Solitary fibrous tumours of the pleura: do we need a different perspective on malignancy?

OBJECTIVES: Solitary fibrous tumours of the pleura (SFTP) are historically considered to be benign soft tissue neoplasms. However, a clinical relevant number of these neoplasms have malignant histological features. The objective of this study was to evaluate the percentage of SFTP presenting unfavourable clinical behaviour in order to predict negative long-term outcome. METHODS: A retrospective review of 74 patients treated at 4 hospitals between 1990 and 2013 was performed. The median follow-up was 10 years (range: 1-20 years). Risk of tumour recurrence and metastases (unfavourable clinical behaviour) with regard to histology using the Kaplan-Meier and Cox proportional hazards methods. RESULTS: The mean age was 61 years (SD 12.75 years). There were 31 male patients (58%) and 43 female patients (42%). Tumour size ranged from 1 to 30 cm (mean 9.09 cm; SD 6.22 cm). Complete resection (R0) was achieved by minimally invasive thoracoscopic resection in 29% and thoracotomy in 57%; 25% of SFTPs showed histological evidence of malignancy, according to England criteria. Recurrence occurred in 21% and 10% of patients had metastases; 83% of patients with metastases and 39% of patients with recurrence died within 5 years. The median recurrence-free survival for histologically benign SFTP was not reached, compared to 8 years for malignant SFTP. The five-year overall survival rate was 84%. Mitotic rate ≥1/10 HPF, high cellularity, nuclear atypia, Ki-67 level >5% and poorly circumscribed (sessile) growth pattern were associated with poor long-term outcome. CONCLUSIONS: Pathological differentiation of SFTP morphology into pedunculated, well circumscribed and poorly circumscribed (sessile) growth pattern is recommended. Due to the misleading classification into histologically benign and malignant, all unpedunculated SFTP should be classified as potentially aggressive. Lifelong follow-up is mandatory.

Solitary pleural fibroma causing IGF-2-mediated hypoglycaemia in a non-diabetic patient.

A patient without a diagnosis of diabetes mellitus presented to the hospital due to a fall and hypoglycaemia on admission. The patient was found to have recurrent nocturnal fasting hypoglycaemia. CT revealed a large lung mass consistent with a solitary pleural fibroma, a rare tumour associated with insulin-like growth factor 2 (IGF-2) production. This case is an important reminder that potential causes of hypoglycaemia should be considered in non-diabetic patients.

Anesthetic management of thoracotomy for massive intrathoracic solitary fibrous tumor of the pleura: a case report.

BACKGROUNDS: Solitary fibrous tumor of the pleura (SFTP) is a rare thoracic tumor and usually asymptomatic. Massive SFTP may affect adjacent organs and tissues including pulmonary vasculature, bronchus and heart. A thoracotomy for massive SFTP is necessary in severe case. Therefore, it is important for anesthesiologists to understand the condition of patients with massive SFTP and develop an appropriate anesthetic management strategy. A 76-year-old woman with massive SFTP presented to our clinical center and was evaluated as requiring thoracotomy. She received multidisciplinary cooperation treatment from the radiology, cardiac, thoracic surgery and anesthetic teams. The perioperative management of anesthesiologists played a crucial role in the great prognosis of this woman. CONCLUSIONS: This case report demonstrates the importance of comprehensive and meticulous perioperative management and provides guidance to the multidisciplinary team on the potential risk and the rational treatment strategy of patients with massive SFTP during the perioperative period.

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)

Our experience with solitary fibrous tumors in the chest area.

The article reports on three patients with a solitary fibrous tumor of the chest. The first patient had a tumor in the area of the dome of the right pleural cavity which was radically resected together with the chest wall around its origin. In the second case, the tumor was attached by a vascular pedicle to the lower lobe of the right lung. This tumor was resected atypically, via thoracotomy, along with a margin of healthy lung tissue at the base of its pedicle. The last patient had a tumor of the lower lobe of the right lung, surrounding the lower pulmonary vein, which did not have a clear margin of healthy lung tissue. This finding required right lower lobectomy via posterolateral thoracotomy. The presented cases describe rare types of tumors in the chest area which at the time of detection often reach large dimensions, necessitating extensive surgical procedures. Due to the biological nature of these tumors, long-term patient follow-up is advisable.

