Taquipnea persistente como manifestación inicial de hiperplasia de células neuroendocrinas: reporte de un caso clínico / Persistent tachypnea as initial manifestation of hyperplasia of neuroendocrine cells: case report

Se realiza revisión de la literatura y presentación de un caso clínico de Hiperplasia de Células Neuroendocrinas en paciente lactante masculino que inicia su padecimiento a los 3 meses de vida con dificultad respiratoria caracterizada por retracciones subcostales y taquipnea persistente, posterior-mente a los 8 meses de edad se agrega hipoxemia respirando aire ambiente que requiere uso de oxígeno suplementario continuo. Tiene antecedente de tres hospitalizaciones, con diagnóstico de Bronquiolitis y Neumonía atípica, realizándose panel viral respiratorio con reporte negativo. El paciente persiste con sintomatología respiratoria a pesar de tratamientos médicos, por lo que se deriva a neumología pediátrica, unidad de enfermedad pulmonar intersticial del lactante, iniciando protocolo de estudio, se realiza tomografía tórax de alta resolución, que evidencia imágenes en vidrio despulido en lóbulo medio y región lingular, además de atrapamiento aéreo. Se concluye el diagnóstico de Hiperplasia de Células neuroendocrinas con base a la clínica y hallazgos tomográficos. La Hiperplasia de Células Neuroendocrinas es una patología pulmonar intersticial poco frecuente, cuyo diagnóstico es clínico y radiológico, en la minoría de los casos se requiere biopsia pulmonar para confirmación. Puede ser fácilmente confundida con otras enfermedades respiratorias comunes, por lo que es importante sospecharla para realizar un diagnóstico precoz. La mayor parte de los casos evolucionan con declinación de los síntomas, mejorando espontáneamente en los primeros años de vida.

A review of the literature and presentation of a clinical case of Neuroendocrine Cell Hyperplasia in a male infant patient who begins his condition at 3 months of age with respiratory distress characterized by subcostal retractions and persistent tachypnea is presented. After 8 months of age hypoxemia is added requiring continuous oxygen therapy. He has a history of three hospitalizations, with a diagnosis of bronchiolitis and atypical pneumonia, respiratory viral panel has a negative report. The patient persists with respiratory symptoms despite medical treatments, so it is referred to pediatric pulmonology, initiating study protocol for interstitial lung disease of the infant. A high resolution chest tomography is performed, which evidences images in polished glass in the middle lobe and lingular region, in addition to air entrapment. The diagnosis of neuroendocrine cell hyperplasia is concluded based on clinical and tomographic findings. Neuroendocrine Cell Hyperplasia is a rare interstitial pulmonary pathology, whose diagnosis is clinical and radiological. Lung biopsy is required only in the minority of cases for confirming diagnosis. It can be easily confused with other common respiratory diseases, so it is important to suspect it to make an early diagnosis. Most cases evolve with decline in symptoms, improving spontaneously in the first years of life.

Taquipnea persistente de la infancia: hiperplasia de células neuroendocrinas, una revisión / Persistent tachypnea of infancy: neuroendocrine cell hyperplasia, a review

La hiperplasia de células neuroendocrinas de la infancia (HCNEI) constituye una de las enfermedades intersticiales más frecuentes en pediatría. Tanto su etiología como los mecanismos fisiopatológicos involucrados son inciertos. Suele presentarse en pacientes por lo demás sanos, durante los primeros meses de vida con taquipnea, retracciones costales, rales e hipoxemia. En la tomografía axial computada de tórax de alta resolución (TACAR) presenta imágenes características en vidrio esmerilado de distribución central y zonas de atrapamiento aéreo. Para el diagnóstico, además de la clínica y la TACAR, podemos recurrir a la biopsia en casos atípicos. Los hallazgos histológicos reflejan una arquitectura pulmonar normal y un aumento en el número de células neuroendocrinas. El manejo global es con medidas de sostén, ya que no se cuenta con un tratamiento específico. La sintomatología suele mejorar con la edad y el pronóstico es favorable.

