Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. They are classified as functional or non-functional tumors according to the presence of associated clinical symptoms. The majority are non-functional tumors. For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. Chromogranin A is the most sensitive marker but has insufficient specificity. In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. Multiphasic magnetic resonance imaging can detect PNETs smaller than 2 cm and small liver metastasis compared with other modalities. Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. Positron emission tomography (PET) scan with 18F-fluorodeoxyglucose cannot visualize PNETs, but PET with 68-Ga DOTATATE can. Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy. In this article, we review the characteristics of grading systems and diagnostic modalities commonly used for PNETs.
Biopsy ; Biopsy, Fine-Needle ; Chromogranin A ; Classification ; Diagnosis* ; Endosonography ; Incidence ; Liver ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors* ; Positron-Emission Tomography ; Radionuclide Imaging ; Receptors, Somatostatin ; Sensitivity and Specificity ; World Health Organization

Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are a group of malignant tumors with varying degrees of neuroectodermal differentiation. Although it may develop in any organs, ES/PNET originating from small intestine is exceedingly rare. We experienced a 9-year-old girl presenting with abdominal pain, melena, and iron deficiency anemia. Imaging work-up showed multiple masses in the small bowel and omentum with disseminated peritoneal seeding nodules, indicating lymphoma as the most likely diagnosis. Pathological reports from explorative diagnostic laparoscopic biopsy showed tumors comprising small round cells with CD99 expression and EWS-FLI1 translocation leading to the diagnosis of ES/PNET. Tumor burden decreased gradually during five consecutive cycles of systemic chemotherapy. The patient received segmental resection of jejunum, followed by adjuvant chemotherapy. This is the first pediatric case of ES/PNET found in small intestine in Korea.
Abdominal Pain ; Anemia, Iron-Deficiency ; Biopsy ; Chemotherapy, Adjuvant ; Child ; Diagnosis ; Drug Therapy ; Female ; Humans ; Intestine, Small ; Jejunum ; Korea ; Lymphoma ; Melena ; Neural Plate ; Neuroectodermal Tumors ; Neuroectodermal Tumors, Primitive ; Omentum ; Pediatrics ; Sarcoma, Ewing ; Tumor Burden

Accurate diagnosis of pancreatic solid lesions is often difficult using conventional imaging modalities. With the recent introduction of contrast-enhanced harmonic endoscopic ultrasound (CEH-EUS), it is now possible to evaluate the microvascular environment and dynamic enhancement of a variety of pancreatic lesions. With CEH-EUS, three patterns of pancreatic lesion enhancement compared with the normal pancreatic tissue (fast, simultaneous, or slow), two washout patterns (fast or slow) and two distribution patterns (homogeneous, inhomogeneous) can be described. By evaluating the microvasculature, enhancement speed, and washout pattern, CEH-EUS may help to differentiate pancreatic adenocarcinoma from other masses and differentiate between pancreatic neuroendocrine tumor (pNET) and inflammatory masses. The finding of a hyperenhancing lesion on CEH–EUS, both with homogeneous and inhomogeneous patterns, was a strong predictor of histology different from adenocarcinoma (94% positive predictive value). pNET was the most common hyperenhancing lesions overall. Although CEH-EUS is useful for ruling out pancreatic ductal adenocarcinoma, making the differential diagnosis between pNETs and pseudotumoral pancreatic masses is difficult because both may share an isovascular or hypervascular appearance. Currently the interpretation of CEH-EUS findings is examiner-dependent. In the future, digital image analysis by image-processing techniques should allow more objective interpretation.
Adenocarcinoma ; Diagnosis ; Diagnosis, Differential ; Microvessels ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors ; Pancreatic Ducts ; Ultrasonography*

OBJECTIVETo explore CT and MRI manifestations of the axial area peripheral primitive neuroectodermal tumors (pPNETs) in order to improve the knowledge of this disease.

