Biomedical subjects

Bartley G Cilento

Publications and source records attributed to Bartley G Cilento.

10 recordsLinked to original sources

PubMed2005

Renal cell carcinoma: Children's Hospital Boston experience.

OBJECTIVES: To review our experience to better define pediatric renal cell carcinoma (RCC). Pediatric RCC is rare, and recent data suggest it may be a unique disease. METHODS: A retrospective review was conducted of hospital and pathology records from 1965 to 2003. Patients with RCC were identified, and the clinical and pathologic data were extracted. RESULTS: Since 1965, 11 patients with RCC were treated, accounting for 3% of all renal tumors. In the first 20 years, 191 patients with renal tumor were treated, of whom 3 had RCC. In the most recent 15 years, 172 patients with renal tumor were treated, of whom 8 had RCC. The mean age at presentation was 14.7 years (range 9 to 17 years), with a female predominance (2.7:1). The clinical signs and symptoms included hematuria in 36%, flank pain in 27%, and an abdominal mass in 9%; 36% were discovered incidentally. Of the 11 RCC tumors, 45% were papillary and 55% were clear cell carcinoma. Papillary tumors presented at a worse stage and displayed more aggressive clinical behavior. Of 10 patients with available follow-up data, 6 had no evidence of RCC, 1 had died of other causes, 2 had died of metastatic RCC, and 1 was alive with recurrent RCC at a mean follow-up of 4.9 years. CONCLUSIONS: The clinical presentation, pathologic characteristics, and clinical behavior of pediatric RCC are different than those for adult RCC. A possible increasing incidence of RCC in children would parallel an increased incidence in adults. Our findings warrant additional and coordinated efforts to better characterize RCC in children.

Adolescent↗

PubMed2005

Intermittent testicular torsion: diagnostic features and management outcomes.

PURPOSE: Intermittent testicular torsion (ITT) is a poorly characterized condition but harbors potentially serious implications with regard to testicular viability. We report better characterization of the diagnostic features of ITT. MATERIALS AND METHODS: We performed a retrospective review of all patients 1 to 18 years old seen from 1997 to 2003 at our institution diagnosed with ITT. Patients with acute scrotal pain and spontaneous resolution who underwent bilateral testicular fixation were included in the study. RESULTS: A total of 50 patients meeting the inclusion criteria were identified with mean age at presentation of 12.2 years (range 4 to 17). The mean number of painful episodes before surgery was 4.3 (range 1 to 30). The most common presenting features were severe pain of rapid onset and resolution. Nausea and/or vomiting was reported in a quarter of the patients. Finding of a horizontal lie of the testes on examination was significantly associated (p <0.05) with the existence of the bell-clapper deformity. All patients underwent surgical fixation of the testes. There were no postoperative complications. Of 38 patients (97%) for whom followup was available 37 had complete resolution of symptoms (mean followup 7.9 months). CONCLUSIONS: ITT should be a diagnostic consideration in patients who present with recurrent acute scrotal pain with rapid spontaneous resolution. Recurrent severe pain with rapid onset and resolution seems to be highly characteristic. Horizontal lie on examination is highly correlated with the bell-clapper deformity at surgical exploration. Surgery may be recommended in these patients as it appears to result in pain relief in the majority, is likely to prevent future testicular infarction and is associated with low morbidity.

Adolescent↗

PubMed2005

Testicular hypotrophy does not correlate with grade of adolescent varicocele.

PURPOSE: Testicular hypotrophy is the most widely accepted indication for correcting adolescent varicocele. Previous studies in adolescents have shown a relationship between increasing grade of varicocele and the likelihood of testicular hypotrophy. As this relationship has significant clinical implications, we studied the correlation between grade and testicular volume disproportion in our adolescent varicocele population. MATERIALS AND METHODS: We reviewed the adolescent varicocele database at our institution. A total of 168 patients 8 to 21 years old were studied. We routinely calculated testis volumes using scrotal ultrasound. Testicular disproportion was calculated using the equation [(size of unaffected testis) - (size of affected testis)]/(size of unaffected testis) x 100%. Disproportion was categorized as less than 10%, 10% to 20% and more than 20%. Varicoceles were graded by an attending urologist with the patient standing, using the system of Dubin and Amelar. Analysis of variance and Pearson chi-square indicated no significant differences in volume differential between varicocele grades. RESULTS: Mean +/- SD volume differential was 18% +/- 15% for grade I, 25% +/- 20% for grade II and 19% +/- 14% for grade III. ANOVA revealed no significant difference in mean volume differential between the 3 varicocele grades (p = 0.10). When categorizing patients into 3 levels of volume differential (less than 10%, 10% to 20%, more than 20%) no significant correlation was observed between varicocele and volume differential (p = 0.48, chi-square test). CONCLUSIONS: Grade of varicocele does not correlate with presence or severity of testicular disproportion in adolescent boys with varicocele as measured by scrotal ultrasound.

