SIR 2008 Annual Meeting Film Panel Case: Radiation-induced Angiosarcoma

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1 Special Communications SIR 2008 Annual Meeting Film Panel Case: Radiation-induced Angiosarcoma Daniel Y. Sze, MD, PhD, and Judy H. Huang, MD J Vasc Interv Radiol 2008; 19: HISTORY From the Division of Interventional Radiology (D.Y.S.), Stanford University Medical Center, Stanford; and Department of Pulmonary Disease and Critical Care (J.H.H.), Palo Alto Medical Foundation, Palo Alto, California. Received April 7, 2008; final revision received April 8, 2008; accepted April 9, Address correspondence to D.Y.S., Division of Interventional Radiology, Stanford University Medical Center, H-3646, 300 Pasteur Drive, Stanford, CA ; dansze@stanford. edu D.Y.S. is a paid consultant to MediGene, Inc., Jennerex Biotherapeutics, Inc., and Pain Therapeutics, Inc.; and serves on the medical advisory board to Lunar Design, Inc. None of these disclosures are pertinent to this article. SIR, 2008 DOI: /j.jvir Figure 1. Chest radiograph at the time of onset of hemoptysis showed dense consolidation of the medial upper left lung, presumably from radiation fibrosis (asterisk). Surgical clips were seen at the site of a previous supraclavicular lymph node biopsy (white arrow), and a calcified plaque (black arrow) was seen overlying the left hemidiaphragm, likely from previous radiation and splenectomy. THE patient was a 37-year-old white woman who was married and nulliparous because of infertility. She had a history of depression and hypothyroidism that had been successfully managed medically. Twelve years before the current presentation, she was treated for Hodgkins disease with chemotherapy and mantle-field irradiation. She was in full remission with no evidence of disease by cross-sectional imaging and positron emission tomography imaging. However, she developed sequelae of radiation myocarditis with 2 aortic and mitral valve regurgitation and an ejection fraction of 45%, and was chronically treated with oral -blockade. She also lost pulses to her left upper extremity. After resolution of an apparent right-sided community-acquired pneumonia, she developed increasing hemoptysis to a maximum of 15 episodes a day of 5 10 ml of bright red blood mixed with mucus. Dyspnea also increased, although her hematocrit level was stable at 32% and her oxygen saturation on room air at rest was 98%. Bronchoscopy showed fresh blood throughout the left lung without endobronchial lesions. Bronchoalveolar lavage revealed no evidence of infection or malignancy. Chest radiography (Fig 1) and computed tomography (CT) were performed (Fig 2). She was empirically given a brief course of corticosteroids for possible vasculitis and developed steroid psychosis without any improvement in hemoptysis. Bronchial arteriography and embolization were performed (Fig 3), and hemoptysis resolved for 3 weeks, then gradually recurred. Bronchoscopic findings were unchanged, but her dyspnea progressed until she was wheelchair- 1133

2 1134 SIR 2008 Film Panel: Radiation-induced Angiosarcoma August 2008 JVIR DIAGNOSIS At autopsy, a diagnosis was made of angiosarcoma of the visceral and parietal pleura and pericardium, infiltrating the lung, that was presumably radiation-induced. Figure 2. Contrast medium enhanced CT images were obtained 3 months after the onset of hemoptysis. The entire left hemithorax was smaller than the right, likely because of asymmetric radiation field and subsequent fibrosis. The left apex and medial upper lung were densely consolidated, consistent with radiation pneumonitis and fibrosis. Ground-glass opacity was observed peripherally, likely representing aspirated blood (arrow). A right aortic arch with aberrant origin of the left subclavian artery were also discovered. A severe stenosis of the origin of the left subclavian artery was also attributed to radiation damage. bound and oxygen-dependent. Nonocclusive bilateral lower-extremity deep vein thromboses were identified on ultrasound, and chest radiography and CT were repeated (Figs 4,5). The patient was electively intubated for progressive respiratory failure. Pulmonary angiography (Fig 6), inferior vena cava filter placement, and pleural biopsy were then performed, but biopsy revealed only organized thrombus, benign alveolar tissue, and pleural plaque with no evidence of malignancy or infection. Two months after the first embolization, another CT examination (Fig 7) and bronchial arterial embolization (Fig 8) were performed. Despite decreased hemoptysis after the embolization, respiratory failure progressed and care was withdrawn 3 days later. DISCUSSION Radiation-induced sarcomas account for approximately 5% of all sarcomas (1-5). Angiosarcomas comprise 15% 60% of all sarcomas occurring in irradiated fields, compared with only 6% in the absence of radiation. The latency period for development of radiation-induced sarcomas averages 5 10 years. Malignancies most commonly associated include breast and gynecologic cancers, lymphoma, and prostate cancer. Radiation-induced sarcomas are much more common in women than in men, even after treatment of sex-independent primary malignancies such as lymphoma. Incidence is decreased by surgical or chemical elimination of ovarian function, suggesting a hormonal cofactor. Incidence is also increased by previous exposure to chemotherapy. The standardized incidence ratio (ie, observed incidence divided by calculated expected incidence) reported for the irradiated Figure 3. Thoracic aortography and bronchial arteriography were performed 4 months after the onset of hemoptysis. (a) Aortogram confirmed right-sided arch and delayed flow into the aberrant left subclavian artery. (b) Late filling of the left subclavian artery from collateral vessels delineated the long segment stenosis (arrowheads). Note subtraction artifact from sloshing blood pooled in the left main bronchus (arrow). (c) Moderately hypertrophied left bronchial and supreme intercostal arteries apparently supplying the fibrosed lung in the left apex and medial upper lung were embolized to stasis with polyvinyl alcohol particles.

