Journal of Clinical Oncology, Vol 20, Issue 11
(June), 2002: 2745-2751
© 2002 American Society for Clinical Oncology
Unusual Pulmonary Lesions
Case 1. A Juvenile Bronchopulmonary Fibrosarcoma
P. W.A. Kunst,
G. Sutedja,
R. P. Golding,
E. Risse,
G. Kardos,
P. E. Postmus
Vrije Universiteit Medical Center, Amsterdam, the Netherlands
CASE 1. A JUVENILE BRONCHOPULMONARY FIBROSARCOMA
A 12-year-old girl had been treated for asthma during the past year. In the week before admittance, she experienced a sudden pain in her right thorax and was dyspneic. On admittance to a local hospital, she was mildly cyanotic with respiratory wheezing. Chest x-ray showed a complete atelectasis of the right lung (Fig 1). The physician considered the presence of a mucous plug caused by asthma exacerbation and treated her with bronchodilators and corticosteroids. Bronchoscopy was performed as atelectasis persisted. A cherry-red polyp, completely occluding the right main stem bronchus, was seen. She developed acute respiratory failure immediately afterward and had to be intubated. The patient, mechanically ventilated, was referred to our hospital. Computed tomography scanning of the thorax showed complete obstruction of the right main stem bronchus without any abnormality of the bronchial wall or lymph node enlargement (Fig 2). Rigid bronchoscopy under general anesthesia showed the tumor to originate from the right upper lobe carina. Electrocautery was used to remove the tumor completely. Histologic diagnosis was a juvenile primary bronchopulmonary fibrosarcoma (PBPF) (Fig 3A, hematoxylin and eosin stain, x40; Fig 3B, reticulin stain, x40). A computed tomography scan (Fig 4) and specimens of the bronchial wall taken after electrocautery did not show any residual tumor; therefore, a wait-and-see approach was considered justified.1,2 Follow-up has now been over 5 years, and the patient is still free of disease.
Primary fibrosarcoma in children is rare. It usually develops in middle-age adults.3 The most common localization is the distal parts of the extremities. The trunk, head, and neck are less commonly affected.4,5 PBPF is even less common. Twenty-six cases have been reported, most as case reports.3 PBPF is usually localized in the lung parenchyma as a lobulated mass. A review of the literature shows that PBPF is a low-grade malignancy with a relatively good prognosis, which is determined by tumor size, its morphologic characteristics, and the extensive nature of disease. Metastases are uncommon (7%).
Endobronchial PBPF has been reported to have an even better prognosis. Nine of the 10 patients previously reported are still alive after long-term follow-up. This is probably because symptoms caused by bronchial obstruction, such as dyspnea, cough, hemoptysis, and chest pain, led to early detection. All patients underwent surgery.4,6
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Lilleng PK, Monge OR, Walloe A, et al: Fibrosarcoma in children. Acta Oncol 36: 438-440, 1996
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Goldthorn JF, Duncan MH, Kosloske AM, et al: Cavitating primary pulmonary fibrosarcoma in a child. J Thorac Cardiovasc Surg 91: 932-934, 1986[Abstract]
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