Adolescent↗

PubMed2004

Urachal inflammatory myofibroblastic tumor with ALK gene rearrangement: a study of urachal remnants.

OBJECTIVES: Abnormalities of the urachus are rare among children and include a patent tract and cyst formation. These structures can also be affected by infection and abscess development. They are usually diagnosed during infancy and treated by surgical resection. Involvement of this remnant by either benign or malignant tumors is very infrequent. A few cases of mesenchymal tumors, such as desmoid tumor and leiomyoma, involving the urachus have been described in published reports. METHODS: We studied an inflammatory myofibroblastic tumor arising from the urachus in a 10-year-old boy. In addition, we reviewed 101 cases of urachal remnants retrieved from the surgical pathology and autopsy files in the Department of Pathology at the Children's Hospital Boston diagnosed in the past 82 years. RESULTS: The urachal inflammatory myofibroblastic tumor showed anaplastic lymphoma kinase (ALK) rearrangement by immunohistochemistry and fluorescence in situ hybridization techniques. No other neoplasms were diagnosed in the analyzed population. CONCLUSIONS: We describe an example of inflammatory myofibroblastic tumor involving the urachus. Involvement of the urachus by tumors is rare, but these should be considered in the differential diagnosis of urachal lesions.

Anaplastic Lymphoma Kinase↗

PubMed2004

Intratesticular varicocele in adolescents: a reversible anechoic lesion of the testis.

PURPOSE: We describe ultrasonographic and clinical findings in adolescents with intratesticular varicocele. MATERIALS AND METHODS: Three adolescent boys 12 to 16 years old each had a large intratesticular multicystic lesion on scrotal Doppler ultrasound for a large extratesticular varicocele. The presence of active Doppler flow within the anechoic lesions supported the diagnosis of intratesticular varicocele. RESULTS: All 3 boys underwent spermatic vein ligation for varicocele. In each case scrotal Doppler ultrasound at 3 months postoperatively demonstrated resolution of the intratesticular anechoic lesions and Doppler flow, confirming the diagnoses of intratesticular varicocele. CONCLUSIONS: Intratesticular varicocele is a clinically occult lesion that may occur in conjunction with extratesticular varicocele. This entity is apparent on scrotal Doppler ultrasound as an intratesticular anechoic lesion with active Doppler flow, and has been shown to resolve following spermatic vein ligation. Its clinical significance has not yet been defined.

Adolescent↗

PubMed2004

Is adolescent varicocele a progressive disease process?

PURPOSE: We determined whether there was evidence of progression in either varicocele grade or testicular volume differentials over time in our adolescent varicocele population. MATERIALS AND METHODS: A total of 41 boys 7.3 to 19 years old (mean age 13.7, SD 2.5) were seen at sequential visits. At each visit varicocele grade was determined by the attending urologist and ultrasound calculated testicular volume differential was determined by an attending ultrasonographer. A senior staff statistician performed statistical analysis of the sequential data. RESULTS: The varicocele grade data were analyzed using the nonparametric Wilcoxon test and no significant evidence of progression on sequential visits was demonstrated. Similarly, the serial volume differential data were assessed using the paired t test and no significant evidence for progression of testicular volume differentials was demonstrated. CONCLUSIONS: In our study population neither varicocele grade nor change in testicular volume differentials increased over time in a statistically significant manner. These results argue against the concept that adolescent varicocele is a progressive disease process.

Adolescent↗

PubMed2004

Prepubertal testis tumors: actual prevalence rate of histological types.