Neuroendocrine cell hyperplasia of infancy (NEHI) is one of the most common interstitial lung diseases of childhood. The etiology and pathophysiological mechanisms involved are uncertain. It usually presents in otherwise healthy patients during the first months of life with tachypnea, rib retractions, crackles, and hypoxemia. High-resolution chest computed tomography (HRCT) shows ground-glass opacities of central distribution and areas of air trapping. For diagnosis purposes, in addition to clinical and HRCT features, a lung biopsy is indicated for atypical cases. Histological findings reflect normal architecture and an increased number of neuroendocrine cells. The management consists of supportive and preventive care, since there is no specific treatment. Symptoms usually improve with age and the prognosis is favorable.

Hiperplasia de células neuroendocrinas: reporte de caso / Neuroendocrine cell hyperplasia: case report

A clinical case of Neuroendocrine Cell Hyperplasia is presented with a bibliographic review. An infant patient with respiratory distress syndrome, characterized by nasal flaring, retractions, and tachypnea with temporary resolution with the use of bronchodilators. However, the patient requires oxygen. With complementary examinations (negative viral panel twice) and epidemiology it is classified as a viral Bronchiolitis. Without improvement, extrapulmonar pathologies were suspected, discarding hearth disease, epilepsy, pathological gastroesophageal reflux. New tests were performed to rule out other pathologies, including immunological disorders. Those results were normal, so a high-resolution chest tomography was done which allowed the diagnosis of Neuroendocrine Cell Hyperplasia. During the follow up the child had improved and required oxygen until he was two years old. Neuroendocrine Cell Hyperplasia belongs to a huge group of less common interstitial disorders, which diagnosis is clinical and radiological. It can easily be confused with common respiratory disorders. For this reason, it is important to know about this disease to make an early diagnosis. Most of the cases had a gradual (months to years) improvement.

Se presenta un caso clínico de Hiperplasia de Células Neuroendocrinas y la revisión de la literatura. Paciente lactante menor con cuadro de dificultad respiratoria, caracterizado por aleteo nasal, retracciones y taquipnea persistente acompañada de desaturación. Sin adecuada respuesta al uso de broncodilatadores. Por exámenes complementarios, panel viral negativo en dos ocasiones y epidemiología, se le diagnostica una bronquiolitis viral. Por no presentar mejoría se completan estudios, descartándose neumonía atípica, cardiopatía, epilepsia, reflujo gastroesofágico patológico y compromiso inmunológico. El diagnóstico fue determinado en base a la clínica, junto con imágenes en vidrio esmerilado característicos en lóbulo medio y língula. En su seguimiento mejora paulatinamente, requiriendo soporte de oxígeno hasta los dos años. La Hiperplasia de Células Neuroendocrinas es una patología intersticial pulmonar poco frecuente, cuyo diagnóstico es clínico y radiológico. Puede ser fácilmente confundida con desórdenes respiratorios comunes, por lo que es importante sospecharla para realizar un diagnóstico precoz. La mayor parte de los casos evolucionan con declinación de los síntomas, mejorando espontáneamente en meses o en los primeros años de vida.

Hiperplasia de células neuroendocrinas que simula tumor sólido de páncreas / Neuroendocrine cell hyperplasia mimicking a solid pancreatic tumor

RESUMEN La hiperplasia de células neuroendocrinas pancreáticas es una patología donde se produce un aumen to en el número de células de los islotes de Langerhans y a veces puede simular un proceso tumoral. Caso clínico: presentamos el caso de un paciente con tumor sólido de cola de páncreas, sintomático, al que se le realizó esplenopancreatectomía corporocaudal laparoscópica. El resultado anatomopatoló gico posterior informó una hiperplasia neuroendocrina. Conclusión: la hiperplasia de células neuroen docrinas debería considerarse en el diagnóstico diferencial de tumores sólidos de páncreas. La alterna tiva quirúrgica laparoscópica es factible cuando no es posible establecer el diagnóstico prequirúrgico con estudios de imágenes o biopsia.

ABSTRACT Pancreatic endocrine cell hyperplasia is defined as an increase in the number of cells of Langerhans islets and can sometimes mimic a tumoral process. Case report: a male patient with a symptomatic solid tail of pancreas tumor underwent laparoscopic distal pancreatectomy and splenectomy. The pathological examination reported neuroendocrine cell hyperplasia. Conclusion: pancreatic endocrine cell hyperplasia should be considered in the differential diagnosis of solid pancreatic tumors. Laparoscopic surgery is feasible when the preoperative diagnosis with imaging tests of biopsy is not possible.

