Localized fibrous tumours of the pleura: 15 new cases and review of the literature

Objective: To present a series of localized fibrous tumours of the pleura (LFTP), to define the clinical and histopathological diagnostic criteria of this tumour, and to determine the optimal treatment and follow-up. Methods: Review of the charts of the patients with the diagnosis of LFTP (formerly called benign fibrous mesothelioma), as well as of all the histological sections, including immunohistochemical stains. Review of the literature with special emphasis on the clinical and histological criteria of malignancy. Results: During the last 30 years, we found 15 patients with a complete clinical chart and histological material, particularly paraffin blocks of the tumour. The mean age was 57 years (range 27-79). Eight patients were asymptomatic, and the remaining seven presented with non-specific symptoms. All but one had complete resection of the tumour, including partial lung resection in two and partial chest wall resection in three. The diagnosis was confirmed by histological review in 15 cases. Immunohistochemical stainings showed positivity for vimentin in all cases, for CD 34 in 80%, but were consistently negative for cytokeratins. Nine tumours were histologically classified as malignant. Among them, five recurred, two of which were responsible for death. One benign tumour recurred after 1 year, and was treated successfully by repeat resection and radiotherapy. Overall, 13 patients (86%) were alive with no evidence of disease between 10 months and 27 years after the first resection. Conclusions: LFTP is a rare tumour which has a benign clinical course in over 80% of the cases, and is asymptomatic in half the patients. The diagnosis is difficult to establish before operation. Treatment consists of complete resection including adjacent structures if necessary. The clinical behaviour of LFTP cannot be predicted on the basis of histological aspects only. If histologically malignant tumours are more prone to recurrence and poor outcome, broad-based and locally invasive tumours bear a higher risk of recurrence. Long term follow-up is therefore mandatory in all cases in order to perform early re-resection when recurrence occur

Pulmonary sequestration: a review of 26 cases

Objectives: Pulmonary sequestration is a continuum of lung anomalies for which no single embryonic hypothesis is yet available. The aim of this study was to assess the diagnostic tools and treatment for the rare condition, pulmonary sequestration, in an unspecialised centre. Methods: We performed an analysis of 26 cases of pulmonary sequestration (paediatric and adult) operated at the Centre Hospitalier Universitaire Vaudois between May 1959 and May 1997. A review of the extralobar and intralobar types of sequestrations is discussed. Angiography is compared to other diagnostic tools in this condition, and treatment is discussed. Results: Twenty-six cases of pulmonary sequestrations, a rare congenital pulmonary malformation, were operated on in the defined time period. Seventy-three percent (19) of the cases were intralobar and 27% (seven) extralobar. Extralobar localisation was basal in 71% and situated between the upper and the lower lobe in 29%. In six cases, the diagnosis was made by exploratory thoracotomy. In the other 20 cases, diagnosis was evoked on chest X-ray and confirmed by angiography. Lobectomy (46%) was the most common treatment procedure. Segmental resection was performed in 30% of the cases and bilobectomy in 4%. Post-operative morbidity was low. The most significant complications were pleural empyema, haemothorax and haemopneumoperitoneum in case of extralobar sequestration. There was no evidence of metaplasia or pre-neoplastic changes. Conclusions: Despite its rarity, some radiological features are sufficiently suggestive of diagnosis of pulmonary sequestration. Investigations are necessary in order to avoid unexpected pathology at the time of operation. Resection of the involved lung leads to excellent results and the long-term outcome is highly favourabl

