No abstract

Objective Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. Limited data are available to guide management decisions. To address this limitation, we present a large retrospective analysis of angiosarcoma patients treated at a single institution over a 25-year period. Methods To identify factors that impact angiosarcoma outcomes, we reviewed demographic, tumor and treatment characteristics of angiosarcoma patients evaluated at the University of Wisconsin Hospital between 1987 and 2012. Results The cohort included 81 patients diagnosed at age 19–90 yo (median 67). Fifty-five (68%) patients presented with localized disease while 26 (32%) presented with metastases. The primary sites were visceral/deep soft tissue (42%), head and neck/cutaneous (37%), breast (16%) and limbs in the setting of Stewart-Treves (5%). The 5-year overall survival (OS) was 40% with a median of 16 months. By univariate analysis, significant adverse predictors of survival included metastases at presentation, visceral/deep soft tissue tumor location, tumor size > 5 cm, tumor necrosis and the absence of surgical excision. A trend toward prolonged survival was observed with radiation therapy and for chemotherapy in patients with metastases. Age, sex, and prior radiation showed no correlation with survival. Conclusions Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease.

Certain monomorphic cellular tumors that occur in the dermis have been called trabecular carcinomas or Merkel cell tumors. Forty‐six cases have been reported to date and the literature on these is reviewed here, with six additional cases reported. Cytologic features include sparse cytoplasm, dispersed chromatin with inconspicuous nucleoli in round nuclei and many mitoses. Trabeeulae and pseudorosettes may be identified. Electron microscopy is required for definitive diagnosis. Like normal Merkel cells, tumor cells contain electron‐dense granules (80–200 nm), 10 mm filaments and desmosomes. Filament‐rich cytoplasmic spikes were found in four tumors. These resemble corresponding protrusions of normal Merkel cells and have not been described in other APUDomas. Cancer 52:238‐245, 1983.

Chondrosarcoma of the larynx is an unusual tumor, with only approximately 200 cases having been reported. This tumor is relatively nonaggressive and tends to remain localized in the larynx. Two cases with aggressive clinical behavior manifested by regional or distant metastasis are presented. In the first case, cervical node metastasis appeared 6 months after total laryngectomy. A dedifferentiated chondrosarcoma was identified by pathologic examination. In the other case, clavicular metastasis developed 7 years after initial local excision. Despite additional treatments, the patient later developed cervical node, lung, and vertebral metastasis. Transformation from low-grade to dedifferentiated chondrosarcoma was verified by pathologic examination. We believe this case to be the first reported laryngeal chondrosarcoma with bone metastasis. A review of the literature regarding regional and/or distant metastasis of laryngeal chondrosarcoma, including the present observations, reveals a total of 20 cases. In all cases, tumor grade has been directly correlated with the development of metastasis.

No abstract