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.

Non-islet-cell tumor induced hypoglycemia:one case report and literature review / 中华内分泌代谢杂志

A patient with severe hypoglycemia due to insulin-like growth factor ( IGF)-IIsecreted by a giant solitary fibrous tumor of the pleura ( SFTP) was investigated through comprehensively reviewing his medical history and clinical records. The patient had severe hypoglycemia accompanied with significantly decreased serum insulin level. A solitary fibrous tumor of the pleura was found, and right pneumonectomy removed this giant tumor. Two years after the operation, the patient was fit and well with no further hypoglycemia episodes. Non-islet-cell tumor hypoglycemia should be considered in patients who have hypoglycemia episodes accompanied with significantly decreased serum insulin level.

Diagnosis and treatment of intraperitoneal hemorrhage from spontaneous rupture of mixed pancreatic cancer / 中华消化外科杂志

Pancreatic ductal carcinoma accounts for 85％-90％ in pancreatic cancer, followed by the pancreatic cellendocrine tumors and pancreatic acinar cell carcinoma.In addition, mesenchymal cell carcinoma of pancreas is rare, and a mixed pancreatic cancer from 3 cells carcinoma is extremely rare.And pancreatic cancer always assume hypovascular tumor,spontaneous rupture of pancreatic cancer is rarely reported.A patient with a mixed duct-acinar-islet cell tumor of pancreas was admitted to the Second Affiliated Hospital of Harbin Medical University in June 2014, and underwent emergent operation of intraabdominal bleeding due to spontaneous rupture of tumor.The follow-up was done up to January 20, 2015.The patient died of intraabdominal widespread implantation metastasis of pancreatic cancer.A mixed duct-acinar-islet cell tumor of pancreas is extremely rare and easy to metastasis and diffusion of tumor with a rapid disease progression and a poor prognosis of patient,while operation is the key to terminate deterioration of the condition, and is the last line of defense to save lives.

Precise positioning and enucleation for the treatment of functional islet cell tumor / 中华消化外科杂志

Objective To investigate the clinical value of intraoperative ultrasound guided precise positioning and enucleation of the functional islet cell tumor.Methods The clinical data of 20 patients with functional islet cell tumor who were admitted to the First Affiliated Hospital of Xinjiang Medical University from January 2005 to December 2011 were retrospectively analyzed.The method of precise positioning,surgical approach and prognosis of the patients were reviewed.Results The accurate rates of computed tomography (CT),magnetic resonance imaging (MRI) and transabdominal B ultrasound in detecting the position of the functional islet cell tumors were 12/18,2/6 and 7/13,respectively,and the diameters of the tumors were (1.7 ±0.8)cm,(1.3 ±0.2)cm and (1.9 ±0.9)cm,respectively.The accurate rates of arterial stimulation venous sampling and pancreatic perfusion CT imaging were 100％,and the diameters of the tumor detected were (0.7 ± 0.3) cm and (0.9 ± 0.4) cm.The accurate rate of intraoperative B ultrasound examination was 14/14,and the diameter of the tumor was (1.5 ± 0.6)cm.Routine surgery was carried out on 6 patients,and 2 patients were complicated with grade C pancreatic fistula,and 1 was complicated with grade A pancreatic fistula.Fourteen patients received precise enucleation of islet cell tumor,and 4 patients were complicated with grade A pancreatic fistula.Twenty patients were followed up.The general condition of the patients was good till April 2012,and no death,tumor recurrence and metastasis were detected.Conclusions Combination of pre-and intraoperative imaging positioning could precisely locate functional islet cell tumor.If the distance between the tumor and main pancreatic duct is above 3 mm,precise enucleation of the islet cell tumor should be considered.

Imaging Finding of Multiple Endocrine Neoplasia Type 1: Case Report

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited syndrome with characteristic clinical and radiological manifestations. Many reports on MEN1 have been published; however, no cases of radiologically diagnosed MEN1 have been reported. Therefore, we report on a radiologically diagnosed case of MEN1 with clinical symptoms of gastroduodenal ulcer.

Nonfunctional islet cell tumor: a report on 26 cases and review of the literature / 中华肝胆外科杂志

Objective To review our experience in the diagnosis and treatment of nonfunctional islet cell tumors(NICT).Method The clinicopathological data of 26 cases of NICT were retrospectively analysed and the medical literature was reviewed.Results In addition to the 26 cases seen in our center,there were 569 cases published in 37 articles in the medical literature.In China,NICT was more common in women.The male to female ratio was about 3 ∶ 7.The mean age of onset of the disease was 35 years old.Most NICT were solitary and malignant,surgery was effective in prolonging long-term survival.Conclusions NICT is rare and it had no specific clinical presentation.BUS and CT are useful for diagnosis and surgery is effective.

