Tumor neuroectodérmico maligno del tracto gastrointestinal: a propósito de un caso / Malignant neuroectodermal tumor of the gastrointestinal tract: case report

El tumor neuroectodérmico maligno del tracto gastrointestinal es una neoplasia rara con pocos casos reportados en la literatura, especialmente en América Latina. Descrito por primera vez en 2003, se trata de una entidad sin tratamiento estandarizado y de pobre pronóstico. Se presenta el caso de una paciente de 22 años de edad que acude a la consulta por dolor abdominal, anemia y masa abdominal palpable. Luego de estudios pertinentes se decide la conducta resectiva y el posterior tratamiento oncológico. (AU)

Malignant gastrointestinal neuroectodermal tumor (GNET), formerly known as clear cell sarcoma of the gastrointestinal tract, is an extremely rare tumor of mesenchymal origin, which presents great microscopic and molecular similarity to clear cell sarcoma found in other parts of the body, such as tendons and aponeurosis. It is characterized by its rapid evolution, high recurrence rate and frequent diagnosis as metastatic disease.1,2 (AU)

Tumor neuroectodérmico primitivo periférico de riñón, reporte de caso

Introducción: El tumor neuroectodérmico primitivo periférico de riñón (PNETk) es una enfermedad rara muy agresivo que afecta mayormente a varones jóvenes. Reporte de caso: paciente varón de 19 años con cuadro clínico dolor abdominal, hematuria y masa palpable, en la tomografía se evidencia una enorme tumoración renal izquierda, Se le realiza nefrectomía radical convencional y se envía a estudio patológico más histoquímica resultando de PNETk. Luego paciente siguió su manejo por oncología para quimiterapia inicialmente. Conclusión: El PNETk que describimos representa el primer caso reportado en nuestro país, constituye una entidad clínica única por su rareza siendo un reto hacer diagnóstico y su comportamiento y manejo se sigue basando a reportes de casos debido a su poca frecuencia.

Introduction: Peripheral primitive neuroectodermal tumor of the kidney (PNETk) is a very aggressive rare disease that mainly affects young men. Case report: A 19-year-old male patient with symptoms of abdominal pain, hematuria and a palpable mass, the tomography shows a large left renal tumor. Conventional radical nephrectomy was performed and sent for pathology study plus histochemistry, resulting in PNETk. The patient then continued his oncology management for chemotherapy initially. Conclusion: The PNETk that we describe represents the first case reported in our country, it constitutes a unique clinical entity due to its rarity, being a challenge to make a diagnosis and its behavior and management is still based on case reports due to its infrequency.

Ewing sarcoma arising in colon and ovary––Two rare sites of presentation

Ewing sarcoma is a rare aggressive malignant round cell tumor, primarily presenting in bone and soft tissues. This study presents two cases of this tumor in unusual locations, one in right colon which presented with intussusception and other in ovary which presented clinically as carcinoma ovary. Both the cases showed histomorphology of primitive round cell tumor with characteristic immunohistochemical profile and was confirmed on molecular analysis. We aim to highlight the importance of considering Ewing sarcoma in the differential diagnoses in these locations as they have dismal prognosis with no standard treatment modality.

Tumor neuroectodérmico primitivo primario de ovario

El tumor neuroectodérmico primitivo del ovario es un sarcoma de tejido blando de células redondas pequeñas, raro y agresivo, de origen neural que generalmente se asocia con una alta morbilidad y mortalidad. La inmunohistoquímica es un complemento útil en el diagnóstico diferencial. Se describe un caso de tumor neuroectodérmico primitivo del ovario en paciente nulípara de 21 años que refería dolor y aumento de la circunferencia abdominal. La ecografía mostró tumoración de contenido heterogéneo sólido-quística que aparentemente se originaba del anexo izquierdo. La resonancia magnética confirmó la presencia de tumoración que se extendía hacia la fosa iliaca izquierda sin afectación de órganos locales ni metástasis regionales o a distancia. Los marcadores tumorales estaban todos dentro del rango normal. Durante la laparotomía se observó tumoración de ovario izquierdo con ovario derecho normal. Se realizó salpingoforectomía izquierda debido al tamaño del tumor, resección en cuña de ovario derecho, linfadenectomía pélvica y omentectomía. El examen histopatológico reveló tumor compuesto por láminas de células redondas. Las células tumorales fueron positivas para cromogranina A, sinatrofisina, vimentina y enolasa específica de neuronas, lo que confirmó el diagnóstico de tumor neuroendocrino primitivo de ovario izquierdo, que se originaba de teratoma quístico inmaduro. La paciente rechazó la quimioterapia postoperatoria.

