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.

[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.

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).

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.

[Hypoglycemia induced by a solitary fibrous tumor of the lung or Doege-Potter syndrome: Report of one case]. / Hipoglicemia inducida por tumor fibroso solitario pulmonar: Síndrome de Doege-Potter.

We report the case of a 75-year-old man who was admitted to the hospital with symptoms of severe hypoglycemia. He had a history of solitary fibrous tumor diagnosed by Pathology after its complete surgical resection eight years before. The laboratory examination reported hypoglycemia with inhibited Insulin secretion. A computed tomography of the thorax revealed a large solid heterogeneous mass in the left hemithorax. Solitary fibrous tumor is a rare neoplasm. The association of solitary fibrous tumor and paraneoplastic hypoglycemia is known as Doege-Potter syndrome and occurs in less than 5% of all solitary fibrous tumors.

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.

Solitary fibrous tumour of the pleura.

Solitary fibrous tumour (SFT) of the pleura is a rare, usually benign primary tumour of the pleura. Spectrum of presentation can vary from an incidental finding on chest radiograph done for some other purpose, features of compression of surrounding structures to symptoms resulting from the tumour per se. We report a case of a female who presented with complaints of cough and chest pain in whom a diagnosis of SFT was confirmed on tru-cut biopsy and immunohistochemistry studies. The patient underwent thoracotomy and successful removal of the tumour.

Benign localized fibrous tumor of the pleura: report of 25 new cases.

OBJECTIVE: Benign localized fibrous tumors (BLFT) of the pleura are very rare slow-growing neoplasms that generally have a favorable prognosis. The aim of this manuscript is to evaluate the predictors of outcome with the review of the literature in a series of 25 patients with BLFT. METHODS: Between January 1985 and November 2009, 25 patients underwent an operation due to BLFT. Of these patients, 14 (56%) were male; mean age was 41.1 (25 to 64) years. All patients underwent thoracotomy. Left thoracotomy approach was used in 16 patients. The mass lesions were totally excised. The histopathological examinations were performed with hematoxylin-eosin and immunohistochemical staining methods. RESULTS: Of the patients, 18 (72%) were symptomatic. Symptoms were cough in 36%, shortness of breath in 32%, and chest pain in 20% of the patients. One patient (4%) appeared to have some symptoms (pain and swelling of the joints) associated with pulmonary osteoarthropathy. Seven patients (28%) underwent an operation due to mass lesion detected at routine control visits. None of the patients had a history of exposure to asbestos. Radiological investigations revealed 16 (64%) mass lesions in the left. Of the lesions found on exploration, 5 (20%) were intrapulmonary localized lesion without pedicle and 20 were pedicled. Of the pedicled masses, 5 were connected to parietal pleura and 15 to visceral pleura and all were intrathoracic extrapulmonary localized lesions. Eight (32%) lesions connected to left lower lobe. Additionally, three pedicled lesions were located in the lung fissure. Pedicled lesions were totally excised together with their pedicles. Intraparenchymal mass lesions were resected using wedge resection. The diameter of the resected masses was ranging between 3 and 22 cm (mean: 8.7). Macroscopically, all were encapsulated with a homogeneous cut surface. Intraoperative mortality and morbidity was not observed. The average hospitalization duration for all patients was 8.6 days (5 to 12). The mean follow-up was 33.6 (9 to 142) months with no recurrence. CONCLUSIONS: Benign localized fibrous tumors are uncommon and treated by surgical means. According to our data obtained from literature review, clinicians should be aware of recurrence possibility even after complete resection of benign localized fibrous tumor and the risk of malign transformation.

Malignant fibrous tumor of the pleura: case report and literature review.

Solitary fibrous tumours (SFT) of the pleura are uncommon and are incidental findings or discovered in patients with non-specific respiratory symptoms. We report a case of a 74 year old man diagnosed with a mesenchymal pleural neoplasm, associated with typical hypertrophic osteoarthropathy, referred to as Pierre-Marie-Bamberg syndrome. As reported in the literature, complete surgical resection is the gold standard for treatment of such lesions and recurrences. Radiotherapy and chemotherapy are of limited value in the curative treatment of pleural SFT. In our case surgical excision of the mass was performed. After a disease-free period of 3 years a second intervention was necessary because of recurrence. Until now our patient is free of complaints and no signs of reappearance were noted. Based on our experience and on literature findings we would like to underline the importance of regular long-term follow-up because of the substantial risk of recurrence.

