Solitary fibrous tumors of the pleura

Solitary fibrous tumor of the pleura is a mesenchymal tumor that has been increasingly recognized over the past few years. The tumor was initially described in the pleura, but it has been reported in many other sites lately. Although the majority of these tumors have a benign course, the malignant form still remains enigmatic. Indeed, the behavior of these tumors is often unpredictable and does not always correlate with histologic findings. In addition, benign tumors may remain unproblematic for several years before changing into a malignant form. In order to define more precisely the clinical behavior of solitary fibrous tumors of the pleura, we reviewed the literature with particular attention to the clinical presentation, histopathologic characteristics, and cytogenetic differentiation of these tumors. A staging system and an algorithm for the management and follow-up of these patients are proposed.     

Solitary fibrous tumors of the pleura presenting satellite tumors

A 29-year-old man presented with a mass in the left lower lung field on a chest radiograph obtained during a medical checkup. Computed tomography revealed a tumor adjacent to the diaphragm. A sessile tumor measuring 10.5 × 8.5 × 4.5 cm arising from the parietal pleura was resected. The tumor was accompanied by several little tumors on the nearby diaphragm. Pathologically, the major tumor consisted of typical spindle-shaped cells with myxoid degeneration. There was no increased cellularity, cellular pleomorphism, or a high mitotic count. In immunohistochemical studies, the spindle cells showed positive staining for CD34 and were negative for bcl-2. The smaller tumors also consisted of myxoid degeneration. We diagnosed benign solitary fibrous tumor of the pleura with satellite tumors. We must be aware of the possibility of satellite tumors when we treat patients with a benign solitary fibrous tumor.     

Solitary fibrous tumour of the pleura: an ultrastructural and immunohistochemical study.

Solitary fibrous tumour of the pleura is a benign neoplasm differing from diffuse pleural mesothelioma. It was originally thought to be a variant of mesothelioma because it consists of a spindle cell stroma associated with branching tubular structures lined by cuboidal cells. In this study of two cases the tubular structures were lined with ciliated and non-ciliated cuboidal cells. Ultrastructurally most of the non-ciliated cells had the features of type II pneumocytes, while the stromal cells had all the characteristics of fibroblasts. Immunohistochemical staining showed the epithelial cells to be positive for both keratin and carcinoembryonic antigen, whereas the stromal cells were negative. The findings support the theory that these tumours are fibroblastic in origin, and that the biphasic pattern is due to entrapment of non-neoplastic bronchiolar and alveolar epithelial cells.     

Solitary fibrous tumor of the pleura

Solitary fibrous tumor of the pleura is a rare clinical entity. Approximately 600 such cases have been reported in the literature. The purpose of this study was to review the surgical outcome of this disease. This is a retrospective review of seven cases of solitary fibrous tumor of the pleura. There were five women and two men. Their ages ranged from 24 to 65 years. Symptoms included cough, shortness of breath, and chest pain. Diagnostic studies included chest X-ray, computed tomography (CT) scan, bronchoscopy, and CT-guided needle biopsy. All the tumors were pedunculated. The sizes ranged from 5.0 to 11.0 cm. All were resected by thoracotomy. The largest of these tumors weighed 68 g. All patients remain disease free. Follow-up ranged from 2 to 20 years. This is a rare disease. Complete resection offers the best chance for long-term care.     

Solitary fibrous tumours of the pleura

Solitary fibrous tumours of the pleura are rare. They are mesenchymal in origin. Initially, they were described in the pleura, but lately they have been reported in many other sites. Although the majority of these tumours are benign, some of them are malignant. Their unpredictable clinical course is probably related to their histological and morphological characteristics. The benign tumours may remain unproblematic for several years before changing into a malignant form. In order to define more accurately the clinical behaviour, diagnosis, management and outcome of these rare tumours, we reviewed the literature with particular attention to clinical presentation, methods of diagnosis, treatment and outcome. Furthermore, a modified algorithm was proposed for the management of these tumours.     

Solitary fibrous tumor of the pleura

Solitary fibrous tumors of the pleura are an uncommon but increasingly recognized neoplasm derived from mesenchymal cells located in the submesothelial lining of the pleural space. They may be differentiated from mesotheliomas and other pleural tumors due to unique immunohistochemical characteristics. The vast majority of these tumors are benign, and complete surgical resection remains the mainstay of therapy for both the benign and malignant variants. In this review, the clinical presentation, radiologic features, histologic findings, and treatment options for this tumor are described.     

