Eur J Cardiothorac Surg 2002;22:654-655
© 2002 Elsevier Science NL
Angiosarcoma of the chest wall in a patient with fibrous dysplasia
K. Eguchia*,
S. Ishib,
H. Sugiurac,
K. Nogab
a Department of General Thoracic Surgery, Kawasaki Municipal Hospital, 12-1 Shinkawa-tori, Kawasaki 210-0013, Japan
b Department of Surgery, Kawasaki Municipal Hospital, 12-1 Shinkawa-tori, Kawasaki 210-0013, Japan
c Department of Pathology, Kawasaki Municipal Hospital, 12-1 Shinkawa-tori, Kawasaki 210-0013, Japan
Received 19 April 2002;
received in revised form 17 June 2002;
accepted 21 June 2002.
* Corresponding author. Tel.: +81-45-4818833; fax: +81-44-2459600
e-mail: egukei{at}aol.com
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Abstract
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We have experienced a case of angiosarcoma of the chest wall in a patient with fibrous dysplasia. The patient was a 64-year-old man and had large bilateral thoracic masses. Each tumor, resected metachronously with the chest wall, was diagnosed as angiosarcoma. One of these tumors had a histological component of fibrous dysplasia. The patient died from brain metastasis 19 months after the first operation. Angiosarcoma associated with fibrous dysplasia of the chest wall is very rare.
Key Words: Angiosarcoma • Fibrous dysplasia • Chest wall reconstruction
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1. Introduction
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A 64-year-old man with large bilateral thoracic masses was referred to our hospital because of dyspnea on exertion. The patient said that the left elastic and soft tumor, measuring 22x11x10 cm, rapidly increased in size over a period of 2 months, while the right elastic and hard tumor, measuring 15x7x6 cm, had been swelling gradually since he was 44 years old. He had no evident etiologic factors, such as trauma, irradiation, foreign bodies, or pyothorax. A computed tomographic (CT) scan of the chest demonstrated a left lateral thoracic mass with massive pleural effusion and a right lateral thoracic mass involving an expansion into the bony cortex. The pleural fluid was bloody, and its cytology was negative (Fig. 1
). The main cause of dyspnea seemed to be slight anemia (Hb 9.2 g/dl) and respiratory restriction resulting from the pleural effusion and the left tumor. The intra-operative findings showed that the tumor was largely covered with the smooth parietal pleura and no pleural or pulmonary malignant lesions were observed; consequently, the left tumor was resected, along with a 3 cm margin. The excised specimen consisted of the tumor, skin, soft tissue, the serratus anterior muscle and the eighth, ninth and tenth ribs. The chest wall was reconstructed with polypropylene mesh and a pedicled rectus abdominis musculocutaneous flap. Microscopical examination of the tumor revealed angiosarcoma with multiple anastomosing blood vessels lined by atypical cells. Epithelial immunostains were positive for factor VIII-related antibody and CD34. All margins were free from the tumor. The patient's dyspnea improved after the operation.
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Fig. 1. CT scan shows bilateral prominent tumors with massive pleural effusion in the left pleural cavity.
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The right tumor began to rapidly increase in size, measuring 15x11x9 cm 10 months after the operation. A chest CT scan showed the right lateral mass and two metastatic tumors of the left lung, though no local tumor recurrence on the left chest wall was found. A CT scan of the head and abdomen and a total body bone X-ray examination revealed no evidence of metastasis. Resection of the right tumor, along with the eighth and ninth ribs, and chest wall reconstruction using a polypropylene mesh and a pedicled dorsalis musculocutaneous flap was performed. The pathological diagnosis of the right thoracic tumor was angiosarcoma with fibrous dysplasia (Fig. 2
). The lung metastatic tumors were resected 4 months after the second operation.
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Fig. 2. Histological findings of the resected right chest wall tumor demonstrate angiosarcoma lesion (left side) with fibrous dysplasia lesion (right side).
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The patient died from brain metastasis 19 months after the first operation. Right before his death, metastases to the liver and ribs were identified by radiology.
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2. Discussion
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The most common therapy for localized angiosarcoma of the chest wall is surgical resection. The present case was rather advanced at the time of referral to our hospital, but only surgical treatment was performed because effective postoperative radiotherapy or chemotherapy protocol for this disease was not established [1–3]. A preoperative angiogram with embolization, which is useful for decreasing tumor vascularity, was not performed in this case, but severe bleeding did not occur during the operations for the less invasive tumors.
Unlike most reported cases, the patient did not complain of pain, and his clinical appearance was so unique that the progress of the disease can only be speculated. We suspect that a sarcomatous transformation of fibrous dysplasia may have occurred in the right chest wall tumor and that the left tumor may have been a metastasis from the right tumor. Some reports have described sarcomatous transformations of fibrous dysplasia [4–6], but an angiosarcoma of the chest wall arising from fibrous dysplasia has not been previously reported. Further evidence regarding this potential mechanism of pathogenesis is needed.
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References
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- Fukuroku J., Kusuzaki K., Murata H., Nakamura S., Takeshita H., Hirata M., Hashiguchi S., Hirasawa Y. Two cases of secondary angiosarcoma arising from fibrous dysplasia. Anticancer Res 1999;19:4451-4458.[Medline]
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Abstract
1. Introduction
