Pancreatic hemangioma manifesting as variceal gastroesophageal bleeding during pregnancy: case report

There are only 10 reported cases of pancreatic hemangiomas in adults, only one of them causing digestive bleeding. We present a case of variceal bleeding and portal hypertension caused by a pancreatic hemangioma. The patient had 19 year-old and was received at her 16th week of pregnancy. She had massive hematemesis, controlled after variceal band ligation. Her image exams revealed a cystic lesion of 164 cm³ in the pancreas tail and signs of portal hypertension. Two months after, the ultrassonographic exam documented the lesion growth, achieving 200 cm³ at that time. The patient was submitted to distal pancreatectomy, and the histopathological analysis revealed a pancreatic hemangioma of 11 x 9 x 8 cm. Therefore, we report the second case of digestive bleeding caused by a pancreatic hemangioma, which had a well documented growth during the pregnancy. Additionally, we review the previous reports of pancreatic hemangiomas and discuss the hypothesis of hormonal infl uence on the natural history of these tumors.

Multidetector CT Evaluation of the Postoperative Pancreas

Several pancreatic diseases may require surgical treatment, with most of these procedures classified as resection or drainage. Resection procedures, which are usually performed to remove pancreatic tumors, include pancreatoduodenectomy, central pancreatectomy, distal pancreatectomy, and total pancreatectomy. Drainage procedures are usually performed to treat chronic pancreatitis after the failure of medical therapy and include the Puestow and Frey procedures. The type of surgery depends not only on the patient's symptoms and the location of the disease, but also on the expertise of the surgeon. Radiologists should become familiar with these surgical procedures to better understand postoperative changes in anatomic findings. Multidetector computed tomography is the modality of choice for identifying normal findings after surgery, postoperative complications, and tumor recurrence in patients who have undergone pancreatic surgery. (C)RSNA, 2012 . radiographics.rsna.org

Hyperinsulinemic hypoglycemia of the infancy. Analysis of clinical data from a Brazilian sample

Objective: To review the presentation of hyperinsulinemic hypoglycemia of the infancy (HHI), its treatment and histology in Brazilian pediatric endocrinology sections. Materials and method: The protocol analyzed data of birth, laboratory results, treatment, surgery, and pancreas histology. Results: Twenty-five cases of HHI from six centers were analyzed: 15 male, 3/25 born by vaginal delivery. The average age at diagnosis was 10.3 days. Glucose and insulin levels in the critical sample showed an average of 24.7 mg/dL and 26.3 UI/dL. Intravenous infusion of the glucose was greater than 10 mg/kg/min in all cases (M:19,1). Diazoxide was used in 15/25 of the cases, octreotide in 10, glucocorticoid in 8, growth hormone in 3, nifedipine in 2 and glucagon in 1. Ten of the cases underwent pancreatectomy and histology results showed the diffuse form of disease. Conclusion: This is the first critic review of a Brazilian sample with congenital HHI. Arq Bras Endocrinol Metab. 2012; 56(9): 666-71

Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1

Surgical approaches to pancreatic endocrine tumors associated with multiple endocrine neoplasia type 1 may differ greatly from those applied to sporadic pancreatic endocrine tumors. Presurgical diagnosis of multiple endocrine neoplasia type 1 is therefore crucial to plan a proper intervention. Of note, hyperparathyroidism/multiple endocrine neoplasia type 1 should be surgically treated before pancreatic endocrine tumors/multiple endocrine neoplasia type 1 resection, apart from insulinoma. Non-functioning pancreatic endocrine tumors/multiple endocrine neoplasia type 1 >1 cm have a high risk of malignancy and should be treated by a pancreatic resection associated with lymphadenectomy. The vast majority of patients with gastrinoma/multiple endocrine neoplasia type 1 present with tumor lesions at the duodenum, so the surgery of choice is subtotal or total pancreatoduodenectomy followed by regional lymphadenectomy. The usual surgical treatment for insulinoma/multiple endocrine neoplasia type 1 is distal pancreatectomy up to the mesenteric vein with or without spleen preservation, associated with enucleation of tumor lesions in the pancreatic head. Surgical procedures for glucagonomas, somatostatinomas, and vipomas/ multiple endocrine neoplasia type 1 are similar to those applied to sporadic pancreatic endocrine tumors. Some of these surgical strategies for pancreatic endocrine tumors/multiple endocrine neoplasia type 1 still remain controversial as to their proper extension and timing. Furthermore, surgical resection of single hepatic metastasis secondary to pancreatic endocrine tumors/multiple endocrine neoplasia type 1 may be curative and even in multiple liver metastases surgical resection is possible. Hepatic trans-arterial chemo-embolization is usually associated with surgical resection. Liver transplantation may be needed for select cases. Finally, pre-surgical clinical and genetic diagnosis of multiple endocrine neoplasia type 1 syndrome and localization of multiple endocrine neoplasia type 1related tumors are crucial for determining the best surgical strategies in each individual case with pancreatic endocrine tumors.