Therapeutic potential of sunitinib in the treatment of malignant islet cell tumor / 中华内分泌代谢杂志

Malignant islet cell tumor, a rare type of neuroendocrine carcinoma, biologically behaves in an aggressive way and is difficulty to be treated. Sunitinib malate, a novel tyrosine kinase inhibitor, demonstrates a high efficacy in treating malignant islet cell tumor as shown by promising results in recent trials.

Non-Functioning, Malignant Pancreatic Neuroendocrine Tumor in a 16-Year-old Boy: A Case Report

We report the case of a 16-year-old boy with a solid pancreatic mass which proved to be a nonfunctioning, malignant pancreatic neuroendocrine tumor (PNET). In pediatric patients, malignant pancreatic tumors are rare, especially malignant PNET. When dynamic contrast enhanced MRI showed a well enhancing solid pancreatic tumor on arterial and delayed phases and combined with malignant features, such as vascular invasion, invasion of adjascent organs, and lymphadenopathy, we should include malignant pancreatic neuroendocrine tumor in the differential diagnosis of childhood pancreatic tumors.

Clinical Analysis of Insulinoma / 대한내분비외과학회지

PURPOSE: Insulinoma is a rare disease for which early diagnosis followed by proper surgical management provides a chance for cure. Analyses of clinicopathological features of patients can help optimize the surgical approach in the treatment of insulinoma. METHODS: The records of 13 patients (seven male, six female mean age 44.3 years; age range 17~62 years) who were diagnosed clinically and pathologically with insulinoma and who underwent surgery between March 1997 and April 2007 at the Department of Surgery, Seoul National University Hospital. Hospital in English please were retrospectively examined. RESULTS: All patients had findings compatible with Whipple's triad. Mean fasting blood sugar was 40.5 mg/dl, serum insulin level was 33.5µU/ml, and insulin-to-glucose ratio was 0.6. A prolonged starvation test was performed on six patients. Tumors were localized in 10 patients with a computed tomography (CT) scan and in three patients with CT angiography. Five tumors were located in the pancreas head and uncinate process, five in the body, and four at the body-tail border and tail. Patients underwent resection of tumorby enucleation, distal pancreatectomy, pylorus-preserving pancreaticoduodenectomy, laparoscopic distal pancreatectomy, and duodenum-preserving resection of pancreas head. Four immediate postoperative complications (fluid collection, pancreatic fistula, delayed gastric emptying) occurred. No symptoms or recurrences were apparent during the median 15 month follow-up. CONCLUSION: Insulinoma is difficult to diagnose correctly without a prolonged duration of symptoms. Localization of insulinoma can be aided by a CT scan and/or CT angiography. Less aggressive operative procedures such as simple enucleation might be a sufficient and feasible procedure for curative resection of benign insulinomas.

A Case of Gastrointestinal Stromal Tumor with Recurrent Hypoglycemia

Non-islet cell tumor induced hypoglycemia (NICTH) is attributable to overproduction of insulin-like growth factor-II (IGF-II) by solid tumors, and these tumors usually originate from mesenchymal or epithelial cells. Gastrointestinal stromal tumor (GIST) is a rare mesenchymal tumor and most commonly find in the gastrointestinal tract. It is usually expresses the CD117 (stem cell factor receptor, c-kit) detected by immunohistochemistry. Hypoglycemia associated with GIST is very rare and this has not yet been reported in Korea. A 72-year-old man was hospitalized due to frequent episodes of confusion. It was observed that non-hyperinsulinemic hypoglycemia, an elevated serum IGF-II level and a huge liver mass. The histology of liver mass showed c-kit (CD117) positivity, which was consistent with GIST, but it was surgically unresectable. He was treated with imatinib mesylate. Although he recieved palliative treatment, he still experienced intermittent fasting hypoglycemia. After 2 months, the serum IGF-II level was even higher than before. We changed imatinib mesylate to sunitinib malate and performed radiotherapy on the liver mass. Although the change of the liver mass was not significant, he did not suffer from hypoglycemia for three months afterwards.

