Pancreatic Tumors In Children Research Articles

P-228 - Surgical treatment of pancreatic tumors in children

P-228 - Surgical treatment of pancreatic tumors in children

Laparoscopic Resection of Pancreatic Tumors in Children: Results of a Multicentric Survey.

This study aimed to report the results of a multicentric survey about laparoscopic treatment of pancreatic tumors in children. The data of patients operated using minimally invasive surgery (MIS) for a pancreatic tumor in 5 International centers of Pediatric Surgery in the last 5 years were retrospectively reviewed. We recorded data relating to the clinical presentation, diagnostic evaluation, surgical technique, and outcome. Fifteen patients (average age 2.2 years) were identified. The most common symptoms at presentation were related to the hypoglycemic hyperinsulinism, followed by abdominal pain and vomiting. Tumor types were insulinoma (n = 4), congenital hyperinsulinism of infancy (CHI) diffuse type (n = 3), CHI focal type (n = 3), solid pseudopapillary tumor (n = 2), and cystic malformation (n = 3). The diagnostic assessment was completed using ultrasound associated with computed tomography (CT) scan in all centers; 18FDOPA positron emission tomography in combination with CT was adopted in 2 centers. The MIS procedures performed were as follows: tumor enucleation (n = 4), distal pancreatectomy (n = 8), subtotal pancreatectomy (n = 2), and pancreatico-jejunostomy (n = 1). Average operative time was 110 minutes. As for postoperative complications, we recorded 1 persistent hypoglycemia, requiring redo-surgery (IIIb Clavien-Dindo) and 1 thrombosis of splenic vein, not requiring any treatment (I Clavien-Dindo). Laparoscopic resection can be considered a safe and effective treatment with minimal morbidity and excellent outcomes for most pediatric pancreatic tumors. Suspension of the stomach with a transparietal stitch and use of new hemostatic devices as Starion TLS3 or Ligasure are key factors for the success of the procedure. A long-term follow-up is mandatory in these patients to evaluate postoperative complications and long-term outcome.

Pancreatoblastoma in a child

Pancreatoblastoma (PB) is a rare pancreatic tumor in children. Its biologic behavior is aggressive with frequent local invasion, recurrence, and metastasis, for which there has been no standard treatment regimen. Complete surgical resection has been considered for long-term survival of patients with PB. We present here a case of PB in a 3-year-old male child.

Pancreatic tumors in children and young adults with tuberous sclerosis complex.

Pancreatic neuroendocrine tumors are not included in the diagnostic criteria for tuberous sclerosis complex, although an association has been described. To investigate the association of pancreatic neuroendocrine tumor in children and young adults with tuberous sclerosis complex and define MRI characteristics of the tumor. We retrospectively evaluated the abdominal MRI scans of 55 children and young adults with tuberous sclerosis complex for the presence of a pancreatic mass. The scans were performed over a period of 7years to monitor renal pathology. We obtained each patient's clinical history and treatment protocol from the hospital's electronic medical records. A solid pancreatic mass was identified in 5/55 (9%, 95% confidence interval [CI] 3-20%) patients (4 male) with a mean age of 12.6years. Four of the lesions were located in the pancreatic tail and one in the pancreatic body. All of the lesions were solid, ovoid and well demarcated, with a mean diameter of 3.1cm. The masses uniformly demonstrated T1 and T2 prolongation, but their diffusion behavior and post-contrast enhancement varied. The two surgically resected lesions were synaptophysin (+) non-functional pancreatic neuroendocrine tumors on pathology. Two of the patients who did not have surgery were treated with everolimus; one of the lesions has shown interval decrease in size and the other has remained stable. Pancreatic tumor is relatively common in children and young adults with tuberous sclerosis complex.

Pancreatic surgery for tumors in children and adolescents.

Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies. We conducted a retrospective review of patients ≤21years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015. Nineteen patients were identified with a median age at operation of 16.6years (IQR 13.5-18.9). The most common histology was solid pseudopapillary neoplasm (SPN) (n=13), followed by pancreatic neuroendocrine tumor (n=3), serous cystadenoma (n=2) and pancreatoblastoma (n=1). Operative procedures included formal pancreatectomy (n=17), enucleation (n=1) and central pancreatectomy (n=1). SPNs were noninvasive in all but one case with perineural, vascular and lymph node involvement. Seventeen patients (89.5%) are currently alive and disease free at a median follow-up of 5.7 (IQR 3.7-10.9)years. Two patients died: one with metastatic insulinoma and another with SPN who developed peritoneal carcinomatosis secondary to a concurrent rectal adenocarcinoma. Pediatric pancreatic tumors are a heterogeneous group of neoplastic lesions for which surgery can be curative. SPN is the most common histology, is characterized by low malignant potential and in selected cases can be safely and effectively treated with a tissue-sparing resection and minimally invasive approach.

