Evolution of Pleural Solitary Fibrous Tumors Causing Severe Hypoglycemia after Exceptionally Long Asymptomatic Periods: Report of Two Surgical Cases.

Non-islet cell tumor hypoglycemia (NICTH) is one of the paraneoplastic syndromes manifesting severe hypoglycemia caused by aberrant production of high-molecular-weight insulin-like growth factor 2 (big-IGF2). Two surgical cases of extremely large thoracic solitary fibrous tumors (SFT) with unusual history of NICTH are presented. One case manifested severe hypoglycemia after four years of the first complete surgical resection of the tumor with potential malignant transformation, and the other case showed severe hypoglycemia after ten years of the first detection of the tumor. Meticulous laboratory testing, including serum endocrinological tests and western immunoblotting before and after surgery was performed, and both cases were diagnosed as NICTH. Both patients underwent open thoracic surgery. The patients showed normal glucose and hormone levels immediately after the resection of responsible tumors with elevated blood insulin concentration. SFTs are generally considered benign; however, life-threatening hypoglycemia can happen regardless of treatment. Careful follow-up of the tumor growth is warranted.

[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.

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.

[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.

Extrameningeal solitary fibrous tumors-surgery alone or surgery plus perioperative radiotherapy: A retrospective study from the global solitary fibrous tumor initiative in collaboration with the Sarcoma Patients EuroNet.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare mesenchymal malignancy. Although surgery is potentially curative, the local relapse risk is high after marginal resections. Given the lack of prospective clinical trial data, the objective of the current study was to better define the role of perioperative radiotherapy (RT) in various SFT presentations by location. METHODS: This was retrospective study performed across 7 sarcoma centers. Clinical information was retrieved from all adult patients with extrameningeal, primary, localized SFT who were treated between 1990 and 2018 with surgery alone (S) compared with those who also received perioperative RT (S+RT). Differences in treatment characteristics between subgroups were tested using analysis of variance statistics and propensity score matching. Local control and overall survival rates were calculated from the start of treatment until progression or death from any cause. RESULTS: Of all 549 patients, 428 (78%) underwent S, and 121 (22%) underwent S+RT. The median follow-up was 52 months. After correction for mitotic count and surgical margins, S+RT was significantly associated with a lower risk of local progression (hazard ratio, 0.19: P = .029), an observation further confirmed by propensity score matching (P = .012); however, this association did not translate into an overall survival benefit. CONCLUSIONS: The results from this retrospective study investigating perioperative RT in patients with primary extrameningeal SFT suggest that combining RT with surgery in the management of this patient population is significantly associated with a reduced risk of local failures, especially in patients who have less favorable resection margins and in those who have tumors with a high mitotic count.

Pleural Solitary Fibrous Tumors-A Retrospective Study on 45 Patients.

Introduction: The purpose of this paper is to study the type, the clinical presentation, and the best diagnostic methods for pleural solitary fibrous tumors (PSFTs), as well as to evaluate which is the most appropriate treatment, especially as PSFTs represent a rare occurrence in the thoracic pathology. Material and Method: A retrospective study was conducted on a group of 45 patients submitted to surgery between January 2015 and December 2019. In most cases, the diagnosis was established through imaging studies-thoracic computed tomography (CT) scan with or without contrast-but also using magnetic resonance imaging (MRI) or positron emission tomography (PET) scans when data from CT scans were scarce. All patients were submitted to surgery with curative intent. Results: Most patients included in this study were asymptomatic, with this pathology being more common in patients over 60 years of age, and more common in women. The occurrence of malignant PSFT in our study was 17.77% (8 cases). All cases were submitted to surgery with curative intent, with a single case developing further recurrence. In order to achieve complete resection en bloc resection of the tumor with the chest wall, resection was performed in two cases, while lower lobectomy, pneumectomy, and hemidiaphragm resection, respectively, were needed in each case. Postoperative mortality was null. Conclusion: Thoracic CT scan remains the most important imagistic investigation in diagnosing. MRI is superior to thoracic CT, especially in cases that involved the larger blood vessels within the thorax, spinal column, or diaphragm. Complete surgical resection is the gold standard in treatment of PSFT, and the prognosis in benign cases is very good.

Solitary fibrous tumor of the pleura as a rare cause of severe hypoglycemia: Doege-Potter syndrome.