[Doege-Potter syndrome: hypoglycemia secondary to solitary fibrous tumor of the pleura. Report of one case]. / Síndrome de Doege-Potter: hipoglicemia secundaria a tumor fibroso solitario de la pleura.

Doege-Potter syndrome is characterized for hypoglycemia associated with solitary pleural fibrous tumors. We report a 38-year-old woman with a history of weight loss, malaise and edema. After an episode of symptomatic hypoglycemia, she was admitted to the hospital, where she had new episodes of hypoglycemia. A Chest X ray and scan showed a right pleural tumor that was surgically excised. After surgery the episodes of hypoglycemia subsided. The pathological study of the tumor revealed a solitary fibrous pleural tumor. After 15 months of follow up, the patient is symptom free and without evidence of tumor relapse.

GIANT THORACIC MASS - THE STORY OF A SOLITARY FIBROUS TUMOUR.

The tumours of the pleura are a vast and diverse field. One of the lesser known and less common tumour is the solitary fibrous tumour of the pleura, representing about 5% of these types of tumours. The authors present the case of a woman admitted to the emergency department with symptoms of dizziness and vomits. Imaging studies showed a giant thoracic mass on the left hemithorax, with a biopsy indicating a solitary fibrous tumour. The patient was referred for surgery, which was performed via thoracotomy. In the postoperative period she developed an acute pulmonary oedema secondary to lung reexpansion and fluid overload, with a good response to fluid restriction and intravenous diuretics.

DOEGE-POTTER SYNDROME BY PLEURAL SOLITARY FIBROUS TUMOR.

INTRODUCTION: Solitary fibrous tumor of the pleura (SFTP) is a rare neoplasm that accounts for less than 5% of all pleural tumors. We present the case of a 73-year-old man with a history of recurrent episodes of severe hypoglycemia secondary to a large malignant SFTP. This paraneoplastic manifestation of SFTP occurs in less than 5% of cases and is referred to as Doege-Potter syndrome. Although rare, this is an important and reversible cause of hypoglycemia, which is resolved by complete surgical resection of the tumor. We describe the pathogenesis, diagnosis, and treatment of Doege-Potter syndrome. Key imaging findings and pathologic correlation are shown.

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.

Localized pleural mesothelioma causing cranial vena cava syndrome in a dog.

A 9-year-old female crossbred dog was presented to the Hospital Universitario Veterinario Rof Codina (Universidad de Santiago de Compostela, Lugo, Spain) for acute onset of severe, progressive swelling of the head, neck, and cranial trunk. Survey radiographs and ultrasonography revealed a large, heterogeneous mass in the cranial mediastinum, compressing or growing into a large blood vessel within the cranial mediastinum and displacing the heart dorsocaudally. At postmortem examination, the mass was diagnosed as a large, localized mesothelioma. Localized mesotheliomas are rare neoplasms in dogs but should be considered as a possible differential diagnosis for cranial vena cava syndrome. The anatomic distribution and clinical features of mesothelioma in the present report are similar to other cases in humans.

Solitary fibrous pleural tumor associated with loss of consciousness due to hypoglycemia.

The patient suffered loss of consciousness, dysarthria and right sided hemiparesis. The CT scan and MRI scans were negative. These findings are more in keeping with a diagnosis of Transient Ischemic Attack (TIA) or mild CVA. Hypoglycemia per se does not usually cause hemiparesis. The blood glucose level was low but I am not sure if one can conclude that hypoglycemia caused the above noted neurological signs and symptoms. The authors do not present any data to prove that this patient had "hypoglycemic coma".

Largest known malignant solitary fibrous tumour of the pleura-extended resection warranting cardiopulmonary bypass support.

This case reports the largest known malignant solitary fibrous tumour of the pleura treated with en bloc surgical resection warranting the use of cardiopulmonary bypass support. A 60-year-old male presented with dyspnoea and a dry cough. Following extensive investigations, a radiological and histologic diagnosis of malignant solitary fibrous tumour of the pleura was made. This 4.3 kg tumour occupied the entire left hemithorax, involved the left lung and infiltrated into the pericardial cavity. Although the postoperative course was uneventful with a 12-day length of stay, the patient opted not to undergo adjuvant radiotherapy to a single positive margin site and died 6 months later due to local recurrence.