A method of reconstruction after pancreaticoduodenectomy for pancreatic malignancies in very young children: Two cases reports

Abstract Pancreatic tumors are very rare diseases in very young children. Most information about those diseases in children was published in cases reports. Due to the rare nature of pancreatic tumors in children, there remains the absence of diagnostic algorithms, clear radiographic and morphological assessments as well as evidence based best treatment options. Because of the young age of patients and the rare occurrence of pancreatic neoplasms, tumor detection remains poor. For malignancies affecting the head of the pancreas the only possibility for achieving clear surgical margins is performing a pancreaticoduodenectomy (PD). We describe two cases of diagnostic and treatment of pancreatic tumor of very young children what was done in our institute.

Pancreatic tumours in children: diagnosis, treatment and outcome.

Pancreatic tumours in children are exceedingly rare and as a result constitute a diagnostic and therapeutic challenge to paediatric surgeons. We reviewed our experience with these rare entities. Retrospective single institution study on all paediatric pancreatic tumours over a period of 38 years (1973-2011) and literature review. We recorded data relating to the clinical features at presentation, diagnostic evaluation, treatment and outcome. Fourteen patients were identified (eight male). The most common symptoms at presentation were abdominal pain, anorexia and vomiting. Two cases were discovered incidentally. There were 12 primary and 2 metastatic tumours. Tumour types were solid pseudopapillary neoplasm (n = 6), insulinoma (n = 3), pancreatoblastoma (n = 1), congenital pancreatic cyst (n = 1), Burkitt lymphoma of the pancreas (n = 1) and metastatic lesions of other primary tumours (n = 2). Twelve were treated with surgical resection including 2 laparoscopically. Post-surgical complications included acute pancreatitis (n = 2) and pancreatic pseudocyst (n = 2). There was one death from a metastatic tumour and treatment is ongoing in one patient. The remainder has survived. Paediatric pancreatic tumours are rare entities and are usually benign. Clinical symptoms are often non-specific and presentation may be late due to tumour inactivity in case of endocrine neoplasms. For most tumours, surgical resection is the optimal treatment which may be successfully performed laparoscopically when the lesion is in the body or tail of the pancreas. Long-term outcome is generally good.

Single-incision laparoscopic excision of pancreatic tumor in children

PurposeSingle-incision laparoscopic surgery (SILS) as a surgical approach in treatment of pancreatic disease has recently been reported in adults. However, its application in pancreatic surgery in children is limited. In this article, we report our preliminary experience of SILS in children with pancreatic disease. MethodsThree children with pancreatic tumor underwent single-incision laparoscopic partial pancreatectomy between July 2011 and August 2013. Two of three children were girls, and one was a boy. The ages ranged from 2 to 10months, with an average age of 6.7months. ResultsAll operations were successfully performed. There was no conversion to the conventional multi-incision surgery. The mean operation time of the 3 cases was 153.3minutes (range 120–200 minutes). The postoperative hospital stay was 7days. The drainage tubes were kept for 3 to 4days after surgery. There was no pancreatic juice leak in this case series. All patients were followed up and there was no recurrence. ConclusionsSingle-incision laparoscopic partial pancreatectomy for children with pancreatic tumor is feasible.

Imaging features of primary malignant pancreatic tumors in children.

The purpose of this article is to describe the sonographic, CT, and MRI features of primary malignant pancreatic tumors of childhood. Most children with a pancreatic tumor present with a solid pseudopapillary tumor that is usually well marginated and has solid and cystic areas surrounded by a fibrous capsule. Pancreatoblastoma is more aggressive than solid pseudopapillary tumor. The imaging features are those of a large heterogeneous tumor. Ductal adenocarcinoma is rare in children and has a poor prognosis.

