Gorham's Disease With Chest Wall Involvement: A Case Report and a Review of the Literature.

INTRODUCTION: Gorham's disease is a rare disorder characterized by osteolysis and abnormal vascular growth within bones. Diagnosis of Gorham's disease is often delayed and for accurate and early diagnosis high clinical suspicion is crucial. No specific treatment is available. Management options include surgery, radiation therapy and medical therapy. We aimed to present the first case of Gorham's disease with chest wall involvement in Iran. By review of the literature we discussed important issues of this rare disease including clinical findings, diagnosis and treatment options. CASE PRESENTATION: We present a 48-year-old man with a history of dyspnea following a blunt chest trauma who was admitted to our clinic several times due to reaccumulation of pleural fluid and chylothorax. Gorham's disease was finally established according to clinical manifestations and radiological findings including massive osteolysis in his left ribs and also histological examination. DISCUSSION: According to review of the literature and considering all treatment modalities the patients was successfully treated with a combination of radiotherapy, pamidronate and thalidomide. We suggest that this disease should be considered among differential diagnoses of patients with chest pain, pleural effusion and/or chylothorax with an unknown reason and more importantly history of chest trauma. In suspected cases, it is essential to examine biopsy specimens of the bone adjacent to the inflammated tissues in order to confirm diagnosis.

Primary major airway tumors; management and results.

OBJECTIVE: Primary major airway tumors are rare. A retrospective analysis of referral centers experience could be helpful for their management. METHODS: Fifty-one patients, including 44 (86%) malignant and seven (14%) benign with primary tumors of subglottis, trachea, carina, and main stem bronchi, were managed in a 14-year period. Based on computed tomography (CT) scan and rigid bronchoscopy findings, those who evaluated as resectable underwent airway resection and reconstruction. The others were managed by one or a combination of these methods: core out, laser, chemotherapy, radiotherapy, and tracheostomy. Follow-up was completed in 88.2%, mean (35.2 ± 33.2 months). RESULTS: Extraluminal extension of the tumor found in CT scan was significantly associated with unresectability (p = 0.006). Thirty-two patients underwent resection with three complications (9%) and one mortality (3%). Nineteen were managed by non-resectional methods; of these, 15 were found unresectable, because of tumor length, extensive local invasion or diffuse distant metastases, and four due to risk-benefit ratio or patient preference. Among 18 patients with adenoid cystic carcinoma 13 (72%) were resected (seven with negative margins). Overall 1-, 2-, 5-, and 8-year survival was 90.9%, 90.9%, 77.9%, and 19.5%, respectively. In unresectable tumors with adenoid cystic carcinoma, overall 1- and 2-year survival was 60% and 40%, respectively. Data analysis found significant association of long-term survival with resection (p = 0.005) but not with negative margins in adenoid cystic carcinoma. Among 15 patients with carcinoid tumors, all were alive at the end of follow-up, except one who died after surgery. CONCLUSIONS: Airway resection, if feasible, may extend survival and may even be curative, with low morbidity and mortality, in most patients with major airway tumors.

Extralobar sequestration in anterior mediastinum with pericardial agenesis.

We report a very rare case of extralobar sequestration and pericardial agenesis in a 22-year-old man. A computed tomographic (CT) scan demonstrated an anterior mediastinal mass. No aberrant artery was preoperatively identified. The patient underwent surgery with an impression of thymoma. An extralobar sequestration receiving its blood supply from the left pulmonary artery, accompanied with pericardial agenesis, was noted at the time of operation. The anterior mediastinum is an unusual site for extralobar sequestions. It is recommended to include extralobar sequestration in the differential diagnosis of anterior mediastinal masses, even if the aberrant artery is not recognized on the computed tomographic scan.

The etiological factors of recurrence after tracheal resection and reconstruction in post-intubation stenosis.