INTRODUCTION: Doege-Potter syndrome is a rare syndrome characterized by hypo-insulinemic hypoglycemia. It is caused by excessive ectopic secretion of insulin-like growth factor II from a solitary fibrous tumors of intrapleural or extrapleural origin. Laboratory tests reveal low levels of C-peptide and insulin, on the contrary insulin-like growth factor II level is elevated, which is characteristic for Doege-Potter syndrome. Majority of solitary fibrous tumors present no symptomatology, recurrent hypoglycemia is relatively rare, but it may be the only clinical manifestation. The therapy is surgical, consisting of radical en-bloc tumor resection. CASE REPORT: Authors present a case report of a patient with recurrent hypoglycemia caused solely by solitary fibrous tumor. Hypoglycemia resolved immediately after surgical resection and there were no recurrences. CONCLUSION: Doege-Potter syndrome should be considered as the differential diagnosis in a patient with suspicion on thoracic malignancy if accompanied by features suggestive of hypoglycemia. Prolonged follow up is strongly advised because of the risk of disease recurrence, even in patients with benign solitary fibrous tumors of the pleura (SFTP).

[Solitary fibrous tumor of pleura (symptoms, diagnosis, treatment)]. / Solitarnaya fibroznaya opukhol' plevry (klinika, diagnostika, lechenie).

OBJECTIVE: Optimization of diagnosis and treatment of patients with solitary fibrous tumor of pleura, analysis of overall survival and disease-free survival, predictors of recurrence. MATERIAL AND METHODS: There were 66 patients with solitary fibrous tumor of pleura (26 men and 40 women) aged 57.6 years (range 26-80 years). Asymptomatic course was found in 29 (44%) patients, various symptoms - in 37 (56%) patients. Thoracotomy was applied in 36 patients, thoracoscopy - in 30 patients. Immunohistochemical examination included analysis of definition of Stat6 expression. RESULTS: Benign variant of SFT was diagnosed in 50 (75.7%) patients, malignant variant - in 16 (24.3%) patients. STAT6 expression was observed in all cases. Postoperative morbidity was 9%, mortality - 1.6%. Recurrence was diagnosed in 2 (4%) patients with benign variant of disease and in 5 (31.2%) patients with malignant variant (2 of them died from progression of disease). Progression-free survival was 89.4%, overall survival - 95.4%. Predictors of recurrence are tumor dimension over 10 cm, necrosis and/or hemorrhagic component of tumor, mitotic count of at least four per 10 high-power fields. CONCLUSION. S: Olitary fibrous tumor of pleura is a rare mesenchymal fibroblastic neoplasm growing from submesothelial layer. Differential and preoperative morphological diagnosis of SFT is difficult and demands a special immunohistochemical examination with analysis of Stat 6 expression. Surgery is preferred for tumor de novo and recurrent neoplasm.

[Reexpansion Pulmonary Edema and Atrial Fibrillation after Resection of a Giant Solitary Fibrous Tumor of the Pleura].

We report a case of giant solitary fibrous tumor( SFT) of the pleura postoperatively complicated with probable reexpansion pulmonary edema and atrial fibrillation. An 85-year-old woman was diagnosed to have a 13 cm sized intrathoracic neoplasm. Upon thoracotomy, the tumor was found to pedunculate from the right lung with no direct invasion to the surrounding structures. Complete resection of the tumor and expansion of the right lung was obtained. Histopathology revealed the tumor was a benign SFT arising from the visceral pleura. An infiltrative shadow in the right lower lung field soon after the surgery suggested a reexpansion pulmonary edema, which eventually recovered within a week. The patient suffered from a refractory atrial fibrillation that led to a congestive heart failure requiring an intensive medical treatment. It is emphasized that thoracic surgeons should be aware of these postoperative complications in treating such a case like this.

Surgical outcomes and clinical courses of solitary fibrous tumors of pleura.

AIM: This study's aim is to define the clinicopathological and surgical outcomes, clinical courses, and results of long-term follow-up of cases that underwent surgical treatment for solitary fibrous tumors of the pleura (SFTp). SUBJECTS AND METHODS: Clinical and long-term follow-up records of 16 consecutive patients who had surgery for SFTp between 2006 and 2016 were reviewed in the retrospective chart review. There were nine males (56%) and seven (44%) females with an average age of 60.5 ± 17.1 (range 30-87 years). Etiological factors, diagnostic procedures, clinical and surgical outcomes, 5-year overall survival (OS), and disease-free survival (DFS) in these patients were researched. RESULTS: There was no remarkable common etiological factor. Nine of the cases were asymptomatic. Other symptoms were chest pain, dyspnea, cough and hypertrophic osteoarthropathy, respectively. Thoracotomy for the removal of pleural mass was carried out in 15 (94%) cases. Additional resection procedures included the chest wall resection in two cases and lobectomy in two. Video-assisted thoracoscopic surgery (VATS) resection was performed in one (6%) case. Complete surgical excision was performed in 74% of cases. Nineteen percent of cases were malignant SFTp (mSFTp). One of the mSFTp cases died in the 19th month after the diagnosis. The mean follow-up time was 50.6 ± 34.2 months (2--114 months). Mean survival of mSFTp patients was 40.6 ± 19.08 months (19--55 months) and that for benign SFTp (bSFTp) was 52.9 ± 37.05 months (2--114 months). Five-year OS--DFS were 93.5% and 74%, respectively. CONCLUSION: SFTp is an uncommon benign neoplasm but it can have malignant features. Even in the case of recurrence, the main treatment is total surgical excision. Oncologic treatments can be tried in unresectable and metastatic cases. VATS can be used in surgical total excision for small diameter and appropriate tumors. Understanding the nature of these tumors, immunohistochemical, and genetic studies may be a guide in future.

