Case Report Respiration 1994;61:109-112 Masahiro Nomura'' Yutaka Nakayaa Ken Saito* Hirokazu Miyoshi“ Fumiko Kixhia Shingo Hibinoa Tetsuya Saijyoa Susumu Ito" Koji Nakagawab Hideki Nakanishib Hiroaki Nagaeh Noriyuki Todab Shinji Tanaka*1 Hiroshi Hciradab Kazuya Matsumotob Tadashi Hasegawac Hemopneumothorax Secondary to Multiple Cavitary Metastasis in Angiosarcoma of the Scalp Second Department o f Internal Medicine, Department o f Dermatology and First Department o f Pathology, School o f Medicine. The University o f Tokushima, Japan Abstract Key Words Angiosarcoma Lung tumor Cavitary metastasis Hemopneumothorax We report a case o f hemopneumothorax secondary to multiple cavitary metastasis in the angiosarcoma o f the scalp in an 86-year-old woman, who died o f respiratory failure. At autopsy, multiple cavities were found in both lungs. Histologic specimen o f the cavitary metastasis of the lung showed that tumor cells proliferated forming several tubular spaces and these tubular spac es seemed to communicate with the central cyst. These findings suggested that imperfect vessel-like structures of the cavitary metastasis are likely to break down and finally grow up to large thin-walled cavities. Angiosarcoma is a relatively rare malignant tumor originating in vessels, and its prognosis is reported to be extremely poor 11-3]. An angiosarcoma is frequently ac companied with pulmonary metastasis, but thin-walled cavernous pulmonary metastases are extremely rare |4], We report here an autopsy case with angiosarcoma o f the scalp, who died of respiratory failure caused by hemo pneumothorax due to pleural infiltration o f angiosarcoma and metastases to the pulmonary cavity. Case Report An 86-year-okl woman was admitted to the hospital because of erythematous nodule on her forehead in 1988. Histological exami nation disclosed angiosarcoma o f the right scalp. Tumor was ex- Received: March I. 1993 Accepted alter revision October I. 1993 eised and skin was grafted. An X-ray film o f the chest showed no cystic pulmonary disease at the time o f operation. On March 1991. erythematous tumor reappeared near the operative scar and tumor was excised again. On November 1991. she was hospitalized in our department because o f severe dyspnea. X-ray film o f the chest showed multiple thin-walled cavitary lesions and pleural effusion in both sides (fig. 1). Body temperature was 36.5 ° C . blood pressure was 110/70 mm H g. heart rate was 90 per min. Heart sound was clear but the breath sounds were decreased bilaterally. Surfacial lymph node could not be palpated. Chest C T scan image showed multiple cavities and hydropneumothorax. Thoracic drainage and continuous aspiration were performed immediately al ter admission. The pleural effusion was bloody and cloudy and its specific gravity was 1.032. The number o f cells was 7.680/3. protein 3.589 mg/dl. glucose 109 mg/dl. and LD H 1,095 IU/I. Malignant cells were not observed, but tumor markers (C'EA and CA125) showed higher values, suspicious o f pleural infiltration o f angiosarcoma. After both lungs were expanded by the continuous thoracic drainage, pleural adhesion (OK-432) was carried out. but air leak could not be prevented. She died o f respiratory failure on December 26. 1991. Masahiro Nomura. M l) O 1994 S. Knrger A G . Basel The Second Department o f Internal Medicine School of Medicine Tlie University o f Tokushima (X)25- 7931/94/0612-0109 $ 5.00/0 2-50 Kuramolo cho. Tokushima. T70 (Japan) Downloaded by: Vanderbilt University Library 184.108.40.206 - 10/27/2017 7:22:58 PM Introduction Biopsy Findings o f the Primary Head Skin Lesion at the Operation Extended proliferation o f intradennal tumor with bleeding was observed along with ulceration o f the head skin. In the corium. epithelioid tumor cells contained abundant alveoli diffusively prop- agated. forming irregular blood vessels. Heterotypic mitosis was frequently encountered. A ntopsy Findings In the head skin, malignant cells o f angiosarcoma W'ere not found. Cystic lesions were found in both lungs (fig. 2). Several metastatic lesions could be observed in upper, middle and lower right lobes and lower left lobe. In addition, congestive lesions were found over both lungs. Autopsy Histological Findings Spindle cells proliferated solitary in the primary head skin le sion. but proliferated in slit-like shape in the metastatic lesions in lungs (fig. 3). forming clear boundary to the surrounding normal tissue around cystic space. In the cavities, small amounts o f exudate tinted