Primitive neuroectodermal tumor of the ovary is a rare and aggressive small round cell soft tissue sarcoma of neural origin that is usually associated with high morbidity and mortality. Immunohistochemistry is a useful adjunct in the differential diagnosis. We describe a case of a primitive neuroectodermal tumor of the ovary in a 21-yearold nulliparous patient who reported pain and increased abdominal circumference. Ultrasonography showed a solid-cystic heterogeneous tumor apparently originating from the left adnexa. Magnetic resonance imaging confirmed the presence of a tumor extending into the left iliac fossa without local organ involvement or regional or distant metastases. Tumor markers were all within the normal range. During laparotomy, a left ovarian tumor was observed with a normal right ovary. Left salpingo-oophorectomy was performed due the size of the tumor, right ovarian wedge resection, pelvic lymphadenectomy and omentectomy. Histopathologic examination revealed tumor composed of sheets of round cells. The tumor cells were positive for chromogranin A, synaptophysin, vimentin and neuron-specific enolase, which confirmed the diagnosis of a primitive neuroendocrine tumor of the left ovary originating from immature cystic teratoma. The patient refused postoperative chemotherapy.

Cervical Primary Ewing's Sarcoma:Report of One Case / 中国医学科学院学报

Ewing's sarcoma in the cervix is characterized by extremely rare occurrence,high degree of malignancy,and rapid progression.The diagnosis of this disease is based on pathology and immunohistochemistry. The main image of the case reported in this paper showed the cervical cyst with solid mass,large volume,and uneven density and signal,and the solid part can be strengthened in enhanced scanning.Because of the rapid growth,the lesion is prone to liquefaction necrosis and bleeding.Since the metastasis occurs early,timely diagnosis is essential.

Bioinformatics analysis of gene expression profile of central nervous system primitive neuroectodermal tumors / 西安交通大学学报(医学版)

【Objective】 To analyze the gene expression profile of central nervous system primitive neuroectodermal tumors （CNS-PNETs） by bioinformatics methods so as to explore the possible pathogenesis of CNS-PNETs at the molecular level. 【Methods】 The gene expression profile of CNS-PNETs was downloaded from the GEO database， GSE35493 and GSE74195. The differentially expressed genes （DEGs） were screened by the online analysis tool of GEO2R and Venn software， DEGs were analyzed by using the online analysis tools of David database， such as Gene Ontology （GO） and pathway enrichment （KEGG）. The protein interaction network analysis （PPI） of CNS-PNETs was made by using STRING online analysis tool， Cytoscape software and its plug-in cytohubba to find the key genes. 【Results】 We obtained 262 DEGs， including 49 upregulated genes and 213 downregulated genes. The analysis of GO function and KEGG signal pathway enrichment showed that DEG was involved in DNA transcription and mitosis， cell division， synaptic signal transmission and other biological processes， and associated with cell cycle， tumor-related pathway， p53 signal pathway， synapsis-related signal pathway， cAMP signal pathway and calcium ion signal pathway. Ten key genes， namely， CDK1， CDC20， MAD2L1， KIF11， ASPM， TOP2A， TTK， NDC80， NUSAP1 and DLGAP5 were screened out by STRING analysis. 【Conclusion】 Ten key genes including CDK1 may play an important role in the initiation and progression of CNS-PNETs， providing new clues for exploring the pathogenesis of CNS-PNETs.

Melanotic neuroectodermal tumor of infancy: a case report / Tumor neuroectodérmico melanocítico de la infancia: reporte de caso

Abstract Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a rare neoplasm originating from neural crest cells, which generally affects pediatric patients, most frequently during the first year of life. The behavior of MNTIs is benign, locally aggressive, with a recurrence of 10-15% and eventually malignant in 6.97%. This study describes the clinical, imaging, histopathological, immunohistochemical characteristics and the management of MNTI in a 5-month-old girl, whose lesion was resected and monitored. The present case illustrates the benefits of multidisciplinary integration for a correct diagnosis to ensure adequate therapeutic management, in addition to providing a report on this rare and understudied pathology.