Solitary fibrous tumors of the pleura: clinical characteristics, surgical treatment and outcome

Objective: The aim of this paper is to study clinical characteristics, surgical treatment and outcome of patients with solitary fibrous tumor of the pleura operated in our institutions in a 20-year period. Methods: Clinical records of all patients operated for solitary fibrous tumors of the pleura between 1981 and 2000 were reviewed retrospectively. Tumors were classified as malignant in the presence of at least one of the following criteria: (1) high mitotic activity; (2) high cellularity with crowding and overlapping of nuclei; (3) presence of necrosis; (4) pleomorphism; otherwise they were considered as benign. Results: Sixty patients (mean age 55 years) were operated in this period. None had asbestos exposure. Symptoms were present in 31 cases. Surgical approaches included thoracotomy (n=53), video-assisted thoracoscopy (n=6), and median sternotomy (n=1). Tumors originated from visceral pleura in 48 cases, from parietal, mediastinal or diaphragmatic pleura in seven, two and three cases, respectively; their mean diameter was 8.5 cm. Tumors could be resected with their implantation basis in 49 patients. In the remaining 11, extended resections were performed, including lung parenchyma (lobectomy, n=4, pneumonectomy, n=2), osteomuscular chest wall structures (n=2), diaphragm (n=2), and pericardium (n=1). Two postoperative deaths (due to myocardial infarction and pulmonary embolism, respectively) occurred. Tumors were pathologically benign in 38 cases and malignant in 22 cases. Mean follow-up was 88 months. Resection was complete in all the patients with benign tumors and no recurrence occurred. Resection was considered as complete in 21/22 malignant tumors. Local recurrence was observed in two cases. Both could be successfully managed by iterative exeresis (no extended resection had been initially performed). Metastatic disease (responsible for patient's death) was observed following the only incomplete resection. Actuarial 5- and 10-year survival rates were 97% for benign tumors and 89% for malignant ones. Conclusions: Surgical resection provided cure in all the patients with benign tumors. As insufficiency of exeresis is associated with all recurrences in malignant tumors, completeness of resection is in our experience the best prognostic factor in these forms.     

Solitary fibrous tumour of the pleura: surgical treatment.

OBJECTIVE: Solitary fibrous tumours (SFT) of the pleura are rare tumours originated from the mesenchimal tissue underlying the mesothelial layer of the pleura. This tumours present unpredictable clinical course probably related to their histological and morphological characteristics. METHODS: Twenty-one patients affected by SFT of the pleura were referred to us for surgical resection from September 1984 to April 2000. They were 15 males and six females with median age of 51 (range 15--73) years. Nine patients (43%) were symptomatic and predominant clinical symptoms or signs were dyspnoea (19%), coughing (14.3%), chest pain (28.5%), finger clubbing (14.3%) and hypoglycaemia (14.3%). Hypoglycaemia was related to a pathological incretion of insulin-like growth factor 2 by the tumour. Chest radiograph and computed tomography of the chest revealed intra-thoracic homogeneous sharply delineated round or lobulated mass sometimes associated with ipsilateral pleural effusion (19%) or causing pulmonary atelectasis with opacification of the complete hemithorax (19%). Surgical excision required 14 posterolateral thoracotomies, six anterior thoracotomies and one video-assisted thoracoscopy. Thirteen tumours arose from visceral pleura and wedge resection was performed, seven tumours arose from parietal pleura and extrapleural resection was carried out without any chest-wall resection, one tumour growth within the upper left lobe and required lobectomy. Tumours weighted from 22 to 1942 g and measured from 22x12x8 to 330x280x190 mm. At cut section seven cases (34%) revealed focal necrosis and hemorrhagic zones and on light microscopy six cases (28.5%) were characterized by high mitotic count: characteristics related with uncertain clinical behaviour. Immuno-histochemical reactions were in all cases positive for CD34. RESULTS: In all our patients resections were complete. Paraneoplastic syndromes like hypoglycaemia and clubbing receded after surgery. No intraoperative or perioperative medical or surgical complications occurred. Median chest-drain duration timed 3 (range 2--5) days and median hospital stay was 5 (range 4--7) days. Perioperative mortality rate was 0%. Median follow-up was 68 (range 2--189) months: during this period patients were submitted to chest X-ray with 6-months interval to evaluate possible local recurrence. Only one patient experienced tumour recurrence after 124 months follow-up: the tumour was suspected after observation of finger clubbing. The tumour was detected and excised by redo-thoracotomy. CONCLUSIONS: Surgical resection of benign solitary fibrous tumours is usually curative, but local recurrences can occur years after seemingly adequate surgical treatment. Malignant solitary fibrous tumours generally have a poor prognosis. Clinical follow-up and radiological follow-up are indicated for both benign and malignant solitary fibrous tumours.     