Hiperplasia neuroendocrina en colitis linfocitica y colitis ulcerativa / Neuroendocrine hiperplasia in linfocitic and ulcerative colitis

Introducción: Algunos autores han demostrado incremento de células neuroendócrinas en colitis microscópica y colitis ulcerativa. Objetivo: El objetivo del presente estudio fue evaluar la presencia de células neuroendócrinas en colitis linfocítica, colitis colagenosa y colitis ulcerativa en comparación a controles. Materiales y métodos: Se usó inmunohistoquímica para identificar a las células neuroendócrinas a través del marcador cromogranina A. El estudio incluyó 10 casos de cada diagnóstico de colitis linfocítica, colitis colagenosa y colitis ulcerativa. Resultados: Se encontró diferencia estadísticamente significativa en el conteo de células neuroendocrinas en colitis linfocítica (p=0,019104) y colitis ulcerativa en comparación con los controles (p=0,0077). En colitis colagenosa, se encontró un incremento de células neuroendocrinas pero no pudimos demostrar diferencias estadísticamente significativa. Conclusión: Se demostró hiperplasia de células neuroendocrinas en colitis linfocítica y colitis ulcerativa, lo que confirma lo reportado por los pocos estudios anteriores realizados sobre el tema.

Introduction: Some authors have found increase of neuroendocrine cells in microscopic colitis and ulcerative colitis. Objective: The aim of this study is to evaluate the presence of neuroendocrine cells in ulcerative colitis and lymphocytic colitis and collagenous colitis. Materials and methods: Immunohistochemistry was performed to identify neuroendocrine cells through marker chromogranin A (CgA). The study included 10 cases of each diagnosis of Lymphocytic colitis, collagenous colitis and ulcerative colitis. Results: There was statistically significant difference in the count of neuroendocrine cells, between lymphocytic colitis and control (p=0.019104), and between ulcerative colitis and controls (p=0.0077). In collagenous colitis there was an increase in neuroendocrine cells but we failed to find statistical differences. Conclusion: We could observe neuroendocrine cell hyperplasia in lymphocytic colitis and ulcerative colitis compared with controls, which confirm previous studies.

Current Consensus on I-131 MIBG Therapy / 대한핵의학회잡지

Metaiodobenzylguanidine (MIBG) is structurally similar to the neurotransmitter norepinephrine and specifically targets neuroendocrine cells including some neuroendocrine tumors. Iodine-131 (I-131)-labeled MIBG (I-131 MIBG) therapy for neuroendocrine tumors has been performed for more than a quarter-century. The indications of I-131 MIBG therapy include treatment-resistant neuroblastoma (NB), unresectable or metastatic pheochromocytoma (PC) and paraganglioma (PG), unresectable or metastatic carcinoid tumors, and unresectable or metastatic medullary thyroid cancer (MTC). I-131 MIBG therapy is one of the considerable effective treatments in patients with advanced NB, PC, and PG. On the other hand, I-131 MIBG therapy is an alternative method after more effective novel therapies are used such as radiolabeled somatostatin analogs and tyrosine kinase inhibitors in patients with advanced carcinoid tumors and MTC. No-carrier-aided (NCA) I-131 MIBG has more favorable potential compared to the conventional I-131 MIBG. Astatine-211-labeled meta-astatobenzylguanidine (At-211 MABG) has massive potential in patients with neuroendocrine tumors. Further studies about the therapeutic protocols of I-131 MIBG including NCA I-131 MIBG in the clinical setting and At-211 MABG in both the preclinical and clinical settings are needed.