Resumen El Tumor Neuroectodérmico Melanocítico de la Infancia (TNEMI) es una neoplasia infrecuente derivada de las células de la cresta neural, que afecta generalmente pacientes pediátricos y se presenta con mayor frecuencia durante el primer año de vida. Su comportamiento es benigno, localmente agresivo, con una recurrencia de 10% a 15% y eventualmente maligno en un 6.97%. En este estudio se describen las características clínicas, imagenológicas, histopatológicas, inmunohistoquímicas y el manejo del TNEMI en una niña de 5 meses de edad, a la cual se le realizó resección de la lesión y seguimiento. El presente caso ilustra el beneficio de la integralidad multidisciplinaria que permite establecer un diagnóstico correcto para asegurar un manejo terapéutico adecuado, además de aportar un reporte sobre esta patología poco frecuente y estudiada.

Tumor neuroectodérmico recidivante. A propósito de un caso y revisión bibliográfica / Recurrent neuroectodermal tumor. A case report and literatura review

Resumen ANTECEDENTES: La relación entre cáncer y embarazo supone 0.07% de las complicaciones gestacionales. Cuando estas situaciones coinciden el tratamiento del tumor se dificulta. El tumor neuroectodérmico primitivo es una neoplasia relacionada con el sarcoma de Ewing y su incidencia es excepcional durante el embarazo. CASO CLÍNICO: Paciente de 34 años, con 36.3 semanas de embarazo, que ingresó a la unidad hospitalaria por dolor abdominal irradiado al miembro inferior derecho. A la exploración física se palpó una tumoración de gran dimensión en la fosa iliaca derecha. La ecografía abdominal objetivó una imagen compatible con un mioma. La resonancia magnética reportó una masa de 16 x 13 x 17 cm, retroperitoneal, paravertebral, coincidente con tumor neuroectodérmico, sarcoma y tumor neurogénico. La paciente tuvo parto eutócico, sin administración de analgesia epidural, del que nació una niña de 2950 g, con Apgar 8-9. Se efectuó una biopsia por aspiración con aguja gruesa, que reportó un tumor neuroectodérmico primitivo. El tratamiento consistió en quimioterapia con protocolo VAC (vincristina, dactinomicina y ciclofosfamida [14 ciclos]) y adriamicina (6 a 8 ciclos de inducción). Actualmente padece dolor neuropático en la pierna derecha y permanece en rehabilitación, con tratamiento médico. CONCLUSIONES: Los tumores neuroectodérmicos primitivos son neoplasias excepcionales durante el embarazo. Se requieren estudios complementarios para conocer la relación exacta entre este tipo de tumores y el embarazo, y de esta forma establecer el protocolo de tratamiento adecuado.

Abstract BACKGROUND: The relationship between cancer and pregnancy accounts for 0.07% of gestational complications. This aspect makes treatment difficult and has a negative impact on pregnant patients. The primitive neuroectodermal tumor is a neoplasm related to Ewing's sarcoma and its incidence is exceptional during pregnancy. CLINICAL CASE: A 34-year-old patient, 36.3 weeks pregnant, who was admitted to the hospital unit due to abdominal pain radiating to the right lower limb. Physical examination revealed a large tumor in the right iliac fossa. The abdominal ultrasound showed an image compatible with a myoma. Magnetic resonance imaging revealed a mass of 16 x 13 x 17 cm, retroperitoneal, paravertebral, coinciding with neuroectodermal tumor, sarcoma and neurogenic tumor. The patient had eutocic delivery, without administration of epidural analgesia, from which a girl of 2950 g was born, and Apgar 8/9. An aspiration biopsy was performed with a thick needle, which reported a primitive neuroectodermal tumor. The treatment consisted of chemotherapy with VAC protocol (vincristine, dactinomycin and cyclophosphamide [14 cycles]) and adriamycin (6 to 8 induction cycles). He currently suffers from neuropathic pain in the right leg and remains in rehabilitation, with medical treatment. CONCLUSIONS: Primitive neuroectodermal tumors are exceptional neoplasms during pregnancy. Complementary studies are required to know the exact relationship between this type of tumors and pregnancy, and in this way establish the appropriate treatment strategy.