METHODSThe clinical data of 10 patients with pPNETs underwent pathologically confirmed were retrospectively analyzed from October 2008 to May 2014. There were 7 males and 3 females, aged from 8 to 49 years old with median of 23.6 years. The preoperative multi-slice spiral CT scan was completed in 3 cases, plain CT scan and enhancement in 4 cases; MRI and enhancement scanning in 5 cases; and among them, 2 cases underwent both MRI and CT scan.

RESULTSIn-bone type was found 6 cases and out-bone type was found 4 cases. Three cases occurred in sacral vertebrae, 2 cases in lumbar vertebrae, 1 case in cervical vertebrae, 1 case in cervical spinal canal, 1 case in coccyx, 1 case in the right iliac bone, 1 case in presacral space. Cross sectional the smallest tumor maximum level was 1.1 cmx 1.2 cm in size, the biggest tumor was 8.0 cm x 9.2 cm, the median size was 4.4 cm x 5.7 cm, of them, the tumor of maximal diameter larger than 5 cm had 6 cases. Except 2 cases-without destruction of bone, the other 5 cases with osteolytic destruction, 2 cases with calcification, 1 case with mixed. Equidensite was main in CT scan, 1 case with uniform density, other 6 cases with uneven density,in which 3 cases with "floating ice" change; 1 case with moderate strengthening, other 3 cases with obviously strengthening, 2 cases with multiple small blood vessels in enhancement scanning. MRI of 5 cases showed the signal of isointensity on T1WI, the slightly high signal on T2WI and the signal was not uniform; after enhancement scan, the signal of 5 cases obviously enhanced. Two patients complicated with vertebral compression fractures, no periosteal reaction was found in all patients, and no the destruction of intervertebral disk was found in 5 patients of MRI scan.

CONCLUSIONThe axial area pPNETs is common among children and the youth, and the mass often is huge. The mass of in-bone type often envelopes the vertebral body, and main located on prevertebral space, all associated with bone destruction, osteolytic destruction is common, and primary vertebral bodies also is common, attachment primary or involvement is few found, it can involve the spinal canal and anterior wall of spinal canal is common, some cases complicate with multiple newly born small vessels. The mass of out-hone type in deep soft tissue is common, minority primary spinal canal, many complicated with vertebral bone destruction, osteolytic destruction was main. The intervertebral disk was not invaded and intervertebral space has not stenosis. CT scan offer complicate with "floating ice" sign, and in-bone type is common. Isointensity is main on MRI TlWI and slightly longer signal is main on MRI T2WI, strengthening signal is obvious.

Adolescent ; Adult ; Child ; Cross-Sectional Studies ; Diagnosis, Differential ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; diagnostic imaging ; Radionuclide Imaging ; Tomography, X-Ray Computed ; Young Adult

Humans ; Infant, Newborn ; Male ; Mediastinal Neoplasms ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Tomography, X-Ray Computed

Medulloblastoma is a common pediatric tumor typically diagnosed before the age of fifteen. Initial therapy includes surgical resection and radiation of the entire neuro-axis. Recurrence is common and typically occurs within 2 years of initial diagnosis. Those fitting Collin's Law is considered tumor-free. We report a case of single supratentorial recurrence 13 years after initial diagnosis. Here we present a 22 year old male presenting 13 years after initial diagnosis with isolated septum pellucidum recurrence. He underwent complete resection of the tumor. Medulloblastoma is a common in the pediatric population. Late recurrence to the ventricular system is uncommon. Long term follow-up is recommended in these patients.
Diagnosis ; Follow-Up Studies ; Humans ; Jurisprudence ; Male ; Medulloblastoma* ; Neuroectodermal Tumors, Primitive ; Recurrence* ; Septum Pellucidum

