Differentiating pediatric cystic nephroma from common renal multicystic lesions: A case report

Pediatric cystic nephroma is a rare, clinically benign, renal tumor. Pediatric renal cystic lesions are complex. Imaging findings and tumor appearance are often nonspecific, and careful pathological examination is necessary. We discuss diagnosis of pediatric cystic nephroma and how to differentiate it from multicystic dysplastic kidney and cystic partially differentiated nephroblastoma.

Tumor de Wilms en displasia renal multiquística

RESUMEN Introducción: La displasia renal multiquística es una anomalía congénita caracterizada por un riñón afuncional con quistes de diferentes tamaños. Es considerada como una anomalía del desarrollo, aunque se han descrito casos hereditarios. Se ha puntualizado la degeneración maligna de esta displasia, pero su asociación es muy rara. Objetivo: Describir una paciente con tumor de Wilms asociado a una displasia renal multiquística. Presentación del caso: Paciente de sexo femenino y 10 años de edad que los ultrasonidos prenatales no detectaron anomalías del tracto urinario y a los 7 años un estudio ecográfico detectó quistes en el riñón izquierdo. No cumplió el seguimiento clínico y ultrasonográfico indicado y abandonó la consulta; los 10 años asistió al médico por síntomas respiratorios agudos y al palpar el abdomen se comprobó masa en flanco izquierdo de consistencia leñosa, no dolorosa y con contacto lumbar. Se realizó biopsia renal y se diagnosticó tumor de Wilms, se impuso tratamiento citostático y se realizó nefrectomía que confirmó el diagnóstico. Entre los antecedentes familiares se informa un hermano fallecido antes de las 72 horas de nacido por displasia renal multiquística bilateral y madre con nefropatía por reflujo vesicoureteral. Conclusiones: La paciente que se describe reúne tres características raras: un tumor maligno asociado a una displasia renal multiquística, la posibilidad hereditaria por el antecedente del hermano con la displasia bilateral y un tumor de Wilms a los 10 años de edad, lo que demuestra la importancia del seguimiento en estos pacientes.

ABSTRACT Introduction: Multicystic renal dysplasia is a congenital anomaly characterized by an afunctional kidney with cysts of different sizes. It is considered a developmental abnormality, although hereditary cases have been described. The malignant degeneration of this dysplasia has been pointed out, but its association is very rare. Objective: Describe a patient with Wilms tumor associated with multicystic renal dysplasia. Case Presentation: 10-year-old female patient to whom prenatal ultrasounds did not detect urinary tract abnormalities and at age 7 an ultrasound study detected cysts in the left kidney. She did not comply with the indicated clinical and ultrasonographic follow-up and left the consultation; at 10 years old, she attended the doctor for acute respiratory symptoms and when palpating the abdomen there was a mass on the left flank of woody consistency, not painful and with lumbar contact. A renal biopsy was conducted and Wilms tumor was diagnosed; cytostatic treatment was indicated, and a nephrectomy was performed that confirmed the diagnosis. Among the family history is reported a brother who died before 72 hours of birth due to bilateral multicystic renal dysplasia and a mother with nephropathy by vesicoureteral reflux. Conclusions: The described patient has three rare characteristics: a malignant tumor associated with multicystic renal dysplasia, the hereditary possibility due to the sibling's history of bilateral dysplasia, and a Wilms tumor at 10 years of age, which demonstrates the importance of follow-up in these patients.