Incidence and clinical characteristics of gastroenteropancreatic neuroendocrine tumor in Korea: a single-center experience

BACKGROUND/AIMS: Neuroendocrine tumors (NETs) may originate from heterogeneous neuroendocrine cells. The incidence is increasing worldwide, and World Health Organization (WHO) updated its classification in 2010. We investigated clinical characteristics of gastroenteropancreatic NETs in a single center. METHODS: Clinicopathologic characteristics of patients with pathologically confirmed gastroenteropancreatic NET in Seoul St. Mary Hospital from March 2009 to August 2011 were retrospectively analyzed. The grade and stage were determined according to WHO 2010 classification and TNM Staging System for Neuroendocrine Tumors (7th ed., 2010) of American Joint Committee on Cancer. RESULTS: One hundred and twenty-five patients (median age, 50; male, 61.3%) were analyzed. Among 100,000 patients who visited the hospital, incidence was 24.1. Only two patients (1.6%) had a functional NET. The rectum (n = 99, 79.8%) was most common primary site and found in early stage. The prevalence by stages was 84.7% stage I, 8.9% stage IV, 4.8% stage II, and 1.6% stage III. The pathology grading was 74.5% grade 1, 12.7% grade 2, and 12.7% grade 3. Tumor stage correlated positively with pathologic grade (Spearman’s rank correlation coefficient, 0.644). CONCLUSIONS: Wide range of clinicopathological features of Korean gastroenteropancreatic NETs were demonstrated using WHO 2010 classification. Rectal NET was most frequent and found in early stage.

Incidence and clinical characteristics of gastroenteropancreatic neuroendocrine tumor in Korea: a single-center experience

BACKGROUND/AIMS: Neuroendocrine tumors (NETs) may originate from heterogeneous neuroendocrine cells. The incidence is increasing worldwide, and World Health Organization (WHO) updated its classification in 2010. We investigated clinical characteristics of gastroenteropancreatic NETs in a single center. METHODS: Clinicopathologic characteristics of patients with pathologically confirmed gastroenteropancreatic NET in Seoul St. Mary Hospital from March 2009 to August 2011 were retrospectively analyzed. The grade and stage were determined according to WHO 2010 classification and TNM Staging System for Neuroendocrine Tumors (7th ed., 2010) of American Joint Committee on Cancer. RESULTS: One hundred and twenty-five patients (median age, 50; male, 61.3%) were analyzed. Among 100,000 patients who visited the hospital, incidence was 24.1. Only two patients (1.6%) had a functional NET. The rectum (n = 99, 79.8%) was most common primary site and found in early stage. The prevalence by stages was 84.7% stage I, 8.9% stage IV, 4.8% stage II, and 1.6% stage III. The pathology grading was 74.5% grade 1, 12.7% grade 2, and 12.7% grade 3. Tumor stage correlated positively with pathologic grade (Spearman’s rank correlation coefficient, 0.644). CONCLUSIONS: Wide range of clinicopathological features of Korean gastroenteropancreatic NETs were demonstrated using WHO 2010 classification. Rectal NET was most frequent and found in early stage.

A Pediatric Case of Mixed Acinar-Neuroendocrine Carcinoma / 소아외과

Mixed acinar-neuroendocrine carcinoma (MANEC) is a malignant pancreatic tumor that rarely occurs in children. It is diagnosed pathologically according to the proportion of neuroendocrine cells present, highlighting the need for surgical biopsy. A 13-year-old boy presented with a 10-cm palpable mass on CT. Surgical resection was performed, and the pathological diagnosis was MANEC. There were no postoperative complications, and the patient was discharged from the hospital 10 days after surgery. He is presently undergoing adjuvant chemotherapy. We reviewed historical MANEC cases published in the English literature. We concluded that pathological analysis of a surgically resected specimen is necessary for an accurate diagnosis of MANEC, and that publication of more cases is needed to determine the optimal management strategy for MANEC.

Immunohistochemical evaluation of the goat forestomach during prenatal development

Here we report the detection and distribution of synaptophysin (SPY), non-neuronal enolase (NNE), glial fibrillary acidic protein (GFAP), vimentin (VIM), neuropeptide Y (NPY), and vasoactive intestinal peptide (VIP) expression in the goat forestomach during prenatal development. A total of 140 embryos and fetuses were examined to evaluate protein expression from the first stage of prenatal life until birth. In all cases, SPY immunoreactivity was detected at 53 days gestation in the lamina propria-submucosa, tunica muscularis, serosa, and myenteric plexuses. Immunoreactivity to NNE was observed at 64 days gestation in the same locations as well as the epithelial layer. Glial cells were found at 64 days as indicated by signals corresponding to GFAP and VIM at 39 days. Positive staining for NPY and VIP was observed at 113, 75, and 95 days in the rumen, reticulum, and omasum, respectively, in the lamina propria-submucosa, tunica muscularis, and myenteric plexuses of each of these gastric compartments. These findings indicate possible preparation of the fetal goat forestomach for postnatal function. Compared to other ruminant species, neuroendocrine cells, glial cells and peptidergic innervations markers were detected earlier compared to sheep but at around the same stage as in deer.

