Congenital lung malformations, primary and secondary, contribute to an important portion of pediatric thoracic surgery. One purpose of this report is to outline the close relation in terms of embryology and clinical presentation of congenital parenchymatous pulmonary malformations. In a retrospective study we also aim to evaluate our experience with the diagnosis and surgical management of congenital parenchymatous bronchopulmonary malformations and to compare our data with the literature. From January 1979 to December 1996 a series of 48 patients, 30 males (62.5%) and 18 females (37.5%), were operated on for congenital bronchopulmonary malformations. Pulmonary sequestration, bronchogenic cysts, congenital lobar emphysema, and congenital cystic adenomatoid malformation were seen in 16, 13, 5, and 14 patients, respectively. The first clinical symptoms occurred at a mean age of 8.8 years (1 day to 62 years), and the mean age at the time of surgical intervention was 9.3 years (1 day to 62 years). The maximum time between first symptoms and surgical treatment was 27 years. A lobectomy was performed in 22 cases; in the other patients more lung-preserving surgery such as enucleation or sequestrectomy was performed. Only one postoperative death occurred following lobectomy for pulmonary sequestration, and it was due to pulmonary hypoplasia and pulmonary hypertension. Eleven other patients presented with postsurgical complications: pneumothorax (n = 5), pleural effusion (n = 3), prolonged air leak (n = 2), portal vein thrombosis (n = 1), and hemorrhage requiring reintervention (n = 1). We conclude that any thoracic cystic lesion expanding on chest radiography should be an indication for surgical resection, even if asymptomatic, because of the risk of pulmonary compression, infection, or malignant degeneration. In the few cases of a fetal intrathoracic mass, prenatal diagnosis and intrauterine intervention may be indicated, and these indications are also discussed.

Case reportA 66-year-old man was treated for a malignant brous histiocytoma of the proximal left tibia, without metastases, 16 years previously.After one course of neoadjuvant chemotherapy with bleomycin-dactinomycin-cyclophosphamide and two cycles of high-dose methotrexate, an above-knee amputation of the left lower limb was performed.Postoperatively the patient received adjuvant chemotherapy with CDDP-adriamycin (doxorubicin)-bleomycin-cyclophosphamide and dactinomycin.During one of the chemotherapy administrations, extravasation of adriamycin occurred in the tissues around the basilic vein at the left elbow. No skin necrosis occurred. After a period of local erythema and swelling, a multinodular sclerotic lesion developed, consisting of four to ve noduli of 0.5-1 cm diameter, xed to the skin and underlying biceps muscle which also became indurated.The lesions were situated along the basilic vein from the elbow up to where the adriamycin extravasation had occurred (Fig. 1).Over a period of 16 years there was no evidence of tumour recurrence. The patient remained in good health and the nodules at his left elbow did not change in size or number. The patient consulted this year because of a fast growing mass above the left elbow at the extravasation site. One of the nodules had signicantly increased in size over a period of 1 month.On examination, the mass was 6 cm in diameter, xed to the underlying biceps muscle and overlying skin, multinodular and non-tender.Magnetic resonance imaging showed a tumour of 2 ´2.5 ´4 cm on the ulnar side of the biceps muscle with thickening of the overlying skin (Fig. 2). Computerized tomography of chest and abdomen did not show metastases except for a small nodular lesion at the pleura of the left upper lobe.A resection of the mass en bloc with the medial head of the biceps, part of the brachialis muscle, and the subcutaneous tissue and overlying skin was performed. A split thickness skin graft was used to cover the defect.Postoperative wound healing was somewhat delayed because of partial loss of the skin graft due to a hematoma.Two months after the procedure a new CT scan of the chest showed a clear increase in volume of the previously described nodule. Even more pleural nodules were seen, which were highly suggestive for pleural metastasis. The patient was subsequently treated with four cycles of ifosfamide with partial response: only the initially described nodule was still present. Via a left thoracotomy, a resection of the nodule en bloc with the lateral side of ribs 4 and 5 was done. A second nodule was palpated intraoperatively and resected with the lateral side of rib 3. Multiple scars in the left lung, especially in the lingula, were seen and one of these lesions was excised. A recon- AbstractWe report the case of a 66-year-old man presenting with a high-grade pleomorphic sarcoma at the left elbow 16 years after the extravasation of adriamycin given for a malignant brous histiocytoma of the tibia. We suggest that this sarcoma originated in a multistep way over many year...