Cutaneous Angiosarcoma Simulating as Pyoderma in a Middle-aged Indian female: A Rare Case Report.

Sir,

Angiosarcoma is a rare and highly malignant vascular tumor with an incidence of approximately 0.01/100,000. It may vary from highly differentiated tumors to those with a significant anaplasia. It mainly occurs in fair skinned males (sex ratio of 3:1), at an average age ranging from 60 to 80 years.[1] Very few Indian case reports, mostly males, have been documented till date.

We report a rare case of a 47-year-old female who presented to the outpatient department of a tertiary care center in southwest Rajasthan with 2-month history of a painful lesion on her scalp. Examination showed a well-defined, violaceous, indurated, and a highly tender plaque on the right side of the temporo-occipital region. The plaque was approximately 2 cm × 1 cm, with a central yellowish crusting with clear boundaries [Figure 1]. Examination of the whole integument, nail, and oral mucosa was normal. She was previously diagnosed with pyoderma and was given multiple courses of antibiotics with no relief. She had no history of exposure to radiation and no significant family and past medical history. Routine laboratory investigations were within normal limits.

Figure 1

Well-defined violaceous plaque with central yellowish crusting on the right temporo-occipital region

An incisional skin biopsy was performed. Hematoxylin and eosin stained section [Figures 2 and 3] showed tumor cells arranged in nests and sheets. Conventional microscopic sections demonstrated irregular rudimentary and congested vascular channels lined by a single layer of enlarged endothelial cells infiltrating between collagen bundles and dissecting into surrounding dermis. Few endothelial cells appeared large, hyperchromatic, and pleomorphic protruding within the vascular lumina. Mitotic activity was noted. On immunohistochemistry, the neoplastic cells were intensely immunopositive for CD31 [Figure 4] and CD34 [Figure 5]. A diagnosis of cutaneous angiosarcoma (CA) was made. The patient was referred to the Oncology Department for complete workup of metastasis. However, the patient absconded and was lost to follow-up.

Figure 2

H and E stained section under ×10 view showing neoplastic endothelial cells infiltrating in the surrounding dermis

Figure 3

H and E stained ×40 view showing tumor cells arranged in nests and sheets with rudimentary vascular channels

Figure 4

Immunohistochemistry showing strongly positive CD31 marker

Figure 5

Immunohistochemistry showing strongly positive CD34 marker

Angiosarcoma is an aggressive malignant tumor of vascular endothelial origin that comprises approximately 2% of soft tissue sarcomas. The most common form of angiosarcoma is CA, which occurs exclusively in three clinical settings, namely, idiopathic angiosarcoma of face, scalp, and neck; angiosarcoma associated with chronic lymphedema (Stewart–Treves syndrome); and postradiation angiosarcoma.[2] It was first described by Livingston and Klemperer in 1926 and described in detail by Wilson Jones in 1964. The diagnosis of angiosarcoma is based on histopathological evaluation. Histologically, angiosarcoma is characterized by abnormal and pleomorphic malignant endothelial cells. Neoplastic cells range from spindle-to-epithelioid cells, with large irregular nuclei, coarse chromatin, and eosinophilic cytoplasm. In addition, angiosarcoma is associated with positive immunohistochemical markers, including CD31, CD34, and factor VIII-related antigen.[3] In one of the largest series to date, Perez et al.[4] reported on 88 patients with CA, the median age at diagnosis for the entire cohort was 70 years (72.5 years for the head and neck), with 95% of the patients being fair skinned. Of those with head and neck angiosarcoma, 82% were male. To the best of our knowledge, only few Indian case reports[56] of CA are published till date, in which mainly elderly males were involved with the exception of a single case report of Shetty et al.[7] who reported a case of a 67-year-old female of CA. In that way, our case of middle-aged immunocompetent female is very unique. In the clinical setting, the appearance of CA can be variable. Typical manifestations of CA have been described as raised purplish-red papules, rosacea-like lesions, and bruise-like lesions. Due to the variability in the appearance of CA, the correct diagnosis can often be delayed. Our patient also presented with ecthyma type of lesion and had been previously misdiagnosed. Differential diagnosis of angiosarcoma includes intravascular papillary endothelial hyperplasia, Kaposi's sarcoma (multiple hemorrhagic sarcoma), kaposiform hemangioendothelioma, Kimura's disease, angiolymphoid hyperplasia, and amelanotic melanoma.

Our observation presents several particularities as follows:

CA of scalp in a middle-aged (47 years) Indian female of skin Type IV

Clinically, it presented as well-defined violaceous, indurated, and highly tender plaque, with central yellowish crusting simulating as pyoderma

Mitotic activity was noted in histopathological findings in our patient with very short history of 2 months.

This case is being reported because it is a rare tumor in the Indian population. Moreover, the atypical presentation of our patient in terms of age and gender further made timely diagnosis difficult, which is extremely important for a good prognosis. It is important for clinicians to have an index of suspicion for angiosarcoma (especially in case of head and neck lesions), and take the help of histopathology, whenever in doubt.

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Conflicts of interest

There are no conflicts of interest.