Tumor fibroso pleural ¿son realmente benignos? ¿Cuándo deben operarse? / Pleural fibrous tumor, are they really benign? When should they have operation?

El tumor fibroso pleural es un tumor habitualmente asintomático, benigno y de lento crecimiento, que en un pequeño porcentaje de los casos tiene un comportamiento más agresivo. Para definir los criterios de malignidad es necesario un análisis inmunohistoquímico. El tratamiento de elección es la resección quirúrgica completa con márgenes de seguridad ya sea por toracotomía o por videotoracoscopia en función del tamaño. Se encuentra en estudio la utilización de terapia adyuvante radioterápica o quimioterápica que en el momento actual no cuenta con resultados significativos. Presentamos dos casos que fueron tratados de forma quirúrgica, con extirpación completa siendo el primero definido como maligno según los criterios histológicos y el segundo benigno, pero de alto riesgo. Solo uno de ellos recibió terapia adyuvante. (AU)

Solitary fibrous pleural tumor is a usually asymptomatic, benign and slow-growing tumor, which in a small percentage of cases has a more aggressive behavior. To define the malignancy criteria, an immunohistochemical analysis is necessary. The gold standard treatment is a complete surgical resection with safety margins either by thoracotomy or by videothoracoscopy depending on the size. The use of radiotherapy or chemotherapy as an adjuvant therapy is under study, which at the present time does not have significant results. We present two cases that were treated surgically, with complete removal, one being classified as malignant according to histological criteria and the other benign but high risk. Only one of them received adjuvant therapy. (AU)

Localized malignant mesothelioma, an unusual and poorly characterized neoplasm of serosal origin: best current evidence from the literature and the International Mesothelioma Panel.

Localized malignant mesotheliomas (LMM) is an uncommon and poorly recognized neoplasm. Its pathologic diagnosis is often surprising in patients with serosal/subserosal based localized tumors that are clinically suspicious for metastatic lesions or primary sarcomas. Once a tumor is diagnosed as "mesothelioma", LMM is often mistaken for diffuse malignant mesothelioma (DMM). Best currently available evidence about LMM was collected from the literature and cases diagnosed by members of the International Mesothelioma Panel (IMP). One hundred and one (101) LMM have been reported in the English literature. Patients had localized tumors with identical histopathologic features to DMM. Patients ranged in age from 6 to 82 years; 75% were men. Most (82%) of the tumors were intrathoracic. Others presented as intrahepatic, mesenteric, gastric, pancreatic, umbilical, splenic, and abdominal wall lesions. Tumors varied in size from 0.6 to 15 cm. Most patients underwent surgical resection and/or chemotherapy or radiation therapy. Median survival in a subset of patients was 29 months. Seventy two additional LMM from IMP institutions ranged in age from 28 to 95 years; 58.3% were men. Sixty tumors (83.3%) were intrathoracic, others presented in intraabdominal sites. Tumors varied in size from 1.2 to 19 cm. Median survival for 51 cases was 134 months. Best evidence was used to formulate guidelines for the diagnosis of LMM. It is important to distinguish LMM from DMM as their treatment and prognosis is different. A multidisciplinary approach is needed for the diagnosis of LMM as it shows identical histopathology and immunophenotype to DMM.

Solitary fibrous tumors of the pleura: A single center experience at National Cancer Center, China.

BACKGROUND: This study explored the clinicopathological features, predictive factors of malignancy, effectiveness of video assisted thoracic surgery (VATS), and prognosis of solitary fibrous tumor of the pleura (SFTP). METHODS: A single-center retrospective study of the data of 82 patients with SFTP who were surgically treated in our department between January 2003 and December 2015 was conducted. RESULTS: A total of 82 SFTPs (70 benign, 12 malignant) were included and all patients underwent complete en bloc resection. SFTPs originated from the visceral pleura in 47 (57%) and the parietal pleura in 35 (43%) patients. In our cohort, malignant tumors were often symptomatically large, and the patients with malignant SFTPs (mSFTPs) often had a family history of neoplasms. Patients in the VATS group (n = 22) had tumors with significantly smaller diameters, required a shorter surgical duration and shorter hospital stay, and experienced less intraoperative blood loss and less postoperative chest tube drainage compared to the thoracotomy group (n = 60). No tumor recurrence was found in benign SFTP (bSFTP) patients. The long term survival and disease-free survival rates of mSFTP patients were 76% and 53%, respectively. CONCLUSION: Larger tumor diameter and a family history of neoplasm may be predictive factors for mSFTP; however, this conclusion needs to be verified in large cohort. VATS is safe and reliable for treating selected SFTP patients. Local recurrence is associated with mSFTP patient death, thus close follow-up of such patients is crucial.

