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A Unique Collision of Epithelioid Hemangioendothelioma and Bronchioloalveolar Carcinoma in a 54-Year-Old Japanese Woman Vfe present a unique collision of epithelioid hemangioendothelionm and bronchioloalveolar carcinoma. A 54-year-old Japanese woman seen for a small nodular shadow on chest X-ray in December 1989 was found in chest computed tomography to have multiple small nodules in bilateral lung fields. At thoracotomy through a median sternotomy, 9 nodules from 0.3 cm to 1.2 cm in diameter detected were all resected. The pathological diagnosis was epithelioid hemangioendothelionm of the lung. A focus of bronchioloalveolar carcinoma 1.4 cm in diameter existed adjacent to the focus of epithelioid hemangioendothelioma in the lingular portion of the left lung. The initial surgical margin was not clear, and a complete lingulect o m y was conducted through a left lateral thoracotomy. The patient is currently alive with no evidence of recurrence at 13 years after the first appearance. (Jpn J Thorac Cardiovasc Surg 2003; 51: 311-313) K e y words:
epithelioid hemangioendothelioma, bronchioloalveolar carcinoma, collision tumor
Kenro Takahashi, MD, and Mitsuyo Nishimura, MD.*
I n 1982, Weiss and Enzinger 1 reported 41 cases of u n c o m m o n epithelioid vascular tumor apparently biologically on the border between hemangioma and angiosarcoma, for which the term epithelioid hemangioendothelioma (EH) was proposed. Sporadically reported cases hmze included isolated pulmonary EH and lung cancer. 2'3 We present a rare case of an unusual admixture, or "collision," of pulmonary EH and bronchioloalveolar carcinoma (BAC). Case
A 54-year-old Japanese woman seen for an abnormal shadow on chest X-ray in December 1989 was as~nptomatic and had normal laboratory data, Chest computed tomography (CT) showed 6 small nodular
From the Division of Thoracic Surgery, OurLmaCancer Center Hospital, Ota, and *Division of Thoracic SurgeD; National Cancer Center Hospital East, Kashiwa, Japan. Received for publication October 25, 2002. Accepted for publication January 10, 2003. Address for reprints: Kenro Takahashi, MD, Division of Thoracic SurgeD,, Ounma Cancer Center Hospital, 61%1 Takabayashi-Nishi, Ota 373-8550, Japan.
shadows in bilateral lung fields. Transbronchial lung biopsy and transneedle aspiration cytology showed no positive results. In March 1990, the patient underwent diagnostic thoracotomy through a median sternotomy. Partial initial resection of the right upper lobe was pathologically diagnosed on a fi-ozen section as EH. Nine foci of the lesion detected on palpation were resected. Two foci of EH were resected from the right upper lobe, 3 from the right lower lobe, 1 from the left upper lobe, and 3 from the left lower lobe, ranging from 0.3 cm to 1.2 cm in diameter. Macroscopically, each sectioned nodule w,as graywhite and almost cartilaginous. Adjacent to a wellmargined grayish-whitish focus of EH in the lingular portion of the left lung was ill-margined BAC 1.4 cm in diameter (Fig. 1). Retrospectively, an ill-defined shadow" was seen adjacent to a clear-bordered nodule of EH in the lingular portion of the left lung on chest CT at the first appearance (Fig. 2). Postsurgical pathological findings showed concomitant BAC adjacent to a nodule of EH in the lingular portion of the left lung, with the surgical margin not clear. Complete lingulectomy was conducted through a left lateral thoracotomy in May 1990. Figure 3 show's the border between EH and BAC.
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Takahashi et al.
Fig. 1. Serial sectioned specimen resected from the lingular portion of the left lung showing a well-margined grayish-whitish lbcus of EH and an ill-margined focus of BAC 1.4 cm in diameter. Fig. 2. Chest CT at first appearance. Note the ill-bordered shadow adjacent to a clear-bordered nodule of EH in the lingular portion of the left lung.
Discussion
Fig. 3. Microphotographs of border between EH and BAC (hematoxilin & eosin). Note viable minor cells at the periphery. of EH nodules extending into alveolar lumens (left). In a highpower field (right), tumor cells have large, polygonal, eosinophilic cytoplasm and relatively incgular shaped nuclei, with occasional intracytoplasvnicvacuoles.
Viable tumor cells at the nodule periphery extend into the alveolar lumens. Tumor cells penetrate from the alveolus to the next through the pores of Kohn micropotypoidally, expanding to the alveolar lumens. Under a high-power field, tumor cells have large, polygonal, eosinophilic cytoplasm and relatively irregularly shaped nuclei, with occasional intracytoplasmic vacuoles. Factor VIII-related antigen stained positively (data not shown). Electron microscopic examination was not done. Annual chest CT have shown neither EH recurrence in the lung nor BAC recurrence for 13 years.
In 1975, Dail and Liebow: presented 20 cases of a peculiar lung tumor they considered as a rare variant of bronchioloalveolar tumor with intravascular invasion, terming it intravascular bronchioloalveolar tumor (IVBAT), because 1 case showed BAC existence. In 1979, Corrin et al.4 studied 3 cases of IVBAT ultrastructurally and proposed that 1VBAT was of endothelial origin. Immunohistochemical examination using antifactor VIII-related antigen antibody revealed this tumor was derived from vascular endothelium. 3'5 In 1982, Weiss and Enzinger 1 published the outcome of their review on a tumor originating in soft tissue, which they termed EH. Its histological characteristics were identical to that of IVBAT, and some cases had pulmonary lesions. They concluded that IVBAT was a pulmonary analog of EH. Dail et al.6 analyzed 20 cases of IVBAT in 1983, referring to the tumor as a peculiar sclerosing endothelial tumor. It is generally accepted that the pulmonary tumor, originally described as IVBAT, should be referred as pulmonary EH. 7 Kitaichi et al. 8 reported 21 cases of pulmonary EH, describing pleural effusion as a significant prognostic marker in patients with EH. They also mentioned symptomatic patients associated with a relatively unfavorable prognosis, histologically considering, fibrinofibrous pleuritic lesions with extrapleural proliferation
Volume 51 Number 7 July 2003
o f tmnnor cells and the presence of spindle tumor cells to be markers of an uaffavorable prognosis. According to Kitaichi's criteria, the present case is not considered associated with an tuffavomble prognostic marker, Since no proof exists of improvement on sui~.,ival with complete resection o f the lesion, 8 biopsy specimens would have been resected less invasively tBrough video-assisted thoracic surgery (VATS) in this case, if it had been available. The association of lung cancer among patients with EH is rare, 6'8 with only 2 cases reported having concurrent pulmonary malignancies with EH, One was a 61-year-old woman, who had BAC 6 and the other was a 61-year-old woman, who had 1.5 cm subpleural small cell carcinoma of the lung] To our knowledge, no other cases with a collision tumor consisted o f EH and B A C have been reported. The term <
The association of EH mad pulmonary malignancies is apparently rare and has not, to our knowledge, been associated with a collision style, with which it is considered to be incidental. We thank Dr. Tetsuro Kodama, National Cancer Center Hospital, for the pathological diagnosis and all those working for National Matsudo Hospital before its closure on June 30, 1992.
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