Mesotelioma peritoneal multiquístico benigno: reporte de un caso y revisión de la literatura / Benign multicystic peritoneal mesothelioma: case report and literature review

INTRODUCCIÓN: El mesotelioma peritoneal multiquístico benigno (MPMB) es una entidad con muy baja incidencia que se presentan con mayor frecuencia en mujeres jóvenes. CASO CLÍNICO: Paciente de 17 años que consulta por amenorrea secundaria. Además, refería pérdida de peso, estreñimiento y molestias abdominales. La exploración fue normal y en las pruebas de imagen se objetivó una pelvis ocupada por una masa multiquística, de unos 20 cm de diámetro máximo, de paredes finas y contenido anecoico. Se solicitaron marcadores tumorales de los cuales sólo se encontró elevado el CA 19.9 (35,2 U/mL). Se decidió realizar una laparoscopia exploradora para establecer un diagnóstico y ante los hallazgos y al considerarse la masa fácilmente resecable, en el mismo acto quirúrgico se extirpó por completo para su estudio anatomopatológico, que confirmó un MPMB. Se desestimaron tratamientos adicionales y actualmente se encuentra en seguimiento y asintomática. CONCLUSIÓN: La patogénesis del MPMB no está clara. Algunos autores defienden que se trata de una reacción peritoneal a un daño inflamatorio, otros apoyan que se trata de una neoplasia benigna. Esta naturaleza incierta, junto con la escasa experiencia, hacen que el manejo resulte complejo. Es necesario individualizar cada caso e intentar ser conservadores especialmente en pacientes jóvenes. Si se opta por un manejo activo, lo más acertado es la citorreducción completa seguida de quimioterapia hipertérmica peritoneal. El seguimiento, en cualquier caso, debe ser exhaustivo y multidisciplinar principalmente con pruebas de imagen.

BACKGROUND: Benign Multicystic Peritoneal Mesothelioma (BMPM) is an entity with a very low incidence that occurs more frequently in young women. CASE REPORT: A 17-year-old woman attended for secondary amenorrhea. She also referred to weight loss, constipation and abdominal discomfort. The examination was normal and echography revealed a pelvis occupied by a 20 centimeters multicystic mass, with thin walls and anechoic content. A preoperative study with tumor markers was requested in which only a CA 19.9 elevation was observed (35.2 U/ml 0-27). It was decided to perform an exploratory laparoscopy to establish a diagnosis. Due to the findings and because of the fact that the mass was considered easy to remove, it was completely removed in the same surgical act. The pathological result confirmed a BMPM. Additional treatments have been dismissed and are the patient is currently being followed up without symptoms. CONCLUSION: The pathogenesis of BMPM is unclear. Some authors argue that it is a peritoneal reaction to inflammatory damage, others support that it is a benign neoplasm. This uncertain nature coupled with limited experience make management complex. It is necessary to evaluate each case and try to be conservative especially in young patients. If surgery management is chosen, complete cytoreduction followed by hyperthermic peritoneal chemotherapy is the most successful option. The follow-up in any case must be exhaustive and multidisciplinary, mainly with imaging tests.

Urine hepcidin, netrin-1, neutrophil gelatinase-associated lipocalin and C-C motif chemokine ligand 2 levels in multicystic dysplastic kidney / Níveis de hepcidina, netrina-1, lipocalina associada a gelatinase neutrofílica e ligante de quimiocina C-C motif na urina do rim displásico multicístico

ABSTRACT Introduction: Glomerular hyperfiltration may lead to proteinuria and chronic kidney disease in unilateral multicystic dysplastic kidney (MCDK). We aimed to investigate the urine neutrophil-gelatinase-associated lipocalin (NGAL), netrin-1, hepcidin, and C-C motif chemokine ligand-2 (MCP-1/CCL-2) levels in patients with MCDK. Methods: Thirty-two patients and 25 controls were included. The urine hepcidin, netrin-1, NGAL, and MCP-1/CCL-2 levels were determined by ELISA. Results: The patients had higher serum creatinine (Cr) levels, urine albumin, and netrin-1/Cr ratio with lower GFR. There were positive correlations between urine protein/Cr, MCP-1/CCL-2/Cr, and netrin-1 with NGAL (r = 0.397, p = 0.031; r = 0.437, p = 0.041, r = 0.323, p = 0.042, respectively). Urine netrin-1/Cr was positively correlated with MCP-1/CCL-2/Cr (r = 0.356, p = 0.045). There were positive associations between the presence of proteinuria and netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr [Odds ratio (OR): 1.423, p = 0.037, OR: 1.553, p = 0.033, OR: 2.112, p = 0.027, respectively)]. ROC curve analysis showed that netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr had high predictive values for determining proteinuria p = 0.027, p = 0.041, p = 0.035, respectively). Urine hepcidin/Cr was negatively correlated with tubular phosphorus reabsorption and was positively correlated with urine NGAL/Cr (r = -0.418, p = 0.019; r = 0.682, p = 0.000; respectively). Conclusions: MCP-1/CCL-2 may play a role in the development of proteinuria in MCDK. Netrin-1 may be a protective factor against proteinuria-induced renal injury. Urine hepcidin/Cr may reflect proximal tubule damage in MCDK. Urine NGAL/Cr may be a predictor of tubule damage by proteinuria.