[Consideration of Surgical Procedure for Repeated Pulmonary Metastases of Solitary Fibrous Tumor].

We report a case of a solitary fibrous tumor with repeated lung metastases after resection of the primary tumor. The patient was a 58-year-old man who had a left upper lobe lung tumor resected in 2018. The tumor was a solitary fibrous tumor arising from the visceral pleura. During the subsequent follow-up, the tumor repeatedly metastasized into the lung, and a total of three surgeries were performed. From the specimen at third surgery, the possibility of the enucleation of the tumor was speculated from the macroscopic and microscopic findings. Since repeated resections of the lung may cause the gradual deterioration of pulmonary function, the possibility of tumor enucleation will be discussed for the future treatment plan.

[Solitary Fibrous Tumor of Pleura Communicating with Pulmonary Artery and Vein].

A 68-year-old man was noted to have an abnormal shadow on chest X-ray. Chest computed tomography (CT) showed a 100 mm mass in the lower right thoracic cavity. The mass was lobulated and compressed the surrounding lung tissue and diaphragm. Contrast-enhanced CT showed that the mass was heterogeneously enhanced and contained expanded blood vessels within it. The expanded vessels communicated with the pulmonary artery and vein via the diaphragmatic surface of the right lung. The mass was diagnosed as a solitary fibrous tumor of the pleura (SFTP) by CT-guided lung biopsy. We performed partial resection of the lung including the tumor via right eighth intercostal lateral thoracotomy. Intraoperative examination showed that the tumor was pediculated from the diaphragmatic surface of the right lung. The stem was about 3 cm long and easily cut with a stapler. The tumor was definitively diagnosed as a malignant SFTP. No recurrence was found for 12 months postoperatively.

Resection of giant malignant solitary fibrous pleural tumor after interventional embolization: a case report and literature review.

BACKGROUND: Solitary fibrous tumor of the pleura (SFTP) is a rare mesenchymal tumor that arises at various sites and typically originates from the pleura. Most patients with SFTPs are asymptomatic, unless the tumor is large. Approximately 20% of SFTP cases are malignant. There are few reports on imaging diagnoses and interventional treatments of SFTP. Here, we report a case of a giant SFTP that exhibited malignant behavior and underwent successful resection after embolization of the main supply artery of the tumor. CASE PRESENTATION: We report a clinical case of a giant SFTP in a 66-year-old Chinese female patient complaining of chest tightness and cough for more than 2 months. Ten years ago, the patient had undergone a chest CT scan at a local hospital for cough. Computed tomography (CT) had revealed a mass in the right thoracic region, which was misdiagnosed as a pulmonary abscess by CT-guided biopsy. Therefore, the patient did not receive appropriate/complete treatment at that time. She was hospitalized again, because CT showed significant enlargement of the right thoracic mass, which caused her obvious symptoms of discomfort. The pathological results of CT-guided biopsy at our hospital confirmed SFTP. Considering the large size of the tumor and the rich blood supply, some of the main blood vessels were treated with embolization before surgical resection. A large tumor, about 23 cm × 16 cm × 15 cm in size, was then successfully removed by thoracic surgery. The diagnosis of malignant SFTP was confirmed by surgical pathology and immunohistochemistry. CONCLUSION: Imaging findings of SFTPs are not characteristic, especially when a tumor is large, the diagnosis is difficult, and the final diagnosis still depends on histological and immunohistochemical examinations. The two-stage surgical treatment described here, which involves first embolization of the main supplying artery of the large tumor and then complete surgical resection, is effective and safe for SFTPs. Whether needle biopsy or vascular embolization is performed, intervention plays a crucial role in the diagnosis and treatment of patients with SFTPs.

Malignant solitary fibrous tumor of the pleura.