p27 Loss Is Associated with Poor Prognosis in Gastroenteropancreatic Neuroendocrine Tumors / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) represent a heterogeneous disease group originating from the neuroendocrine cells. Identification of prognostic markers, related to neuroendocrine tissue-selective tumorigenesis, is necessary to find therapeutic targets. MATERIALS AND METHODS: A total of 327 patients with GEP-NETs were included in this study; there were 49 gastric, 29 duodenal, 49 pancreatic, 12 hepatobiliary, 33 appendiceal, 5 proximal colon, and 150 distal colon cases. We performed immunostaining with the tissue microarray method for menin, p27, and p18. RESULTS: We observed negative staining for menin, p27, and p18 in 34%, 21%, and 56% of GEP-NETs, respectively. The loss of p27, but not menin, was positively correlated with the grade of Ki-67. Menin-/p27-, menin-/p27+, menin+/p27-, and menin+/p27+ phenotype groups included 13%, 22%, 8%, and 57% of patients, respectively. A dichotomized comparison showed that menin- or p27- tumors were significantly associated with foregut and midgut localizations, high World Health Organization (WHO) grade, lymph node metastasis, and more advanced stage as compared to menin+/p27+ patients. Kaplan-Meier analysis for the overall survival showed that p27 loss was significantly associated with decreased survival. Multivariate analysis showed that p27 loss is an independent factor for poor overall survival. CONCLUSION: Our results revealed that the loss of p27 is associated with poor prognosis and the menin-p27 pathway is important in the tumorigenesis of GEP-NETs.

Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series / Critérios diagnósticos e seguimento em hiperplasia de células neuroendócrinas do lactente: uma série de casos

OBJECTIVE: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease characterized by tachypnea, retractions, crackles, and hypoxia. The aim of this study was to report and discuss the clinical, imaging, and histopathological findings in a series of NEHI cases at a tertiary pediatric hospital, with an emphasis on diagnostic criteria and clinical outcomes. METHODS: Between 2003 and 2011, 12 full-term infants were diagnosed with NEHI, based on clinical and tomographic findings. Those infants were followed for 1-91 months. Four infants were biopsied, and the histopathological specimens were stained with bombesin antibody. RESULTS: In this case series, symptoms appeared at birth in 6 infants and by 3 months of age in the remaining 6. In all of the cases, NEHI was associated with acute respiratory infection. The most common initial chest HRCT findings were ground-glass opacities that were in the middle lobe/lingula in 12 patients and in other medullary areas in 10. Air trapping was the second most common finding, being observed in 7 patients. Follow-up HRCT scans (performed in 10 patients) revealed normal results in 1 patient and improvement in 9. The biopsy findings were nonspecific, and the staining was positive for bombesin in all samples. Confirmation of NEHI was primarily based on clinical and tomographic findings. Symptoms improved during the follow-up period (mean, 41 months). A clinical cure was achieved in 4 patients. CONCLUSIONS: In this sample of patients, the diagnosis of NEHI was made on the basis of the clinical and tomographic findings, independent of the lung biopsy results. Most of the patients showed clinical improvement and persistent tomographic changes during the follow-up period, regardless of the initial severity of the disease or type of treatment. .