A Case of Non-islet Cell Tumor Hypoglycemia / 대한내분비학회지

Mesenchymal tumors including hemangiopericytomas, hepatocellular tumors, adrenal carcinomas, and a variety of other large tumors have been reported to produce excessive amounts of insulin-like growth factor (IGF) type II precursor, which binds weakly to insulin receptors and strongly to IGF-I receptors, leading to insulin like actions. In addition to increased IGF-II production, IGF-II bioavailability is increased due to complex alterations in circulating binding proteins. The authors of this article diagnosed non-islet cell tumor hypoglycemia from an 81-year-old male patient suffering from repetitive fasting hypoglycemia while he has not received any treatment for pulmonary hemangiopericytoma diagnosed in the past. Moreover, this topic is getting reported as the authors have experienced a significant improvement of catamnesis by a treatment with glucocorticoid.

A Case of Pancreatic Somatostatinoma / 대한소화기학회지

Somatostatinoma is a rare neoplasm usually arising from the pancreas and duodenum which typically presents with indolent, nonspecific symptoms in the absence of systemic neuroendocrine manifestations that characterize somatostatinoma syndrome. It accounts for less than 1% of all gastrointestinal endocrine tumors with an annual incidence of 1 per 40 million. It is often associated with regional and/or portal metastasis at the time of diagnosis, and complete tumor resection is possible only in 60% to 70% of cases. We experienced a case of pancreatic somatostatinoma recently. A 51-year-old woman presented with right upper quadrant abdominal pain and loose stool for one month. A hypermetabolic lesion in the pancreatic head was detected on positron emission tomography-CT (PET-CT) scan. The tumor was resected by pylorus preserving pancreaticoduodenectomy. Immunohistochemical staining of the tumor tissue exhibited diffuse positivity for somatostatin, but was negative for insulin and glucagon. Herein, we report a case of pancreatic somatostatinoma diagnosed postoperatively.

Islet Cell Tumor Arising from Heterotopic Pancreas in the Duodenum: A Case Report

It is difficult to distinguish an islet tumor originating from heterotopic pancreas tissue from the other submucosal tumors. Although the malignant transformation of a heterotopic pancreas, including islet cell tumor, is extremely rare, it remains an important consideration in the differential diagnosis of duodenal submucosal masses. We have demonstrated the radiologic appearance and the clinical-pathologic findings of a highlighted, rare case of islet cell tumor arising from a heterotopic pancreas in the duodenal wall.

A case of gastric variceal bleeding associated with pancreatic endocrine tumor in the pancreas tail / 대한내과학회지

Isolated obstruction of the splenic vein leads to segmental portal hypertension, which is mainly originated from pancreatic disease, such as chronic pancreatitis, pancreatic pseudocyst, pancreatic cancer. The clinical manifestation are devoid of ascites and encephalopathy, but presented with gastric variceal bleeding. We experienced 27-year-old male patient who complained of hematochezia and melena. After work-up with CT and angiography, we performed operation with the impression of pancreatic tumor associated with splenic vein occlusion. It resulted nonfunctioning pancreatic endocrine tumor. Often called islet cell tumor, pancreatic endocrine tumor has various names according to secreted hormones, such as insulinoma, Zollinger-Ellison syndrome and glucagonoma. It was classified as nonfunctioning tumor if there were any evidence of hormone secretion by radioimmunoassay and immunohistochemistry. Surgical excision and adjuvant medical therapy is the mainstay of therapy. We performed tumor resection and splenectomy for control of gastric variceal bleeding due to splenic vein occlusion.

The effectiveness of EUS in detecting islet cell tumor / 中华消化内镜杂志

Objective To study on the effectiveness of endoscopic ultrasonography(EUS)in detec- ting insulinoma preoperatively.Methods Fifteen patients with clinical and biochemical signs of insulinoma were examined by EUS using a radial-scanning ultrasound endoscope and abdominal ultrasonography,CT, DSA prior to surgery.The outcome was evaluated on the basis of surgery and examination of the resected specimens.Results Fifteen patients with 16 lesions of insulinoma were identified by surgery and pathology. The aceuraey of diagnosis with EUS was 13/15(86.7%),and that with B-US,CT,DSA was 3/15(20%), 5/15(33.3%),9/14(64.3%)respectively.In the 14 lesions identified by EUS,10 lesions were depicted to be hypoechogenic,1 lesion was isoechogenic and 3 lesions were hyperechogenie.All 14 lesions were well demarcated and surrounded by normal pancreatic tissue.The minimum size of the lesion visualized by EUS was 0.5cm.Ten lesions were correctly detected by EUS with size of 0.5～2.0cm.EUS missed diagnosis in 2 lesions not for their small size.EUS falsely indicated a 10mm lesion from two lesions inside the head of pancreas.One lesion outside the pancreatic tail and one lesion in the pancreatic head were missed by EUS in another case.Conclusion EUS is superior in assessing the location of pancreatic insulinoma than other ima- ging methods such as B-US,CT,DSA.