Streptozotocin-induced diabetes mellitus in pigs

We induced, as a precondition for a pancreas transplant, insulin-dependent diabetes mellitus in 67 Yorkshire Landrace pigs by administering streptozotocin. A dosage of 150 mg/kg body weight gave rise to a long-lasting diabetes mellitus that persisted with time (follow-up period: 26 weeks). Consecutive measurements of serum glucose and plasma insulin, before and up to 30 hours after administering streptozotocin, revealed triphasic behavior: initial hyperglycemia (1st to 3rd hour), pronounced hypoglycemia (12th to 18th hour), then hyperglycemia (22nd hour on). IVGTTs done 1 to 7 days after administering streptozotocin revealed a reduction of the K-value (glucose disappearance rate) from 0.3 (day 2) to 0.07 (day 4). Immunohistochemical studies revealed a complete loss of all beta-cells, concomitantly with a relative increase in glucagon- and somatostatin-positive cells. We also observed a complete loss of pp (pancreatic polypeptide)-positive cells. Diabetes induced by streptozotocin at 150 mg/kg body weight is complete and permanent; our mortality rate was 0%. Given the high morbidity rate after pancreatectomy, streptozotocin should be the method of choice for inducing diabetes mellitus in pigs.

Long-term survival after multidisciplinary management of resected pancreatic adenocarcinoma.

INTRODUCTION: Actual 5-year survival rates of 10-18% have been reported for patients with resected pancreatic adenocarcinoma (PC), but the use of multimodality therapy was uncommon in these series. We evaluated long-term survival and patterns of recurrence in patients treated for PC with contemporary staging and multimodality therapy. METHODS: We analyzed 329 consecutive patients with PC evaluated between 1990 and 2002 who underwent resection. Each received a multidisciplinary evaluation and a standard operative approach. Pre- or postoperative chemotherapy and/or chemoradiation were routine. Surgical specimens of 5-year survivors were re-reviewed. A multivariate model of factors associated with long-term survival was constructed. RESULTS: Patients underwent pancreaticoduodenectomy (n = 302; 92%), distal (n = 20; 6%), or total pancreatectomy (n = 7; 2%). A total of 108 patients (33%) underwent vascular reconstruction, 301 patients (91%) received neoadjuvant or adjuvant therapy, 157 specimens (48%) were node positive, and margins were microscopically positive in 52 patients (16%). Median overall survival and disease-specific survival was 23.9 and 26.5 months. Eighty-eight patients (27%) survived a minimum of 5 years and had a median overall survival of 11 years. Of these, 21 (24%) experienced recurrence, 7 (8%) after 5 years. Late recurrences occurred most frequently in the lungs, the latest at 6.7 years. Multivariate analysis identified disease-negative lymph nodes (P = .02) and no prior attempt at resection (P = 0.01) as associated with 5-year survival. CONCLUSIONS: Our 27% actual 5-year survival rate for patients with resected PC is superior to that previously reported, and it is influenced by our emphasis on detailed staging and patient selection, a standardized operative approach, and routine use of multimodality therapy.

Protection of nonobese diabetic mice from autoimmune diabetes by reduction of islet mass before insulitis.

Nonobese diabetic mice spontaneously develop diabetes that is caused by autoimmune cell-mediated destruction of pancreatic beta cells. Here we report that surgical removal of 90% of pancreatic tissue before onset of insulitis induced a long-term diabetes-free condition in nonobese diabetic mice. Pancreatectomy after development of moderate insulitis had no effect on the course of diabetes. The effect of pancreatectomy was abrogated with subsequent development of diabetes by infusion of islet-cell-specific T lymphocytes and by transplantation of pancreatic islets. Lymphocytes from pancreatectomized diabetes-free mice exhibited low response to islet cells but responded normally to alloantigens. These results suggest that the islet cell mass plays a critical role in development of autoimmune diabetes.

The outcome in Australian children with hyperinsulinism of infancy: early extensive surgery in severe cases lowers risk of diabetes

