clinical diagnosis is often delayed until the lesions are advanced.5
Confirmation of diagnosis usually requires immunohistopathology.6
Even very small tumours can extend microscopically far beyond the visible boundaries of the lesion.7
Therefore, early diagnosis is essential, and can determine prognosis.8
Treatment options are limited.
Surgery is the mainstay of treatment for the primary disease, but it is not curative in most cases since multifocal invasion with rapid distant spread is common.9
Radiotherapy of the margins is recommended as an adjunct to surgery, but there is limited difference in the overall outcome.10
Recent published data show the usefulness of chemotherapy in the treatment of cutaneous angiosarcoma,11
but overall prognosis is still poor.12
There have been brief accounts of primary congenital angiosarcoma of the brain.13,14
A few case reports of angiosarcoma with primary lesions of the heart, pericardium and spleen leading to metastatic brain involvement show the characteristics of rapid deterioration and adverse outcome.15–17
Scalp angiosarcoma causing brain metastasis is an extremely rare and lethal disease. Kyu-Sun et al
and Ellegala et al
reported two case of angiosarcoma brain metastases arising from the scalp.18,19
Our case illustrates probable scalp angiosarcoma primary with brain metastases as an unusual clinical conundrum and highlights a difficulty, rarely reported, in distinguishing between brain haemorrhage and brain angiosarcomata.
Matsuno et al
have reported a case of cerebellar angiosarcoma, which was cited as cerebral haemorrhage until the surgery was performed and histopathology confirmed angiosarcoma.20
The vascular nature of the tumour makes it radiologically difficult to distinguish from haemorrhage. Clinical symptoms, course of illness, and brain CT findings of angiosarcoma can easily masquerade as a bleed.21,22
Therefore, clinicians and radiologists should be aware of this potentially significant differential diagnosis.23
Our case was challenging as smaller metastatic angiosarcomatous lesions, which were reported on the follow-up CT scan, were not distinguishable until the probable primary lesion became fully evident on the scalp. Without visible lesions, reaching a definite diagnosis could well have been impossible despite suspicions on retrospective analysis of the CT scans. Management of anticoagulation was a real dilemma in view of the mitral valve prosthesis and the patient’s high risk of thromboembolic event. Due to the late diagnosis and aggressive nature of the pathology, there was a lack of therapeutic options and the outcome was understandably unfavourable.
- Angiosarcoma is a rare tumour.
- It is a very vascular tumour and therefore can mimic cerebral haemorrhage of the brain.
- Prompt diagnosis is important due to the aggressive nature of the tumour.