Solitary fibrous tumors of the meninges

Central nervous system solitary fibrous tumors are a new pathological entity. To our knowledge, only 60 meningeal solitary fibrous tumors both in the spinal cord and in the brain have been described in the literature. The 56 previously reported cases of meningeal solitary fibrous tumors are critically reviewed. In addition, we report four new cases of solitary fibrous tumors of the meninges. There is a slight male prepoderance. Meningeal solitary fibrous tumors show a tendency to arise in the posterior fossa (26%) and spine (25%). The treatment was mainly total surgical excision. Radiotherapy was given only to four patients with tumors involving the cerebral parenchyma. Sporadic cases of recurrence and distant metastasis have been reported. The prognosis of meningeal solitary fibrous tumors is still unknown because the follow-up of the reported cases is short. It is probable that cases of solitary fibrous tumors of the meninges have been misdiagnosed as other tumors in the past. The best management of these tumors seems to be total surgical excision whenever possible. It is important that every new case of meningeal SFT be reported to throw light on this particular tumor and to affirm its status as a clinicopathological entity.     

Localized fibrous tumors of the pleura: clinical and surgical evaluation

BACKGROUND: Localized fibrous tumors of the pleura, which are considered to originate from submesothelial connective tissue, are rare. The purpose of this study was to investigate the clinical features of these tumors and to determine the optimal treatment and follow-up. METHODS: The records of 10 consecutive patients with localized fibrous tumors of the pleura operated on at the Ataturk Center for Chest Diseases and Thoracic Surgery between 1995 and 2001 were retrospectively reviewed. Diagnostic procedures, clinical courses, and outcomes of these patients were studied. Total excision through a thoracotomy was performed in all of the patients. Pneumonectomy was required in 1 patient because of a giant intraparenchymal localized fibrous tumor of the pleura. Additional partial chest wall resections were done in 2 patients. RESULTS: The size of excised tumors ranged from 10 x 8 x 4 cm to 30 x 25 x 20 cm. Malignant transformation was seen in 1 patient 22 months after resection of a benign tumor. Four cases were pathologically considered to be malignant. All patients except 1 are alive (follow-up 16 to 57 months after resection). CONCLUSIONS: Although localized fibrous tumors of the pleura are considered histologically benign tumors, because of the risk of recurrence and malignant transformation, complete surgical resection is indicated and long-term follow-up is recommended in all patients.     

Solitary fibrous tumor of the pleura; report of a case

An 81-year-old male appealed against a feeling of dyspnea, and consulted the hospital. The giant tumor discovered in the thorax and it was enlarging gradually for 7 years. The tumor shadow with a diameter of about 15 cm was noted in right lower lung field on the chest X-ray. A definite diagnosis was not obtained by the needle biopsy. The tumor was found to exist between upper lobe and lower lobe and pressed lower lobe at surgery. The tumor was completely excised with partial resection of the collapsed lower lobe. The tumor was 1,050 g in weight and 18 cm in maximum diameter. Pathological examination showed the irregular and plan-like arrangement of the spindle-shape cell. Immunohistochemical study revealed positive findings for bcl-2 and CD34, negative findings for desmin, ketatin, and alpha-actin. The tumor was diagnosed as malignant solitary fibrous tumor of the pleura due to highly atypical nuclear finding with an abundant nuclear fission or histology.     

Solitary fibrous tumour of the pleura: Recurrence presented with severe clinical hypoglycaemia

Aim

To highlight an interesting case report of a rare cause of surgically treated hypoglycemia.

Background

Solitary fibrous tumors of the pleural are rare tumors, accounting for less than 5% of all pleural tumors. Interestingly, they are associated with hypoglycemia due to secretion of insulin growth factor II.

Case report

An 80 year old female who had been operated in the past for solitary fibrous tumor of the pleura in the right pleural cavity, developed severe symptomatic episodes of hypoglycemias. Her diagnostic workup revealed multiple ipsilateral intra-thoracic masses. She had been re-operated for and, indeed, multiple tumors were found and excised.

Results

The histologic identity of these tumors were in accordance with histology of the tumors which had been excised at her first operation.The patient recovered well and the hypoglycemic episodes have been completely subsided thereafter.

Conclusion

Hypoglycemia, which is considered a common though sometime severe symptom, needs a thorough diagnostic workup and when assessing it with an intrathoracic mass, solitary fibrous tumor of the pleural should always be kept in mind, since it is reversible cause.     

Solitary fibrous tumors of the central nervous system: report of five cases with unusual clinicopathological and outcome patterns

Background  

This is a retrospective study of 11 patients harboring a solitary fibrous tumor (SFT) of the central nervous system (CNS), with special emphasis on unusual clinicopathological and outcomes patterns.