AIMS Hyperinsulinism of infancy (HI) is characterized by unregulated insulin secretion in the presence of hypoglycaemia, often resulting in brain damage. Pancreatic resection for control of hypoglycaemia is frequently resisted because of the risk of diabetes mellitus (DM). We investigated retrospectively 62 children with HI from nine Australian treatment centres born between 1972 and 1998, comparing endocrine and neurological outcome in 28 patients receiving medical therapy alone with 34 who required pancreatic resection to control their hypoglycaemia. METHODS History, treatment and clinical course were ascertained from file audit and interview. Risk of DM (hazard ratio) attributable to age at surgery (< vs. greater than or equal to 100 days at last pancreatectomy) and extent of resection (< vs. greater than or equal to 95%) were calculated using Cox proportional hazards regression and categorical variables compared by the chi(2) -test. Neurological outcome (normal, mild deficit or severe deficit) was derived from the most authoritative source. RESULTS Surgically treated patients had a greater birthweight, earlier presentation and higher plasma insulin levels. Of 18 infants < 100 days and 16 greater than or equal to 100 days of age at surgery, four (all greater than or equal to 100 days) became diabetic as an immediate consequence of surgery and five (two < 100 days and three greater than or equal to 100 days) became diabetic 7-18 years later. Surgery greater than or equal to 100 days and pancreatectomy greater than or equal to 95% were associated with development of diabetes (HR = 12.61, CI 1.53-104.07 and HR = 7.03, CI 1.43-34.58, respectively). Neurodevelopmental outcome was no different between the surgical and medical groups with 44% overall with neurological deficits. Patients euglycaemic within 35 days of the first symptom of hypoglycaemia (Group A) had a better neurodevelopmental outcome than those still hypoglycaemic > 35 days from first presentation (Group B) (P = 0.007). Prolonged hypoglycaemia in Group B was due either to delayed diagnosis or to need for repeat surgery because of continued hypoglycaemia. Within Group A, medically treated patients (who presented later with apparently milder disease) had a higher incidence of neurodevelopmental deficit (n = 15, four mild, three severe deficit) compared with surgically treated patients (n = 18, two mild, none severe deficit) (P < 0.025). CONCLUSIONS Poor neurodevelopmental outcome remains a major problem in hyperinsulinism of infancy. Risk of diabetes mellitus with pancreatectomy varies according to age at surgery and extent of resection. Patients presenting early with severe disease have a better neurodevelopmental outcome and lower risk of diabetes if they are treated with early extensive surgery.

Neoplasia papilar cística do pâncreas

Papyllary cystic tumor of the pancreas, so-called Frantz’s tumor, is rare. Clinical presentation of this disease is usually a slowly growing abdominal mass with or without abdominal pain, affecting predominantly young females. Its pathogenesis is still unknown . Surgical resection is usually curative, and prognosis is excellent. The authors report two pancreatic tumor cases(Frantz’s tumor) in women aged 26 and 31 years old. Pre operative assessment showed a solid-cystic tumor of the tail and body of the pancreas. An extended distal pancreatectomy was performed without splenic preservation

Pancreatic large mucinous cystoadenoma with invasive ductal carcinoma in pregnancy. Case report

Background. Cystic tumour of the pancreas are infrequent and malignancy of the pancreas during pregnancy is extremely rare. Mucinous cystoadenomas is the most frequent cystic pancreatic neoplasm and it is seen mainly in women suggesting a sex hormone influence. Its presentation during pregnancy is extremely rare and entails difficulties in diagnosis and therapy. Case report. A 28 year old woman was referred to our service for abdominal mass. She had given birth to her second child two weeks previously. Ultrasound and CT scan showed a large cystic lesion, with sepitation and inner solid growth portions, involved mainly the left sovramesocolic space. An ultrasound-guided aspiration of the cystic fluid showed high level of CEA and CA. 19-9. The patient underwent laparotomic body-tail pancreatectomy and splenectomy. The histological examination showed mucinous cystoadenoma with associated invasive ductal carcinoma, with ovarian-like stroma and a well delimited fibrous capsule. Hystochemical study revealed a strong positivity for progesterone receptors. Conclusions. To our knowledge this is the eighth case of mucinous cystoadenoma reported in English literature and the forth with an invasive adenocarcinoma associated. This pathological entity should always be kept in mind in case of patient with an hepigastric mass during or soon after pregnancy. Aggressive approach is mandatory.

Neoplasia papilar cística do pâncreas

Papyllary cystic tumor of the pancreas, so-called Frantz’s tumor, is rare. Clinical presentation of this disease is usually a slowly growing abdominal mass with or without abdominal pain, affecting predominantly young females. Its pathogenesis is still unknown . Surgical resection is usually curative, and prognosis is excellent. The authors report two pancreatic tumor cases(Frantz’s tumor) in women aged 26 and 31 years old. Pre operative assessment showed a solid-cystic tumor of the tail and body of the pancreas. An extended distal pancreatectomy was performed without splenic preservation

Hyperinsulinaemic hypoglycaemia - a diagnostic challenge. A report of two atypical cases

Objectives: The authors describe 2 atypical cases of patients with hypoglycaemia, suspected for insulinoma. Methods: The 2 reports are accompanied by a concise review of the literature. Results: Patient 1 had a distal pancreatectomy performed for suspected insulinoma, and was diagnosed with a glucagonoma and beta-cell hyperplasia (nesidioblastosis). To the authors’s knowledge, co-existing glucagonoma and nesidioblastosis had not been previously reported. Patient 2 was diagnosed with a benign insulinoma and 5 years later with metastatic disease. Conclusion: The authors conclude that insulinomas are rare entities which often present a diagnostic and therapeutic challenge. In such cases, patient referral to tertiary multidisciplinary centers is recommended.

Insulinoma em furões

Dissertação de Mestrado Integrado em Medicina Veterinária