Pulmonary sequestration: a review of 26 cases

OBJECTIVES: Pulmonary sequestration is a continuum of lung anomalies for which no single embryonic hypothesis is yet available. The aim of this study was to assess the diagnostic tools and treatment for the rare condition, pulmonary sequestration, in an unspecialised centre. METHODS: We performed an analysis of 26 cases of pulmonary sequestration (paediatric and adult) operated at the Centre Hospitalier Universitaire Vaudois between May 1959 and May 1997. A review of the extralobar and intralobar types of sequestrations is discussed. Angiography is compared to other diagnostic tools in this condition, and treatment is discussed. RESULTS: Twenty-six cases of pulmonary sequestrations, a rare congenital pulmonary malformation, were operated on in the defined time period. Seventy-three percent (19) of the cases were intralobar and 27% (seven) extralobar. Extralobar localisation was basal in 71% and situated between the upper and the lower lobe in 29%. In six cases, the diagnosis was made by exploratory thoracotomy. In the other 20 cases, diagnosis was evoked on chest X-ray and confirmed by angiography. Lobectomy (46%) was the most common treatment procedure. Segmental resection was performed in 30% of the cases and bilobectomy in 4%. Post-operative morbidity was low. The most significant complications were pleural empyema, haemothorax and haemopneumoperitoneum in case of extralobar sequestration. There was no evidence of metaplasia or pre-neoplastic changes. CONCLUSIONS: Despite its rarity, some radiological features are sufficiently suggestive of diagnosis of pulmonary sequestration. Investigations are necessary in order to avoid unexpected pathology at the time of operation. Resection of the involved lung leads to excellent results and the long-term outcome is highly favourabl

Intrathoracic tumours in von Recklinghausen's neurofibromatosis

We review our cases of a rare pathology, intrathoracic tumours in von Recklinghausen's neurofibromatosis, involving 6 patients operated upon in our Surgery Department for intrathoracic neurogenic tumors. All had had a positive history of von Recklinghausen's neurofibromatosis prior to thoracic surgery. The management of this rare condition is discussed. We describe the presentation of the symptoms, the operative technique and the long-term outcome, when obtainable. Our results are compared with those of other authors, and the management of these cases is reconsidered. In conclusion, we recommend that thoracic surgery should only be considered when the patient becomes symptomatic, since malignant changes in the tumour are possible due to operative traum

Computed tomography of leiomyosarcoma of the inferior vena cava

Leiomyosarcoma of the inferior vena cava is a rare lethal malignancy. The authors report the seventy-sixth case of the literature and the sixth displayed by computed tomography. CT is presently the radiological modality of choice to localize the site and extension of the tumour, but a cavogram must also be obtained since it displays, much better than CT, the collateral veins and gives a synthetic longitudinal view of the tumour

Léiomyosarcome de la veine cave inférieure. A propos de trois observations [Leiomyosarcoma of the inferior vena cava. Three cases].

We report three new cases of leiomyosarcoma of the inferior vena cava observed between 1985 and 1990. Two of them developed in women and one in a man. The age of the patients were 56, 29 and 57 years. The three tumors have been removed completely. One patient received postoperative radio- and chemotherapy and is well 68 months after excision. One patient developed liver metastasis 18 months after resection despite chemotherapy and died after 3 years. The last one refused postoperative radio- and chemotherapy, developed massive local recurrence and pulmonary metastasis three months postoperatively, and is still alive with disease after 8 months. The diagnostic modalities of these rare tumors as well as the problems created by their surgical resection and the need for a complementary treatment are discussed

Chirurgie iterative en cas de tumeur germinale. [Iterative surgery in germ cell tumor]

Between 1979 and 1988, iterative surgery was performed on 13 patients for a germ-cell tumor. Apart from orchidectomy, surgery was not the first modality of treatment. On the other hand, 21 surgical procedures were performed for residual tumoral masses after chemotherapy or recurrences. In one third of the cases, only necrosis of fibrosis was resected. Out of 13 patients, 7 died of tumor; 6 are alive (3 with complete remission). Surgery is always indicated in stage IIA to IV nonseminomatous tumors in case of residual tumoral deposits after chemotherapy. For seminoma, surgery is carried out only for retroperitoneal residues larger than 3 cm in diameter. Surgery is also indicated for persistent pulmonary or mediastinal metastases following chemotherapy. Complete surgical excision of residual masses may be technically difficult but is of prime importance as combined chemotherapy and surgery doubles the complete remission rate in comparison to chemotherapy alone