Resumo Introdução: A hiperfiltração glomerular pode causar proteinúria e doença renal crônica no rim displásico multicístico unilateral (RDM). Nosso objetivo foi investigar os níveis de lipocalina associada à gelatinase neutrofílica na urina (NGAL), netrina-1, hepcidina e quimiocina C-C com ligante-2 (MCP-1/CCL-2) em pacientes com RDM. Métodos: Trinta e dois pacientes e 25 controles foram incluídos. Os níveis urinários de hepcidina, netrin-1, NGAL e MCP-1/CCL-2 foram determinados por ELISA. Resultados: Os pacientes apresentaram níveis séricos mais elevados de creatinina (Cr), albumina na urina e relação netrina-1/Cr com menor TFG. Houve correlação positiva entre proteína na urina/Cr, MCP-1/CCL-2/Cr e netrina-1 com NGAL (r = 0,397, p = 0,031; r = 0,437, p = 0,041, r = 0,323, p = 0,042, respectivamente). A netrina-1/Cr na urina foi correlacionada positivamente com MCP-1/CCL-2/Cr (r = 0,356, p = 0,045). Houve associações positivas entre a presença de proteinúria e netrina-1/Cr, MCP-1/CCL-2/Cr e NGAL/Cr [Odds ratio (OR): 1,423, p = 0,037, OR: 1,553, p = 0,033, OR: 2,112, p = 0,027, respectivamente) ]. A análise da curva ROC mostrou que netrina-1/Cr, MCP-1/CCL-2/Cr e NGAL/Cr apresentaram altos valores preditivos para determinar a proteinúria p = 0,027, p = 0,041, p = 0,035, respectivamente). A hepcidina/Cr na urina foi correlacionada negativamente com a reabsorção tubular de fósforo e positivamente com a NGAL/Cr na urina (r = -0,418, p = 0,019; r = 0,682, p = 0,000; respectivamente). Conclusões: MCP-1/CCL-2 pode ter participação no desenvolvimento de proteinúria no RDM. A Netrina-1 pode ser um fator protetor contra lesão renal induzida por proteinúria. Hepcidina/Cr na urina pode refletir danos em túbulos proximais no RDM. O valor de NGAL/Cr urinário pode ser um preditor de danos nos túbulos por proteinúria.

Immunohistochemical expression of p53 and murine double minute 2 protein in odontogenic keratocyst versus variants of ameloblastoma

Introduction: Oncogenes and tumor suppressor genes play a major role in cancer formation, growth, and progression. One of the important findings in this area is that murine double minute 2 (MDM2) oncogene is a negative regulator of wild-type p53. In tumors, expressing wild-type p53, inhibition of MDM2 expression will stabilize p53 and allow it to perform its proapoptotic function, while simultaneously preventing MDM2 from exerting its p53-independent oncogenic effects. The intracellular levels of p53 are tightly regulated by MDM2, as it is a key player in autoregulatory feedback loop under nonstressed conditions. The p53-MDM2 relationship is vital not only for essential functions of the cell, but it also appears to be an integrated part of the complex cellular network which supports the importance of this affair and is a hallmark for its coexistence. Subjects and Methods: This study was designed to identify immunohistochemically the expression of p53 and MDM2 gene using monoclonal antibody in 60 cases of formalin-fixed paraffin-embedded tissue blocks, of which 20 cases were of solid multicystic ameloblastoma (SMA), 20 cases were of odontogenic keratocyst (OKC), and 20 cases were of unicystic ameloblastoma (UA). Results: Immunoexpression of p53 and MDM2 was highest in OKC followed by SMA and was minimum in UA. Further results showed positive correlation between both the molecules. Conclusion: The studied showed that the relationship has a significant role in cancer etiology and progression and therefore is an important topic for future research which should help in the development of new therapeutic agent against cancer