3 Volume 19 Number 8 Sze and Huang 1135 Figure 5. Because of lower-extremity deep vein thrombosis and progressive dyspnea, a CT angiogram of the chest was obtained, which showed small pulmonary emboli and confirmed cystic degeneration of the embolized previously consolidated medial left lung (arrow). Pleural thickening was increased bilaterally. Groundglass opacity of the right lung likely represented aspirated blood. Figure 4. Repeat chest radiography was performed 1 month after embolization, 5 months after the original onset of hemoptysis. This showed dense consolidation in the peripheral left lung where there was previously clear airspace, and new lucency in the left suprahilar region where there was previously radiation fibrosis. Figure 6. A catheter pulmonary angiogram was obtained at the time of inferior vena cava filter placement, confirming subsegmental pulmonary emboli throughout the right lung (arrowheads). Pulmonary arterial flow was nearly entirely to the right lung despite injection of contrast medium in the left main pulmonary artery. The pulmonary arterial vascular tree of the left lung was severely atrophied, with nearly static flow and no flow to the upper lung. Pulmonary arterial pressure was 32/18 mm Hg (mean, 26 mm Hg). A biopsy of the pleural rind was nondiagnostic. Figure 7. Repeat CT was performed 2 months after embolization, 6 months after the original onset of hemoptysis. The patient had been electively intubated for respiratory failure and to facilitate repeat bronchoscopy. This follow-up CT image showed progressive consolidation of the left lung, including the region of cystic degeneration, and of the posterior right lower lobe. Pleural thickening and effusions were increased bilaterally. Numerous small pulmonary emboli were again seen, including in the right lower lobe (arrow).

4 1136 SIR 2008 Film Panel: Radiation-induced Angiosarcoma August 2008 JVIR Figure 8. A second bronchial embolization procedure was performed. (a) Angiography revealed a recanalized supreme intercostal artery. (b) Parenchymal phase of supreme intercostal arteriogram revealed diffuse chest wall hypervascularity, which was not present at the time of the first embolization. This and four other intercostal arteries were embolized to stasis with polyvinyl alcohol particles. (c) A newly hypertrophied left inferior phrenic artery was found, supplying irregular hypervascularity of the pericardial and diaphragmatic surfaces. (d) A newly hypertrophied left fifth intercostal artery was also found with collateral connections all supplying irregular hypervascularity of the chest wall. This artery and the inferior phrenic were also embolized to stasis. population ranges from 6 to 26. Tumors are treated by excision if possible, even for palliative effect. Adjuvant therapies include additional radiation therapy when feasible and/ or chemotherapy that usually includes doxorubicin alone or in combination with other agents. Radiation therapy and chemotherapy, although commonly administered, have not been shown to prolong survival even though objective response rates are high (64%). Median survival in all patients with angiosarcomas is only approximately 7 months, but 5-year sur-

5 Volume 19 Number 8 Sze and Huang 1137 vival rates for surgical candidates range from 24% to 40%. References 1. Virtanen A, Pukkala E, Auvinen A. Angiosarcoma after radiotherapy: a cohort study of 332,163 Finnish cancer patients. Br J Cancer 2007; 97: Abraham JA, Hornicek FJ, Kaufman AM, et al. Treatment and outcome of 82 patients with angiosarcoma. Ann Surg Oncol 2007; 14: Cha C, Antonescu CR, Quan ML, et al. Long-term results with resection of radiation-induced soft tissue sarcomas. Ann Surg 2004; 239: van Leeuwen FE, Klokman WJ, Hagenbeek A, et al. Second cancer risk following Hodgkin s disease: a 20-year follow-up study. J Clin Oncol 1994; 12: Brady MS, Gaynor JJ, Brennan MF. Radiation-associated sarcoma of bone and soft tissue. Arch Surg 1992; 127: