Management of ampullary tumours in children: still a challenge.

This study describes the case of the youngest patient ever reported with ampullary adenoma. The incidence of ampullary adenomas in childhood is unknown. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound are used in adults to assess and treat these lesions, although there are no instruments designed specifically for use in young children. A six-year-old girl was admitted for abdominal pain, vomiting, pruritus and weight loss. Abdominal ultrasound showed biliary tree (8mm) and pancreatic duct dilatation (4mm). Magnetic resonance cholangiopancreatography and computed tomography confirmed these findings, and also showed displacement of the ampulla to the left upper quadrant. An upper endoscopy confirmed a large ampullary adenoma. A laparotomy was performed and a 5cm villous tumour arising from the ampulla was excised. The postoperative course was uneventful. The histology demonstrated adenoma of the ampulla (intestinal type) without low-grade dysplasia. all clinical and radiological parameters are normal at 20 months follow-up. We describe the case of the youngest patient ever reported with ampullary adenoma. Pancreaticoduodenectomy carries high morbidity and mortality rates, and therefore it should be avoided in absence of histologically proven malignancy. We believe that surgical ampullectomy is a safe and oncologically correct procedure until better endoscopic instruments for peadiatric use will be designed.

A randomized phase III trial comparing induction chemotherapy followed by chemoradiotherapy versus chemoradiotherapy alone as treatment of unresectable head and neck cancer.

BACKGROUND: Concurrent chemoradiotherapy (CCRT) is the standard treatment for patients with unresectable, nonmetastatic locoregionally advanced squamous-cell carcinoma of the head and neck (LASCCHN). This randomized, open-label, phase III clinical trial compared the efficacy between standard CCRT and two different induction chemotherapy (ICT) regimens followed by CCRT. PATIENTS AND METHODS: Patients with untreated LASCCHN were randomly assigned to ICT (three cycles), with either docetaxel (Taxotere), cisplatin and 5-fluorouracil (TPF arm) or cisplatin and 5-fluorouracil (PF arm), followed by CCRT [7 weeks of radiotherapy (RT) with cisplatin 100 mg/m(2) on days 1, 22 and 43]; or 7 weeks of CCRT alone. The primary end points were progression-free survival (PFS) and time-to-treatment failure (TTF). RESULTS: In the intention-to-treat (ITT) population (n = 439), the median PFS times were 14.6 (95% CI, 11.6-20.4), 14.3 (95% CI, 11.8-19.3) and 13.8 months (95% CI, 11.0-17.5) at TPF-CCRT, PF-CCRT and CCRT arms, respectively (log-rank P = 0.56). The median TTF were 7.9 (95% CI, 5.9-11.8), 7.9 (95% CI, 6.5-11.8) and 8.2 months (95% CI, 6.7-12.6) for TPF-CCRT, PF-CCRT and CCRT alone, respectively (log-rank P = 0.90). There were no statistically significant differences for overall survival (OS). Toxic effects from ICT-CCRT were manageable. CONCLUSION: Overall, this trial failed to show any advantage of ICT-CCRT over CCRT alone in patients with unresectable LASCCHN.

Compromised fronto-striatal functioning in HIV: an fMRI investigation of semantic event sequencing.

The human immunodeficiency virus (HIV) damages fronto-striatal regions, and is associated with deficits in executive functioning. We recently developed a semantic event sequencing task based on the Picture Arrangement subtest of the Wechsler Adult Intelligence Scale-III for use with functional magnetic resonance imaging (fMRI) and found recruitment of dorsolateral prefrontal cortex and basal ganglia in healthy participants. To assess the impact of HIV on the functioning of the basal ganglia and prefrontal cortex, we administered this task to 11 HIV+ and 11 Control participants matched for age and education. Neuropsychological evaluation demonstrated that the HIV+ group had mild impairment in memory retrieval and motor functioning, but was not demented. Morphometric measurements suggested no atrophy in basal ganglia regions. The results of the fMRI analysis revealed hypoactivation of the left caudate, left dorsolateral prefrontal cortex, and bilateral ventral prefrontal cortex in the HIV+ group. Functional connectivity analysis demonstrated less functional connectivity between the caudate and prefrontal cortex and basal ganglia regions in the HIV+ group. In contrast, the HIV+ group demonstrated increased activation of right postcentral/supramarginal gyrus, and greater connectivity between the caudate and this same anterior parietal region. The results of this study extend previous investigations by demonstrating compromised function of the caudate and connected prefrontal regions in HIV during cognition. This disruption of fronto-striatal circuitry likely precedes the development of cognitive impairment in HIV.

Putamen hypertrophy in nondemented patients with human immunodeficiency virus infection and cognitive compromise.

BACKGROUND: Documented death and dysfunction of basal ganglia cells in patients seropositive for human immunodeficiency virus (HIV) suggest that the virus may cause structural compromise to these regions. OBJECTIVES: To examine subcortical volumes in nondemented patients seropositive for HIV (HIV+) by means of a novel automated neuroanatomic morphometric analysis tool, and to investigate relationships among cognitive function, immune health, and subcortical volumes. DESIGN AND SETTING: Cross-sectional study of subcortical morphometry and cognitive function conducted at the Boston University Center for Memory and Brain and the Massachusetts General Hospital Athinoula A. Martinos Center for Biomedical Imaging. PATIENTS: Twenty-two nondemented HIV+ patients and 22 age- and education-matched healthy control participants. MAIN OUTCOME MEASURES: Subcortical segmentation volumes, neuropsychological performance, and immunological variables. RESULTS: Nondemented HIV+ patients demonstrated relative and isolated putamen hypertrophy compared with control participants. Putamen volume enlargement in HIV+ patients was related to motor slowing and immune status, such that higher CD4 lymphocyte levels were associated with larger putamen volumes. There were no other subcortical volume differences between the groups. CONCLUSIONS: This study suggests that basal ganglia hypertrophy accompanies HIV-related mild cognitive compromise. These findings may represent a structural imaging parallel to functional imaging studies demonstrating basal ganglia hypermetabolism in HIV+ patients with mild cognitive compromise and early HIV-associated brain disease.

IRAP and REMAP assessments of genetic similarity in rice.

Rice is a model genome for cereal research, providing important information about genome structure and evolution. Retrotransposons are common components of grass genomes, showing activity at transcription, translation and integration levels. Their abundance and ability to transpose make them good potential markers. In this study, we used 2 multilocus PCR-based techniques that detect retrotransposon integration events in the genome: IRAP (inter-retrotransposon amplified polymorphism) and REMAP (retrotransposon-microsatellite amplified polymorphism). Markers derived from Tos17, a copia-like endogenous retrotransposon of rice, were used to identify genetic similarity among 51 rice cultivars (Oryza sativa L.). Genetic similarity analysis was performed by means of the Dice coefficient, and dendrograms were developed by using the average linkage distance method. A cophenetic correlation coefficient was also calculated. The clustering techniques revealed a good adjustment between matrices, with correlation coefficients of 0.74 and 0.80, or lower (0.21) but still significant, between IRAP and REMAP-based techniques. Consistent clusters were found for Japanese genotypes, while a subgroup clustered the irrigated Brazilian genotypes.

Neutron measurements in a Varian 2,100C LINAC facility using a Bonner sphere system based on passive gold activation detectors.

The use of high-energy linear electron accelerators (LINACs) for medical cancer treatments is widespread on an international scale. The associated bremsstrahlung X rays may produce neutrons as a result of subsequent photonuclear reactions with the different materials constituting the accelerator head. The generated neutron field is highly variable and depends strongly on the beam energy, on the accelerator shielding, on the flattering filter as well as on the movable collimators (jaws) design and on the irradiation field geometry. An estimate of this photoneutron component is, thus, of practical interest to quantify the radiological risk for the working staff and patients. Due to high frequency electromagnetic fields, and also to the presence of abundant leaked and scattered photons in these installations, measurements of the corresponding neutron fields by active dosemeters are extremely difficult. A modified version of the Bonner sphere system, based on passive gold activation detectors, has been used to perform neutron measurements at two points in a Varian 2,100C LINAC facility. A home-made unfolding procedure (CDM) has been utilised to determine the neutron spectra present at the measurement points. Results indicate that the giant dipole resonance process is the most adequate model to explain neutron production in the LINAC and that a thermal component is present at the measurement points.

Altered hippocampal-prefrontal activation in HIV patients during episodic memory encoding.

OBJECTIVE: To investigate the integrity of hippocampal-prefrontal circuitry during episodic encoding in patients with HIV. METHODS: Functional MRI was used to observe changes in blood oxygenation level-dependent (BOLD) signal in 14 HIV-positive participants and 14 age- and education-matched control subjects while performing an episodic encoding task. Subjects also completed neuropsychological measures of attention and memory. RESULTS: Behavioral results revealed no significant differences in neuropsychological performance. The fMRI results revealed that while both groups recruited brain regions known to be important for successful encoding, including bilateral medial temporal lobes and inferior prefrontal gyri, the HIV group demonstrated significantly reduced signal intensity changes in the right posterior hippocampus, right inferior frontal gyrus, and left lingual gyrus. Additionally, the HIV group exhibited more activity within lateral frontal and posterior parietal regions. CONCLUSIONS: This study demonstrates altered integrity of hippocampal-prefrontal regions during episodic encoding in HIV-positive patients. These results extend previous studies that have documented the effects of HIV on fronto-striatal circuits, and suggest the virus functionally impacts the hippocampal system as well.

[Supernumerary intrascrotal ectopic spleen: discontinuous splenogonadal fusion]. / Bazo ectópico supernumerario intraescrotal: fusión esplenogonadal discontinua.

Splenogonadal fusion is a rare congenital malformation that results from abnormal connection of splenic tissue with the gonad or the mesonephric structures during embryonic development. It is usually detected in males and is classified into two types, continuous and discontinuous. We present the case of a 3-year-old boy with an asymptomatic scrotal mass that was suspicious for discontinuous splenogonadal fusion on Doppler ultrasonography. The diagnosis was confirmed by surgical excision and histological analysis. Splenogonadal fusion is a benign anomaly that has sometimes led to unnecessary orchidectomy because of suspicion that the mass represented a malignant tumor. Consequently, it is essential to include this malformation in the differential diagnosis of scrotal masses in children.

Uterus didelphys with obstructed hemivagina and multicystic dysplastic kidney.

Müllerian malformations include a broad range of anomalies, resulting from the incomplete formation and/or differentiation of the Müllerian ducts. The uterus didelphys with obstructed hemivagina represents the result of a lateral nonfusion of the Müllerian ducts with asymmetric obstruction, and it is almost always associated with renal agenesis. We report a case of incidental discovery of this anomaly in a 12-year-old girl during a routine renal ultrasound. A right multicystic dysplastic kidney had been detected in the prenatal and neonatal period with no evidence of uterine anomaly. Postnatal ultrasound examinations performed regularly had never detected either right renal tissue or the uterine anomaly before menarche. Hematocolpos after menarche made uterine anomaly detection easier. Magnetic resonance imaging performed to evaluate the uterine malformation detected a dysplastic right kidney. We review the present knowledge and recommendations for the diagnosis and treatment of this anomaly, and we emphasize the use of magnetic resonance imaging not only for the evaluation and classification of Müllerian malformations, but also for the detection of the persistence of renal tissue missed with other studies.

Pathology of pyogenic liver abscess in children.

Little is known about preexisting lesions in livers of children with pyogenic liver abscess (PLA). Study of these lesions may elucidate possible predisposing factors for the disease. In Vitória, state of Espirito Santo, Brazil, PLA in children is frequently associated with helminthic infections and eosinophilia. We hypothesize that nematode infection with larvae migrating through the liver is a predisposing factor for PLA, because the infection induces immunomodulation and likely trapping of bacteria in liver granulomas. In this report, we describe observations of 22 cases of PLA in children studied at autopsy (16 cases) or in surgical biopsies (6 cases), including 17 boys and 5 girls ranging in age from 1 to 13 years (mean 4.6 years, median 3.5 years). Multiple abscesses in both lobes were found in 13 cases and a single abscess was found in the right lobe in 10 cases. All cases showed histologically classical pyogenic inflammation without morphological evidence of amoebiasis. In six cases there were granulomas similar to those caused by larva migrans visceralis (from Toxocara or other nematodes) in liver tissue not affected by the abscess. Nematode antigens in central areas of necrosis of granuloma in all six cases and fragments of a larva, possibly of Toxocara, were found on samples immunohistochemically stained with polyclonal anti-Toxocara antibodies. There were numerous eosinophils in abscesses with Charcot-Leyden crystals. Eosinophils were found frequently in portal triads far from the abscess wall. In four cases, in which bile duct ascariasis was found, worms were noted in the bile ducts, and eggs were found in liver parenchyma surrounding the abscess in two cases. Foreign-body granulomas were found in one case in which penetrating trauma was the cause of abscess. In one case there was one histiocytic granuloma whose origin was not determined. The observation of six cases of granuloma similar to larva migrans visceralis (or produced by other nematode larva) in liver tissue not directly affected by the abscess supports the hypothesis that helminth infections with larva migrating through the liver are a predisposing factor for pyogenic hepatic abscess in children.

Frequency of myocarditis in cases of fatal meningococcal infection in children: observations on 31 cases studied at autopsy.

The frequency of myocarditis associated with meningococcal disease in children was reported only in two autopsied series (United States and South Africa). Here we report the frequency of associated myocarditis in 31 children who died of meningococcal infection at Hospital Infantile N.S. da Glória in Vitória, Espirito Santo State, Brazil. The diagnosis was confirmed by isolation of Neisseria meningitidis. At least three sections of fragments of both atria and ventricles were studied using the Dallas Criteria for the morphologic diagnosis of myocarditis. The mean age was 47.6 +/- 39.8 months and the mean survival time after the onset of symptoms was 46.1 +/- 26.5 h (12-112 h). Myocarditis was present in 13 (41.9%) patients, being of minimal severity in 11 cases and of moderate severity in 2 cases. There were no cases with severe diffuse myocarditis. The frequency of myocarditis was not influenced by sex, presence of meningitis, survival time after the onset of symptoms or use of vasoactive drugs. The frequency of myocarditis reported here was intermediate between the values reported in the only two case series published in the literature (57% in the United States and 27% in South Africa). Although our data confirm the high frequency of myocarditis in meningococcal disease, further investigations are necessary to elucidate the contribution of myocarditis to myocardial dysfunction observed in cases of meningococcal infection in children.

[Second primary tumors in patients with pharyngo-laryngeal carcinoma]. / Segundos tumores primarios en pacientes con carcinoma faringolaríngeo.

The risk of second primary tumors of the respiratory and upper digestive tract developing in patients treated for head and neck cancer is well known. In these cases, the entire mucous membrane surface of the tract is affected and predisposed to metachronous or synchronous neoplasms. The mucosa of these patients is extremely susceptible to external carcinogenic stimuli. We report the results obtained in 83 patients with cancer of the oral cavity, pharynx, or larynx who experienced at least one second primary tumor. The most common site of the second primary was the lung (43.2%). We reviewed tobacco and alcohol use, treatments, and survival.

Primary lingual abscess presenting as acute swelling of the tongue obstructing the upper airway: diagnosis with MR.