OBJETIVO: A hiperplasia de células neuroendócrinas do lactente (HCNEL) é uma forma de doença pulmonar intersticial da infância caracterizada por taquipneia, retrações, estertores e hipóxia. O objetivo deste estudo foi descrever e discutir os achados clínicos, histopatológicos e de imagem em uma série de casos de HCNEL em um hospital pediátrico terciário, enfatizando critérios de diagnóstico e desfechos clínicos. MÉTODOS: Entre 2003 e 2011, 12 lactentes nascidos a termo foram diagnosticados com HCNEL, com base em critérios clínico-tomográficos e acompanhados por 1-91 meses. Quatro lactentes foram submetidos a biopsia pulmonar, e as amostras histopatológicas foram coradas com anticorpo para bombesina. RESULTADOS: Nesta série de casos, os sintomas surgiram ao nascimento em 6 lactentes e em até 3 meses de idade nos outros 6. Em todos os casos, HCNEL estava associada com infecção respiratória aguda. Os achados iniciais em TCAR de tórax foram opacidades em vidro fosco em lobo médio e língula, em 12 pacientes, e em outras regiões medulares, em 10. O aprisionamento aéreo foi o segundo achado mais frequente, em 7 pacientes. As TCAR de controle (realizadas em 10 pacientes) revelaram resultados normais (em 1) e melhorias (em 9). Os achados de biopsia foram inespecíficos, e os resultados para bombesina foram positivos em todas as amostras. A confirmação de HCNEL baseou-se principalmente em achados clínico-tomográficos. Os sintomas melhoraram durante o seguimento (média, 41 meses). Quatro pacientes apresentaram cura clínica. CONCLUSÕES: Os achados clínicos e tomográficos permitiram o diagnóstico de HCNEL nesta série de pacientes, independentemente dos resultados da biopsia pulmonar. A maioria mostrou melhora clínica e ...

Células neuroendocrinas en yemas gustativas primordiales / Neuroendocrine cells in primordial taste buds

El objetivo del trabajo fue identificar la presencia de células neuroendócrinas en yemas gustativas primordiales (fetales) humanas. Fueron utilizadas 15 lenguas fetales humanas obtenidas de abortos espontáneos (tiempo de gestación 23 semanas) de pacientes ingresados en el Hospital General, Chihuahua, México. Una muestra representativa del ápex lingual fue embebida en parafina y cortada a 3 micras para ser procesadas con la técnica inmunohitoquímica utilizando los siguientes anticuerpos: Anti-sinaptofisina; anti-proteína neurofilamento; anti-cromogranina; anti-citoqueratina 20; y anti proteínas-S100. El protocolo de investigación fue aprobado por los comités de bioética de las instituciones participantes. Se obtuvo la autorización de los padres para la utilización del material biológico. Las células gustativas fetales fueron fuertemente positivas a cromogranina, y negativas a sinaptofisina. Las papilas gustativas fetales humanas mostraron inmunorreactividad positiva contra citoqueratina 20, mientras que fibras nerviosas intragemulares mostraron inmunorreactividad contra anticuerpos anti-proteína neurofilamento. Las fibras nerviosas subyacentes al epitelio gustativo fueron positivas a proteína S100. Se muestra evidencia inmunohistoquímica de la presencia de células neuroendócrinas gustativa en yemas gustativas linguales primordiales humanas. Esto sugiere una probable participación neuroendocrina o paracrina en el desarrollo de las yemas gustativas humanas.

The objective was to identify the presence of neuroendocrine cells in primary taste buds (fetal) cells. We used 15 human embryionic fetal tongues derived from human spontaneous abortions (23 weeks gestation time) of patients admitted to the General Hospital, Chihuahua Mexico. A representative sample of lingual apex was embedded in paraffin and cut to 3 microns processed by immune histochemical technique using the following antibodies: anti-synaptophysin, neuro filament anti-protein, anti chromogranin; anti-citokeratin 20 and S100 anti proteins. Research was approved by bioethics committees of the participating institutions. Permission was obtained from the parents to use the biological material. The taste bud fetal cells were significantly positive for chromogranin and synaptophysin negative. The human fetal taste buds showed positive immunoreactivity against cyto keratin 20, while nerve fibers underlying the gustatory epithelium were positive for S100 protein. Immunohistochemical evidence shows the presence of neuro endocrine cells in human primordial taste bud papilla. This suggests a probable neuro endocrine or paracrine participation in the development of human taste buds.

