Giant Solitary Fibrous Tumor of the Ascending Aortic Wall Causing Reversible Heart Failure: A Case Report and Review of the Literature.

A 56-year-old woman was admitted to our hospital with a 2-week history of chest tightness and fatigue, and an echocardiogram revealed a massive polyserous cavity effusion. A massive (13.5 cm maximum diameter) intrapericardial mass was discovered using computed tomography (CT) and cardiovascular magnetic resonance imaging (MRI) in the ascending aortic wall. A pericardial biopsy was performed and diagnosed as a solitary fibrous tumor (SFT). After successful mass resection, an immunohistochemical test was positive for CD34, STAT-6, CD34, and Bcl2, which indicates a giant benign solitary fibrous tumor of the ascending aortic wall. After three years of follow-up, the patient is symptom-free, and histological indications of malignancy were absent. A giant benign solitary fibrous tumor is extremely rare in the heart, especially from the ascending aorta wall, and experience with this tumor location is limited, so close follow-up at regular intervals is considered necessary. We present this case, followed by a literature review on SFTs involving the heart and management approaches.

Big insulin-like growth factor 2-producing multiple solitary fibrous tumors treated with debulking surgery: A case report.

Background: Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome caused by a tumor-producing high molecular weight form of insulin-like growth factor 2 (IGF2) known as big IGF2. The only curative treatment for this condition is surgical resection of the responsible tumors. However, this may not be feasible in cases with multiple metastases at diagnosis of NICTH, and no standard treatment strategy for multiple tumors has been established. The effects of pharmacological therapies including somatostatin analogs are often inefficient and remain difficult to predict. Case description: A 68-year-old man was admitted to our hospital due to impaired consciousness and severe hypoglycemia. His medical history included diagnosis of a left temporal solitary fibrous tumor (SFT) at the age of 48 years, after which local recurrent and metastatic tumors were repeatedly resected. Four years before admission, multiple intraabdominal and subcutaneous tumors were detected and, being asymptomatic, were managed conservatively. Laboratory exam on admission demonstrated hypoglycemia accompanied with low serum insulin and IGF1 levels. Computed tomography (CT) scan revealed multiple intraabdominal and subcutaneous tumors increasing in size. Serum big IGF2 was detected on immunoblot analysis, and he was diagnosed as NICTH. In addition, tumor uptake was observed on 68Ga-labelled 1,4,7,10-tetraazacyclododecane-N,N',N'',N'''-tetraacetic acid-d-Phe1-Tyr3-octreotide positron emission tomography/CT (DOTATOC-PET/CT). Since larger tumor is more suspicious about responsible producibility of big IGF2, we planned to resect large ones preferentially and reduce the amounts of residual tumors. Debulking surgery was performed by removing eleven intraabdominal tumors; the hypoglycemia was then completely corrected. Histological analyses revealed the resected tumors to be metastases of SFT having somatostatin receptor 2 expression. In immunoblot analysis, the resected tumors were found to be positive for big IGF2; serum big IGF2 was undetectable after surgery. Conclusion: We present a case of NICTH with multiple metastatic SFTs. We strategically performed debulking surgery, which led to remission of hypoglycemia. This result demonstrates a pioneering practical solution for NICTH cases with multiple tumors. In addition, in cases of SFTs presenting with NICTH, positivity of DOTATOC-PET/CT as well as single-dose administration of octreotide may be predictive of the efficacy of somatostatin-based therapy.

Doege-Potter syndrome in a patient with a giant abdominal solitary fibrous tumor: a case report and review of the literature.

A 63-year-old man with spells of reduced consciousness in the morning and a giant abdominal mass presented to our institution for a second opinion. Investigation revealed non-diabetic hypoinsulinemic hypoglycemic events. Removal of the abdominal mass solved the hypoglycemia. Anatomopathological examination confirmed a solitary fibrous tumor (SFT). Doege-Potter syndrome was diagnosed. Doege-Potter syndrome is a potentially life-threatening rare paraneoplastic syndrome characterized by recurrent hypoinsulinemic hypoglycemia due to the overproduction of a prohormone form of insulin-like growth factor-II (pro-IGF-II) from a solitary fibrous tumor. First, we describe the clinical, laboratory and radiologic findings of the case. Second, a brief literature review on Doege-Potter syndrome is provided.