Multimodality Treatment of Malignant Nonfunctioning Islet Cell Tumor of the Pancreas: A Case Report / 한국간담췌외과학회지

Nonfunctioning islet cell tumor of the pancreas is mostly malignant and has a poor prognosis. But, even in case of hepatic metastasis, the multimodality treatment including surgical resection, transhepatic arterial chemoembolization(TACE), radiofrequency interstitial tissue ablation(RITA), and systemic chemotherapy provides a better prognosis. We report a case of the multimodality treatment for malignant nonfunctioning islet cell tumor of the pancreas. The patient was 38-year old male whose diagnosis was made by routine health check-up. Abdominal computed tomography revealed about 20cm sized hypervascular mass in the tail of the pancreas. After preoperative selective arterial embolization for the purpose of reducing surgical bleeding, we explored his abdomen and found the mass originating from the tail of the pancreas and expanding to the spleen and transverse colon. We performed distal pancreatectomy combined with total gastrectomy, splenectomy, and transverse colectomy. Also, we found multiple bilobar hepatic metastatic nodules and performed the wedge resection for the lesion of the left lateral segment. Postoperatively, we underwent the TACE and the RITA for the remaining right hepatic metastatic nodules, then performed the systemic chemotherapy with interferon-alpha. At postoperative 22 months, the patient was alive with regular follow up of viable right hepatic metastatic nodules.

Pheochromocytoma with pancreatic islet cell tumor: a case report / 한국간담췌외과학회지

A 17-years old woman with a combination of unilateral pheochromocytoma and an asymptomatic islet cell tumor of the pancreas is presented. The unusual coincidence of pheochromocytoma and pancreatic islet cell tumor in the patient is of interest as a possible crossover between MEN I and MEN II. It has been suggested that MEN I represents an abnormality of the APUD(amine precursor uptake and decarboxylation) cells of ectodermal origin. However, the possibility of a common progenitor cell in the neural crest for all APUD cells has been suggested by Pearse and Polak(1971). Recent clinical reports suggest that overlap between the two syndroms may occur. The overlapping of elements of the classical endocrine neoplasia should alert clinicians to the possibility of such associations in any particular patients. Since islet cell tumors may occur in association with pheochromocytomas and may be clinically silent, exploration of the pancreas during surgery for pheochromocytomas would seem to be useful.

Hypoglycemia associated with the abnormal expression of insulin like growth factor-Ⅱ in a pleural solitary fibroma / 中华内分泌代谢杂志

High expressions of IGF Ⅱ mRNA and protein were demonstrated by RT PCR and IRMA analysis in tumor tissue of a patient with non islet cell tumor induced hypoglycaemia (NICTH). Northern blotting results displayed abundant 6.0, 5.3, 5.0, 4.8 kb IGF Ⅱ transcripts in the tumor. It suggests that autosecretive production of IGF Ⅱ of the tumor plays an important role in the pathogenesis of tumor induced hypoglycemia.

Solid and Papillary Neoplasm and Nonfunctioning Islet Cell Tumor: Differential Diagnosis and Study of Histopathogenesis by Immunohistochemical Staining

