Wilson-Jones Angiosarcoma Presenting as Cerebriform Swelling Over Scalp

Sir, A 69-year-old male presented to the skin department with asymptomatic, gradually progressing, reddish elevated swelling over the scalp for 4 months. There was no history of photoaggravation, weight loss or night sweats. The patient was a known case of diabetes mellitus-2 and hypertension for 1 year. On local examination single, cerebriform, erythematous plaque of size approximately 5 × 7 cm, soft to firm in consistency, is present over the left frontoparietal region of the scalp [Figure 1a and b]. Hair, nails, oral cavity and genital mucosa were normal. No lymphadenopathy was noted. Differentials of lymphangioma circumscriptum, lymphangiectasis, acquired progressive lymphangioma, cerebriform sebaceous nevus, syringocystadenoma papilliferum, trichoblastoma, tricholemmona, sebaceous epithelioma and basal cell epithelioma were kept. Dermatoscopy done with DermLite DL4 showed pinkish areas and reddish polymorphous vessels [Figure 2a] with white and yellow circles [Figure 2b].Figure 1: Single, cerebriform, erythematous plaque of size approximately 5 × 7 cm, soft to firm in consistency, present over left (a) frontal (b) parietal scalpFigure 2: Dermatoscopy showed. (a) Pinkish areas (blue arrow) and reddish polymorphous vessels (Red arrow). (b) Dark red and purple clods scattered around the hair follicles appearing as white (white arrow) and yellow circles (yellow arrow)Punch biopsy from the lesion showed a focal thinned out epidermis with few horn cysts and hair follicles in the papillary dermis surrounded by chronic inflammatory infiltrate and areas of haemorrhage. A definitive opinion was not formed from this; hence, a wedge excision biopsy was done, showing a dermal tumour with many horn cysts. Stroma showed prominent plasmacytoid cells having enlarged nuclei and occasional mitoses [Figure 3]. Immunohistochemistry (IHC) staining revealed a malignant tumour with epithelioid morphology. The tumour was seen insinuating between the adnexal structures in solid sheets and anastomosing sinusoidal spaces. Tumour cells had enlarged hyperchromatic nuclei and prominent eosinophilic cytoplasm with mitotic activity of approximately 7–8/10HPF. The lesional cells express ERG and CD31 and are immune-negative for cytokeratin, EMA, CD138, MUM-1, desmin and CD34.Figure 3: Histopathology showed dermal tumour with many horn cysts. The stroma showed prominent plasmacytoid cells, many of them having enlarged nuclei with occasional mitoses (H&E Stain 10X)From this clinical impression of cutaneous angiosarcoma, TNM stage IV-B was made. Wide excision with left radical node dissection and parotidectomy was done followed by 30 cycles of adjuvant radiotherapy. Angiosarcoma is a rare (1–3% of adult soft tissue sarcomas),[1] highly malignant tumour of vascular endothelial origin and has particular predilection for skin and superficial soft tissue. Cutaneous angiosarcoma follows an aggressive course, with an early propensity for metastasis, having an abysmal prognosis. Three distinct clinical variants are idiopathic angiosarcoma of head and neck (Wilson-Jones angiosarcoma), chronic lymphedema-associated angiosarcoma (Stewart-Treves syndrome) and post-irradiation angiosarcoma (radiation-associated angiosarcoma).[2] Wilson-Jones angiosarcoma is the most common, representing 50% of all cases. It is seen in older individuals with a median age of 70–75 years, male predominance and predilection for the scalp and central face which is observed in this case. It has diverse manifestations with solitary or multiple lesions being erythematous to violaceous macules, patches, papules, nodules or plaques, thus considered a great mimicker.[3] Early lesions simulate bruise, haemangioma, infection or inflammation. In advanced lesions, ulceration and bleeding are observed. It has the highest rate of lymph node and distant metastasis with the lung being the most common site followed by the liver.[3] Dermatoscopy according to different studies showed clusters of violet areas and areas with a combination of colours in vascular lesions within the visible light spectrum showing rainbow patterns.[4] The nodular part shows white lines and white-yellowish clods (follicle openings). Our case showed polymorphous vessels, white and yellow circles and polygonal lines. The high-grade lesions are poorly differentiated and consist of sheets of pleomorphic cells with areas of haemorrhage, disordered architecture and cells with hyperchromatic and pleomorphic nuclei with prominent mitotic activity. The pattern of dissecting proliferation between collagen bundles and nuclear projection towards the lumen are characteristic. Angiosarcoma typically expresses endothelial markers, such as CD34 and VEGF. Prognostic factors include age, extent, primary site, size and histological grade. Because of the difficulty of resection with free margins, tumours located in the head or neck have worse prognosis. Wide excision of lesions to achieve tumour-free margin gives a good prognosis. Various reconstructive options are split-thickness skin grafts and local and free flaps.[5] Unresectable tumours or evidence of metastasis require palliative chemotherapy. Other treatment options including Paclitaxel with/without bevacizumab, VEGF-A monoclonal antibodies and tyrosine-kinase seem promising.[6] Clinical diversity of cutaneous angiosarcoma hinders the diagnosis; thus, biopsy and IHC is performed in time to ensure accurate and timely recognition of this aggressive tumour. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.