[Doege-Potter syndrome with giant malignant solitary fibrous tumour of the pleura]. / Doege­Potter-szindróma a mellhártya óriás malignus szoliter fibrosus tumorával.

Infrequent solitary fibrous tumours of the pleura are associated with hypoglycaemia only in a few percent of the cases; this condition is called Doege-Potter syndrome, named after its first descriptors. Our 63 years old male patient has previously undergone clinical treatment for intrathoracic fluid accumulation on the left side caused by a giant tumour-like mass in the lower left lobe detected by CT scan. In the course of further investigations performed due to increasing load-induced dyspnoea, lung core biopsy verified low grade sarcoma in the tumour. Tumour board suggested surgery. The patient was transferred from the intensive care unit into the operation theater due to increasing dyspnoea and repeated hypoglycaemic periods in rapidly worsening general condition. Pneumonectomy and removal of the tumour was performed on the left side. Histology showed solitary fibrous tumour of the pleura corresponding to Doege-Potter syndrome. The patient was discharged without complications and underwent adjuvant chemotherapy due to pleural dissemination of the tumour observed intraoperatively. One year after surgery the patient underwent surgical removal of a locally recurrent tumour. In spite of repeated chemotherapy local and multiplex contralateral pulmonary progression was observed. Three-year survival was noted from the time of the first surgery. Orv Hetil. 2018; 159(41): 149-153.

Doege-Potter syndrome: hypoglycaemic coma in a 90-year old due to a solitary fibrous tumour.

Solitary fibrous tumour of the pleura (SFTP) is a rare primary tumour of the pleura associated with 4% of cases with a paraneoplastic hypoglycaemia, termed Doege-Potter syndrome (DPS). We report a case of DPS presenting with severe coma in a 90-year-old woman. The cause was a malignant SFTP treated with surgical resection, from which the patient made a full recovery with prevention of recurrent hypoglycaemia. Surgical resection of the SFTP presenting with symptomatic hypoglycaemia should be considered even in elderly patients.

Solitary Pleural Fibroma: A Case Report and Review of Literature.

Solitary fibrous tumors ofthe pleura (SFTP) are uncommon. They tend to be discovered incidentally or during workup for unexplained cough or paraneoplastic effects. It is important to recognize the entityand perform a surgical excision because of the possibility of subsequent malignant transformation and local compressive effects. We present the case of a SFTP discovered on chest imaging. Our patient had surgical excision with good response. A review of the literature is also presented.

Challenges in diagnosis and management of giant solitary fibrous tumour of pleura: a case report.

BACKGROUND: Majority of patients with solitary fibrous tumours of the pleura (SFTP) are asymptomatic. Acute presentation with symptoms resulting from mass effect due to rapid expansion of tumour size has not been reported before. CASE PRESENTATION: This report chronicles the case of a giant SFTP in a 76-year-old lady who presented with acute onset of haemoptysis, left-sided pleuritic chest pain and hoarseness of voice. Her chest radiograph showed a large left upper hemithorax mass with an ipsilateral effusion. Computed tomography (CT) scan of the thorax confirmed the presence of a pleural-based mass lesion in the left apex measuring 9.7 cm with close apposition to the aortic arch. The mass demonstrated neovascularization and there was also presence of a moderate-sized heterogeneous-appearing left pleural effusion. Thoracocentesis yielded deeply haemoserous pleural fluid with a pleural aspirate hematocrit closely approaching that of peripheral blood hematocrit and alongside a 2 unit decrease in haemoglobin, was indicative of a haemothorax. Repeat CT 10 days from initial presentation showed reduction in size of the left apical mass as well as resolution of the left effusion. This was consistent with the occurrence of an intra-tumoural bleed resulting in rapid increase in the size of the SFTP, causing rupture of superficial blood vessels on the tumour surface (haemothorax) and consequential compression of the lung parenchyma (haemoptysis) and left recurrent laryngeal nerve (hoarseness of voice). The patient eventually underwent an uneventful surgical resection. CONCLUSION: A benign SFTP can present acutely with compressive symptoms as a result of spontaneous intra-tumoural bleed causing sudden increase in its size. It is important to allow temporal regression of these acute changes before deciding on surgical resectability.

