Solitary fibroma of the pleura - clinical findings and prognosis

Solitary fibrous tumours of the pleura can arise from either the visceral or the parietal pleura. Female and male subjects are affected equally; solitary tumours of the pleura are found at all ages with a main incidence in the sixth and seventh decades. The current literature describes the origin of both benign and malign tumour cells as being from multipotential submesothelial fibrous cells. The clinical appearance is unspecific and characteristic symptoms are lacking; cough, feeling of pressure in the thoracic chest or dyspnoea are the main leading symptoms and correlate with tumour size. A CT scan of the chest is the diagnostic tool of choice. Frequently, a solitary tumour of the pleura is found incidentally. Paraneoplastic syndromes are described and hypertrophic pulmonary osteoarthropathia (HPO) is the most common in cases of a solitary fibrous tumour of the pleura. The therapy of choice is complete surgical resection. There are no data supporting a neoadjuvant or adjuvant therapy. There are no known risk factors and complete resection is the only factor of prognostic relevance. Patients with a benign solitary fibrous tumour of the pleura have a far better survival compared to those with the malignant variant. In two recent reviews, the 5- and 10-year survival rates are described as being 97 and 90 %, respectively, for benign tumours compared with 89 and 88 % for malignant fibromas.     

A case report of an intracaval extrathoracic solitary fibrous tumour

Solitary fibrous tumours are infrequent neoplasms based in the pleura that are predominantly benign with malignant pathology and behaviour described in 10–36% of cases. Extrathoracic solitary fibrous tumours (ESFTs) have been considered separately to their intrathoracic counterparts and comprise a third of all solitary fibrous tumours. The extrathoracic location was identified as an adverse prognostic factor for local recurrence but not for metastatic disease. So far, there have not been any reports of solitary fibrous tumours demonstrating caval infiltration. We present a case of a benign ESFT infiltrating into the perirenal inferior vena cava. Together with extrauterine leiomyomas, ESFTs should also be considered as a differential diagnosis for the rare benign lesions invading the inferior vena cava.     

Solitary fibrous tumour of prostate: evaluation and management of a rare tumour

Solitary fibrous tumours, which are commonly described as arising from the pleura, are rarely seen in the lower urinary tract. Although malignant changes have been reported, these tumours are usually benign. This case report discusses the radiological features, management strategy and histopathology of a solitary fibrous tumour arising from the prostate.     

An unusual presentation of solitary fibrous tumour of the pleura: right atrium and inferior vena cava compression.

Solitary fibrous tumours of the pleura are rare and usually present with unpredictable clinical signs. We report a 58-year-old patient presenting with signs of cardiac decompensation and external compression of the right atrium and inferior vena cava by a voluminous solitary fibrous tumour arising from the diaphragmatic pleura. Marked clinical improvement was obtained after excision of the mass with complete disappearance of the cardiac signs.     

Metachronous benign solitary fibrous tumours of the pleura (localized “mesotheliomas”): a case report

Solitary fibrous tumours are uncommon pleural tumours that are not related to mesotheliomas. They are typically benign and pedunculated and may grow to massive sizes. Surgical resection is usually curative. Tumour recurrence and metachronous development of multiple tumours are unusual. In this report a patient was treated for 3 benign solitary fibrous tumours of the pleura over 23 years. The authors hypothesize that this represents multifocal tumorigenesis as opposed to local tumour recurrence. The importance of complete surgical excision and lifelong radiographic follow-up are stressed.     

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.     

Thoracoscopic resection for benign solitary fibrous tumor of the parietal pleura

We have experienced thoracoscopic surgery for benign solitary fibrous tumor of the parietal pleura. A 46-year-old woman was admitted to our hospital because of chest abnormal shadow. Under thoracoscopy the tumor that was connected to the parietal pleura with a wide pedicle was completely resected with combined parietal resection of the pleura. Pathological diagnosis was a benign solitary fibrous tumor developed from the connective tissues under the parietal pleura. Thoracoscopic surgery is well indicated for a solitary fibrous tumor and wide excision of the tumor with combined resection of the pleura is important to prevent a local recurrence.     

Pedunculated solitary fibrous tumor of the pleura manifesting as a migrating chest mass: report of a case

We report the case of solitary fibrous tumor of the pleura, which appeared to change its location. A computed tomography (CT) scan done at a previous hospital showed a tumor in the posterior mediastinum, suggesting that it was neurogenic. However, on the initial preoperative CT scan, the tumor seemed to have moved anteriorly, but when contrast material was injected; the tumor appeared in its original position. Video-assisted thoracoscopic surgery (VATS) revealed a pedunculated and free-moving tumor, originating from the visceral pleura. We diagnosed this unusual migrating tumor as a pedunculated solitary fibrous tumor of the pleura.     