We report the sudden development of an inflammatory process thought to be of the floor of the mouth with subsequent involvement of the respiratory airway. MR imaging showed an enhancing posterior lingual lesion, consistent with an abscess. Immediate surgical drainage relieved the symptoms. MR imaging allowed accurate diagnosis, which was essential for surgical planning in a condition that was otherwise difficult to diagnose.

[Orbital cellulitis and permanent amaurosis: clinical case]. / Celulitis orbitaria y amaurosis permanente: caso clínico.

Orbital cellulitis is a rare, potentially serious but complication of acute sinusitis. It is more frequent and benign in children, but in adults usually requieres surgical drainage of the affected sinus. We report a case of aggressive evolution with permanent blindness in an adult without general or local risk factors, in spite of adequate treatment. The literature is reviewed.

[Localized laryngeal amyloidosis. Treatment with CO2 laser]. / Amiloidosis laríngea localizada. Tratamiento con láser CO2.

Amyloidosis of the upper airways is rare. The larynx is the most common site of involvement. Primary amyloidosis may be localized or generalized. We report a case of laryngeal amyloidosis in which amyloid deposits were confined to the larynx (right false vocal cord and subglottis) and successfully treated with CO2 laser.

[Subdural empyema induced by frontal sinusitis]. / Empiema subdural inducido por sinusitis frontal.

Nowadays the complications arising from sinusitis are rare. However its seriousness can even cause death. Intracranial complications are much more uncommon than the orbital ones. However, slight symptoms can appear, which may disjunct the correlation between clinical-radiology and the severity of the infection. The diagnosis is fundamentally based on the TAC, and early treatment with intravenous anti-biotherapy should be implemented, being followed by drainage surgery of the sinus and empyema. We have presented a case which evolved favourably and revised all the literature pertaining to it.

[Adjuvant chemotherapy in the treatment of pharyngolaryngeal cancers in advanced stages]. / Tratamiento quimioterápico adyuvante en carcinomas laringo-faríngeos en estadios avanzados.

Since 1980 we use adjuvant chemotherapy in advanced laryngopharyngeal carcinomas. These cases were resectable and unresectable lesions with tumoral extent to the pharynx as a common characteristic. We analyze the survival of the first 50 cases treated with chemotherapy followed by: A) surgery and radiotherapy (36 cases) and B) radiotherapy alone (14 cases). Results of this not randomized study are compared with a historic group of advanced laryngopharyngeal carcinomas (T4/N+) treated with conventional therapy (surgery +/- radiotherapy). Response to chemotherapy was complete or partial (> 50% reduction) in 56 percent of the patients. There was improvement in overall survival and five-years disease-free survival in the latter group compared with those who did show any response (p < 0.01). We would point out that disease-free survival of the group A was better than historic group (60% vs 36%, p < 0.05), although these result should be carefully interpreted. Laryngeal preservation was achieved in 47 percent in the group A by modification of the initially scheduled radical surgery, in selected cases.

Ascaris lumbricoides: reinfection in children bearing an established worm burden.

To clarify the existence of reinfection in children bearing an established Ascaris lumbricoides infection, the authors evaluated the weight and the length of worms collected from ten cases of ascaridiasis. The worm burden was greater than 27 worms in nine cases. In seven cases the weight and the length of worms showed little variation, with unimodal distribution of values, suggesting that all the worms in each case belong to the same population, originated from a single brood infection or from successive infections over small time intervals. In three cases there was great variation in worm size indicated by the different values for the means and medians and by the high values for the standard deviation and coefficient of variation. In these three cases there was a bimodal distribution of worm's size suggesting the coexistence of two distinct populations: one, less numerous, composed of mature worms and the other, more numerous, composed of immature worms, in two cases, and two distinct populations of immature worms in one case. The existence of worms in different stages of maturation indicates that the less mature population was acquired when the mature worms were established in the gut. These results indicate that the reinfection with Ascaris in children bearing an established infection is not rare and resistance induced by a preexisting infection is not the rule.