Detection and clinicopathologic significance of microsatellite alteration and p53 mutation of neuroendocrine cells in colorectal adenocarcinoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the possible clonal origin of neuroendocrine cells in colorectal adenocarcinoma.</p><p><b>METHODS</b>Twenty-six microsatellite loci were screened using laser capture microdissection, DNA extraction and whole genome amplification. Microsatellite instability (MSI) and loss of heterozygosity (LOH) in adenocarcinoma cells and neuroendocrine cells amongst 30 cases of colorectal carcinoma with neuroendocrine differentiation were detected using polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP)-silver staining. The mutation status of p53 was evaluated by PCR-sequencing. The clonal origin of neuroendocrine cells in colorectal adenocarcinoma was determined.</p><p><b>RESULTS</b>Amongst the 30 cases studied, the prevalence of MSI was 16.9% while that of LOH was 8.5%. The rate showed no statistically significant difference between adenocarcinoma cells and neuroendocrine cells. In 6 cases, the microsatellite alteration was entirely consistent. In 23 cases, the rate of microsatellite alteration consistency was greater than that of inconsistency. In 1 case, the consistency and inconsistency rates were identical. There was statistically significant difference between consistency and inconsistency of microsatellite alteration. The prevalence of p53 mutation was 16.7% which was the same for both adenocarcinoma cells and neuroendocrine cells.</p><p><b>CONCLUSIONS</b>Adenocarcinoma cells and neuroendocrine cells in colorectal adenocarcinoma with neuroendocrine differentiation have similar biologic changes. It is likely that they are of identical origin.</p>

A Carcinoid Tumor Arising from a Normal Kidney in a Young Man / 대한내과학회지

Carcinoid tumors are low-grade malignant tumors arising from neuroendocrine cells. Primary renal carcinoid tumor is very rare due to the absence of neuroendocrine cells in the kidney and ureter. Therefore, little is known about the management and prognosis of renal carcinoid. Here, we report a case of a primary renal carcinoid tumor arising from a normal kidney in a 21-year-old man. He presented with a left renal mass, which was found accidentally. Abdominal computed tomography (CT) showed a 5.5 x 5.0-cm cystic mass with calcification. We suspected a cystic renal cell carcinoma and performed a laparoscopic radical nephrectomy. However, the histology revealed a well-differentiated neuroendocrine tumor. We concluded that it was a primary renal carcinoid tumor with no distant metastasis and did not administer chemotherapy or radiation therapy. He is recurrence-free after 8 months.

Variable Clinical Classifications and Diagnostic Coding Systems of Colorectal Neuroendocrine Tumor

The incidence of colorectal carcinoid tumor is recently increasing as screening colonoscopy increased. Traditional carcinoid tumor had been known as low grade, malignant neuroendocrine cell orign tumor. In 2000, World Health Organization (WHO) suggested that carcinoid was called well-differentiated neuroendocrine tumor (NET). It recently updated in 2010 by WHO; according to the differentiation and malignant potential, NET classified with NET Grade 1, Grade 2, and neuroendocrine carcinoma. They suggested that NET had malignant potential in accordance with histopathologic characteristics. Therefore, WHO recommended the behavior code of NET as malignant. However, European Neuroendocrine Tumor Society (ENETS) proposed the behavior of NET to four grades based on the histopathologic features; benign, benign or low grade malignant, low grade malignant, and high grade malignant. Also, American Joint Committee on Cancer (AJCC) suggested that topography codes of NET were defined as malignant. Korean Standard Classification of Diseases (KCD) described the different codings of carcinoid (NET). The discrepancies of behavior code or coding system exist among WHO, ENETS, AJCC and KCD. Also, there were differences in the perception for topographic coding system between clinicians and pathologists. NETs of colorectum were reported with the variable clinical characteristics (especially, metastasis) and long term prognosis from many studies. Especially, risk of metastasis and long term prognosis of small sized NET (<1 cm) had some discrepancies and should be investigated prospectively. Therefore, the consensus about topographic codes of NET should be needed with multidisplinary approach among gastroenterologists, pathologists and surgeons.

Acinar Cell Carcinoma of the Pancreas: Clinical and Cytomorphologic Characteristics

Acinar cell carcinoma is a rare malignant epithelial neoplasm with predominantly exocrine acinar differentiation and is seen primarily in older men (mean age, 62 years). The presenting symptoms are usually non-specific, and jaundice is often not present. Symptoms relating to the overproduction and release of lipase into the circulation are present in 10-15% of patients. Characteristic cytomorphologic features include a population of cells with minimal pleomorphism, eccentrically placed nuclei with a single prominent nucleoli and moderate hyperchromasia. The cytoplasm is finely granular, and the background may contain granular debris secondary to cytolysis. A significant proportion of the cases also have a minor neuroendocrine component or scattered neuroendocrine cells. Approximately 50% of patients have metastatic disease at presentation, often restricted to the regional lymph nodes and liver. The prognosis is poor, only slightly better than that of pancreatic ductal adenocarcinoma.