Solitary fibrous tumour of the pleura presenting as a giant intrathoracic mass.

Solitary fibrous tumours (SFTs) are relatively rare neoplasms thought to originate from the submesothelial connective tissue. SFTs have been described in a variety of sites, including the pleura, orbit, lower respiratory tract, peritoneal cavity and heart. These neoplasms are usually benign, though locally aggressive, and metastatic behaviour has been observed in some cases. We describe a case of a 61-year-old man presenting with weight loss, poor appetite, malaise, worsening dyspnoea on exertion and lower extremity oedema, who was found to have a gigantic-21×21 cm-tumour occupying the entire right hemithorax causing compression and displacement of the mediastinum and liver. Transthoracic CT-guided biopsy revealed SFT of the pleura. The patient underwent preoperative angiography and embolisation of the tumour followed by successful surgical resection via thoracotomy.

Embolic Brain Infarcts: A Rare Fatal Complication of Preoperative Embolization of a Massive Solitary Fibrous Tumor of the Pleura.

Solitary fibrous tumor of the pleura (SFTP) is a rare intrathoracic neoplasm, often giant in size and highly vascular, which can make surgical resection very challenging. Preoperative percutaneous embolization before surgical removal can significantly reduce the risk of uncontrollable intraoperative hemorrhage. However, a rare potential life threatening complication could result from embolization of SFTP and must be taken into consideration. This report describes a 69-year-old female with a large right thoracic SFTP, who underwent preoperative angiography and embolization and developed diffuse embolic brain infarcts immediately after the administration of polyvinyl alcohol particles.

Assessing the multimodal management of advanced solitary fibrous tumors of the pleura in a routine practice setting.

BACKGROUND: Solitary fibrous tumors of the pleura (SFTP) refer as to a heterogeneous group of mesenchymal malignancies with various anatomic and histology features. Upfront surgical resection is the standard approach, but recurrences may be aggressive and difficult to treat. The most widely accepted staging system has been proposed by De Perrot et al. Because SFTPs are rare, evidence to support a role for perioperative chemotherapy is scarce. Likewise, the predictive or prognostic relevance of the De Perrot system may be questioned. METHODS: Multicenter retrospective study of patients with histologically proven SFTP with complete follow-up from surgical diagnostic to tumor recurrence and death. RESULTS: Sixty-eight patients were included. Tumor stage was 0/I for 29 (43%) patients, II for 23 (34%) patients, III for seven (10%) patients, and IV for nine (13%) patients. Postoperative chemotherapy was given to seven patients, mostly with stage III/IV SFTP, mostly consisting of doxorubicin-based regimen. Recurrence rate and median relapse-free survival after surgery were 3%, 52%, 71%, and 80% (p < 0.001), and 107, 70, 29, 11 months (p < 0.001) for stage 0/I, II, III, and IV tumors, respectively. At time of tumor recurrence, 14 patients received exclusive chemotherapy. Highest disease control rates were observed with trabectedin, and gemcitabine-dacarbazine combination. CONCLUSION: Our study confirms the prognostic value of the De Perrot staging system, as well as its possible predictive value for perioperative chemotherapy decision-making, whereas the efficacy of currently available regimens to significantly reduce the risk of tumor recurrence remains questionable. Trabectedin may be of interest for recurrent tumors.

Intraorbital metastasis from solitary fibrous tumor.

Solitary fibrous tumor (SFT) is a rare spindle cell tumor of mesenchymal origin that usually arises from pleura or pericardium but can also arise from many extraserosal sites. Although more than 50 cases of primary SFT of the orbit have been reported, there are no reports to date of a malignant nonophthalmic SFT metastasizing in the orbital soft tissues (although sphenoid wing bony involvement has been reported). The authors report here the first case of a patient with intraorbital metastasis of a CD34-positive malignant SFT. The patient was a 57-year-old man with a history of malignant pleural SFT and a prior kidney metastasis. He presented with the rapid appearance of proptosis and massive conjunctival chemosis preventing eyelid closure, and he was found to have a well-circumscribed metastasis to his lateral rectus muscle. Surgical excision cured his ocular symptoms, although he died 3 months later from brain and widespread metastases.