Successful Surgical Treatment of a Recurrent Pelvic Solitary Fibrous Tumor of Uterine Origin Accompanied by Doege-Potter Syndrome: A Case Report.

BACKGROUND Solitary fibrous tumors (SFT), rare soft-tissue neoplasms, are usually found in the thoracic cavity, and a uterine origin is extremely rare. SFTs with insulin-like growth factor-II (IGF-II) production induce non-islet cell tumor-induced hypoglycemia (NICTH), referred to as Doege-Potter syndrome. CASE REPORT A 70-year-old woman presented with urinary retention, and imaging revealed a huge mass occupying almost the entire pelvic space. She had a history of hysterectomy for leiomyoma of the uterus 7 years earlier. In her present course, she developed hypoglycemia, and NICTH was suspected. Her previous uterine specimen was reexamined, and immunohistochemistry (IHC) revealed the specimen to be CD34-positive and alpha-smooth muscle actin-negative, indicating that the uterine specimen was not leiomyoma but SFT. Therefore, the present pelvic tumor was considered to be a recurrence of SFT with NICTH, namely Doege-Potter syndrome. Surgical resection was performed, and the pathological examination showed the same histologic features as the previous uterine specimen, while IHC revealed the present specimen to be positive for CD34, signal transducers and activator of transcription 6, and IGF-II, consistent with the diagnosis of recurrent SFT with IGF-II production. The patient's hypoglycemia improved after tumor resection. To confirm the IGF-II secretion from the SFT, we conducted immunoblotting of the patient's perioperative serum, with results showing that the strong band of IGF-II in the preoperative serum disappeared after surgery. CONCLUSIONS Because SFTs, especially those with Doege-Potter syndrome, often recur, sometimes with a very long interval, long-term cautious surveillance is required, even after complete tumor resection.

IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature.

BACKGROUND: Non-islet cell tumour hypoglycemia (NICTH) is rarely encountered in clinical practice. Insulin-like growth factor 2 (IGF2) is the most common cause of NICTH observed in the setting of mesenchymal and epithelial neoplasia. This is a paraneoplastic syndrome caused by IGF2 activation of the insulin receptor. CASE PRESENTATION: An 80 year old female presented with a short history of recurrent episodes of confusion with laboratory confirmed hypoglycemia with a plasma glucose of 2.7 mmol/L on fasting which fulfilled Whipple's triad. Diagnostic clues to the aetiology at presentation include the fasting pattern of hypoglycemia, hypokalaemia and the absence of weight gain. A 72 hour fast with results showed early hypoglycemia and suppression of serum insulin, c-peptide, and proinsulin. Serum insulin antibody was not detected. Subsequent measurement of the serum IGF2:IGF1 ratio was elevated at 22.3 and consistent with IGF-2 mediated hypoglycemia and imaging studies demonstrated a pelvic mass. Dietary intervention and oral prednisolone abated hypoglycemia prior to surgery. Ultimately, hypoglycemia resolved following operative intervention and steroid therapy was successfully withdrawn. Histopathology was remarkable for dual neoplastic processes with uterine solitary fibrous tumour (SFT) confirmed as the source of IGF2 hypersecretion on IGF-2 immunohistochemistry and a coincidental invasive high grade serous carcinoma involving the fimbria of the right fallopian tube. CONCLUSION: The paradox in this case is that the benign solitary fibrous tumour accounted for patient morbidity through secretion of IGF2 and without treatment, posed a mortality risk. This is despite the synchronous presence of a highly malignant fallopian tube neoplasm. This case reinforces the need for thorough clinical evaluation of hypoglycemia to allow prompt and definitive management.

The challenge to prove a rare cause of secondary arterial hypertension. A case report of a pediatric renal solitary fibrous tumor.