Carcinoma en tejido mamario ectópico axilar con diferenciación neuroendocrina: reporte de un caso / Carcinoma in ectopic axillary breast tissue with neuroendocrine differentiation: case report

Presentar un caso de carcinoma en tejido mamario ectópico axilar. Paciente femenina de 38 años de edad, quien consultó por aumento de volumen y nódulo en región axilar de un año de evolución y punción aspiración por aguja fina previa no diagnóstica. En la evaluación se observó mamas axilares bilaterales, palpando en la derecha tumor duro de superficie irregular, se realizó mamografía, C y biopsia por aguja gruesa. Por el diagnóstico de la biopsia por aguja gruesa, se practicaron estudios de extensión y se trató con neoadyuvancia y cirugía. El estudio histopatológico de la pieza quirúrgica concluyó carcinoma ductal infiltrante con patrón neuroendocrino y metástasis en 2 de 18 ganglios, recibió adyuvancia y se mantiene libre de enfermedad

A case of female patient 38 years of age, who consulted for increased volume and axillary node in a year of evolution and prior non-diagnostic FNA. The evaluation noted bilateral axillary breasts, feeling hard lump on the right an irregular surface, we performed mammography, PAAF and core needle biopsy. For the diagnosis of the biopsy needle, extension studies were performed and treated with neoadjuvant therapy and surgery. Histopathological examination of the surgical specimen concluded infiltrating ductal carcinoma neuroendocrine pattern and metastatic in 2 of 18 nodes, received adjuvant therapy and remains free of disease

Pulmonary neuroendocrine cell hyperplasia and tumorlets in bronchiectasis: a clinicopathologic study of 22 cases with review of literature / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinical and pathological features of pulmonary neuroendocrine cell hyperplasia and tumorlets with bronchiectasis.</p><p><b>METHODS</b>Both the clinicopathologic changes and immunohistochemical findings were examined with microscopy and EnVision method in 22 cases of pulmonary neuroendocrine cell hyperplasia and tumorlets.</p><p><b>RESULTS</b>The average age of the 22 patients was 53 years, with a male to female ratio of 9:13. On macroscopic examination the lungs showed bronchiectasis; one case was accompanied by gray-white, soft nodules (diameter < 5 mm). Microscopy of the HE sections showed the basic pathologic change was bronchiectasis, accompanied by neuroendocrine cell hyperplasia and tumorlet formation in the pulmonary parenchyma surrounding the bronchioles, presenting as single nodule (10 patients), or multifocal nodules (12 patients), with average size of 1.6 mm in diameter. No tumor cells were identified in the lymph nodes. Sixteen of 22 patients were disease-free after an average follow-up period of 58 months (17 - 117 months); one patient died suddenly after surgery; and five were loss of follow up. Immunohistologically, the tumor cells were positive for CgA (18/18), Syn (16/16), AE1/AE3 (16/16) , TTF-1 (14/15), and CD56 (14/14), and Ki-67 index was < 2% in 12 cases.</p><p><b>CONCLUSIONS</b>Immunohistological staining for CgA, Syn, CD56, TTF-1 and AE1/AE3 can confirm the diagnosis. Early detection, pulmonary resection and follow-up help prevent the progression of these diseases.</p>

Prostatic neuroendocrine cells and their association with chronic prostatitis / 中华男科学杂志

Neuroendocrine cells are abundant in all the body tissues and organs as well as the nervous system, either the central or the peripheral nervous system. In the normal prostate tissue, there are a few neuroendocrine cells, too, in addition to basal and epithelial cells. Prostatic neuroendocrine cells play the function of regulating the development, secretion and differentiation of the prostate. Recent studies show that prostatic neuroendocrine cells may be involved in the pathogenesis of chronic prostatitis through their activity and secreted products. This article presents an overview on the origin, distribution, morphology, structure, secretion and functions of prostatic neuroendocrine cells and their association with chronic prostatitis.