Possible malignant transformation of arteriovenous malformat
Angiosarcoma is a rare malignant tumour, which accounts for 1–2% of all malignant soft-tissue tumours. Most cases of angiosarcoma arise spontaneously, and malignant transformation of vascular malformation to angiosarcoma is extremely rare.

A 70-year-old Japanese woman was referred to department. Her medical history indicated surgery, radiation therapy and intravascular treatment for AVM in her right upper limb since she was 17 years old (Fig. 1).

One month before initial presentation, she noticed frequent haemorrhagic masses in her oral cavity. She complained of general malaise and loss of appetite. The masses bled readily upon scraping the hard palate (Fig. 2A). Similar masses were found on her left lower gingiva (Fig. 2B).

Routine blood tests revealed normocytic normochromic anaemia of 4.6 g/dl, while no platelet or coagulation abnormalities were found. Open incisional biopsy under general anaesthesia was performed after conservative management, including blood transfusion, nutrition and oral care. The masses had grown rapidly and covered the hard palate and had spread to the upper gingiva, maxillary bone and maxillary sinus (Fig. 2C and D).

The specimen consisted of bloody, dark-red and partially sponge-like lesions. Histologically, necrosis and haemorrhage were evident. In areas where viable cells were present, atypical, vascular endothelial cells with pleomorphic nuclei formed a solid lesion or a network to form a narrow lumen filled with blood cells (Fig. 3). Immunohistochemical staining was positive for CD31, CK (AE1 + 3), ERG, D2–40 and CK (CAM 5.2) and negative for CD34, FactorVIII, αSMA, MyoD1, Desmin, Myogenin, S100, HMB45, MelanA, CK (5/6), p63, p40 and EMA. MIB-1 staining showed a proliferation index as high as 50%. These pathohistological findings revealed the tumour as AS (FNCLCC Histological Grade 3).

Positron emission tomography–computed tomography (CT) revealed high fluorodeoxyglucose (FDG) accumulation in her oral cavity and right shoulder in accordance with the location of the AVM (Fig. 4). High accumulation was also evident in her left lung, lumbar spine, piriformis and lymph nodes, suggesting multiple metastases. We diagnosed the masses in the oral cavity as metastatic AS, with the primary tumour located in the right shoulder, where the massive AVM was suspected to have malignantly transformed.

The patient desired the best supportive care and declined additional treatments such as radiotherapy and chemotherapy. She died 4 months after initially noticing the intraoral masses. An autopsy was not performed.

Source: https://academic.oup.com/jscr/article/2019/12/rjz375/5680738
R●●●●●t k●●●r S●●u and 21 others like this6 shares
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Dr. A●●u A●●●u
Dr. A●●u A●●●u Internal Medicine
Nice presentation
Dec 26, 2019Like
V●●●l J●●●o
V●●●l J●●●o General Medicine
Wow. Interesting case!
Dec 26, 2019Like