BACKGROUND: Childhood hypertension is getting more attention in recent years. We present a case report of a rare cause of secondary arterial hypertension in a teenage girl - a solitary fibrous tumor of the kidney. The case demonstrates that standard imaging techniques, computed tomography and magnetic resonance imaging, are not fully reliable in the diagnosis of renovascular hypertension. CASE: A 15-year old girl was admitted to the Pediatric Department because of episodes of stiffness in the limbs, accompanied by pale skin and lips, dated 4 months back. During these episodes, high blood pressure up to 160/100 mmHg was measured. A 24-hour blood pressure monitoring demonstrated arterial hypertension stage II. Renovascular hypertension was suspected, but the computed tomography examination of the abdomen showed normal-sized renal arteries. In the left kidney hilum, an intraparenchymal formation was discovered. The data presented a non-specific lesion with a wide differential diagnosis. Given the fact that the patient had been treated with an ACE-inhibitor, serum renin level could not be correctly interpreted. The lesion was removed through a laparoscopic intervention. Intraoperatively, the tumor was compressing a small intra-renal vessel - a finding that hadn`t been discovered by the previous imaging studies. The final pathologist diagnosis was: solitary fibrous tumor. During the next six months of follow-up, the maximal blood pressure values of the patient were up to 120/80 mmHg. CONCLUSIONS: Solitary fibrous tumors of the kidneys are infrequent in children. The presented case displays a rare form of initial clinical manifestation of this tumor. It is also a demonstration that standard imaging techniques are not able to get a precise visualization of the small intra-renal vessels. At the same time, the decision of whether or not to perform a more invasive procedure should be based on the clinical conditions and risks of the individual patient.

Solitary fibrous tumor of the left adrenal gland associated with Doege-Potter syndrome. Case report.

We present the case of a 71-year-old man with a history of type 2 diabetes mellitus manifesting abdominal pain, weight loss, dyspnea on small efforts, muscle weakness, and left subcostal swelling. Associated with this, he developed frequent episodes of syncope, diaphoresis, and night sweats, during which blood glucose levels below 50 mg/dL and improvement were documented after the administration of hypercaloric beverages or intravenous dextrose. Abdominal computed tomography revealed the presence of a tumor of approximately 20 cm dependent on the left adrenal gland. Histopathological diagnosis of a solitary retroperitoneal fibrous tumor was obtained, confirmed by immunohistochemistry.

Se presenta el caso de un varón de 71 años con antecedente de diabetes mellitus tipo 2 que manifiesta dolor abdominal, pérdida de peso, disnea de pequeños esfuerzos, debilidad muscular y aumento de volumen subcostal izquierdo. Asociado a esto desarrolló episodios frecuentes de síncope, diaforesis y sudoraciones nocturnas, durante los cuales se documentaron cifras de glucemia < 50 mg/dl y mejoría tras la administración de bebidas hipercalóricas o dextrosa intravenosa. La tomografía computarizada abdominal reveló la presencia de una tumoración de aproximadamente 20 cm dependiente de la glándula suprarrenal izquierda. Se obtuvo diagnóstico histopatológico de tumor fibroso solitario retroperitoneal confirmado por inmunohistoquímica.

A rare case of rectal bleeding and Fusobacterium mortiferum sepsis due to solitary fibrous tumour originating from the mesentery.

Solitary fibrous tumours (SFTs) are rare mesenchymal tumours that are mostly seen in the pleura. Lately, they have also been described in other locations. Recent discovery of the NAB2-STAT6 fusion gene which is specific for SFTs has led to an accurate diagnosis of SFTs. The occurrence of SFTs in the mesentery is very rarely reported in the literature. We report a case of a 63-year-old female who presented with abdominal pain, rectal bleeding and Fusobacterium bacteraemia, who was ultimately found to have a mesenteric SFT.

Small bowel occlusion secondary to a giant abdominal solitary fibrous tumor.

A 30-year-old male with no medical history was admitted to the hospital with abdominal pain, vomiting, and inability to pass gas through the rectum. In the physical examination a non-mobile, smooth, firm mass was palpated in the lower abdominal quadrants. An abdominal mass was detected by a CT scan. Thus, the patient underwent laparotomy and the mass was excised together with a bowel segment, and a terminal anastomosis was performed. No peritoneal or liver seeding was observed, and the patient recovered uneventfully.

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 in the emergency department with symptoms of dizziness and vomits. Imaging studies show 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.

Extraordinarily long-inactive solitary fibrous tumor transformed to produce big insulin-like growth factor-2, leading to hypoglycemia and rapid liposarcoma growth: a case report.