with blood were observed. The density o f tumor cells was I It) Nomura et al. Fig. 3. Photomicrograph showing metastatic lung tumor. The density o f tumor cells is high and they proliferated forming several tubular spaces. These tubular spaces seem to communicate w ith the central cyst. a x i l ) , b 660. Cavitary Metastasis Downloaded by: Vanderbilt University Library 220.127.116.11 - 10/27/2017 7:22:58 PM Fig. 1. Multiple thin-walled cavitary lesions (arrows) and pleural effusion are seen in both sides. Fig. 2. Cystic lesion o f the lung. high and they proliferated forming several tubular spaces. These tubular spaces seemed to communicate with the central cyst. On the relatively large tubes, the tumor cells lined the wall so as to form epithelioid endothelium. Factor V III staining o f the tumor cells determined by PAP method was negative. Autopsy findings were as follows: ( I ) angiosarcoma o f skin me tastasis. both lungs: (2) bronchopneumonia: (3) pulmonary edema: (4) pulmonary congestion; (5) hypertrophy o f left ventricle; (6) re tention cysts o f kidney; (7) paraovarian cyst. Discussion Angiosarcoma is a malignant tumor that originates from endothelial cells of the lymph or blood vessels, and occurs most frequently in the head and face skin o f the aged [5, 6|. Recurrence and lung metastasis appear at an early stage and the prognosis is very poor [71. This disease is so far reported as 'angiosarcoma', hemangioendothelioma’ , ‘ malignant angioendothelioma’ or ‘ lymphangiosarcoma’ . It was difficult to determine whether angiosarcoma originates from a blood vessel or from a lymphatic vessel. However, in recent years, intra cellular Weibel-Palade’s granules [8. 9J. factor VIII in cells by immunofluorescence technique  and the PAP method have been developed as the specific markers for endothelial cells o f blood vessels. In our case, the factor VIII o f tumor cell by the PAP method was negative, but this does not rule out the possibilities o f blood vessel en dothelium as origin 1111. Pulmonary metastasis forming the cavity o f an angio sarcoma has often been reported 112. 13]. There have been few reports o f the cases, in which hemopneumothorax due to cavitating pulmonary metastases was com bined with pleural infiltration and only reported by Cardozo et al. [14|. Starshak 115] and Lanese et al. . It is suggested that our case was accompanied with metastatic pleural infiltration because of the remarkable increase in tumor marker in spite of no malignant cells in the pleural exudation. The frequency o f cavity formation in primary lung cancer is 20-30% 116. 17]. and moreover, the squamous cell carcinoma of the head and neck frequently produces cavitary metastasis in the lung, but that in metastatic lung cancer is very rare. Dodd and Boyle 118] and Honma et al. 1171 reported that its frequency is 4 and 1%, respec tively. The primary organs causing metastatic lung cancer are often colon and rectum, and the mechanism o f patho genesis developing to the cavity is tumor necrosis . The various pathogenesis o f thin-walled cavity forma tion were so far reported, but definite conclusions remain unclear. Dines et al. 119] and Ohno et al.  reported that the mechanism o f development of pneumothorax in metastatic sarcoma was a rupture o f peripheral necrotic tumor into a bronchus and the pleural space, creating bronchopleural fistula. Moreover, they reported the other possibility that tumor embolus with infarction o f the lung formed the air leak. Peabody et al.  and Crow and Brogdon [211have also suggested that cavitating pul monary metastases could arise in a lung cyst. With regard to the mechanism o f cavity formation in our case, we can postulate additional pathogenesis. Ne crotic substances did not exist in the thin-walled cavities, and the chest radiograph at the first operation did not re veal any cystic lesion. The histopathological findings in autopsy disclosed that the spindle cells proliferated solid ly in the primary lesion o f the head skin, but on the other hand the slit-like proliferation was observed in the meta static lesion o f the lung. Although these malignant cells have a tendency to form vessels, the lining o f these ves sels was incomplete. These imperfect vessel-like struc tures could break down, coalesce, and form the larger thin-walled cavities observed in the study. References 4 Lanese D M . Pacht ER: Unusual presentation o f metastatic scalp angiosarcoma: Diagnosis by transbronchial lung biopsy. 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