Cutaneous Angiosarcoma of Head and Neck: A New Predictive Score for Locoregional Metastasis

Abstract

OBJECTIVES: Cutaneous angiosarcoma of head and neck (cAS-HN) is a malignant neoplasm with deficient data on prognostic factors. The aim of this study is to present our monocenter database on cAS-HN so far and a new predictive score for locoregional metastasis (LRM). METHODS: Retrospectively, tumor characteristics and outcome of 103 consecutive patients with cAS-HN were analyzed. The main predictors of LRM (identified by univariate and multivariate statistics) were combined to a LRM risk score. The prognostic values of stratification into high-, medium-, and low-risk groups concerning disease-specific survival (DSS), distant metastasis (DM), and progression-free survival (PFS) were evaluated. RESULTS: LRM (n = 29) and control (n = 74) groups differed significantly concerning several tumor characteristics and outcome (DM, PFS, and DSS). Patients developing LRM showed 3-, 5-, and 10-year survival rates of 32%, 16%, and 11% (mean DSS time of 36.7 months [95% confidence interval (CI) 20.5-52.8]) compared to 81%, 73%, and 69% (mean DSS time of 292.4 months [95% CI 208.4-376.5]) in controls without LRM (P < .001). The main predictors were American Joint Committee on Cancer (AJCC) stage, tumor extent, and origin of the primary tumor. The LRM risk score revealed significant higher values for the LRM group [7.14 (SD 1.46) vs 4.88 (SD 1.89), P < .001]. The high-risk group showed significantly higher risk for DM and more unfavorable DSS and PFS. CONCLUSION: The LRM risk score is a simple way to estimate the risk for LRM and DM, to stage patients, and to determine treatment options.

Angiosarcoma of the Nose: A Case Report and Review of the Literature

“…The diagnosis of angiosarcoma is challenging due to the rareness of this tumor, the absence of consistent therapeutic guidelines and its poor prognosis [1]. Therefore, a thorough medical history, a concise physical examination and clinical knowledge are necessary.…”

“…Cutaneous angiosarcoma is typically a malignancy of elderly males [1,4], the nose is a rare location and can be easily mistaken for a benign lesion due to its variable presentations [5]. Different clinical pictures have been described in the literature with multiple reports of angiosarcoma mimicking rhinophyma, rosacea, hemangiomas, contact dermatitis and even sebaceous cyst [6].…”

“…Angiosarcomas are rare malignant mesenchymatous tumors that originate in the endothelium of blood vessels and represent approximately 1% to 2% of all head and neck soft tissue sarcomas [1].…”

“…Its poor prognosis is due mostly to its rapid growth and a high rate of lymphoid and systemic metastasis [1]. No standard treatment has been described but surgery followed by wide-field radiotherapy seems to be the optimal choice while chemotherapy has a palliative role.…”

Cutaneous Angiosarcoma

“…Surgical resection with ample margins is the treatment of choice and the only potential cure if free margins are reached. In most cases however, complete excision is not possible due to the extension and multifocal nature of the disease [2, 5]. …”