Van Wyk Grumbach Syndrome: A Rare Cause of Precocity in a Common Condition

@#<p>A nine-year old myxedematous girl with short stature, isosexual pseudoprecocious puberty and bilateral multicystic ovaries underwent pelvic laparotomy due to an acute abdomen because of adnexal torsion. She also delayed bone, age, severe hypothyroidism, hyperprolactinemia, hyperestrogenism and pituitary adenoma. All of these findings led to the diagnosis of Van Wyk Grumback Syndrome, which is a rare condition characterized by long standing untreated hypothyoidism, pseudoprecocious puberty and nulticystic ovaries. Adminsitration of levothyroxine efected gradual regression and reversal of symptoms. This case highlight the significance of recognizing the clinical features of this syndrome in order to medically treat the child and thus, prevent its undesirable effects in the physial andmental growth an dpossible surgical cimplications that ay affect her future reproductive capacity.</p>

Flexible Fiberoptic Bronchoscopy Directed Interventions in Neonatal Intensive Care Unit

Objective: To describe the utility of flexible fiberoptic bronchoscopy for the diagnosis andmanagement in the neonatal ICU. Methods: A retrospective, medical chart review wasconducted in neonates who underwent flexible fiberoptic bronchoscopy over a period of 7years. Besides demographic data and diagnostic findings, the results of medical and/orsurgical interventions done by treating neonatologist were recorded. Results: 88bronchoscopies were performed in 83 neonates, of which 37 were done throughendotracheal tube. Indications included persistent need for mechanical ventilation (32),persistent atelectasis (21), and stridor (27). Most common airway anomalies diagnosedincluded tracheobronchomalacia (20), laryngomalacia (18), subglottic stenosis (7), choanalatresia (4), laryngeal cleft (4), and tracheoesophageal fistula (4). Surgical interventions wereundertaken in 17 cases (9 tracheostomies and 2 cases of slide tracheoplasty). Conclusion:Flexible fiberoptic bronchoscopy can be beneficial for the diagnosis and management ofneonates with persistent or undiagnosed respiratory problems.

Recurrent aggressive multicystic ameloblastoma with hemangiomatous proliferation and giant cells: A perplexed diagnosis

Hemangiomatous ameloblastoma, a very rare variant of ameloblastoma, was originally described as ameloblastoma in which part of the tumor contained spaces filled with blood or large endothelial-lined capillaries. Ameloblastoma, the most commonly affecting a slow-growing, persistent, and locally aggressive neoplasm of epithelial origin, account for a major chunk of all odontogenic tumors. A 43-year-old female reported to our outpatient department with a rare association of multicystic ameloblastoma with unusual hemangiomatous proliferation (both in lining and the stroma) along with giant cells in the lesional tissue. There are <8 cases documented in the literature with the same histologic features and most are in the middle age with the mandibular location.

Multicystic Encephalomalacia:Report of One Case / 中国医学科学院学报

The magnetic resonance imaging findings of multicystic encephalomalacia are featured by bilateral frontal large cystic lesion with corpus callosum involvement,evident heterogeneous enhancement of the lesion margin,ring hyperintensity on diffusion weighted imaging,and high choline peak and low N-acetylaspartate peak of the enhanced lesion margin on magnetic resonance spectroscopy.This article reports a case of multicystic encephalomalacia.