Solitary fibrous tumor of the pleura: morphogenesis and progression. A report of 36 cases.

PURPOSE: We attempted to identify the exact point of tumor eruption of a solitary fibrous tumor of the pleura (SFTP). METHODS: We morphologically classified 36 SFTPs into 5 categories. Type A showed a connection that included a bloodstream with the pleura on both sides. Type B only showed a connection that included a bloodstream with the visceral pleura, and had a non-bloodstream connection with the parietal pleura. Type C only showed a connection that included a bloodstream with the visceral pleura, and had no connection with the parietal pleura. Type D showed a non-bloodstream connection with the visceral pleura, and only showed a connection that included a bloodstream with the parietal pleura. Finally, type E had no connection with the visceral pleura, and only showed a connection that included a bloodstream with the parietal pleura. The clinicopathological profiles of the tumors were investigated according to their type. RESULTS: The distribution of the 36 SFTPs was as follows: A (19 %), B (6 %), C (67 %), D (0 %) and E (8 %). The tumors categorized as type A tended to be large in size. CONCLUSIONS: SFTPs commonly arise from the visceral pleura and in accordance with tumor progression they will form a non-bloodstream connection with the parietal pleura. Finally, a vascular pedicle will arise with the parietal pleura.

[Giant solitary fibrous tumor of the pleura: A case report and literature review].

Solitary fibrous tumor (SFT) is a derived mesenchymal tumor from spindle cells, mostly occurred in the pleura. To analyze the clinical features of the SFT, data for a patient with SFT that involved in the pleura were retrospectively analyzed by assisted thoracoscope in the Affiliated Hospital of Zunyi Medical College in August 2015. The male patient was 45 years old, who showed the main clinical symptoms of chest pain, cough, sputum, and dyspnea. Large amount of right pleural effusion, chest space-occupying lesions were found by chest CT, suggesting a malignant tumor with metastasis at the 2nd and 3rd right rib. Immunohistochemical results showed: CD34 (+), cytokeratin (-), cytokeratin 5/6 (-), calretinin (-), epithelial membrane antigen(-), mesothelial cell (-), vimentin (++), Wilm's tumor-1 (+), Bcl-2 (+), CD56 (-), CD99 (+), desmin (-), and thyroid transcription factor-1 (-). It was diagnosed as SFT at right side wall layer pleura. SFT is a rare disease and it may occur at any site in the body. It lacks characteristic clinical symptoms and can be asymptomatic, or displays symptoms such as cough, chest pain, dyspnea, and hemoptysis. SFTs can only be conclusively diagnosed based on histopathologic and immunohistochemical characteristics of the tumor, and they are mostly benign. The main treatment for SFTs is the complete surgical resection. The prognosis for this disease is relatively good.

Determinants of outcome of solitary fibrous tumors of the pleura: an observational cohort study.

BACKGROUND: Solitary fibrous tumors of the pleura (SFTP) are rare and their long-term outcome is difficult to predict, as there are insufficient data which allow accurate characterization of the malignant variant. Thus the aim of this study was to describe the outcome and possible determinants of malignant behavior of SFTPs. METHODS: Data were collected retrospectively from medical records of patients treated at the University Hospital Zurich from 1992 to 2012. Kaplan-Meier and Cox regression analysis were performed to define disease-free survival time (defined as survival without tumor-recurrence or tumor-related death) using the classical histo-morphological criteria (tumor size, localization, pedunculation, tumor necrosis or hemorrhage, mitotic activity and nuclear pleomorphism) and immunohistochemical parameters. RESULTS: 42 patients (20 males) with SFTP (median (IQR) age 62 (56-71) years) could be identified. SFTP were associated with symptoms in 50% of all cases. Complete resection was achieved by video-assisted thoracic surgery or thoracotomy in 20 and 22 patients, respectively. Three SFTP-related deaths (7.1%) and four tumor recurrences (9.5%) were observed. Mean disease-free survival time was 136.2 (± 13.1) months, and 2-, 5- and 10-year disease-free survival was 91%, 84%, and 67%, respectively. Mean disease-free survival inversely correlated with the mean tumor diameter, number of mitotic figures and proliferation rate (Ki-67 expression). Other criteria (tumor necrosis, atypical localization, sessile tumor, and pleomorphism) were not statistically significant prognostic parameters. CONCLUSIONS: Patients with large SFTP with a high mitotic index and high proliferation rate should be followed-up closely and over a prolonged time period in order to recognize recurrence of the SFTP early and at a treatable stage. Future research on this topic should focus on the prognostic role of immunohistochemistry including Ki-67 expression and molecular parameters.