Cutaneous Metastases Can Be the Initial Presenting Sign of a Cardiac Sarcoma

Abstract

To the Editors: We read with interest the authoritative CME article by Alcaraz et al1 on cutaneous metastases from internal malignancies published in the June issue. In their review, the authors describe the clinical, histopathologic, and immunohistochemical features of cutaneous metastasis from internal malignant neoplasms including breast, lung, gastrointestinal tract, oral cavity, genitourinary tract and thyroid carcinomas, carcinoid tumors, neuroblastoma, soft tissue sarcomas, and other origins embraced under the term “miscellaneous group.” A very uncommon origin of a cutaneous metastasis not mentioned by the authors is a cardiac sarcoma. Virtually all types of soft tissue sarcomas can be observed in the heart. However, these tumors are quite rare as only about 10% of surgically resected cardiac tumors are primary sarcomas.2 On the other hand, cardiac sarcomas presenting as metastatic disease are very rare.3 Angiosarcoma is the most common primary malignant neoplasm of the heart. In this, location depicts only 3% of angiosarcomas.4 This rare tumor has a predilection for middle-aged males and a marked predominance in the right side of the heart, specially the right atrium. The location in left atrium is rare because 78%–93% of cardiac angiosarcomas occur in the right atrium.5,6 The tumor commonly causes a nonspecific clinical presentation including congestive heart failure; precordial chest pain; dyspnea; constitutional symptoms of fever, weight loss, or malaise; cardiac murmurs, often of recent onset; and nonspecific electrocardiographic changes. Two-thirds of cardiac angiosarcomas are well- to moderately differentiated, showing vascular channels and papillary structures. However, the remaining one-third of these tumors are poorly differentiated or composed of spindle or anaplastic cells with poorly formed vascular channels.2 Metastatic spread at the time of diagnosis is frequent. The reported incidence of metastases varies from 66% to 89% of patients.5,6 Metastases occur most often to the lung, liver, lymph nodes, bone, adrenal gland, central nervous system, and spleen. Cutaneous metastases are very infrequent. A review of the literature yielded only 2 cases of primary cardiac angiosarcoma that presented as cutaneous metastases.7,8 Pomper et al7 described a 43-year-old man who initially presented with a soft tissue metastases below the left eye in the absence of cardiac symptoms. At surgery, the lesion was found to be invading the orbicularis muscle. The biopsy revealed a high-grade invasive angiosarcoma. One week postoperatively, a similar cutaneous lesion appeared at the superior border of the upper lip. Chest roentgenogram and computed tomographic scan revealed a right atrial mass. A biopsy of this mass showed a high-grade angiosarcoma consisting predominantly of spindle cells. We reported the case of a 51-year-old man who initially presented with multiple cutaneous metastases in the absence of cardiac symptoms.8 Physical examination revealed cutaneous nodules located in the right mandibular angle, left scapular region, thorax, and right hypochondrium. The nodules showed intact overlying skin; they were mobile and non-tender. The abdominopelvic computed tomographic scan revealed nodules in both lungs and liver. The skin biopsy was diagnosed as metastatic undifferentiated sarcoma. The tumor was relatively well circumscribed, was growing within the dermis and hypodermis, and was composed of cohesive islands with an epithelioid appearance. Tumor cells showed sharply demarcated eosinophilic cytoplasm and round to oval large nuclei containing one or two prominent nucleoli. There were moderate nuclear pleomorphism, mitotic activity, and necrotic areas. The stains for cytokeratin, desmin, muscle-specific actin, S100 protein, HMB45, CD45RB, and placental alkaline phosphatase were negative. The stain for CD31 showed intense positivity in the necrotic areas and in many tumor cells and was regarded as misleading. The patient died 26 days later with widely disseminated disease. At autopsy, a tumor arising in the wall of the left atrium and in the interatrial septum was found.8 After an immunohistochemical study including CD31 and CD34 stains, the diagnosis of cardiac pure epithelioid angiosarcoma was made. Unlike most cardiac sarcomas, our case manifested as an infiltrating tumor displaying auricular thickening not protruding or filling the chambers. It must be remembered that classification of cardiac sarcoma is difficult if there is limited tissue. Even in contemporary series, more than 20% of primary cardiac sarcomas are reported as unclassifiable.2,9,10 In conclusion, although cardiac sarcoma is a rare neoplasm, its presence should be suspected in patients with cutaneous metastatic sarcoma without an evident source of the tumor, even in absence of cardiac symptoms.