Peripheral primitive neuroectodermal tumor of the mandible: a case report and literature review / 口腔疾病防治

Objective@# To explore the clinical, imaging and pathological characteristics of mandibular peripheral primitive neuroectodermal tumors, and to review relevant literature to improve the understanding and diagnosis of pPNET in mandible.@* Methods@# The clinical and imaging features, pathological examination, treatment and prognosis of a case of mandibular pPNET diagnosed and treated at the First Affiliated Hospital of Xinjiang Medical University were observed, and analyzed a literature review. @* Results @#The patient′s clinical manifestations were an enlarged mass of the mandible, hard texture, unclear borders, involving loose teeth, and numbness of the lower lip; CT and MRI showed osteolytic and aggressive growth patterns. The mandibular tumor was resected and the mandibular partial truncated resection was performed on the titanium plate. Postoperative pathological sections showed small round cell tumors under HE staining and Vimentin and Fli-1 were positive, and the pathological diagnosis was pPNET. The patient did not undergo chemoradiotherapy after surgery and died of tumor recurrence after 9 months of follow-up. A review of the relevant literature revealed that pPNETs are a group of small round cell tumors, which are more common in children and adolescents. pPNETs have a high degree of malignancy, a short course of disease and fast metastasis. The main route of metastasis is through the blood circulation. Most patients die within 2 years, the 3-year survival rate is only 30%, and the 5-year survival rate is less than 10%. Imaging is generally nonspecific; therefore, pPNETs are easily misdiagnosed. The final tumor type is determined by pathological HE staining and immunohistochemical characteristics. Current treatment methods are mainly complete surgical resection combined with postoperative radiotherapy and chemotherapy, but it is critical to provide individualized treatment to patients when necessary.@*Conclusion@# pPNETs have a high degree of malignancy, easy recurrence and poor prognosis, so early diagnosis and treatment are extremely important.

Imaging Findings and Pathological Characteristics of Primitive Neuroectodermal Tumor / 中国医科大学学报

Objective To investigate the imaging findings and pathological characterislics of primitive neuroectodennal tumor (PNET). Meth-ods Imaging data of 22 cases of PN ET confirmed by pathology were retrospectively analyzed. Results There were 5 cases of central PNET, which were round or lobulated masses, 4 cases with cystic degeneration, 1 case with necrosis, and 4 cases with mild peritu moraledema. Magnetic resonance imaging (MRI) revealed isointensity or slightlyhy pointensity on Tl-and hyperin tensity on T2-weighted imag-es, and marked heterogeneous enhancement. There were 17 cases of peripheral PNET, which were mostly large lobulated soft tissue mass-es, 7cases with cystic degeneration, 3 cases with necrosis, and 8 cases with boned estructio n. Computed tomography (CT) scan showed iso-lowd ensity. MRI revealed isointensity or slightly hypointensity on Tl-and heterogeneous hypeiintensityon T2-weighted images, and marked heterogeneous enhancement. Hematoxylin-eosin staining showed Homer-Wright rosette arrangement of the tumor cells in 13 cases. Immunohistochemical staining revealed expression of CD99 (17/22), CD56 (16/18), Ki-67 (22/22), and vimentin (22/22) by the tumorcells. Conclusion MRI and CT findings of PNET are characteristic and helpful in differential diagnosis of the disease. CD99 is of signif-icant value as a diagnostic biomarker and in the classification of PNET.

Clinical analysis of 18 cases of breast primitive neuroectodermal tumor / 中华内分泌外科杂志

Objective To investigate the clinical pathological characteristics and prognosis of primitive neuroectodermal tumor (PNET) of breast.Methods Patients with breast PNET were retrieved from CNKI,Pubmed,Europe PMC and other databases from Jan.1980 to Dec.2016.The clinical data of one patient with breast PNET in our hospital were analyzed retrospectively.Results 18 cases had painless,rapid growth mass as the main clinical features.The pathological morphology showed small round cell tumors,PAS staining positive.Immunohistochemistry CD99 and Fli-1 characteristic expression were the main indexes for the diagnosis of breast PNET.The positive expression of Vimentin,NSE,Syn and negative expression of CK,EMA,Desmin,CgA,LCA,S-100 also played an important role in the diagnosis of breast PNET.The positive expression of genetic marker EWSRI was the golden standard for diagnosis of breast PNET.The size of the tumor,surgical treatment,lymph node metastasis,distant metastasis and chemotherapy were the important factors that affect the prognosis of the PNET.The survival rates of 1 and 3 years were 71.4％ and 33.3％ respectively.Conclusions Breast PNET is a rare tumor with poor prognosis,and its diagnosis is highly dependent on pathology.Surgery can significantly improve the prognosis of the patients.Surgery should be the main treatment,combined with radiotherapy and chemotherapy.The current study does not show evidence of effectiveness in terms of endocrine or targeted drug therapy for breast PNET patients.

