Angiosarcoma of the of Scalp with Calvarium: CASE REPORT

20 Jan


A 50-year-old African-American man related a nine-month history of a scalp “knot” that measured about 1 cm x 0.5 cm. He scratched the lesion repeatedly, leaving him with an enlarging ulcer. Three weeks later, he presented to his primary care physician because of the foul smelling nature of the lesion. He was initially treated with antibiotics for seven days but with no resolution. He was again seen about two weeks later and was prescribed another regimen of antibiotics for 14 days. The lesion continued to increase in size associated with fluctuance and pruritus. The patient returned a third time and was given a 21-day course of antibiotics along with instruction to clean the lesion four times daily. By this time, the lesion had become an ulcerated mass. He was eventually referred to a dermatologist eight months after the lesion was first noticed. A biopsy of the lesion revealed atypical vascular proliferation that resembled granulation tissue but with focal atypical features including papillary formation and dissection in between the reticular collagen bundles. A few nonatypical mitotic figures were also noted associated with a marked mixed inflammation. Given the age and race of the patient in conjunction with the history, the dermatopathologist favored pyogenic granuloma as the top differential. Others on the differential included deep fungal infection, granulomatous inflammation, squamous cell carcinoma, and basal cell carcinoma. The patient was then referred for surgical evaluation. On physical exam, a fluctuant fungating and foul-smelling mass measuring 3.7 cm x 5.3 cm was noted in the right occipitoparietal area with purulent drainage. No cervical or posterior auricular lymph nodes were palpable, and the rest of the physical exam was otherwise unremarkable. Nine months after the patient first noticed the “knot”, the mass was excised with primary closure (Figure 1). The excised tissue was submitted for surgical pathology consultation.

Figure 1. Primary closure after excision

Figure 1. Primary closure after excision of the lesion (POD #7).

The specimen submitted was an ellipse of hairy skin measuring 8.3 cm x 4.6 cm x 1.7 cm with a central well circumscribed 2.8 cm x 2.7 cm necrotic red-tan area that extended to within 0.1 cm to the deep resection margin. Histopathologic examination by light microscopy showed skin ulceration with superficial necrosis and bacterial colonies within the necrotic tissue debris (Figure 2A). Beneath the ulcer exudate, the lesion comprises a vascular proliferation that resembled granulation tissue with chronic inflammation (Figure 2B). Destruction and replacement of skin adnexal struc­tures were also noted along with presence of abnormal vascular architecture, interconnections between the vascular channels, and dissection through collagen bundles (Figure 2C). High magnifications showed rather large and occasionally bizarre-looking pleomorphic atypical cells with hyperchromatic nuclei, and high nuclear-cytoplasmic ratio lined the vascular channels. Rare mitotic figures were also detected (Figures 2D, ЗА). The tumor was also present at the deep margin of the excised lesion, but the radial surgical margins were all free of tumor. Postoperative CT scan of the head and MRI of the brain revealed invasion of the underlying parietal bone through its entire thickness over an area that measured about 3.5 cm in diameter, but the underlying brain was not involved (Figures 3B, 4A, 4B). Consequently, the patient was taken back to the OR for craniotomy and excision of the involved calvarium with metal capping (Figure 5). Immunohistochemistry confirmed the endothelial origin of these tumor cells by their diffusely strong immunoreactivity with markers for endothelial cells (Factor VIII-Related Antigen and CD31) (Figure 3C). CT of the chest, abdomen, and pelvis did not reveal metastases. The patient is currently receiving radiation and chemotherapy. cialis professional