Cystic kidney disease in children / 中华实用儿科临床杂志

Both the incidence and prevalence of chronic kidney disease are rising rapidly all over the world, which brings heavy economic and mental burden to the patients and the society. A retrospective study of the European Renal Association _ European Dialysis and Transplant Association( ERA _ EDTA) Registry showed cystic kidney disease was one of the three most prevalent rare diseases leading to renal replacement therapy before the age of 20 years. Renal cystic disease encompasses a variety of diseases that cause single or multiple cysts in the kidneys,and can be divided into 3 categories:cystic kidney disease due to fetal renal malformations,hereditary cystic kidney diseases and acquired cystic kidney diseases. Acquired renal cysts rarely occur in the general pediatric population. Renal cystic disease can present during fetus,infancy,childhood or adulthood. The onset of the disease is occult,and the clinical manifestations are heterogeneous. The manifestations may be confined to the kidney or as part of a syndrome. On renal imaging,the kidney size is normal,enlarged or small,and cysts can involve unilateral or bilateral kidneys. Pamily history investigation should be performed when collecting first_hand clinical data. Genetic testing can confirm the diagnosis of hereditary renal cystic disease. Cystic kidney disease is currently treated symptomatically,and patients with end_stage renal disease require renal replacement therapy.

Ameloblastoma maxilar: a propósito de un caso / Maxillary ameloblastoma. Aclinical case

El Ameloblastoma es un tumor odontogenico benigno, poco frecuente, pero con alta tasa de recurrencia debido a su carácter localmente invasivo. Se produce en la región mandibular en la mayoría de los casos, siendo solo en un 20% de localización maxilar. Se presenta el caso clínico de un paciente, hombre, de 60 años con un Ameloblastoma Uniquístico de localización maxilar en región posterior, tratado con una maxilectomía parcial con incisión de Weber Ferguson y reconstrucción de maxilar con malla de titanio y obturador removible. A partir de este se presenta una breve revisión de la literatura con respecto al Ameloblastoma.

Ameloblastoma is an infrequent benign odontogenic tumor with a high recurrence rate due to its locally invasive nature. Most originate in the upper jaw with only 20% found in the lower jaw. We present the clinical case of a sixty year old male with monocystic ameloblastoma located in the posterior maxillary region. We performed a partial maxillectomy using a Weber Ferguson incision and carried out reconstruction using a titanium mesh and removable plug. We present a brief review of the literature concerning ameloblastoma.

Prenatal ultrasound in diagnosis of fetal unilateral polycystic kidney disease / 中国医学影像技术

Objective To investigate the value of ultrasound in diagnosis of fetal simple unilateral multicystic dysplastic kidney (MCDK) disease.Methods Pregnant women who underwent prenatal ultrasound screening and follow-up were analyzed retrospectively,and 29 fetues with MCDK were found.After exclusion of pregnancy syndrome,other structural abnormalities and chromosomal abnormalities,15 fetues willing to continue pregnancy and accepting the follow-up tracking observation were observed to postpartum.Ipsilateral renal ultrasonographic characteristics,contralateral renal morphology and size,growth and development of children and the renal function were analyzed.Results The minimum follow-up time of the 15 fetus was pregnancy to 7 months after birth,the maximum follow-up time was pregnancy to 5 years of age in children.Ultrasound showed that ipsilateral kidney volume became large in fetal period,reduced gradually in late pregnancy,and atrophy in 5 6 months after birth,even could not displayed with untrasound.The shape,size,and sonographic characteristics of the healthy kidney were similar to the normal gestational age kidney.Prenatal fetal growth indicators and amniotic fluid volume were normal.After birth,except for 1 child with overweight,other children's growth and development indicators were almost normal.Conclusion Fetus with simple unilateral MCDK has a good prognosis.Ultrasound has important value in the examination and follow-up.It can provide reliable basis for prenatal diagnosis and consultation.