Melanotic neuroectodermal tumor of infancy: a clinicopathological case report

Abstract Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that affects mainly children under 1 year of age. A 4-month-old boy was referred for evaluation of a lesion with 1 month of evolution. Intra-oral examination detected a firm upon palpation submucosal nodular mass, measuring 1.5 cm in diameter, affecting the anterior maxillary alveolar ridge and covered by a slightly blue mucosa with evident telangiectasia. The patient underwent an incisional biopsy and histological and immunohistochemical analyses revealed nests of AE1/AE3 positive epithelioid cells with abundant melanin pigmentation. Other cell types, resembling neuroblasts, were also present and positive for CD56, synaptophysin and enolase. The diagnosis of MNTI was established and the patient was referred for treatment. Conservative surgical resection was performed along with 3 adjacent teeth under general anesthesia. The patient is in follow-up for 1,5 year without recurrence. Conservative surgical management of MNTI may be an alternative to maxillectomy, contributing to the patient´s quality of life.

Resumo Tumor neuroectodérmico melanótico da infância (TNMI) é um neoplasma raro que afeta principalmente crianças com idade abaixo de 1 ano. Um menino de 4 meses foi referenciado para avaliação de uma lesão com 1 mês de evolução. O exame intra-oral detectou uma massa nodular submucosa firme à palpação, medindo 1,5 cm de diâmetro, afetando rebordo alveolar anterior da maxila e recoberta por mucosa de coloração levemente azulada com telangiectasia evidente. O paciente foi submetido à biopsia incisional e as análises histológica e imunohistoquímica revelaram ninhos compostos por células com abundante pigmento de melanina, positivas para AE1/AE3. Outro tipo celular, semelhante à neuroblastos, também estava presente e foram positivas para CD56, sinaptofisina e enolase. O diagnóstico de TNMI foi estabelecido e o paciente encaminhado para tratamento. Ressecção cirúrgica conservadora sob anestesia geral ao longo de 3 dentes adjacentes foi realizada. O paciente está em acompanhamento há 1 ano e meio sem sinais de recorrência. O tratamento cirúrgico conservador do TNMI pode ser uma alternativa à maxilectomia, contribuindo para a qualidade de vida do paciente.

Primary primitive neuroectodermal tumor of the kidney

Renal primitive neuroectodermal tumor (PNET) is a rare entity. It should be differentiated from other primary renal malignancies. The differentiation of the malignant small round cell tumors may be challenging; however, it is essential for better management of the patient. We report a 45-year-old male having complaints of pain in the abdomen, hematuria, and mass in the abdomen for 6 months. Abdominal and pelvic sonography finding showed cystic-solid, right renal mass suggestive of malignancy. On histopathology diagnosed as malignant small round blue cell tumor suggestive of primitive neuroectodermal tumor. On immunohistochemistry, CD99 positivity confirmed the diagnosis of primary PNET of the kidney. We are presenting this case for its rarity, clinical presentation, and pathological findings.

A mesenteric primary peripheral Ewing's sarcoma/primitive neuroectodermal tumor with molecular cytogenetic analysis: Report of a rare case and review of literature

Rare cases of Ewing's sarcoma/primitive neuroectodermal tumors (EWS/PNETs) arising from mesenteric tissue have been reported. This report describes an EWS/PNET in a 25-year-old woman who presented with abdominal pain lasting 3 days. Radiologic evaluation revealed a 9 cm × 6 cm homogeneous mass in the lower abdomen with homogeneous enhancement and invasion of the ileum. Surgical resection was completed during exploratory laparotomy. Immunohistochemically, the tumor cells revealed CD99, friend leukemia virus integration-1 and NKX2.2 (NK2 Homeobox 2, a protein coding gene) and subsequently showed EWSR1 rearrangement. The histological feature, immunohistochemical results and genetic fluorescence in situ hybridization analysis of this case were confirming the diagnosis of EWS/PNET. Adjuvant chemotherapy was suggested, but the patient was lost to follow-up.