Benign solitary fibrous tumour of the thigh: morphological, chromosomal and differential diagnostic aspects

Solitary fibrous tumours (SFTs) are rare and usually benign neoplasms of mesenchymal origin that are often found in the visceral pleura (fibrous pleural tumour, FPT) or other serosal surfaces. They have also been found in soft tissues. We report the case of an SFT localised in the thigh of an 86-year-old woman. The tumour specimen was examined morphologically, immunohistochemically and molecular genetically, using comparative genomic hybridisation (CGH). The latter detects unbalanced chromosomal alterations in human neoplasms by competitive nucleic acid hybridisation and consecutive computer image analysis. The tumour consists of fibroblast-like cells, arranged in a typical "patternless pattern". Immunohistochemically, the tumour stained positively for vimentin, CD34, CD99, and focally for actin and desmin. No reaction occurred with keratin or S100 protein antibodies. CGH detected a single loss on chromosome 13q.     

Malignant solitary fibrous tumor in the pleura.

We present a case of malignant solitary fibrous tumor of the pleura in an asymptomatic 75-year-old man. A needle biopsy specimen revealed a solitary fibrous tumor of the pleura with suspected. The tumor was resected and the final diagnosis was a malignant solitary fibrous tumor. At one-and-a-half years later, the patient has no clinical or radiological evidence of recurrence. The propriety of a needle biopsy for preoperative diagnosis is discussed.     

Three types of spindle cell tumors of the pleura. Fibroma, sarcoma, and sarcomatoid mesothelioma

The clinicopathologic features of 17 fibrous tumors of the pleura are presented. Eight were benign localized fibrous tumors; all of these were negative when stained with antibodies to keratin. Eight were diffuse malignant tumors that demonstrated intense immunohistochemical staining of the spindle cells with antibodies to keratin. One case presented as a histologically malignant spindle cell tumor that was initially localized but recurred subpleurally three times in 12 years. This spindle cell tumor was consistently negative when stained for keratin. We conclude that there are three types of fibrous tumors of the pleura: (a) a localized, histologically benign, keratin-negative spindle cell tumor that might be termed "fibroma," (b) a diffuse, histologically malignant, keratin-positive neoplasm that might appropriately be termed a "sarcomatoid mesothelioma," and (c) a histologically malignant, keratin-negative, spindle cell tumor with the potential to spread under the pleura that might appropriately be termed a "sarcoma." Immunohistochemistry proved to be useful in distinguishing the sarcomatoid mesothelioma from the sarcoma.     

Complete surgical resection of a huge thoracic mass consisting in a localized fibrous tumor simulating a high-grade malignant neoplasm

Solitary fibrous tumour is an infrequent neoplasm generally arising from the parietal and visceral pleura. The diagnosis may be difficult in the presence of a history of malignant disease owing to the different presentations and to radiological findings of evident invasiveness. The authors report the case of a woman with a right giant fibrous solitary tumour of the pleura twenty years after a subcutaneous mastectomy with axillary dissection and radiation therapy for breast cancer. The biopsy diagnosis was consistent with a probable solitary fibrous tumour of the pleura but the discrepancy with the radiological images and the difficult differential diagnosis versus a malignant sarcoma, possibly radio-induced, prompted us to verify the real features of the disease. The patient was submitted to a right anterolateral thoracotomy and partial sternotomy and the giant mass was resected enbloc with the phrenic nerve and diaphragm which proved to be the only structures tightly adhering to the neoplasm. Histological examination confirmed the diagnosis of a solitary fibrous tumour of the pleura. The patient is still alive and disease-free 30 months after the surgical operation. Fibrous solitary tumour is a disease generally characterised by a good prognosis but in particular cases, with unmistakable radiological findings of invasiveness, a precise diagnosis must be obtained in order to choose the most appropriate therapy.     

Uniportal video-assisted thoracic resection of a solitary fibrous tumor of the pleura

We describe the surgical removal of a pedunculated solitary fibrous tumor of the pleura through single-access (uniportal) video-assisted thoracoscopic surgery. At the end of the procedure, no chest drain was inserted and the patient discharged home after an 8-hour observation period. Uniportal video-assisted thoracoscopic surgery can represent a suitable surgical option for small solitary fibrous tumors of the pleura.     

Telomerase activity in human pleural mesothelioma

BACKGROUND: Gradual telomere erosion eventually limits the replicative life span of somatic cells and is regarded as an ultimate tumour suppressor mechanism, eliminating cells that have accumulated genetic alterations. Telomerase, which has been found in over 85% of human cancers, elongates telomeres and may be required for tumorigenesis by the process of immortalisation. Malignant mesothelioma is an incurable malignancy with a poor prognosis. The disease becomes symptomatic decades after exposure to carcinogenic asbestos fibres, suggesting the long term survival of pre-malignant cell clones. This study investigated the presence of telomerase in pleural malignant mesothelioma, which may be the target for future anti-telomerase drugs. METHODS: Telomerase activity was semiquantitatively measured in extracts from 22 primary pleural mesotheliomas, two benign solitary fibrous tumours of the pleura, four mesothelioma cell lines, and six short term mesothelial cell cultures from normal pleura using a non-isotopic dilution assay of the telomeric repeat amplification protocol. RESULTS: Twenty of the 22 primary mesotheliomas (91%) and all tumour derived mesothelioma cell lines were telomerase positive. Different levels of enzyme activity were observed in the tumours of different histological subtypes. Telomerase activity could not be detected in the six normal mesothelial cell cultures or in the two mesotheliomas. Both benign solitary fibrous tumours showed strong telomerase activity. CONCLUSIONS: Telomerase activity is found in a high proportion of mesotheliomas and anti-telomerase drugs might therefore be useful clinically. The results are consistent with the hypothesis that telomerase activity may be a feature of carcinogenesis in mesotheliomas and possibly in many other cancers.     