Insulinoma in childhood: clinical, radiological, molecular and histological aspects of nine patients

Insulinomas are a rare cause of hyperinsulinaemic hypoglycaemia (HH) in children. The clinical features, investigations, management and histology of these rare pancreatic tumours in children have not been described in a large cohort of patients. We conducted a retrospective review of cases diagnosed between 2000 and 2012, presenting to two referral centres in the United Kingdom. Clinical, biochemical, imaging (magnetic resonance imaging (MRI) and 6-L-¹⁸F-fluorodihydroxyphenylalanine (¹⁸F-DOPA) PET/CT scanning) and histological data were collected. Nine children (age range 2-14.5 years) were diagnosed during the study period at Great Ormond Street Hospital (n=5) and Royal Manchester Children's Hospital (n=4). The combination of abdominal MRI scan (7/8) and ¹⁸F-DOPA PET/CT scan (2/4) correctly localised the anatomical location of all insulinomas. Before surgery, diazoxide therapy was used to treat hypoglycaemia, but only four patients responded. After surgical resection of the insulinoma, hypoglycaemia resolved in all patients. The anatomical localisation of the insulinoma in each patient was head (n=4), uncinate process (n=4) and tail (n=2, one second lesion) of the pancreas. Histology confirmed the diagnosis of insulinoma with the presence of sheets and trabeculae of epithelioid and spindle cells staining strongly for insulin and proinsulin, but not for glucagon or somatostatin. Two children were positive for MEN1, one of whom had two separate insulinoma lesions within the pancreas. We describe a cohort of paediatric insulinoma patients. Although rare, insulinomas should be included in the differential diagnosis of HH, even in very young children. In the absence of a single imaging modality in the preoperative period, localisation of the tumour is achieved by combining imaging techniques, both conventional and functional.

Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital

Pancreatic tumors are rare in children, resulting in limited information regarding their frequency and imaging appearances. To review all pancreatic tumors seen in children over a decade at a large tertiary pediatric institution and to describe multimodality imaging findings. We conducted a retrospective review of imaging studies performed in children with pancreatic tumors between January 2000 and December 2012, analyzing imaging features on available US, CT and MR examinations. We reviewed patient charts for clinical features, management and final diagnosis. We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage. Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.

Pancreatoblastoma with portal vein involvement in a child: A case report

Pancreatoblastoma is a rare pancreatic tumor in children. Its biologic behavior is aggressive with frequent local invasion, recurrence, and metastasis , for which there has been no standard treatment regimen. A 13-year old Japanese girl had pancreatoblastoma arising from the pancreas head and body and invading the portal vein. The tumor was resected with pancreaticoduodenectomy combined with reconstruction of the portal and splenic vein using native left common iliac vein graft, followed by chemotherapy. The tumor recurred in the liver and periportal lymph node but was treated with surgical resection and chemotherapy followed by autologous peripheral blood stem cell transplantation. The reconstructed portal vein has been patent with no sign of stricture for 3.5 years after the first operation. She has been well with a second complete remission for 10 months. Complete surgical resection has been considered for long-term survival of patients with pancreatoblastoma. Portal venous involvement is not a contraindication of surgical resection of pancreatic tumors and may be aggressively treated by pancreaticoduodenectomy with portal venous reconstruction using a venous conduit. Autologous peripheral blood stem cell transplantation has been used in only two other pediatric cases in the literature for treating pancreatoblastoma. Although our follow-up period is short, aggressive surgical and chemotherapeutic regimen may hold therapeutic promise for managing advanced or relapsed pancreatoblastoma in children.

Primary malignant pancreatic neoplasms in children and adolescents: A 20 year experience

BackgroundMalignant pancreatic neoplasms in children and adolescents are rare. The clinical presentation, pathologic characteristics, management, and outcomes at two institutions are discussed. MethodsWe retrospectively reviewed all pediatric patients (age<= 18years) treated for malignant pancreatic neoplasms at two institutions between 1991 and 2011. ResultsThirty-one patients were identified with median age of 14.7years (4–18years). The most common histology was solid pseudopapillary tumor (SPT) (n=22, 71%) followed by neuroendocrine tumors (n=4, 13%), pancreatoblastoma (n=4, 13%), and one unclassified spindle cell neoplasm (3%). Most patients presented with abdominal pain (n=22, 71%). Complications included pancreatic leak, pseudocyst formation, pancreatitis, pancreatic insufficiency, and small bowel obstruction. The overall 1- and 5-year survival was 96% (95% CI 74%–99%) and 78% (95% CI 43%–93%). Median follow-up among patients alive at the end of follow-up was 20months (<1month–16.2years). Patients with SPT had better overall survival compared to patients with neuroendocrine tumors or pancreatoblastomas (Log-rank; p=0.0143). ConclusionThe majority of pediatric and adolescent patients present with SPTs which are usually resectable and associated with an excellent prognosis. Other histologic subtypes more often present with distant metastases and portend a worse prognosis.

