Indian Journal of Dermatology
: 2022  |  Volume : 67  |  Issue : 3  |  Page : 315-

Scalp angiosarcoma with skull roof infiltration and metastasis to the lung

Yaxiong Li1, Conghui Li1, Jian Wang1, Xin Wang2,  
1 Department of Neurosurgery, The First Hospital of Hebei Medical University, Shijiazhuang, Hebei Provence, China
2 Department of Pathology, The First Hospital of Hebei Medical University, Shijiazhuang, Hebei Provence, China

Correspondence Address:
Yaxiong Li
Department of Neurosurgery, The First Hospital of Hebei Medical University, Shijiazhuang, Hebei Provence

How to cite this article:
Li Y, Li C, Wang J, Wang X. Scalp angiosarcoma with skull roof infiltration and metastasis to the lung.Indian J Dermatol 2022;67:315-315

How to cite this URL:
Li Y, Li C, Wang J, Wang X. Scalp angiosarcoma with skull roof infiltration and metastasis to the lung. Indian J Dermatol [serial online] 2022 [cited 2022 Dec 8 ];67:315-315
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A 79-year-old man presented a large painless ulcer in the frontoparietal area of the scalp, with blood extravasation and suppuration. The ulcer had started as a small erythematous lesion approximately 3 months ago; it had gradually increased in size, and rapid growth of the ulcer had occurred in the past 2 weeks. His medical history revealed diabetes. A 5 cm × 4 cm irregular-shaped ulcer resembling cavities and some erythematous lesions were observed in the frontoparietal region of the scalp [Figure 1]. The floor of the ulcer was purulent, and the margin was edematous and bloodshot. The brain magnetic resonance imaging showed a subcutaneous soft tissue mass with irregular margins in the parietal area, and computed tomography (CT) revealed that there was no destruction of the skull bone [Figure 2],[Figure 3],[Figure 4],[Figure 5]. Multiple skin biopsies were performed, and histological examination revealed a benign hemangioma in the other hospitals. Because of the progression of the lesion, the patient was treated with wide local excision; the skin grafts were performed with a free flap taken from the outer side of the right thigh. In the central part of the tumor, the periosteum was invaded. The histopathology revealed that the ulcerated tumor was composed of irregularly anastomosing vascular channels lined by moderately differentiated enlarged endothelial cells permeating between collagen bundles [Figure 6] and [Figure 7]. Furthermore, immunohistochemical analysis revealed that the tumor cells were positive for CD31 and Vimentin; approximately 40% were positive for Ki-67 [Figure 8],[Figure 9],[Figure 10]. The final pathology was consistent with angiosarcoma. Adjuvant chemotherapy and radiotherapy were refused by the patient. At 3 months post-surgery, the patient visited our clinic with headaches and frequent rebleeding of a partial scalp wound. The examination revealed multiple satellite metastasis of the scalp angiosarcoma around the operation region and the skull defect [Figure 11]. Cranial CT showed the invasion of the bone [Figure 12], while chest CT revealed multiple masses in the right lung [Figure 13] with enlargement of the mediastinal lymph nodes. Subsequently, a CT-guided core needle biopsy was performed on the lung lesion, and metastatic angiosarcoma was detected [Figure 14]. The patient and the relatives declined further invasive surgical treatment. Hence, he was given palliative care and passed away within 10 months.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}{Figure 7}{Figure 8}{Figure 9}{Figure 10}{Figure 11}{Figure 12}{Figure 13}{Figure 14}

Angiosarcoma is a malignant neoplasm of vascular endothelial cells.[1] Scalp angiosarcoma mainly occurs in elderly patients, is very aggressive, inclines to recrudesce locally, spread widely, and has a high rate of lymph node and systemic metastasis. It has a wide range of clinical presentations and often has a delayed or incorrect diagnosis.[2] It usually presents as bruise-like patches or violaceous nodules. On progression, the masses may ulcerate and bleed.[1],[2] The patient usually delays seeking treatment because early tumors are benign in appearance and asymptomatic. Skull roof invasion by scalp angiosarcoma is extremely rare, which is less than 20 cases reported worldwide till date.[3] In our patient, the tumor invaded and destroyed the roof of the skull metastasized to the lung. It shows the highly aggressive biological behavior and the strong metastatic potential of the scalp angiosarcoma. Patients with scalp angiosarcoma involving the skull usually have a poor prognosis. The five-year survival rate is less than 30%.[1],[4],[5] In the UCLA review, they reported 50% of the patients dying within 15 months.[5] The optimal management has not yet been defined. After going through the literature, the most effective treatment is complete surgical resection followed by adjuvant radiotherapy and chemotherapy. Promising treatment protocols involve radical resection with a sufficient safety margin (5 cm), radiotherapy with a dose of 70Gy, and paclitaxel (80 mg/m2) chemotherapy.[1],[6],[7]

Declaration of patient consent

The authors certify that they have obtained all appropriate consent forms from the patient. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

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