BACKGROUND: A high-molecular-weight form of insulin-like growth factor-2 (IGF-2), known as "big" IGF-2, is occasionally produced by various tumor types, leading to hypoglycemia. Although solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm, it has been estimated that 4-6% of SFT patients develop hypoglycemia due to circulating big IGF-2. The mean time elapsed from tumor detection until the onset of hypoglycemia is reportedly less than one year (8.5 ± 1.9 months). CASE PRESENTATION: A 68-year-old man was hospitalized for exacerbation of recurring hypoglycemic episodes. He had been diagnosed with an SFT 17 years before the onset of hypoglycemia, and the SFT had already been very large at that time. The tumor, which was non-resectable and refractory to chemotherapies, had slowly increased in size since the initial diagnosis. Half a year before the hypoglycemic episodes manifested, another tumor, adjacent to the left kidney, was newly identified. Fluorodeoxyglucose positron emission tomography-computed tomography scanning, revealed the left peri-renal tumor to show much higher fluorodeoxyglucose uptake than the preexisting SFT, suggesting that it was unlikely to be a metastasis from the SFT. Abundant serum big IGF-2 was detected by western immunoblot analysis, indicating it to be the cause of the hypoglycemia. Since the 17 years between SFT detection and the onset of IGF-2-induced hypoglycemia was an extremely long period as compared with those in previous reports, we initially suspected that the new, peri-renal tumor had produced big IGF-2, but transcatheter arterial embolization of its feeding arteries did not suppress hypoglycemia. Notably, by measuring the tumor volume doubling time, the peri-renal tumor growth was shown to be markedly accelerated in parallel with exacerbation of the hypoglycemia. The patient died of heart failure 21 months after the onset of hypoglycemia. Unexpectedly, autopsy revealed that big IGF-2 had been produced only by the preexisting SFT, not the peri-renal tumor, and that the peri-renal tumor was a dedifferentiated liposarcoma. CONCLUSIONS: We should keep in mind that even a long-inactive SFT can undergo transformation to produce big IGF-2, which then acts on both insulin and IGF-1 receptors, possibly leading to both hypoglycemia and the development/growth of another tumor, respectively.

Outcome of Epidural Hematoma: Lessons from Solitary Fibrous Tumor/Hemangiopericytoma.

BACKGROUND: Intracranial solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is rare. In this report, a case of epidural hematoma (EDH) that eventually evolved into SFT/HPC is presented. We describe the possible association between the 2 diseases, which has not been previously reported. CASE DESCRIPTION: A 40-year-old man suffered from an EDH in the right parietal area 12 years ago and accepted conservative treatment. Follow-up computed tomography (CT) scan shows that the density of the right EDH gradually changed from uniform slightly lower density to mixed density. A new CT scan revealed an epidural mass extending to the subcutaneous with local bone destruction. An operation was performed via a large right parietal craniotomy, and the final diagnosis was World Health Organization grade III SFT/HPC after histopathologic examination and immunohistochemical verification. The patient died of deterioration of brain disease 3 months after the final diagnosis. CONCLUSIONS: To our knowledge, this is the first report that HPC occurred in the epidural cavity. We are the first time to describe the possible association between EDH and HPC.

A Footprint-Like Intracranial Solitary Fibrous Tumor/Hemangiopericytoma With Extracranial Extension and Acute Intratumoral Hemorrhage.

BACKGROUND: Intracranial solitary fibrous tumors/hemangiopericytomas (ISFTs/HPCs) are extremely rare spindle-cell tumors that are mostly benign and derived from mesenchymal tissue. Extracranial metastasis and intratumoral hemorrhage are considered to be special manifestations of malignant tumors, of which extracranial metastases of ISFTs/HPCs have been reported, while tumor hemorrhage has rarely been described. Here, the authors present an interesting case of ISFTs/HPCs with acute intratumoral hemorrhage. CLINICAL PRESENTATION: A 72-year-old woman underwent brain magnetic resonance imaging (MRI) for recurrent headaches and nausea. The MRI scan showed a footprint-like space-occupying lesion in the bilateral parietooccipital lobe. This lesion had multiple cystic components and invaded the superior sagittal sinus, destroying adjacent cranial bones. While waiting for routine surgery, the patient suddenly developed acute tumor bleeding and then underwent emergency surgery. Postoperatively, the diagnosis of ISFT/HPC was confirmed by pathological and immunohistochemical analysis. DISCUSSIONS AND CONCLUSIONS: The diagnosis of ISFT/HPC mainly depends on pathological and immunohistochemical results. Malignant cases with cystic necrosis may be prone to acute hemorrhage. Early total surgical excision can provide a good clinical prognosis. Adjuvant radiotherapy is an effective supplement to surgical treatment. Metastasis and recurrence require long-term follow-up monitoring.