Malignant pancreatic tumors in children: a single-institution series

BackgroundPancreatic tumors in children represent a very rare entity. We reviewed the clinical and pathologic features of pediatric patients with pancreatic tumors at a single institution. MethodsWe conducted a retrospective review of cases diagnosed at the Hospital for Sick Children between 1986 and 2010. ResultsTwenty-seven patients were diagnosed during the study period: 18 with solid pseudopapillary neoplasm (SPN), 6 with pancreatic endocrine neoplasia, and 3 with other tumors. Of the 27 children, 3 had associated syndromes, in specific tuberous sclerosis, von Hippel-Lindau, and polycystic ovarian syndrome. The most common symptoms were pain and vomiting in SPN and hypoglycemia and seizures in insulinomas. Magnetic resonance imaging and computed tomographic scan were equally accurate in determining size and site of origin of the tumor, and both were better than ultrasound. All patients underwent surgery (distal pancreatectomy in 14 cases, Whipple procedure in 8, other procedures in 5), which represented the only treatment for all but 3 cases. Seventeen patients (94%) with SPN are alive (median follow-up, 32 months), 3 of whom had positive margins. Two patients died: 1 male with SPN with malignant transformation and 1 with fibrosarcoma. ConclusionsWe describe the largest single-institution study of pediatric pancreatic tumors. Females with SPN have an excellent outcome, even in presence of positive margins, suggesting that limited surgical resection may be appropriate for these patients.

EUS-FNA for a Pancreatic Neuroendocrine Tumor in a Four-Year-Old Daughter of a Woman Exposed to Radiation at Chernobyl

Pancreatic neoplasms in children are rare. Herein is reported the case of a four-year-old girl whose mother was exposed to radiation at Chernobyl that presented with obstructive jaundice and a mass suspected on CT and diagnosed by endoscopic ultrasound (EUS) with fine needle aspiration (FNA). This child is probably the youngest case of application of linear EUS with biopsy to be described. The diagnosis, management, and followup of children with this rare tumor are discussed.

Malignant Pancreatic Tumors in Children and Young Adults: Evaluation of 228 Patients Identified through the Surveillance, Epidemiology, and End Result (SEER) Database

Malignant pancreatic tumors are rare in young patients, few epidemiologic data are available. We reviewed prognostic factors and outcome of 228 patients <30 years with malignant pancreatic tumors identified through the U.S. National Cancer Institute's SEER (Surveillance, Epidemiology, and End Results) Public-use Database from 1973 to 2004. Cases were grouped using the ICD-O-3. 5-year overall survival (OAS) was assessed by gender, ethnicity, SEER stage, and 5-year age intervals using univariate and Cox regression analysis. 228 patients with malignant pancreatic tumors were identified, resulting in an incidence of 0.46/million (100 carcinomas, 85 endocrine tumors, 8 solid pseudopapillary neoplasms (SPN), 11 pancreatoblastomas) in the USA. OAS was worse in males than females (37% vs. 55%, p=0.005). OAS according to stage was 87%, 68%, 21% for local (n=54), regional (n=42), distant metastatic disease (n=108), respectively. OAS of patients with carcinoma was 33%, endocrine tumors 58%, SPNs 88%, pancreatoblastomas 66%. Cox regression revealed stage (p=< 0.001), histology (p=< 0.001), age group (p=0.05) to be independent prognostic factors. Malignant pancreatic tumors are extremely rare in children and young adults. Entities change over the age groups towards more carcinomas with worse outcome in older patients. Tumor stage, histology and age group are important predictors for outcome. International collaboration is needed to learn more about pediatric pancreatic tumors.

