Case 83 - Discussion

Uploaded: 2007-08-26 Updated: 2007-08-28

   
 

Angiosarcoma of the Scalp

CLINICAL FEATURES
 
  • Most patients presented with a significant delay in diagnosis;
  • Angiosarcoma of the face and scalp is a distinctive subgroup primarily in elderly persons, also known as senile angiosarcoma or malignant angioendothelioma; commonly occurs in elderly white men with a male-to-female ratio of 3:1;
  • Usually occurs in the dermis of the scalp, less often in the upper face, uncommon in the lower face and rarely in the neck;
  • Clinical manifestations vary widely. In the early stages, most lesions appear clinically innocent. Most patients presented with either a bruise-like macule or a nonbruise-like nodular painless lesion.. Extensive local growth is common, and margins are difficult to define surgically. Multifocality is noted in approximately half the patients. Metastasis to regional lymph nodes and to the lungs can occur, often after repeated surgical excisions of the primary growth. In these patients, the prognosis is poor;
  • Most of the cases are sporadic, and some may occur In chronic lymphedematous areas, such as occurs in the upper arm after mastectomy with lymph node dissection or radiotherapy (Stewart Treves Syndrome), and in previously irradiated sites;
GROSS FINDINGS
 
  • In the early stages: ill-defined bruise-like areas with an indurate border; may be single or multifocal; bluish or violaceous; nodules, plaques, or flat infiltrating areas; and occasionally may bleed or ulcerate;
  • Advanced lesions: can be elevated, nodular, or occasionally ulcerated.
MICROSCOPIC FINDINGS
 
  • Early lesions may show benign capillary hemangioma-like structures; Often show extensive involvement of the dermis and even deep structures such as fascia and subcutis; Three histologic patterns: vascular channels, sheets of cells, and cells of undifferentiated morphologic features.
SUBTYPES
 
  • Low-grade angiosarcomas: composed of well-formed, irregular vascular channels, often lined by flattened endothelial cells; distinguished from hemangiomas by their so-called “collagen dissection pattern,” anastomosing architecture,  formation of papillae, vascular channels that are often irregular in shape and size, and larger and more chromatic nuclei with cells often piling up along the lumina. Mitoses uncommon;
  • High-grade tumors: sheets of pleomorphic cells may resemble a carcinoma. The vascular anastomosing channels are lined by atypical endothelium either in a single row or multiple layers. The highly cellular areas may appear solid. Often show areas of hemorrhage, disordered architecture, and large cells with hyperchromatic, pleomorphic nuclei; Often display prominent mitotic activity. Occasionally have well-differentiated areas.
  • Histological variants: spindle cell type, solid epithelioid type, minimal-deviation (hemangioma-like, may express CK8 and CK18), granular cell type, and pleomorphic type.
DIFFERENTIAL DIAGNOSES
 
  • Cutaneous angiosarcoma with prominent lymphocytic infiltrate may be readily mistaken for cutaneous follicle center cell lymphoma or cutaneous pseudolymphoma;
  • Hemangiomas
IMMUNOHISTOCHEMISTRY AND SPECIAL STAINS
 
  • Positive: von Willebrand factor (vWF), CD31 and CD34
TREATMENT AND PROGNOSIS
 
  • The optimal treatment is surgery followed by wide-field radiotherapy although it is difficult to obtain a fee margin;
  • Overall, the prognosis is poor. Angiosarcomas have a tendency for metastasis by lymphatic or hematogenous routes;
  • Patients with T1 (<5 cm) disease had a significantly better long-term outcome than patients with T2 (>5 cm). Thus, the most important factor in determining prognosis seems to be the size of the initial lesions.
  • Other factors have a less substantial effect on prognosis; patients with moderate or marked lymphocytic inflammatory responses survived longer (P = .002); patients with multifocal disease have worse outcome than those with solitary lesion; younger age and postoperative radiation are associated with better outcome; the impact of tumor grade on prognosis is not well-defined;
  • Recurrences and metastases are usually noted within 2 years of diagnosis. In one of the largest series to date, Holden et al reported that only 12% of patients survived 5 years or longer, with half dying within 15 months of presentation.
REFERENCES
 
  • www.emedicine.com/plastic/topic494.htm
  • Cutaneous Angiosarcoma of the Scalp. Cancer 2003;98:1716–26
  • AFIP, series 4. Nonmelanocytic Tumor of the Skin.
  • European Journal of Dermatology. Volume 11, Number 3, 261-3, May - June 2001
  • Am J Dermatopathol 2007;29:342–350)
  • Cutaneous Angiosarcoma. American Journal of Clinical Oncology • Volume 29, Number 5, October 2006