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E-IJD® - CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 3  |  Page : 315
Scalp angiosarcoma with skull roof infiltration and metastasis to the lung


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

Date of Web Publication22-Sep-2022

Correspondence Address:
Yaxiong Li
Department of Neurosurgery, The First Hospital of Hebei Medical University, Shijiazhuang, Hebei Provence
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_9_20

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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

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 Oct 6];67:315. Available from: https://www.e-ijd.org/text.asp?2022/67/3/315/356772




Sir,

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: The erythematous nodules and large ulcer with oozing blood and fester in the frontoparietal region of the scalp

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Figure 2: T1-weighted axial [Figure 2] and sagittal [Figure 4], T2-weighted axial [Figure 3] images show an irregular scalp lesion over the parietal scalp (white arrow), which is T1 slight hyperintense and T2 hyperintense

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Figure 3: T1-weighted axial [Figure 2] and sagittal [Figure 4], T2-weighted axial [Figure 3] images show an irregular scalp lesion over the parietal scalp (white arrow), which is T1 slight hyperintense and T2 hyperintense

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Figure 4: T1-weighted axial [Figure 2] and sagittal [Figure 4], T2-weighted axial [Figure 3] images show an irregular scalp lesion over the parietal scalp (white arrow), which is T1 slight hyperintense and T2 hyperintense

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Figure 5: Brain CT reveals that the skull is intact. CT, computerized tomography

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Figure 6: The dermis was infiltrated by irregular anastomosing vascular channels lined by malignant cells permeating between the collagen bundles (hematoxylin and eosin, ×100)

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Figure 7: Hyperchromatic or enlarged atypical endothelial cells poorly lined the vascular channels (hematoxylin and eosin, ×400)

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Figure 8: Immunohistochemical findings show positive staining for CD31 (×100)

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Figure 9: Immunohistochemical findings show positive staining for Vimentin (×100)

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Figure 10: Tumor cells exhibit a high Ki-67 labeling index (×100)

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Figure 11: Three months after surgery, multiple erythematous nodules (white arrow) and the skull invasion

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Figure 12: Skull defect in brain CT imaging. CT, computerized tomography

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Figure 13: Chest CT demonstrates right pulmonary metastasis (white arrow). CT, computerized tomography

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Figure 14: Histopathological findings of metastatic tumor tissue revealed the multifocal appearance of angiosarcomas (hematoxylin and eosin, ×200)

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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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Choi JH, Ahn KC, Chang H, Minn KW, Jin US, Kim BJ. Surgical treatment and prognosis of angiosarcoma of the scalp: A retrospective analysis of 14 patients in a single institution. Biomed Res Int 2015;2015:321896. doi: 10.1155/2015/321896.  Back to cited text no. 1
    
2.
Ajayi AA, Commins SV, Clarke DE. Metastatic angiosarcoma of the scalp presenting with cystic lung lesions: A case report and review of cystic lung diseases. Perm J 2018;22:17-168. doi: 10.7812/TPP/17-168.  Back to cited text no. 2
    
3.
Chugh AP, Gandhoke CS, Mohite AG, Khedkar BV. Primary angiosarcoma of the skull: A rare case report. Surg Neurol Int 2014;5:92.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Morrison WH, Byers RM, Garden AS, Evans HL, Ang KK, Peters LJ. Cutaneous angiosarcoma of the head and neck. A therapeutic dilemma. Cancer 1995;76:319-27.  Back to cited text no. 4
    
5.
Mark RJ, Tran LM, Sercarz J, Fu YS, Calcaterra TC, Juillard GF. Angiosarcoma of the head and neck. The UCLA experience 1955 through 1990. Arch Otolaryngol Head Neck Surg 1993;119:973-8.  Back to cited text no. 5
    
6.
Byeon S, Song HN, Kim HK, Ham JS, Lee SJ, Lee J, et al. A Korean single-center, real-world, retrospective study of first-line weekly paclitaxel in patients with metastatic angiosarcoma. Clin Sarcoma Res 2016;6:8. doi: 10.1186/s13569-016-0048-0.  Back to cited text no. 6
    
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Miki Y, Tada T, Kamo R, Hosono MN, Tamiya H, Shimatani Y, et al. Single institutional experience of the treatment of angiosarcoma of the face and scalp. Br J Radiol 2013;86:20130439. doi: 10.1259/bjr. 20130439.  Back to cited text no. 7
    


    Figures

  [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]



 

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