Solitary fibrous tumour of the kidney

Solitary fibrous tumours are relatively rare neoplasms initially described in the pleura. Such lesions are now reported in many extrathoracic sites. To our knowledge, only 8 cases have been reported in the kidney. All these cases were benign. We report a patient with a solitary fibrous tumour of the kidney with infiltration of the wall of the renal vein. Microscopic examination showed proliferation of spindle cells with a prominent vascular haemangiopericytoma-like pattern. Hypercellular areas were admixed with hyalinized collagenous zones. The neoplastic cells stained for vimentin and CD34. CD31 was negative. Biologically, we did not find any arguments for malignancy: the flow cytometric study showed diploid histograms, and immunostaining for p53 was negative.     

Solitary fibrous tumor of the pleura with associated Doege–Potter syndrome

Doege–Potter syndrome is a paraneoplastic syndrome characterized by tumor-associated hypoglycemia secondary to a solitary fibrous tumor of the pleura. We present a case of an 84-year-old man, who presented with acute mental confusion and therapy-resistant hypoglycemia. Diagnostic imaging revealed a large sharply defined pleural tumor based on the left diaphragm, after surgical resection the diagnosis was made of a malignant solitary fibrous tumor of the pleura and restoration of the glucose homeostasis was observed.     

Clinical behavior of solitary fibrous tumors of the pleura.

BACKGROUND: Solitary fibrous tumors of the pleura are rare and present unpredictable clinical behavior. METHODS: Between 1981 and 1998, 11 solitary fibrous tumors of the pleura were resected in 10 patients at the University Hospital of Geneva. Their clinical behavior and outcome were reviewed. RESULTS: Seven tumors arose from the visceral pleura, and three arose from the parietal pleura. Tumors arising from the parietal pleura were revealed to be more difficult to resect than those from the visceral pleura because of their size and adhesion to the chest wall requiring extrapleural resection. Eight tumors showed benign features, whereas two showed distinct features of malignity. One additional patient presented marked pleomorphism that could represent an intermediate form before frank malignity. Four tumors had been followed expectantly for 2 to 10 years before surgery. Although three enlarged rapidly, no signs of malignity were observed on histological examination. All patients are alive, from 2 months to 14 years after surgery (mean 55 months). In one case, however, a malignant tumor recurred 6 years after resection of a benign variant. CONCLUSIONS: Although histologically benign, solitary fibrous tumors of the pleura may enlarge rapidly and occasionally transform into malignant variants after several years. Therefore, complete surgical resection and long-term follow-up is recommended for all patients.     

Solitary fibrous tumor of the nasal cavity and paranasal sinuses

We report two solitary fibrous tumors of the nasal cavity and paranasal sinuses that were histologically and immunohistochemically virtually identical to solitary fibrous tumors (fibrous mesotheliomas) of the pleura. One tumor arose in a 48-year-old woman and the other in a 45-year-old woman. Both patients presented with nasal symptoms, and both patients are alive without evidence of disease 6 months and 1 year after excision. The tumors had a disorganized or "patternless" arrangement of spindle cells in a collagenous background and prominent vascular channels of varying size. Immunoperoxidase stains on paraffin sections showed staining of the cells for vimentin only; there was no staining for keratin, S-100 protein, desmin, and actin. Both cases presented some degree of diagnostic difficulty and had to be distinguished from other spindle cell tumors of the nasal cavity and paranasal sinuses, such as hemangiopericytoma, angiofibroma, and fibrous histiocytoma.     

Giant solitary fibrous tumor of the pleura

Fibrous tumors of the pleura are rare, accounting for <5% of all pleural neoplasms. Although over 80% of pleural fibrous tumors have a benign course, local recurrence postsurgery and occasional malignant transformation have been reported; complete excision of the tumor together with postsurgery follow-up of all patients is therefore recommended. We report on a solitary fibrous tumor of the pleura measuring 30?cm and weighing 3560?g.     

Solitary fibrous tumor in bladder wall

The solitary fibrous tumor is a rare mesenchymal tumor, occurring preferentially in pleura, which has recently been described in extrathoracic sites. There are 6 reports on primary solitary fibrous tumor of bladder. They affect preferably men with mean age around 57 years, are usually asymptomatic and, despite eventually presenting morphologic features of malignancy, tumor resection is considered curative. We report the seventh case of solitary fibrous tumor in bladder wall, discussing differential diagnoses, and call the attention to this rarely occurring entity, which has benign behavior and should be managed conservatively.