Insulinomas are the most common pancreatic neuroendocrine tumors. Most insulinomas are benign, small, intrapancreatic solid tumors and only large tumors have a tendency for malignancy. Most patients present with symptoms of hypoglycemia that are relieved with the administration of glucose. We herein present the case of a 75-year-old woman who presented with an acute hypoglycemic episode. Subsequent laboratory and radiological studies established the diagnosis of a 17-cm malignant insulinoma, with local invasion to the left kidney, lymph node metastasis, and hepatic metastases. Patient symptoms, diagnostic and imaging work-up and surgical management of both the primary and the metastatic disease are reviewed.
Aged ; Diagnosis ; Female ; Glucose ; Humans ; Hypoglycemia ; Insulinoma* ; Kidney ; Lymph Nodes ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors

Malignant kidney neoplasms are the most frequently encountered solid kidney masses. Although renal cell carcinoma is the major renal malignancy, other solid malignant renal masses should be considered in the differential diagnosis of solid renal masses that do not contain a macroscopic fatty component. In this pictorial essay, we present the imaging findings of a primitive neuroectodermal tumor, primary liposarcoma of the kidney, primary neuroendocrine tumor, leiomyosarcoma, synovial sarcoma, malignant fibrous histiocytoma, sclerosing fibrosarcoma and renal metastasis of osteosarcoma.
Bone Neoplasms/secondary ; Carcinoma, Renal Cell/pathology/radiography ; Diagnosis, Differential ; Fibrosarcoma/radiography ; Histiocytoma/radiography ; Humans ; Kidney Neoplasms/*pathology/radiography ; Leiomyosarcoma/pathology/radiography ; Magnetic Resonance Imaging ; Middle Aged ; Neuroectodermal Tumors, Primitive/pathology/radiography ; Osteosarcoma/pathology ; Sarcoma ; Sarcoma, Synovial/radiography ; Tomography, X-Ray Computed

OBJECTIVETo study clinicopathologic features, immunohistochemical profile, diagnosis and differential diagnosis of childhood central nervous system primitive neuroectodermal tumors (CNS PNETs) with the features of ependymoblastoma and neuroblastoma.

METHODSThe clinical data, morphologic and immunohistochemical features were analyzed in 4 cases of pediatric CNS PNETs with features of ependymoblastoma and neuroblastoma. EnVision method immunohistochemistry was applied.

RESULTSFour patients including three boys and one girl presented at the age from 12 month to 4 years and three tumors located in cerebrum, one in brain stem. All tumors showed typical combined histological patterns of ependymoblastoma and neuroblastoma, demonstrating zones of true rosettes, occasional pseudovascular rosettes, and undifferentiated neuroepithelial cells in a prominent background of mature neuropils. There was focal expression of glial fibrillary acidic protein (GFAP) consistent with glial differentiation and epithelial membrane antigen (EMA) consistent with ependymal differentiation. Necrosis was seen in three cases and calcification was present in one case. Immunohistochemically, the rosettes and undifferentiated neuroepithelial cells were positive for vimentin, partially positive for GFAP and EMA but negative for synaptophysin. The tumor cells were also positive for synaptophysin in neuropils. The Ki-67 label index ranged from 20% to 60%.

CONCLUSIONSCNS PNETs with the features of ependymoblastoma and neuroblastoma is a rare tumor with poor prognosis. The tumor primarily occurs in childhood, especially infant and belongs to the family of embryonal tumors of the CNS. The morphologic, immunohistochemical and genetic features are important in differential diagnosis from other tumors of the CNS.

Antigens, Neoplasm ; metabolism ; Central Nervous System ; pathology ; Child ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Immunohistochemistry ; Infant ; Male ; Mucin-1 ; metabolism ; Neuroblastoma ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; pathology ; Synaptophysin ; metabolism ; Vimentin ; metabolism

12E7 Antigen ; Adolescent ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Diagnosis, Differential ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Lymph Node Excision ; Lymphatic Metastasis ; Lymphoma ; metabolism ; pathology ; Male ; Neoplastic Cells, Circulating ; Nephrectomy ; Neuroblastoma ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; metabolism ; pathology ; surgery ; Synaptophysin ; metabolism ; Venae Cavae ; pathology ; Vimentin ; metabolism ; Wilms Tumor ; metabolism ; pathology