BACKGROUND: Solitary fibrous tumors of the pleura are rare diseases of the thoracic cavity. They frequently grow unnoticed until they exert compressive effects on adjacent organs. Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease. CASE PRESENTATION: We present a rare case of a 76-year-old female patient with no previous pulmonary history who presented with progressive dyspnea, fatigue, and involuntary weight loss. On chest X-ray and computed chest tomography scan, she was found to have a 16.7 cm × 12.8 cm × 10.1 cm bulky mass occupying the left hemithorax with associated compressive atelectasis of the lung. She underwent a computed tomography guided biopsy that revealed the mass to be a solitary fibrous tumor. The patient underwent left muscle sparing lateral thoracotomy with complete resection of the tumor. Post procedure, the left lung fully expanded. 18 months post-resection, she developed a 3.3 cm × 1.7 cm tumor along the left internal thoracic artery lymph node chain which was histologically identical to the resected tumor. The patient is currently being treated with bevacizumab and temozolomide. CONCLUSION: Solitary fibrous tumors are very rare pleural tumors. Surgical resection is the treatment of choice followed by close post-operative surveillance.

Critical Limb Ischemia as a Rare Presentation of Malignant Solitary Fibrous Tumor of the Pleura.

BACKGROUND Solitary fibrous tumor (SFT) of the pleura is a rare fibroblastic neoplasm. It is commonly found incidentally on imaging and is usually benign but has significant potential to recur as a malignant tumor. Patients present asymptomatically or with pulmonary symptoms such as cough or shortness of breath. Cardiac invasion of an SFT can create an avenue for peripheral tumor embolization and critical limb ischemia, as in this case report. There is no prior published report of recurring malignant SFT presenting as critical limb ischemia. CASE REPORT We report a rare presentation of malignant SFT recurrence in a 57-year-old woman with critical limb ischemia of both lower extremities secondary to bilateral tumor emboli. The patient's primary tumor was treated with surgical resection alone. Upon recurrence, the tumor growth was so extensive that it was no longer amenable to surgical resection at the time of her critical limb ischemia. The patient presented with bilateral numbness and tingling, without any pulmonary symptoms. CONCLUSIONS Although it is sporadic, clinicians should know that an aggressive malignant SFT can embolize and present as critical limb ischemia. The possibility of tumor emboli provides a pressing reason to surgically resect SFT masses in their early stages before any cardiac invasion.

Solitary fibrous tumour of the pleura: an incidental finding.

We herein report a relatively rare case of a woman in her 30s with an incidental lung finding. She subsequently underwent resection for what was considered to be a solitary fibrous tumour of the pleura (SFTP). SFTPs are rare, slow-growing neoplasms from mesenchymal origin. These tumours are histologically characterised by proliferation of bland-looking spindled cells, arranged in a patternless pattern, with hypocellular and hypercellular areas set in a hyalinised stroma. Complete en bloc surgical excision (with margin negativity) is the treatment of choice for both benign and malignant SFTPs. Due to the possibility of local recurrence with benign and malignant SFTPs, clinical and radiological follow-up is recommended.

Tumor fibroso solitario gigante de pleura / Giant solitary fibrous tumor of the Pleura

Resumen Objetivo: Reportar el caso de una masa gigante en hemitórax izquierdo de 19 cm de diámetro en un paciente de 59 años que debutó con disnea, tos y dolor torácico, confirmándose por estudio imagenológico. Materiales y Método: Registro clínico de un paciente al cual se le diagnostica tumor fibroso solitario de pleura, siendo intervenido quirúrgicamente para exéresis de la lesión. Resultados: Se realiza toracotomía posterolateral izquierda para exéresis de tumor gigante, requiriendo además, resección de diafragma y pericardiectomía parcial con evolución favorable. Discusión: El tumor fibroso solitario es una neoplasia rara derivada del mesénquima que afecta más comúnmente a la pleura, típicamente bien circunscrita, pediculada, con vasos dentro del pedículo tumoral, pudiendo llegar a ser de gran tamaño, siendo considerados gigantes cuando tienen más de 15 cm de diámetro. Conclusión: El diagnóstico correcto es de vital importancia, ya que con la resección quirúrgica es potencialmente curable. El tratamiento quirúrgico puede efectuarse por toracotomía o videotoracoscopia, dependiendo del tamaño del tumor. A pesar del comportamiento benigno, requiere seguimiento a largo plazo debido a la tendencia a la recidiva.