PURPOSE: Solid and papillary neoplasms and nonfunctioning islet cell tumors are both rare pancreatic tumors, and their clinical and pathological features are similar which makes it hard to differentiate between them. Because both tumors have different prognoses, it is important to have precise diagnosis. The etiology of solid and papillary neoplasm is not precisely known. The role of sexual hormone has been debated as this tumor occurs mostly in women. METHODS: We retrospectively reviewed the medical records of 13 patients with solid and papillary neoplasm and 11 patients with nonfunctioning islet cell tumors who had been treated by surgical resection between October 1994 and May 1999 at Samsung Medical Center. Immunohistochemical stainings were performed for neuron-specific enolase (NSE), chromogranin, somatostatin, alpha 1-antitrypsin, estrogen (ER), and progesterone (PR) receptors. RESULTS: The average ages of the patients with solid and papillary neoplasms and nonfunctioning islet cell tumors were 39.5 and 47.8 respectively. The male to female ratio was 2 to 11 and 6 to 5, respectively and solid and papillary neoplasms were more common in women. CT showed a cystic mass in 76.9% (10/13) of the solid and papillary neoplasm patients and 20% (2/10) of nonfunctioning islet cell tumor patients. Lymphadenopathy was noted in 0% (0/13) of the solid and papillary neoplasm cases and in 50% (5/10) of the nonfunctioning islet cell tumor cases, and calcifications were present in 46.2% (6/13) and 0% (0/10) of those cases, respectively. The solid and papillary neoplasms were located most commonly inthe tail of the pancreas (7 cases), and nonfunctioning islet cell tumors were located most commonly in the head of the pancreas (5 cases). No malignancies were detected in the solid and papillary neoplasms. Seven cases of the nonfunctioning islet cell tumors (63.6%) were malignant. Both solid and papillary neoplasms and nonfunctioning islet cell tumors stained positive for NSE and alpha 1-antitrypsin in all cases and they were chromogranin positive in 25% (3/12) and 100% (10/10) and somatostatin positive in 25% (3/12) and 60% (6/10) of the cases, respectively. A solid and papillary neoplasm stained positive for ER in 1 case and for PR in 5 cases. However, only 1 case of a nonfunctioning islet cell tumor stained positive for PR. CONCLUSION: A nonfunctioning islet cell tumor is more malignant tumor than a solid and papillary neoplasm, and age, presence of cysts, lymphadenopathy, calcification, and chromogranin staining can all be used for differential diagnoses of these tumors. Both the solid and papillary neoplasms and the nonfunctioning tumors are thought to originate from a stem cell capable of differentiating into endocrine cells. The sexual hormone seems to have a role in the development of solid and papillary neoplasms.

Pancreatic Islet Cell Tumor: Clinical Review of 12 Cases

BACKGROUND: Pancreatic islet cell tumors are very rare tumors that are classified into functioning, secreting hormone with specific symptoms, and nonfunctioning tumors without distinct symptoms. Because of their rarity, studies of them have been limitted. METHODS: We experienced 12 cases of pancreatic islet cell tumor at Samsung Seoul Medical Center from October 1994 to May 1998 and reviewed charts retrospectively. RESULTS: The mean age was 43.3 years; 46.3 years for those with functioning tumors. The most common symptom in nonfunctioning tumors was abdominal pain; other symptoms were abdominal mass, diabetes mellitus, and jaundice. With the functioning tumors, loss of conciousness and hypoglycemic sympathetic symptoms were present in all patients, and a seizure occurred in one case. The tumors were localized with abdominal CT in 91.7% (11 of 12), abdominal US in 87.5% (7 of 8), and selective angiography in 42.9% (3 of 7). The mean size was 1.7 cm in functioning tumors and 7.6 cm in nonfunctioning tumors. In 5 of the 6 cases, where the tumor was located in the pancreatic head, a pancreaticoduodenectomy was performed; enucleation was done in the other case. In the cases receiving a pancreaticoduodenectomy, choledochojejunostomy leakage was the only postoperative complication one case; there was a pancreatic pseudocyst in one case of enucleation. Of the functioning tumors, 3 (25%) were insulinoma; the remaining 9 (75%) tumors were nonfunctioning tumors. There were 4 cases (33.3%) of malignancies, and they were all nonfunctioning tumors. An immunohistochemical staining study showed evidence of multi-hormonal production in 45.5% of the cases, and all tumors showed a positive reaction to at least one hormonal marker. Two of the 3 insulinoma cases showed a positive reaction to insulin. Somatostatin was positive in 81.8% of the tumors, glucagon in 45.5%, and insulin in 18.2%. Chromogranin was positive in 71.4% of the tumors, synaptophysin in 60%, and neuron-specific enolase in 60%. CONCLUSION: There may be more cases of functioning tumors than have been reported and early diagnosis and treatment will reduce the incidence of malignancy. Abdominal CT or US is comparatively useful to localize the site of tumor and spiral CT angiography may be useful for the cases where nothing is detected with routine CT. Immunohistochemical staining is non-specific in classification of each islet tumor, but the positivity of any one of the hormonal markers may be useful in differentiating of pancreatic islet cell tumors from non-ilset cell tumors.