Non-islet-cell tumour hypoglycaemia (NICTH): About a series of 6 cases.

The purpose of this study was to analyse the characteristics of 6 patients managed in a university hospital between 1996 and 2016 for non-islet cell tumor hypoglycemia (NICTH), a form of hypoglycaemia due to the paraneoplastic secretion of IGF-2 or its related substances. RESULTS: Three of these 6 patients (50%), aged over 69 years, including 2 with acromegaloid phenotype, presented with a pleural solitary fibrous tumor (SFT), with median diameter 20 cm (interquartile range, 12.5-20.5) with a low median SUV (3.3 g/mL (QR, 2-7.5)) on 18F-FDG PET. The other 3 patients presented respectively neuroendocrine carcinoma (NEC) of the palate (70-year-old woman), retroperitoneal myxofibrosarcoma (66-year-old man) and meningeal hemangiopericytoma (36-year-old woman). All 3 were inoperable and did not respond to any therapy other than glucose solution. Corticosteroid therapy was effective in the 3 SFTs and the NEC. One of the SFTs recurred 10 years later with asymptomatic hypoglycemia, which resolved after reintervention. Median (IQR) blood glucose levels of the 6 patients was 0.4g/L (QR, 0.31-0.41), with hypoinsulinemia at 0.7mIU/L (QR 0.7-2.0), undetectable GH, low IGF-1, normal IGF-2 level in 5/6 cases, a high IGF-2:IGF-1 ratio at 26.9 (QR, 20.8-37.8), hypokalemia and hypomagnesemia. CONCLUSION: NICTH is a rare syndrome, which should be considered in the presence of hypoinsulinemic hypoglycemia with low GH and IGF-1, and a IGF-2:IGF-1 ratio>10. Corticosteroid therapy was effective in elderly subjects, particularly with solitary fibrous tumor, which was generally operable. Hemangiopericytoma and myxofibrosarcoma had poor prognosis in younger patients.

Localized malignant mesenteric mesothelioma causing small bowel obstruction.

Malignant mesothelioma is an uncommon lethal neoplasm in the serous membrane in which peritoneal mesothelioma is a rarer form. Herein is reported a case of malignant mesothelioma presenting as a localized mass inside the mesentery causing focal luminal obstruction of the small intestine. The diagnosis of malignant mesothelioma was obtained on repeat double balloon endoscopic biopsy. Partial resection of the small intestine along with the mesentery was performed, followed by a course of chemotherapy. No relapse of the disease has been found in the 8 months' follow up radiologically. To the best of the authors' knowledge this is the first reported case of localized malignant mesothelioma arising inside the mesentery. Mesothelioma should be considered as the differential diagnosis when small bowel obstruction occurs with unknown primary neoplasm.

Defective expression of prohormone convertase 4 and enhanced expression of insulin-like growth factor II by pleural solitary fibrous tumor causing hypoglycemia.

A 75-year-old man was admitted to our hospital because of unconsciousness. His plasma glucose was very low, but his serum levels of insulin and IGF-I were also low. He was found to have a giant solitary pleural tumor, which was completely resected, after which his hypoglycemia ameliorated postoperatively. Histologically, the tumor was consistent with the pathological diagnosis of a solitary fibrous tumor derived from the pleura. Immunohistochemical study revealed positive immunostaining for IGF-II in tumor cells. The presence of high molecular weight (HMW) form of IGF-II in the tumor tissue and patient's serum was confirmed by Western blot analysis. Steady-state mRNA levels of IGF-II and prohormone convertases (PC) 4, a potential protease responsible for IGF-II processing, as determined by RT-PCR were about 14-fold greater and 5-fold less in the tumor tissue than those in normal placental tissue, respectively. Therefore, it is suggested that biologically active, unprocessed HMW form of IGF-II generated from the impaired processing of IGF-II precursor by the defective PC4 expression in the tumor was responsible for the non-islet cell tumor hypoglycemia (NICTH) in the present case.