Aim: To report the case of a 19 cm diameter giant mass in the left hemithorax in a 59-year-old patient who presented with dyspnea, cough and chest pain, confirmed by imaging study. Materials and Method: Clinical record of a patient who was diagnosed with a solitary fibrous tumor of the pleura, undergoing surgery to excise the lesion. Results: A left posterolateral thoracotomy was performed to excise the giant tumor, also requiring resection of the diaphragm and partial pericardiectomy with favorable evolution. Discussion: The solitary fibrous tumor is a rare neoplasm derived from the mesenchyme that most commonly affects the pleura, typically well circumscribed, pedunculated, with vessels within the tumor pedicle, and can become large, being considered giant when they are larger than 15 cm diameter. Conclusión: The correct diagnosis is of vital importance, since surgical resection it a potentially curable treatment. Surgical treatment can be performed by thoracotomy or videothoracoscopy, depending on the size of the tumor. Despite the benign behavior, it requires long-term follow-up due to the tendency to recur.

[Monstrous Solitary Fibrous Tumor of the Pleura - A Case Report and Review of the Literature]. / Monströser solitärer fibröser Tumor der Pleura.

Localized solitary fibrous tumors are a rare entity and are mostly found in the thorax, but can also occur in the liver, skin, meninges, peritoneum, and pericardium. Making the diagnosis is often difficult because of the various micromorphologic outlooks. The histopathological assessment with differentiated immunohistochemistry is decisive. Surgical resection of the localized solitary fibrous tumors is the therapy of choice. The recurrence and metastasis rates depend on the histological dignity and are in total very low. Therefore, continuous follow-up examinations are indicated. We report on a 76-year-old female patient with a monstrous solitary fibrous tumor of the pleura who complained of exertional dyspnea and sharp pain in the right thoracic region for several weeks. Computed tomography showed a massive, heterogeneous pulmonary mass 22 cm in diameter in the middle and lower field of the right lung with compression of the diaphragm and mediastinum. The tumor was completely resected through a double thoracotomy in the 5th and 8th ICR. Intraoperatively, the tumor was stalked to the middle lobe. In order to completely remove the tumor, a wedge resection was also performed from the middle lobe. The tumor weighed 2.4 kg. The diagnosis of a solitary fibrous tumor of the pleura was made histologically and immunohistochemically. Postoperatively, the lung has fully expanded. There was no evidence of a relapse at the 3-month follow-up examination. The clinical symptoms, diagnosis, treatment options, risk of recurrence and the prognosis of these tumors are shown and discussed in accordance with the literature.

[Giant Solitary Fibrous Tumour of the Pleura Compressing the Left Ventricle:Report of a Case].

A 71-year-old woman with chest pain and exertional dyspnea was referred to our hospital. Computed tomography revealed a huge intrathoracic tumor with left parietal pleural dissemination. Transthoracic echocardiography showed the left ventricular dysfunction due to external compression by the tumor. After excision of the tumor, marked improvement of the left ventricular dysfunction was obtained for eight years. But the patient died due to cardiac invasion of the tumor 9th year after surgery.

Massive intrathoracic solitary fibrous tumor of the right hemithorax.

Solitary fibrous tumors of the pleura (SFTP) are rare mesenchymal tumors that arise from visceral or parietal tissue. Surgical resection of massive SFTP can be complicated by airway collapse, vascular compression/hemodynamic instability, and hemorrhage. Patients with SFTP may also present with metabolic derangements secondary to paraneoplastic processes. We present a case of successful removal of massive right-sided SFTP via clamshell sternotomy and discuss the perioperative considerations for which providers should be familiar.