PURPOSE: Tumor registries, urological textbooks and literature surveys all assert that yolk sac tumors are the most common primary testicular tumors in boys 12 years and younger. In contrast, several individual institutions have reported that benign tumors are more common than malignant tumors. To clarify these discordant findings, we surveyed the primary pathology records from 4 major pediatric centers. MATERIALS AND METHODS: The pathology records of the contributing centers were culled for primary testicular masses in boys 12 years and younger. Older boys and those with either paratesticular tumors or leukemia were excluded. The prevalence of each histological subtype was calculated from the pooled cases. RESULTS: A total of 98 patients met our criteria. Only 15% had yolk sac tumors. Teratomas comprised 48% of the tumors (mature 44%, immature 4%). Epidermoid cysts were found in another 14% of patients. Gonadal stromal cell tumors represented 13% of the total, divided among granulosa cell (5%), Leydig cell (4%), Sertoli cell (3%) and mixed gonadal stromal cell (1%). Other pathology, including cystic dysplasia (2), lymphoma (4), inflammatory pseudotumor (1) and gonadoblastoma (2), made up 9% of the total number of cases. CONCLUSIONS: We found that benign lesions represent the majority of primary testis tumors (74%), with the most common histological type being teratoma (48%). The reported high prevalence rates of prepubertal yolk sac tumors probably results from a reporting bias, since benign tumors are less likely to be submitted to tumor registries. Therefore, the primary operative approach to the majority of testis tumors in boys 12 years and younger should entail testis sparing surgery. Orchiectomy should be reserved for histologically confirmed malignancy based on increased preoperative alpha-fetoprotein and/or frozen section analysis of the tumor.

Age Factors↗

PubMed2004

Coping, commitment, and attitude: quantifying the everyday burden of enuresis on children and their families.

OBJECTIVE: To develop and evaluate a parent-completed questionnaire for use by clinicians as part of routine care to assess the burden of diurnal and nocturnal enuresis on children and their families. METHODS: The questionnaire consisted of items that measure the impact on the child and his/her parent, the child's coping ability and commitment to treatment, previous treatment success, family frustration and overall cohesion, and parental attitudes about enuresis and its treatment. Questionnaires (n = 208) were completed by parents during the child's scheduled office visit for enuresis at 5 specialty clinics across the United States. Traditional criteria were used to assess reliability and validity of the questionnaire, including analysis of variance. RESULTS: Success rates provide evidence that many of the items in the child scale (79%) and all items in the parent scale (100%) met stringent criteria. alpha values were.62 and.77, respectively. Statistically significant differences were observed for the scales across responses on all but 1 global item, the majority of parental attitude items, whether the child urinated at bedtime, and the number of pads used. These findings suggest that the child's coping ability and commitment and the family's overall cohesion and frustration with the problem influence parental perceptions about the impact of enuresis on the child and the family. CONCLUSIONS: Findings about the performance of the new measure were satisfactory and suggest that, after further refinement, it should prove as a useful tool for clinicians treating enuresis in children.

Adaptation, Psychological↗

PubMed2003

Prospective evaluation of human chorionic gonadotropin in the differentiation of undescended testes from retractile testes.

PURPOSE: We prospectively evaluated the efficacy of human chorionic gonadotropin (HCG) in the treatment of undescended testis and sought to determine whether HCG assists in the differentiation of undescended testis from retractile testis. MATERIALS AND METHODS: Patients with undescended testes were offered HCG. Testis position, laterality and the presence or absence of a hypoplastic scrotum were noted. The same physician (G. W. K.) recorded physical findings prospectively and stated clinical impression of descent. RESULTS: A total of 67 patients with 90 undescended or retractile testes were treated and evaluated with HCG. Of the 64 undescended testes 13 (20%) descended with HCG therapy, with none requiring subsequent surgery. Of the 26 retractile testes 15 (58%) descended with HCG (p <0.001). Based on physical examination, 100% of retractile testes descended if the testis was in the high scrotal position but only 40% descended if the testis was in the superficial pouch or inguinal area. In the undescended testes group no ectopic or nonpalpable testis descended with HCG. Evaluation of HCG with age demonstrated minimal response (15%) to HCG at less than 24 months, and a peak response between ages 2 and 6 years (75%) with response decreasing thereafter. CONCLUSIONS: HCG may have a limited role in the evaluation of undescended testis in patients younger than 2 years. HCG can serve as an adjunct in the clinical diagnosis of retractile testis in older children.

Child↗

PubMed2002

Proximal hypospadias.

Approximately 20% of hypospadiac urethras are located proximally, anywhere from the penoscrotal to the perineal region. Repair of proximal defects is still one of the most challenging surgical procedures facing the hypospadiologist. Modifications of current techniques for the correction of proximal hypospadias as well as new innovative techniques continue to be proposed.

Humans↗