An Elderly Man with Epitheloid Angiosarcoma Presenting as Multiple Scalp Swellings

Dear Editor,

Angiosarcomas are rare, malignant, vascular neoplasms, comprising less than 1% of all sarcomas.[1] Epithelioid angiosarcoma is a highly aggressive endothelial cell malignancy that has a male predilection. Although isolated pediatric cases have been reported, they generally occur in adult life, with the highest incidence in the seventh decade.[2] According to various sources, the exact number of documented cases is difficult to estimate due to their rarity and scattered reports. However, estimates suggest that fewer than 300 cases of epithelioid angiosarcoma have been described in published studies.[3,4,5]

Epithelioid angiosarcoma often demonstrates early nodal and solid organ metastasis, especially to the lungs, bone, soft tissue, and skin. Within 2 to 3 years of diagnosis, more than 50% of patients succumb to the disease, but 20% to 30% remain disease-free.[6] Treatment modalities vary among individual cases of angiosarcoma, but surgical resection of the primary tumor and radiation therapy are usually used.[7] There is evidence that paclitaxel-based chemotherapeutic regimens may improve survival.[8] Additionally, reports have described remission after the combined use of adjuvant radiation therapy and bevacizumab, followed by surgery.[9]

A 72-year-old male patient hailing from Kasargod, farmer by occupation, presented with multiple swellings on the scalp since 6 months. Initially, the lesion started on the vertex and later on increased in number and size to involve the forehead. Lesions were associated with mild itching and pain. For the above complaints, the patient visited a local physician and was prescribed some topical and oral medications (details unknown); however, his symptoms did not subside. There was no history of fever, fluid-filled lesions, insect bite, or any procedures related to these issues. There was no history of similar lesions in the past. The patient was a known case of hypertension and diabetes mellitus and was on medication for these.

On general physical examination, the patient was moderately built and nourished. His blood pressure was 150/90 mmHg and pulse rate was 76 beats per minute. Systemic examination was unremarkable.

Dermatological examination revealed multiple well-defined skin-colored to erythematous nodules with a shiny, pearly white surface, present on the forehead, vertex, and left temporal area of the scalp. The largest nodule measured 3 cm × 3 cm and the smallest measured 0.5 cm × 0.5 cm. Some of the lesions showed ulceration, oozing, and crusting [Figure 1].

Figure 1.

A 72-year-old male with multiple well-defined, skin-colored to erythematous nodules on the scalp

Based on history and clinical examination, subcutaneous mycoses, dermatofibrosarcoma protuberens, appendageal tumors, cutaneous lymphoma and cutaneous metastasis were considered as differential diagnoses.

The patient was admitted and potassium hydroxide mount was done, which did not show any fungal elements, thus ruling out subcutaneous mycoses. Routine blood investigations were sent, which were normal, except for elevated glycosylated hemoglobin. Human immunodeficiency virus antibody and hepatitis B surface antigen were tested non-reactive. Urine analysis showed the presence of sugar (2+). A skin punch biopsy and excisional biopsy of the lesion were done, and the samples were sent for histopathological examination. Histopathological evaluation revealed a subepidermal clear zone, multiple vascular spaces and pleomorphic tumor cells in the dermis, solid sheets of large epitheloid cells with an abundant cytoplasm and a large vesicular nucleus, anastomosing delicate vascular channels with red blood cells, and perivascular proliferating tumor cells [Figures 2 and 3]. Based on histopathology, differential diagnoses of poorly differentiated carcinoma, melanoma and epithelioid angiosarcoma were considered. Immunohistochemistry of the sample was positive for vimentin, CD 31, and negative for pan-cytokeratin (pan-CK). Vimentin positivity indicates that lesions contain mesenchymal tissue. CD 31 positivity shows that lesion contains vascular tissue. Pan-CK is epithelium-specific, and and its negativity here rules out an epithelial origin. Following immunohistochemistry, we determined that the tumor mainly originated from mesenchymal and vascular tissues [Figure 4]. Ultrasonography of the neck showed a hypoechoic lesion in the right parotid region (intraparotid lymph node) and a few nonspecific lymph nodes in bilateral submandibular region. On the basis of clinical, laboratory, and histopathological evaluation, the patient was diagnosed with cutaneous epitheloid angiosarcoma. The patient was referred to a medical oncologist for further management. As the tumor was extensive and aggressive, he was started on palliative management with injection paclitaxel. The patient was followed up after 3 cycles of paclitaxel, but there was not much improvement, and radiotherapy was started. The patient later lost to follow-up.

Figure 2.

Histopathology using hematoxylin and eosin stain showing subepidermal clear zone, multiple vascular spaces, and pleomorphic tumor cells in the dermis under 10x

Figure 3.

Histopathology using hematoxylin and eosin stain showing multiple vascular spaces and pleomorphic tumor cells in the dermis under 40x

Figure 4.

Immunohistochemistry using 3,3- Diaminobenzidine stain showing tumor cells staining positive for vimentin under 10x

Epithelioid angiosarcoma is a rare, aggressive subtype of angiosarcoma. Overall, cutaneous angiosarcoma (CAS) is somewhat more common in elderly men. Several trigger factors have been associated with the development of epithelioid angiosarcoma, like chronic lymphedema, radiation therapy, chronic ultraviolet radiation from sun exposure, environmental and occupational exposure to certain chemicals, immunosuppression, chronic inflammation, and injury. Our patient is a farmer by occupation, so exposure to ultraviolet radiation from sun rays may be the triggering factor. Epithelioid angiosarcoma is characterized by oval or polygonal cells with an abundant, faintly eosinophilic cytoplasm. It has a male predilection and is more common on the head and neck region (scalp and face). Epithelioid angiosarcoma can also occur in atypical sites, like bones, liver, thyroid, and adrenal glands.[3,4,5,10,11] Histopathologically, CAS may be just as subtle and is often mistaken for one of its mimics including traumatized capillary hemangioma, Kaposi sarcoma, spindle cell hemangioma, atypical vascular lesion, hemangioendothelioma, melanoma, carcinoma, and atypical fibroxanthoma. Immunohistochemical studies can be positive for vimentin, CD31, CD34, and factor VIII. Various investigations like histopathological evaluation, immunohistochemistry, magnetic resonance imaging, computerized tomography, and ultrasonography help in diagnosis. Treatment modalities include surgical resection of primary tumors and radiation therapy. There is also evidence that paclitaxel-based chemotherapeutic regimens may improve survival. Targeted therapies, including antiangiogenic agents like bevacizumab (which targets vascular endothelial growth factor) and tyrosine kinase inhibitors like sorafenib, which aims to block the tumor’s blood supply, have been tried.[12]

This case report highlights the importance of correlating clinical history, laboratory investigations, and histopathological evaluation in diagnosing a clinical condition.

Declaration of patient consent

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

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)

The preparation of this manuscript was carried out entirely by the authors without the use of artificial intelligence technologies.

Funding Statement

Nil.