Clinical and immunohistochemical study of melanotic neuroectodermal tumor of infancy in the maxilla / Estudo clínico e imuno-histoquímico de tumor neuroectodérmico melanocítico da infância em maxila

ABSTRACT Melanotic neuroectodermal tumor of infancy is a rare and fast-growing neoplasm. In this study, we describe the case of a 6-month-old female patient, who presented swelling in the anterior maxilla. Tomographic reconstruction showed an unilocular hypodense and expansive area associated with the upper right central primary incisor. The presumptive diagnoses were dentigerous cyst, adenomatoid odontogenic tumor, melanotic neuroectodermal tumor of infancy and rhabdomyosarcoma, and an incisional biopsy was performed. Microscopically, the lesion revealed a biphasic cell population, consisting of small, ovoid, neuroblastic-like cells and epithelioid cells containing melanin. Immunohistochemically, the melanocyte-like component was strongly and diffusely positive for HMB-45 and Melan-A, but weakly positive for S100. Based on these findings, definitive diagnosis of melanotic neuroectodermal tumor of infancy was established. Then, enucleation of the lesion was performed by careful curettage. After 2 year follow-up, no clinical or radiographical evidence of recurrence was verified. The present case highlights the importance of early diagnosis and therapeutic intervention at the appropriate time to achieve a favorable outcome for the patient.

RESUMO O tumor neuroectodérmico melanocítico da infância é uma neoplasia rara e de crescimento rápido. Neste estudo, relata-se o caso de uma paciente do sexo feminino de 6 meses de idade, que apresentou tumefação na região anterior de maxila. A reconstrução tomográfica revelou área unilocular hipodensa e expansiva associada ao incisivo central superior direito decíduo. Realizou-se biópsia incisional, considerando as hipóteses diagnósticas de cisto dentígero, tumor odontogênico adenomatoide, tumor neuroectodérmico melanocítico da infância e rabdomiossarcoma. Microscopicamente, a lesão revelou população celular bifásica, consistindo de células pequenas, ovoides, de aparência neuroblástica, e de células epitelioides, contendo melanina. A análise imuno-histoquímica demonstrou que o componente celular contendo melanina era positivo de forma intensa e difusa para HMB-45 e Melan-A, mas levemente positivo para S100. Com base nestes achados, foi estabelecido o diagnóstico definitivo de tumor neuroectodérmico melanocítico da infância. Em seguida, foi realizada a enucleação da lesão com curetagem cuidadosa. Após 2 anos de acompanhamento, não foram verificadas evidências clínicas ou radiográficas de recorrência. O presente caso destaca a importância do diagnóstico precoce e da intervenção terapêutica no momento apropriado, a fim de alcançar um desfecho favorável para o paciente.

Successful Surgical Treatment of a Recurrent Esophageal Malignant Gastrointestinal Neuroectodermal Tumor

Malignant gastrointestinal neuroectodermal tumor (GNET) is a very rare disease entity, especially in the esophagus. The diagnosis of GNET is based on histologic, immunohistochemical, and genetic findings. The choice of treatment is complete resection, and further treatment options can be considered. Herein, we describe a case of successful surgical treatment of a 23-year-old man with recurrent malignant esophageal GNET.

Survival and prognostic factors analysis of primitive neuroectodermal tumor / 中华肿瘤杂志