Ureterocalycostomy - final resort in the management of secondary pelvi-ureteric junction obstruction: our experience

ABSTRACT: Ureterocalycostomy can be performed in patients in whom desired methods of treating secondary PUJ (Pelvi-Ureteric Junction) obstructions either failed or could not be used. In our study, one child and two adults in whom one redo-ureterocalycostomy and two ureterocalycostomies were performed for severely scarred PUJ. The causes for secondary PUJ obstruction were post-pyelolithotomy in one case, post-pyeloplasty and ureterocalycostomy for PUJ obstruction in the second patient and the third patient had long upper ureteric stricture post-ureteropyeloplasty due to tuberculosis. In all these cases ureterocalycostomy proved to be salvage/final resort for preserving functional renal unit

High-pressure balloon assessment of pelviureteric junction prior to laparoscopic "vascular hitch"

ABSTRACT Aim To assess if calibration of the ureteropelvic junction (UPJ) using a high-pressure balloon inflated at the UPJ level in patients with suspected crossing vessels (CV) could differentiate between intrinsic and extrinsic stenosis prior to laparoscopic vascular hitch (VH). Materials and Methods We reviewed patients with UPJO diagnosed at childhood or adolescence without previous evidence of antenatal or infant hydronephrosis (10 patients). By cystoscopy, a high-pressure balloon is sited at the UPJ and the balloon inflated to 8-12 atm under radiological screening. We considered intrinsic PUJO to be present where a ‘waist’ was observed at the PUJ on inflation of the balloon and a laparoscopic dismembered pyeloplasty is performed When no ‘waist’ is observed we considered this to represent extrinsic stenosis and a laparoscopic VH was performed. Patients with absence of intrinsic PUJ stenosis documented with this method are included for the study. Results Six patients presented pure extrinsic stenosis. The mean age at presentation was 10.8 years. Mean duration of surgery was 99 min and mean hospital stay was 24 hours in all cases. We found no intraoperative or postoperative complications. All children remain symptoms free at a mean follow up of 14 months. Ultrasound and renogram improved in all cases. Conclusion When no ‘waist’ is observed we considered this to represent extrinsic stenosis and a laparoscopic VH was performed. In these patients, laparoscopic transposition of lower pole crossing vessels (‘vascular hitch’) may be a safe and reliable surgical technique.

Management of Large Mandibular Ameloblastoma Crossing Midline: Reconstructed by Bilateral Iliac Crest Graft: A Rare Entity.

Ameloblastoma is a true neoplasm of odontogenic epithelial origin. It is the second most common odontogenic neoplasm. It’s incidence approximately 1% of all oral tumors and 18% of all odontogenic tumors. More than 80% of cases of ameloblastoma occur in mandible. The ameloblastoma occurs in three variants solid or multicystic, unicystic, and peripheral. A painless expansion of the jaws is the most common clinical presentation. The correct diagnosis can be easily made with the help of plain X-rays and tissue biopsy. The standard management of ameloblastoma is marginal resection but sometimes a large tumor requires complete resection of affected part. Untreated tumors may lead to tremendous facial disfigurement, a severe malocclusion and pathological fractures of the jaw. Here we present a case of a young man with the chief complaint of facial asymmetry. The orthopantomogram of the patient was showing a multilocular radiolucency with Buccal and lingual cortical expansion. A diagnosis was made on the basis of the biopsy as multicystic ameloblastoma, and resection of the mandible was carried out. The mandibular primary reconstruction was done with avascular bilateral iliac crest bone graft. Long term prognosis showed satisfactory healing and good facial esthetics.

Benign Multicystic Omental Mesothelioma in Pregnancy: A Case Report and Review of the Literature.

