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.

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.

[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.

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).

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.

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.

A Rare Diagnosis After the Fall of a 96-Year-Old Woman: Doege-Potter Syndrome.

INTRODUCTION: Doege-Potter Syndrome (DPS) is a rare but life-threatening paraneoplastic syndrome, characterized by Non-Islet Cell Tumor-Induced Hypoglycemia (NICTH) secondary to a Solitary Fibrous Tumor (SFT), which secretes an incompletely processed form of Insulin-like Growth Factor 2 (IGF-2). RESULTS: A 96-year-old woman was admitted with head trauma due to an accidental fall. During her hospital stay she experienced frequent hypoglycemic episodes. Multiple injections of 33% dextrose and continuous infusion with 10% dextrose were required to maintain normal blood glucose levels. Biochemical analyses revealed hypoinsulinemic hypoglycemia, low C-peptide levels, suppressed insulin-like growth factor-1, normal insulin-like growth factor-2, and an elevated IGF-2:IGF-1 ratio, all consistent with IGF-2 secretion by a non-islet cell tumor. A contrast-enhanced chest and abdominal CT scans showed a single large pleural mass in the left lower hemithorax measuring 15x14 cm without secondary lesions. Histological analysis of biopsied specimens suggested a solitary fibrous pleural tumor; accordingly, a diagnosis of Doege-Potter syndrome was considered. Due to extensive tumor burden and the advanced age of the patient, supportive and non-invasive management was chosen. Dexamethasone therapy was started, and while receiving this therapy she was able to discontinue glucose infusion and successfully maintain euglycemia. DISCUSSION: In the elderly, a sudden and unexplained fall can be the expression of severe hypoglycemia, usually as a complication of insulin therapy or of oral hypoglycemic agents administered to patients with diabetes. However, in patients without diabetes, other causes should be investigated, and the hypothesis of neoplastic diseases should be considered. CONCLUSION: In this case report we describe an uncommon cause of paraneoplastic hypoglycemia occurring in the oldest patient with a non-islet cell tumor reported thus far.

Pierre-Marie-Bamberger syndrome and solitary fibrous tumor: a rare association.

A solitary fibrous tumor originates in the pleura with variable degrees of invasion. Hypertrophic osteoarthropathy, known as Pierre-Marie-Bamberger syndrome, is characterized by clubbing of the fingers due to bone surface and soft tissue calcification, historically known as a bronchogenic carcinoma paraneoplastic syndrome; however, a few cases have been associated with solitary fibrous tumors. We describe the case of a 38-year-old woman who presented with clubbing of the fingers. Studies revealed an intrathoracic fibrous tumor that was successfully treated with improvement in symptoms.

Solitary fibrous tumour: a rare tumour of the pleural cavity.

Solitary fibrous tumours are a rare group of tumours that originate from connective tissues such as the pleura and mediastinum. When growing within the thoracic cavity these tumours exert pressure on vital organs and large vessels. We have successfully operated on two patients with large solitary fibrous tumours and managed the pathophysiological changes manifested as superior vena cava syndrome resulting from these tumours.

An Involuntary and Unexpected Treatment of Nutcracker Esophagus.

A 76-year-old woman complained of painful dysphagia and loss of weight. Esophagoscopy results were negative, whereas computed tomography (CT) disclosed a 25-mm mediastinal tumor without a connection to the esophagus. A diagnosis of nutcracker esophagus was made on high-resolution esophageal manometry. Peroral endoscopic esophageal myotomy failed to improve the symptoms. Right video thoracoscopy allowed resection of the tumor, which looked like a neurogenic tumor of the posterior mediastinum that developed from the right vagus nerve. The patient's dysphagia dramatically improved postoperatively. Because the pathologic examination disclosed a benign solitary fibrous tumor of the pleura, we hypothesize that the motility disorder would have been resolved by the unilateral vagotomy.

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.

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.

Tumor fibroso pleural gigante / Giant pleural fibrous tumour

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[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.

Hipoglicemia inducida por tumor fibroso solitario pulmonar: síndrome de Doege-Potter / Hypoglycemia induced by a solitary fibrous tumor of the lung or Doege-Potter syndrome: report of one case

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.

A case of Solitary fibrous pleura tumor associated with severe hypoglycemia: the Doege-Potter 's Syndrome.

Solitary fibrous pleura tumor is a rare primary intrathoracic tumor of the pleura. It usually has an indolent clinical course, but sometimes it can have an aggressive behaviour. In 1930 Doege and Potter independently described this neoplasm, presenting with symptoms of hypoglycemia, hence the eponim of Doege-Potter's Syndrome. In this report, we illustrate a case of Doege Potter's Syndrome, treated with complete surgical resection.

Doege-Potter Syndrome.

Doege-Potter syndrome is a rare paraneoplastic syndrome presenting as a hypoinsulinaemic hypoglycaemia from the ectopic secretion of a prohormone of insulin-like growth factor II (IGF-II) from a solitary fibrous tumour. Surgical resection is curative in the majority of cases. If, however, the diagnosis is not suspected and treatment is delayed, it can lead to hypoxic cerebral injury or death. The underlying tumour can be a benign or malignant pleural tumour but may be present in extrapleural sites. For a diagnosis of Doege-Potter syndrome, symptoms attributable to hypoglycaemia and low blood glucose levels should be present along with the secretion of prohormone IGF-II. We report a case of severe hypoglycaemia in a 76-year-old inpatient admitted for resection of a recurrent left-sided pleural tumour. Investigation revealed true hypoglycaemia and Doege-Potter syndrome was diagnosed. The tumour was completely resected and the patient made a full recovery with no further hypoglycaemic episodes.