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Indian Journal of Dermatology
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CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 3  |  Page : 320-322
Angiosarcoma of nose masquerading rhinophyma


1 From the Consultant Dermatologist, Dr. Paruchuri Raja Ram Memorial Skin and Laser Centre, Guntur, Andhra Pradesh, India
2 Consultant Dermatologist, Leelavathi Advanced Skin and Laser Centre, Guntur, Andhra Pradesh, India
3 Consultant Dermatologist, Cosmetic Surgeon and Dermatopathologist, Visakha Institute of Skin and Allergy, Visakhapatnam, Andhra Pradesh, India
4 Department of Histopathology, Warwick Hospital, Lakin Road, Warwick, CV34 5BJ, UK
5 Department of Pathology, Queen Elizabeth Hospital, Hong Kong, China

Date of Web Publication13-Jul-2021

Correspondence Address:
Athota Kavitha
From the Consultant Dermatologist, Dr. Paruchuri Raja Ram Memorial Skin and Laser Centre, Guntur, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_354_19

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How to cite this article:
Kavitha A, Chennamsetty K, Attili S, Carr RA, Elaine CT. Angiosarcoma of nose masquerading rhinophyma. Indian J Dermatol 2021;66:320-2

How to cite this URL:
Kavitha A, Chennamsetty K, Attili S, Carr RA, Elaine CT. Angiosarcoma of nose masquerading rhinophyma. Indian J Dermatol [serial online] 2021 [cited 2021 Jul 27];66:320-2. Available from: https://www.e-ijd.org/text.asp?2021/66/3/320/321315




A 70-year-old man presented with an ulcerating cauliflower-like asymptomatic growth on the nose, gradually increasing in size over a period of 2 years. The patient was repeatedly treated for rhinophyma with topical antibiotic creams without clinical improvement. Physical examination showed an elevated plaque with well-defined indurated margins and ulceration on the tip of the nose [Figure 1]. No enlarged regional lymph nodes were detected. Keratoacanthoma, squamous cell carcinoma, and basal cell carcinoma were considered in the differential diagnosis. Chest X-ray, Computed tomography of neck were normal. Histopathology of the excision biopsy showed an invasive dermal tumor consisting of solid sheets of pleomorphic spindle cells with eosinophilic cytoplasm [Figure 2]. On higher power magnification, fascicles of spindle cells demonstrated moderate nuclear pleomorphism, frequent mitoses, and eosinophilic vacuolated cytoplasm, with focal poorly-formed vascular channels. Red cell extravasation was also seen [Figure 3].
Figure 1: Cauliflower like an elevated plaque with well-defined indurated margins and ulceration on the tip of the nose

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Figure 2: Low-power magnification shows an invasive dermal tumor consisting of solid sheets of pleomorphic spindle cells with eosinophilic cytoplasm

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Figure 3: Higher power magnification shows that the tumor consists of fascicles of spindle cells with moderate nuclear pleomorphism, frequent mitoses, and eosinophilic to vacuolated cytoplasm, with focal poorly-formed vascular channels

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Immunohistological studies showed that the tumor cells were positive for CD 31 and negative for HHV-8, CD 34, Melan A, and S-100 [Figure 4] and [Figure 5]. The diagnosis of cutaneous angiosarcoma was made based on these clinical and histopathological findings. The tumor was further excised with a 10 mm margin and the margins were confirmed free of tumor cells. The patient is asymptomatic with no local recurrences and no enlarged lymph nodes after a follow-up period of 1 year.
Figure 4: Tumor cells show diffuse strong staining for CD31 (a vascular marker) by immunohistochemistry

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Figure 5: Tumor cells are negative for HHV-8 by immunohistochemistry (with positive control shown at the right lower corner)

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Cutaneous angiosarcoma was first described as arising from the scalp and face of the elderly, by Caro and Stubenrauch in 1954.[1] Angiosarcomas are classified as 1) sporadic––involving upper face, scalp, and neck of elderly patients (Wilson-Jones angiosarcoma) 2) following chronic, persistent lymphedema or secondary to a prior surgery (Stewart-Treves syndrome), and 3) areas previously treated with radiotherapy. Various causes like prolonged solar damage, radiation, vinyl chloride, arsenic, insecticides, androgenic steroids, and thorium dioxide exposure have been implicated.

Cutaneous angiosarcoma often presents as an enlarging erythematous plaque, nodule or nonhealing ulceration, and rarely as unexplained facial edema.[2] Cutaneous angiosarcoma can be a great mimicker of arteriovenous malformations, lymphoma, nodular melanoma, sarcoidosis, or facial granuloma. Gallardo et al. described AS developing on rhinophyma[3] and Aguila and Sanchez described AS resembling rhinophyma.[4]

Histologically well-differentiated angiosarcomas show dilated, irregular vascular channels lined with flat endothelial cells that dissect the dermis, interconnecting to form a network. Poorly differentiated basal cell carcinoma, trichilemmal carcinoma, and poorly differentiated squamous cell carcinoma are histopathological differential diagnoses.

A dermoscopic examination can help the clinician by revealing the classic colors of vascular lesions, a graduation of red, purple and blue,[5] absence of well-defined vascular structures, such as lacunae/lagoons and vessels, whitish veil areas with a white or skin-colored central area, and a strengthening of the purple color at the periphery of the lesion.

The survival rate of angiosarcomas arising on the face and neck is low, with 5-year mortality as high as 70%. Local recurrence or metastasis to viscera via lymph vessels and blood is a common and most common cause of poor prognosis. Lung followed by lymph nodes, soft tissue, bone and liver are frequent sites for metastasis.

This case report highlights the importance of histological study and immunohistochemistry of any erythematous enlargement of the skin on the nose, especially when it adopts tumor-like characteristics. Examination with plain films, computed tomography scan, and scintigraphy are required to look for local and distant metastasis before and after treatment. Surgical excision is the gold standard treatment. Larger tumors need radiotherapy and chemotherapy. Doxorubicin is the standard chemotherapy agent.

The clinical picture described in the present case strongly mimicked rhinophyma for a long period of time, to the point that it was repeatedly treated with topicals and oral antibiotics, further delaying the correct diagnosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Jones EW. Malignant angioendothelioma of the skin. Br J Dermatol 1964;76:21-39.  Back to cited text no. 1
    
2.
Mentzel T, Kutzner H, Wollina U. Cutaneous angiosarcoma of the face: Clinicopathologic and immunohistochemical study of a case resembling rosacea clinically. J Am Acad Dermatol 1998;38:837-40.  Back to cited text no. 2
    
3.
Gallardo MA, Bosch RJ, Vidal L, Cabra B, Rodrigo AB, De Galvez MV, et al. Angiosarcoma arising on rhinophyma. Eur J Dermatol 2000;10:555-8.  Back to cited text no. 3
    
4.
Aguila LI, Sánchez JL. Angiosarcoma of the face resembling rhinophyma. J Am Acad Dermatol 2003;49:530-1.  Back to cited text no. 4
    
5.
Oiso N, Matsuda H, Kawada A. Various color gradations as a dermatoscopic feature of cutaneous angiosarcoma of the scalp. Australas J Dermatol 2013;54:36-8.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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