We assessed several factors which might be responsible for the recurrence of post-intubation airway stenosis in a large group of patients who underwent resection and reconstruction surgery by one surgical team. Four hundred and ninety-four patients underwent reconstruction of post-intubation airway stenosis during 1995-2006. The case group comprised patients who had developed recurrence, while controls had no recurrence. The diagnosis of the recurrence was made based on the presence of clinical signs or symptoms and bronchoscopic evaluation. The following variables were compared in both groups: age, sex, duration of intubation, reason for intubation, period of time between intubation and surgery, history of previous tracheotomy, previous therapeutic interventions, subglottic involvement, length of resection, presence of unusual tension at the site of anastomosis and anastomotic infection. Fifty-two patients (10.5%) developed recurrence. Lengthy resection, presence of tension at the site of anastomosis, anastomotic infection and subglottic involvement were significantly higher in the case group. Logistic regression model showed that the three main predictors are anastomotic infection (OR=3.44), subglottic involvement (OR=2.43), and presence of tension (OR=1.97), respectively. It is concluded that the surgeon can play an important role in avoiding recurrence by decreasing tension, preventing infection, and preserving subglottic structure.

Langerhans cell histiocytosis of the lung and thyroid, co-existing with papillary thyroid cancer.

A 24-year-old man presented to our center with a huge goiter compressing his airway. He had a previous diagnosis of Langerhans cell histiocytosis (LCH) of the lung. Core needle biopsy was consistent with histiocytosis. Thyroidectomy was performed. A very invasive mass was encountered at the time of surgery. Histopathology result was consistent with an invasive papillary cancer of thyroid co-occurring with LCH. Although association of LCH with different malignancies has been reported, co-existing invasive papillary thyroid cancer and LCH is a rare combination.

Postintubation multisegmental tracheal stenosis: treatment and results.

BACKGROUND: A number of postintubation tracheal stenoses involve different and separate segments. Treatment of these types of strictures is complicated with obscure results, infrequently reported in literature. METHODS: A total of 648 patients underwent treatment for tracheal or subglottic stenosis from September 1993 through October 2005; of those, 26 cases had two separate stenotic segments. Four types of therapeutic approaches were considered for these 26 patients: one-stage resection of the stenotic sites; two-stage resection of the stenotic sites; resection of one stricture and treatment of the second one by nonresectional methods such as dilatation, laser, stenting, T-tube, or tracheostomy; or treatment of both lesions by nonresectional methods. The therapeutic approach for each patient was determined by the surgeon and was based on the nature and location of stenoses, length of stenoses and the distance between the two stenotic sites. RESULTS: There were 20 male patients (76.9%) and 6 female patients (23.1%), with a mean age of 23.9 years (range, 4 to 64). Fourteen patients had tracheal stenosis and 12 had both tracheal and subglottic involvement. Five patients underwent type 1 therapeutic approach whereas 4, 9, and 8 patients underwent types 2, 3, and 4, respectively. Mean length of resection was 58.9 mm in those who underwent complete resection of the stenotic sites (range, 30 to 90 mm). There were 2 complications, 1 stomal fistula and 1 wound infection. Follow-up was accomplished in all patients with a mean period of 21.5 months (range, 1 to 108). Sixteen patients achieved satisfactory results (good voice and airway), 7 are still under treatment (requiring stent, tracheostomy, or repeated dilatation), and 3 died (2 type 3 and 1 type 4). Two deaths were due to T-tube obstruction, and 1 was due to acute obstruction of the stenotic part. CONCLUSIONS: Resection of both strictures and reconstruction of airway are feasible in some patients with multisegmental tracheal stenosis with good results. When resection of both strictures is not feasible, a combination of resectional and nonresectional managements could be helpful for the vast majority of patients.

A rare inferior middle mediastinal tumor resection under extra-corporeal circulation.

An inferior middle mediastinal mass was observed in a 51-year-old woman initially presenting as pericardial effusion and mimicking lymph node disease. Thoracotomy demonstrated a tumor invading the posterior atrial wall. Complete resection was possible under the extracorporeal circulation. The tumor proved to be a soft tissue myoepithelioma in an exceptional location. Despite the difficult surgical resection due to location, prognosis of this absolutely rare tumor without cytologic features of malignancy should be good.