Surgical Treatment of Pancreatic Tumors in Childhood and Adolescence: Uncommon Neoplasms with Favorable Outcome

Background/Aims: Pancreatic tumors in children and adolescents are uncommon. The aim of the present paper was to analyze short- and long-term outcomes after surgical treatment of primary pancreatic neoplasms in children and adolescents at a single high-volume center for pancreatic diseases. Methods: Retrospective review of medical records and pathology reports of patients younger than 18 years who underwent surgery at Verona University Hospital from 1990 through 2010. Results:The study population consisted of 20 patients. Abdominal pain and palpable mass were the most common presenting symptoms. No patient had a locally advanced, unresectable or metastatic disease. Complete resection (R0) was achieved in 19 patients. There was no postoperative mortality, but postoperative complications occurred in 5 cases (25%). Histological examination showed 12 solid pseudopapillary tumors, 5 neuroendocrine tumors, 2 cystadenomas and 1 epithelial malignant tumor. At a median follow-up of 49.5 months (range: 7–234), there was no tumor recurrence. Postoperative diabetes was diagnosed in 1 patient and 4 other patients developed pancreatic exocrine insufficiency. Conclusion: In the setting of a high-volume surgical center, radical resection of pancreatic tumors in children and adolescents is associated with acceptable postoperative morbidity and favorable long-term outcome.

Cushing syndrome due to ectopic adrenocorticotropic hormone secretion in a 3-year-old child

Ectopic adrenocorticotrophic hormone (ACTH) secretion is a rare cause of Cushing syndrome in paediatric age, due to tumours arising from different tissues. To date, only 11 reports of ACTH-secreting pancreatic tumours in children and adolescents exist in the literature. We present a paediatric case of Cushing syndrome caused by ectopic ACTH secretion. This was caused by a large acinar cell carcinoma that developed in the pancreas of a 3-year-old girl.

Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy

Pancreatoblastoma (PB) is a rare malignant pancreatic tumor in children and approximately 200 cases have been reported in the literature. The overall 5-year survival rate in PB is 43-50% and no standard treatment for PB has been established. This report presents the case of a 6-year-old female with advanced PB treated successfully by a pylorus-preserving pancreatoduodenectomy (PPPD) after induction chemotherapy, radiation and stem cell transplantation (SCT). A 6-year-old girl was hospitalized for abdominal pain, fever, and vomiting. Abdominal computed tomography (CT) scan showed a 9-cm heterogeneous mass located at the pancreatic head and body, and the duodenum was completely compressed. The inferior vena cava, superior mesenteric artery, and vein were encased by the tumor. The tumor had well-defined margins and calcification. She showed severe anemia and her hemoglobin level was 4.0 g/dl, and the serum alpha-fetoprotein (AFP) level was elevated (884.8 ng/ml). Initially, a resection of the tumor was impossible. An open biopsy was performed and the histopathological diagnosis was PB. She underwent five cycles of the induction chemotherapy regimen for advanced neuroblastoma (cyclophosphamide, etoposide, vincristine, pirarubicin and cisplatin), and the tumor size was decreased to a diameter of 7.5 cm. Furthermore, chemotherapy with irinotecan and vincristine, radiotherapy (40 Gy) and SCT (etoposide, carboplatin, melphalan) was administered. The serum AFP level decreased to 41.1 ng/ml, and the tumor size was decreased to a diameter of 6.5 cm. Then she underwent a PPPD and the tumor was completely resected. The patient's recovery was uneventful, and the AFP returned to the normal values (6.2 ng/ml) after surgery. The child was administered mild postoperative chemotherapy using irinotecan and has been disease-free for 4 months and, and her serum AFP levels remain within normal values. This is the first case of PB that was treated with SCT effectively before surgery. The combined therapy including the intensive chemotherapy with SCT and the radiation followed by surgical treatment is thought to be effective for the treatment of advanced PB.

Pancreatoblastoma: Two Case Reports From a Medical Center in Taiwan

Although pancreatoblastoma (PB) is an extremely rare tumor, it is the most common pancreatic tumor in children. We reported 2 cases of PB treated at a medical center in Taiwan. One was a 3.5-year-old boy who presented with abdominal pain. Physical examination demonstrated abdominal masses. Multiple pancreatic and hepatic masses were noted by magnetic resonance imaging. He underwent surgical resection but tumor could not be removed completely, which was confirmed by the pathology. He received chemotherapy consisting of cisplatin and doxorubicin after the surgery but the tumor progressed rapidly. He died of progressive disease and sepsis. The other case was a 4-year-old boy presented with abdominal pain. Computed tomography showed pancreatic tumor. He underwent surgical resection and pathology showed PB. He received chemotherapy after complete tumor resection. He is disease free till now. Complete tumor resection is the major difference of these 2 patients and is the most important factor affecting the outcome.