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A 64-year-old man presented with weakness of his right arm and leg. He had previously had mitral valve replacement, tricuspid annuloplasty, leg deep vein thrombosis (DVT) and femoral embolism. Computed tomography (CT) scan of the brain showed an acute left thalamic haemorrhage. Repeat CT brain showed resolution of the original haemorrhage, but the apparent development of new areas of haemorrhage. Warfarin continued due to high risk of thromboembolism. He was readmitted with the rapid development of a visible swelling at the sternum and on the scalp. Ultrasound scan of the sternum revealed a vascular tumour. Suspected haemorrhages in the past were reported as the metastatic deposits. Biopsy and immunohistochemical staining confirmed angiosarcoma of the scalp. Being vascular tumours, angiosarcoma can mimic a brain haemorrhage. Our case illustrates a clinical conundrum. Diagnosing metastatic angiosarcoma of the brain proved difficult without visible primary and histology. The rapid clinical course of the disease and problems with anticoagulation therapy made treatment options limited and the prognosis worse.
A 64-year-old man was admitted with a history of clumsiness of the right arm and leg. He had undergone mitral valve replacement and tricuspid annuloplasty 6 months previously. Heart surgery revealed right atrial benign cardiac rhabdomyoma which was confirmed with immunocytochemistry. Staining was positive with desmin, myoglobin and sarcometric actin. Smooth muscle actin stained negative with some background staining with S100. He was taking bisoprolol 2.5 mg for paroxysmal atrial fibrillation, and warfarin. There was a history of recurrent leg DVT and 12 years previously he developed a femoral embolism due to a drop in international normalised ratio (INR) to 1.3. He was having regular surveillance cystoscopy for bladder carcinoma diagnosed 15 years previously with no recurrence. He smoked two cigars a day with a total of less than 10 pack years of smoking. Examination showed right sided weakness and normal prosthetic valve clicks. Full blood count and biochemistry were normal. Computed tomography (CT) scan of his brain showed acute left thalamic haemorrhage 1.9×2 cm in size. He was kept on warfarin but the INR was adjusted to be maintained at a target of 1.7–1.9 after discussion with a cardiologist. He was discharged with a plan to have a repeat brain CT scan 2 weeks later, which showed resolution of the original haemorrhage but the apparent development of a new small area of haemorrhage near the right lateral ventricle. Being high risk for thromboembolism, the patient was managed by continuing warfarin at the same target range.
One month later, he was readmitted with severe pain around the right scapula after a fall and the rapid development of a visible swelling at the upper end of the sternum (fig 1). A smaller swelling was noted on the scalp (fig 2). Investigations showed haemoglobin 10.6 g/dl, white blood cell count 26.2×109/l, platelets 264×109/l, creatinine 74 μmol/l, and INR 2.0. Bone scan showed some increased uptake at the knees but plain x-rays of the knees were normal. Tumour markers and prostate specific antigen (PSA) were normal. An ultrasound scan of the lesion at the sternum revealed a very vascular tumour. Repeat CT scan showed 2 cm soft tissue density within the scalp and multiple enhancing foci within both cerebral hemispheres and a large deposit in the left cerebellum. The previous small area of suspected haemorrhage at the right lateral ventricle could be seen as metastatic deposits (fig 3). A large obstructive lesion was noted in the right maxillary antrum and bilateral adrenal metastases were seen.
Disseminated pulmonary metastases and an 8 cm destructive mass at the upper sternum were seen. Abnormal mediastinal, hilar glands and a large lesion involving the right scapula were also seen. Echocardiography showed a possible small lesion on the mitral valve prosthesis, but maintained left ventricular function. A punch biopsy of the scalp lesion confirmed angiosarcoma of scalp as the histological tumour (fig 4). Immunohistochemical stains showed strong positivity for CD31 in the tumour cells (AE1/3 negative) while CD34 highlighted some native vascular channels in background. Dexamethasone was commenced and warfarin was changed to enoxaparin. The patient was transferred to a local hospice where he died a week later.
There was no postmortem examination.
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.
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.