[Solitary fibrous tumour of the pleura: about five cases]. / La tumeur fibreuse solitaire pleurale : à propos de cinq observations.

BACKGROUND: Pleural solitary fibrous tumour (PSFT) is a rare, usually benign tumour, with unpredictable behaviour. PATIENTS AND METHODS: Five cases of PSFT were diagnosed in our department over a 12-year period from January 1999 to December 2010. Clinical, radiological, histological, therapeutic and follow-up information were provided in all cases. RESULTS: Our series comprised four men and one woman. The mean age of the patients at presentation was 55 years. All patients were symptomatic. Radiologic investigations showed a pleural lesion with a mean size of 10.6cm ranging from 3 to 17cm. Histologic diagnosis was made from resected parietal pleura in three cases and visceral pleura in two cases. The histologic features were suggestive of malignancy in two cases and benign in three cases. Immunohistochemical study showed that the tumour cells expressed vimentin, CD34, CD99 and Bcl2. Complete resection was obtained in all patients. The evolution was marked in the two patients with malignant PSFT by the recurrence of the tumour after 6 and 21 months respectively. Both died from the condition. The three patients with a benign form are disease-free after 3, 11 and 2 and half years of follow-up. CONCLUSIONS: PSFT is rare tumour, the diagnosis of which is based on histologic investigations. These tumours require long-term monitoring due to the possibility of local recurrence and malignant transformation.

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.

Giant solitary fibrous tumor of the pleura: an analysis of five patients.

BACKGROUND: Solitary fibrous tumor of the pleura (SFTP) represents a clinical entity rarely encountered, especially in giant forms. Complete surgical resection for giant tumor of pleura is a challenge. The aim of this article is to present five new cases of giant SFTP, and to discuss their clinical characteristics and the treatment strategy of such neoplasms. METHODS: We performed a retrospective review of the clinical records of five patients who underwent surgery for a huge SFTP (>18 cm in diameter) between 2007 and 2009. RESULTS: Four patients were symptomatic. All five patients underwent angiography and embolization of the tumor-supplying vessels within 24 h of surgery. All giant tumors were removed completely by extended postlateral thoracotomy with moderate intraoperative bleeding. Two wedge resections and one lobectomy were performed in three cases where the parenchyma had been encroached. Tumors in three patients were pathologically benign; those in the other two were malignant. The symptoms disappeared in all cases after surgery. CONCLUSIONS: Complete resection remains the mainstay of cure for giant SFTP. We recommend preoperative angiography and embolization for giant SFTP which can reduce the risk of hemorrhage and can contribute to piecemeal removal for radical excision.

Imatinib inhibits in vitro proliferation of cells derived from a pleural solitary fibrous tumor expressing platelet-derived growth factor receptor-beta.

We examined the in vitro effects of imatinib (Novartis Pharma AG, Basel, Switzerland) as a possible inhibitor of PDGFR pathway on cells derived from a recurrence of a pleural malignant solitary fibrous tumor (SFT). Primary cell culture was characterised by immunofluorescence. SFT-derived cells were treated with imatinib at different time points. Western blotting for PDGFR-beta, phospho-PDGFR-beta or smooth muscle actin (SMA) was performed before and after 96 h of treatment with imatinib. SFT-derived cells treated with imatinib for 96 h showed a dose dependent decrease of Ki67 expression. Results were confirmed by growth curve. Western blotting showed that PDGFR-beta was highly expressed and phosphorylated in SFT-derived cells and imatinib treatment reduced PDGFR-beta phosphorylation and SMA expression. With the limit of experimental findings, our results support a possible future application of imatinib as a candidate molecule in the target therapy of malignant SFTs over-expressing wild-type PDGFR.

Solitary fibrous tumor of the pleura with a minute malignant component and diaphragmatic vascular supply: report of a case.

We resected a fist-sized, solitary fibrous tumor of the pleura (SFTP) with a minute malignant component, following percutaneous embolization of its feeding artery. The tumor had macroscopic characteristics of a benign SFTP, and most parts of it were microscopically benign. However, further careful pathological examination revealed a minute malignant component in its periphery. We report this case to show that large and mostly benign SFTPs may contain malignant components, which can be overlooked. Thus, large SFTPs should be resected in consideration of this possibility.