Objective@#To investigate the clinical features and prognosis of primitive neuroectodermal tumor (PNET).@*Methods@#The clinical data of 99 patients with PNET from February 1, 1998 to February 1, 2017 were retrospectively analyzed. Univariate analysis was performed using Kaplan-Meier and Log rank test. Multiviate Cox regression was applied to analyzed independent prognostic factor for patient survival.@*Results@#Among the 99 patients, 81 were peripheral PNET(pPNET) and 18 were central PNET (cPNET) . Biopsy was performed exclusively in 16 cases, with R0 resection in 61 cases, with R1 resection in 4 cases, and with R2 resection in 18 cases. Twelve patients underwent surgery only, nine had chemotherapy only, and one received radiotherapy only. There were 72 patients who had combined treatment including chemotherapy, and 48 patients had combined therapy including radiotherapy. The one-year, three-year and five-year overall survival(OS) rates of the 99 PNET patients were 79.2%, 63.9% and 56.1% respectively, and median OS time was 14.0 months. The one-year, three-year and five-year progression free survival (PFS) rates of these patients were 42.7%, 25.7% and 19.8% respectively, and median PFS time was 8.0 months. The univariate analysis revealed that lymph node metastasis, surgical resection, numbers of cycles of chemotherapy and radiotherapy dose were the main factors affecting the OS (all P<0.05). Gender, age, lymph node metastasis, staging, and chemotherapy cycles were related to PFS in PNET patients (P<0.05). Multivariate analysis showed that the degree of surgical resection, chemotherapy cycle, and radiotherapy dosage were independent influencing factors of OS in PNET patients (risk ratio=1.856, 0.398, and 0.408, respectively, all P<0.05), and gender was an independent factor influencing PFS in PNET patients (risk ratio=0.494, P<0.05).@*Conclusions@#Comprehensive therapy is the main therapy for PNET patients. The surgical resection, cycles of chemotherapy and radiotherapy dosage are independent prognostic factors for patient′s OS.

CT manifestations of primitive neuroectodermal tumor of adrenal gland / 实用放射学杂志

Objective To explore the CT manifestations of primitive neuroectodermal tumor (PNET) of adrenal gland,aiming to increase the cognition on this disease.Methods The CT manifestations of 7 patients who were pathologically diagnosed with adrenal gland PNET were analyzed retrospectively.Results The age of patients was 24-63 years old,with average age of (47.6±13.7) years,including 6 females and 1 male.Location of PNET:4 in left adrenal gland and 3 in right adrenal gland.Clinical manifestations:3 cases had upper abdominal distension,2 cases suffered from pain of waist and back,1 case was discovered in physical examination and another 1 had fever.No abnormality was found in laboratory and endocrinological examinations.After operation,4 cases had recurrence and 2 had distant metastasis.During follow-up,6 patients died and only 1 still alive.CT showed that the tumor,whose maximum diameter was 10.1-19.4 cm,was elliptic or lobulated in shape.The tumor was clear in boundary in 2 patients,but was unclear in boundary and involved adjacent organs in 5 patients.The plain scanning showed equidensity or slightly low density,complicated with necrosis and cystic lesions without calcification.The enhanced scanning suggested progressive uneven enhancement,with multiple interatrial septum-like changes and latticed enhancement inside.Conclusion CT manifestations of adrenal gland PNET can prompt the diagnosis,pathological examination is still required for confirmation.CT can accurately describe the internal structures,adjacent relationship and the metastasis of tumors.

Supratentorial primitive neutoectodermal tumors in adults:imaging findings and analysis on misdiagnosis / 实用放射学杂志

Objective To investigate the CT and MRI features of supratentorial primitive neuroectodermal tumors (sPNET)in adults,and to analyze the reasons of misdiagnosis.Methods The CT and MRI features of 1 5 patients with sPNET confirmed by pathology were analyzed retrospective.13 of 15 patients were underwent plain and contrast enhanced MRI,4 patients were also performed CT scan and the other 2 patients were underwent CT plain scan only.Results Nine of 1 5 lesions were located in the temporal and/or frontal lobe,with clear margin in 12 lesions,and mild to moderate peritumoral was noted in most cases.The solid portion of the lesions were hyperdense on CT,isointense or slightly hypointense on T1WI,and isointense or hyperintense on T2WI compared to the gray matter.Calcification (2/6),necrosis or cystic degeneration(1 3/1 5),hemorrhage (8/1 5)and flow void signal (1 1/1 3)were seen.Twelve lesions showed significantly heterogeneous enhancement and 7 lesions showed irregular ring-enhancement.The solid parts in 5 lesions showed hyperintensity on diffusion-weighted imaging. Preoperatively,the lesions were misdiagnosed as glioma in 10 cases,ependymoma in 2 cases,meningioma in 1 case and germ cell tumor in 1 case, respectively;and 1 case was not diagnosed definitely.Conclusion Supratentorial PNET in adults has characteristic CT and MRI features,and the main reasons accounting for misdiagnosis are its extraordinary low incidence and that radiologists do no master its imaging features.

Pediatric Ewing's Sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) Developed in the Small Intestine: A Case Report / 임상소아혈액종양

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.