Development of nonspecific interstitial pneumonitis associated with long-term treatment of primary pulmonary hypertension with prostacyclin.

A young woman with primary pulmonary hypertension presented with interstitial lung disease approximately 5 years after successful treatment with IV prostacyclin. The pathology was consistent with nonspecific interstitial pneumonitis and was unresponsive to steroids and immunosuppressive medications. We speculate that further cases of this syndrome may be reported as more patients are living beyond 5 years with prostacyclin.

Remission of invasive thymoma due to chemotherapy. Two patients treated with cyclophosphamide, doxorubicin, and vincristine.

Information regarding the effectiveness of chemotherapy in cases of invasive thymoma is limited. Two patients in whom the combination of cyclophosphamide, doxorubicin, and vincristine produced remission of invasive thymoma are described. The durations of remission were eight and seven months, respectively. In both patients, recurrence was observed at the site of bulky disease, and a secondary complete response continuing for 37 months was achieved in one of them with radiation therapy.

Mucoepidermoid carcinoma of the trachea and bronchus: the case for conservative resection.

We report our experience with 5 patients with mucoepidermoid carcinoma treated by conservative resection, and review the literature on the 62 patients previously reported. A visual bronchoscopic diagnosis of adenoma was made in all 5 current patients. Bronchoscopic biopsy was performed in 4 and provided an accurate histological diagnosis in each. Conservative resection was accomplished with bronchoplastic techniques in 4 patients: sleeve lobectomy, 2; lobectomy with plastic bronchial closure, 1; and segmental tracheal resection, 1. The other patient underwent conventional lobectomy. All 5 patients are alive and free from disease 4 to 15 years following operation. The recently demonstrated correlation between histological grading and clinical behavior allows relatively benign variants of mucoepidermoid tumors to be accurately identified by bronchoscopic biopsy prior to thoracotomy. Because these tumors have a propensity to originate centrally in the tracheobronchial tree, bronchoplastic procedures frequently are required in order to accomplish tumor extirpation with limited pulmonary resection.

Management of carcinoid syndrome.

Two patients who had severe carcinoid syndrome are presented. In one patient prednisone relieved symptoms for 20 months, and in the second patient chemotherapy appears to have precipitated fatal carcinoid crisis. Changes in mentation, increased frequency and intensity of flushing, and hypotension indicate increased risk of a chemotherapy induced carcinoid crisis. Since prednisone may produce relatively long control of carcinoid syndrome, it should be tried before chemotherapy in patients who have increased risk of carcinoid crisis. If chemotherapy is given, doses should be decreased by 50%, and patients should be followed closely.

Mucoepidermoid tumor of trachea.

Mucoepidermoid carcinoma of the trachea is rare. Its occurence in a 14-year-old boy is reported here. This case illustrates the typical course of tracheal tumors with clinical manifestations of cough, wheezing, and hemoptysis, the intially reported normal chest roentgenogram, and the common failure to diagnose tracheal tumor for several months. Early use of tomographic studies and bronchoscopic examination in any person with recent onset of airway obstruction unresponsive to bronchodilator therapy is recommended.