Benign Multicystic Peritoneal Mesothelioma (BMPM) is a very rare condition of unknown pathogenesis, that occurs mainly in women in their reproductive age (5:1 female/male ratio). The pathogenesis of BMPM is unclear and doubts regarding its neoplastic and reactive nature exist. Furthermore, the differential diagnosis with other diseases remains uncertain. In most cases the diagnosis is accidental, during a laparotomy performed for other reasons, since the diagnostic imaging does not allow a definite diagnosis preoperatively. Radical surgical resection is the “gold standard” for treatment of BMPM, despite a high rate of recurrence (about 50%). We report on a case of a 30-year-old pregnant woman with multilocular omental cystic mass, 36 x 22 cm in size, underwent elective caesarean section at term of pregnancy. The definitive histologic diagnosis was benign multicystic mesothelioma of the omentum. BMPM detected in pregnancy or during cesarean delivery is very rare; in fact only three other cases have been reported. Particularly, we present the only case, described in the literature, of a young female with benign multicystic omental mesothelioma in pregnancy that was treated solely by surgery, without recurrence after three years follow-up. In this article we also describe diagnostic evaluation, treatment, prognosis of this rare disease as the possible positive effects of adjuvants medical treatments.

Multicystic Dysplastic Kidney: A Retrospective Study.

Objective: To report the renal structural and functional anomalies in children with multicystic dysplastic kidneys. Methods: Retrospective descriptive analysis of 47 children with multicystic dysplastic kidney seen in a pediatric nephrology unit over a period of 6 years. Results: Antenatal diagnosis of multicystic dysplastic kidney was made in 34 (72.3%) patients. On follow up of 31 children for more than 12 months, 21 (68%) had involution, 4 [13%] had non-regression, and 4 (13%) were nephrectomized. Vesico-ureteric reflux (n=13; 28%) was the commonest renal abnormality. The serum creatinine values were higher (P=0.006) in children with contralateral reflux. Sub-nephrotic proteinuria was noted in 9 (29%) and was significantly associated with complete involution (P=<0.023). None of the patients developed hypertension and 2 (6.4%) had renal failure. Conclusion: Close nephrological follow-up is needed in children with multicystic dysplasia of kidneys.

A Female Infant with Segmental Multicystic Dysplastic Kidney

There have only been 35 pediatric cases and one adult case reported on segmental multicystic dysplastic kidney (MCDK) from our search in PubMed, including 19 cases detected antenatally. There is little documentation of segmental MCDK, particularly concerning its natural history. Segmental MCDK can be presented atypically, making diagnosis more difficult. We report an another case with segmental MCDK. Multicystic abdominal mass detected on antenatal sonogram in this infant was diagnosed as segmental MCDK by renal ultrasonography and computed tomography. If a definitive diagnosis of segmental MCDK can be made on imaging, surgery is not required for a diagnostic biopsy.

Displasia renal multiquística, ¿tratamiento conservador o nefrectomía?: is it appropriate to follow the conservative treatment or nephrectomy? / Multicystic renal dysplasia

Introducción: la displasia renal multiquística es una anomalía relativamente frecuente en la que aún se mantienen algunas discrepancias en la conducta médica a seguir. Objetivos: analizar la evolución de los quistes en un grupo de pacientes con diagnóstico prenatal de esta anomalía, en los que se siguió una conducta médica conservadora y expectante. Métodos: durante el período comprendido entre el 1º de enero de 1996 y el 30 de junio de 2011, se recibieron en la consulta de Nefrología del Hospital Pediátrico Universitario "William Soler", 22 recién nacidos o lactantes, en los que, durante el seguimiento de un embarazo normal, se diagnosticó una displasia renal multiquística u otra anomalía, que al estudiarse después del nacimiento, se demostró la displasia. En todos los pacientes se realizó ultrasonido renal al llegar al hospital y se hizo gammagrafía estática (99m tecnecio-DMSA) para precisar función renal del lado afectado y valorar las características del riñón contralateral. En todos los casos se siguió una conducta conservadora y expectante, y en 21 de estos niños se logró el objetivo de este estudio. Resultados: hubo predominio del sexo masculino en 60 %, del riñón izquierdo en 77 % y la forma "típica" de displasia en el 80 %. En 6 pacientes se encontraron otras anomalías asociadas. En 3 fetos se presentó involución prenatal de los quistes, la involución total entre 3 y 72 meses se observó en 9 (50,0 % de los estudiados después del nacimiento), mientras hubo involución parcial en 6 (33,3 %) y no hubo variación en 3 (16,7 %). Conclusiones: el tratamiento conservador debe ser la elección inicial en todo paciente con displasia renal multiquística, ya que la involución total se logra en la mayoría de los casos en un período relativamente corto.

Introduction: multicystic renal dysplasia is a relatively frequent anomaly that still arouses some discrepancies about the medical behaviour to be followed. Objectives: to analyze the evolution of cysts in a group of patients prenatally diagnosed with this anomaly, with whom a conservative and expectant medical behaviour was adopted. Methods: from January 1st, 1996 to June 30th, 2011, the nephrology service of "William Soler" university pediatric hospital assisted 22 newborns and infants, who were diagnosed with multicystic renal dysplasia or other anomaly during the normal pregnancy follow-up, but later dysplasia was confirmed after birth. All these patients underwent renal ultrasound on arrival at hospital, and then static gammagraphy (99m technetium-DMSA) to see the renal function of the affected side and to assess the characteristics of the contralateral kidney. A conservative and expectant behaviour was observed for all the cases; the objective of this study was attained in 21 of these children. Results: males prevailed in 60 % of patients, left kidney was affected in 77 % of cases and the "typical" form of dysplasia was present in 80 % of them. Six infants presented with other related anomalies. There was prenatal regression of cysts in three children, total regression ranging 3 to 72 months in 9 infants (50 % of the infants under study after birth) whereas partial regression occurred in 6 cases (33.3 %) and 3 did not change their condition (16.7 %). Conclusions: the conservative treatment should be the initial choice for every patient with multicystic renal dysplasia since total regression is possible in most of cases in a relatively short period of time.

Mandibular ameloblastoma in a 10-year-old child: case report and review of the literature / Ameloblastoma mandibular en niño de 10 años: reporte de un caso y revisión de la literatura

The ameloblastoma according to the classification of odontogenic tumors by WHO in 2005, is classified as a benign neoplasm of odontogenic epithelial origin. One to three percent of tumors and cysts of the jaws are comprised of ameloblastomas. The tumor is locally aggressive, but often asymptomatic, showing a slow growth which is manifested as a facial swelling or radiographic incidental finding. On clinical examination, the tumor can cause symptoms such as pain, ulceration, tooth mobility, root resorption and malocclusion. Ameloblastomas have a high rate of recurrence if not completely removed. It occurs in almost all age groups, but is mainly diagnosed in the third or fourth decade of life. The tumor is very rare in children. We present an unusual case of a solid/multicystic ameloblastoma of the mandible in a 10-year-old girl. In addition, a brief review of the literature on reported cases of this pathology in children is also presented...

El ameloblastoma según la clasificación de tumores odontogénicos de la OMS del 2005 lo clasifica como una neoplasia benigna de origen epitelial odontogénico. Compromete el 1-3% de neoplasias y quistes maxilares. El tumor es agresivo localmente, pero muchas veces asintomático; presenta un lento crecimiento que se manifiesta como un aumento de volumen facial o un hallazgo incidental radiográfico. Al examen clínico el tumor puede causar síntomas como dolor, ulceración, reabsorción radicular con movilidad dentaria y maloclusión. El ameloblastoma posee gran tasa de recurrencia si no es totalmente removido. Se presenta en casi todos los grupos etarios pero principalmente se diagnostica en la tercera o cuarta década de vida, el tumor es muy poco común en niños. El tratamiento del ameloblastoma es controversial y debido a la distinta incidencia y comportamiento en niños, hace las consideraciones quirúrgicas diferentes a los adultos. Por lo que presentamos un inusual caso de un ameloblastoma solido/multiquístico mandibular en una niña de 10 años. Además de una breve revisión de la literatura sobre casos reportados de esta patología en niños...