Primary malignant pancreatic neoplasms in children and adolescents: A 20 year experience

Background

Malignant pancreatic neoplasms in children and adolescents are rare. The clinical presentation, pathologic characteristics, management, and outcomes at two institutions are discussed.

Methods

We retrospectively reviewed all pediatric patients (age < = 18 years) treated for malignant pancreatic neoplasms at two institutions between 1991 and 2011.

Results

Thirty-one patients were identified with median age of 14.7 years (4–18 years). The most common histology was solid pseudopapillary tumor (SPT) (n = 22, 71%) followed by neuroendocrine tumors (n = 4, 13%), pancreatoblastoma (n = 4, 13%), and one unclassified spindle cell neoplasm (3%). Most patients presented with abdominal pain (n = 22, 71%). Complications included pancreatic leak, pseudocyst formation, pancreatitis, pancreatic insufficiency, and small bowel obstruction. The overall 1- and 5-year survival was 96% (95% CI 74%–99%) and 78% (95% CI 43%–93%). Median follow-up among patients alive at the end of follow-up was 20 months (< 1 month–16.2 years). Patients with SPT had better overall survival compared to patients with neuroendocrine tumors or pancreatoblastomas (Log-rank; p = 0.0143).

Conclusion

The majority of pediatric and adolescent patients present with SPTs which are usually resectable and associated with an excellent prognosis. Other histologic subtypes more often present with distant metastases and portend a worse prognosis.     

Experience with FiberWire for pectus bar attachment

Background

Minimally invasive repair of pectus excavatum has become an established method for repair of pectus excavatum. Bar displacement or rotation remains the most common complication of this repair requiring return to the operating room.

Methods

Retrospective review of all patients at a single institution who underwent repair of pectus excavatum using FiberWire for bar stabilization between December 2009 and March 2013 was undertaken.

Results

93 patients underwent minimally invasive pectus repair using FiberWire during the study period. The patients included 73 males and 20 females, with an average age of 14.6 years (range 7–21 years). Mean operative time was 102 minutes (range 56–198 minutes). No patients developed wound complications, two patients developed pain because of bar migration and required return to the OR, and no patients had recurrence of their pectus defect because of bar migration during the study period. Median length of follow-up was 17 months (range 3–36 months).

Conclusion

Stabilization of pectus bars using circumferential rib fixation with FiberWire at multiple points on both sides of the bar appears to be effective in preventing bar rotation and displacement, and requires minimal change to the operation as it has been previously described. Early experience shows a low rate of complications.     

Management of epididymal cysts in childhood

Background

A retrospective review was carried out to evaluate the clinical presentation of children with epididymal cysts (EC) and outcome of management at our institution.

Methods

There were 49 patients with EC in this series. The diagnosis of EC was made by physical examination and confirmed by ultrasound (US).

Results

The average age at presentation was 10.7 years (2 months–16 years). Scrotal mass (n: 22) and pain (n: 21) were the most frequent symptoms. Seven patients were lost to follow-up. The cysts were solitary in 32 patients and multiple in 10 patients. The mean value of cysts was 6.7 mm (2–20 mm). The cyst localisations were 22 in left, 16 in right, and bilateral in 4 patients. Complete involution of cysts was detected in 14 children. The average involution time was 11.2 months (1–37 months). In 20 cases, a decrease in cyst size was found. Cyst excision was performed in 8 patients with persistent scrotal pain or no cyst involution observed during follow-up.

Conclusion

Conservative management of epididymal cysts is practical. However, surgical excision is recommended in patients with intractable scrotal pain or if the cyst size does not seem to involute.     

Risk factors for persistent pain after urological surgery

Objective

Ten to 50% of patients with post-surgical pain develop chronic pain depending on the type of surgery. The objective of this study was to assess the incidence of persistent post-surgical pain (PPSP) and to identify risk factors following urology surgery.

Design

Retrospective observational study.

Patients

Two hundred and twenty-eight patients scheduled for urology surgery. Reasons for non-inclusions: patients who underwent a procedure not defined as being associated with PPSP.

Methods

Surgical urologic procedures potentially associated with PPSP were defined. All patients who had one of these procedures during the study period received a questionnaire by mail at least 3 months after the surgery. The files of these patients were retrospectively studied.

Results

Eight percent of the patients had preoperative pain. PPSP, assessed approximately 6 months after the surgery, was reported by 24% of the patients. Twenty-five (36%) of them reported neuropathic pain. Patients with PPSP had significantly more preoperative pain and an increased postoperative morphine consumption. Postoperative NSAID administration led to less persistent pain. Multivariate logistic regression analysis identified two independent risk factors of developing persistent pain: preoperative pain (OR = 21.6, 95% CI 6.7–69.5, P < 0.0001), morphine consumption 48 hours after surgery higher than 6 mg (OR = 2.3, 95% CI 1.2–4.3, P = 0.0118).

Conclusion

These findings confirm the role of preoperative pain and morphine consumption in the genesis of PPSP and call for establishing clinical perioperative pathways tailored to the patient.     

Results of operative treatment of avulsion fractures of the iliac crest apophysis in adolescents

Background

Avulsion fracture of the iliac crest apophysis is a rare condition that commonly occurs in adolescent athletes. Conservative treatment for this injury can produce excellent functional outcomes. However, the rehabilitation process requires a rather long immobilisation period. This study aimed to evaluate the use of cannulated screws for fixation of avulsion fractures of iliac crest apophysis.

Methods

Ten patients with avulsion fractures of iliac crest apophysis were treated by open reduction and internal fixation using cannulated screws.

Results

The mean age of patients was 14.6 years (range, 13–15 years). The mean intraoperative blood loss was 14.9 ml (range, 10–25 ml). The mean operative time was 40.3 min (range, 33–52 min). The mean follow-up period was 11.2 months (range, 6–20 months). At the 4-week follow-up, all patients returned to previously normal activity without pain and had no evidence of lower extremity muscle weakness. At the final follow-up, all patients resumed their athletic activity without any complications.

Conclusion

Open reduction and internal fixation for the treatment of avulsion fracture of iliac crest apophysis can be recommended for patients requiring rapid rehabilitation.     

National Institutes of Health Chronic Prostatitis Symptom Index (NIH-CPSI) Symptom Evaluation in Multinational Cohorts of Patients with Chronic Prostatitis/Chronic Pelvic Pain Syndrome

Background

The assessment of patients with chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS) in everyday practice and clinical studies relies on National Institutes of Health Chronic Prostatitis Symptom Index (NIH-CPSI) scores for symptom appraisal, inclusion criteria for clinical trials, follow-up, and response evaluation.

Objective

We investigated multiple databases of CP/CPPS patients to determine the prevalence and impact of pain locations and types to improve our strategy of individualized phenotypically guided treatment.

Design, setting, and participants

Four major databases with CPSI scores for nonselected CP/CPPS clinic patients from Canada, Germany, Italy, and the United States.

Outcome measurements and statistical analysis

Individual question scores and subtotal and total scores of CPSI were described and correlated with each other. Ordinal regression analysis was performed to define pain severity categories.

Results and limitations

A total of 1563 CP/CPPS patients were included. Perineal pain/discomfort was the most prevalent pain symptom (63%) followed by testicular pain (58%), pain in the pubic area (42%) and penis (32%); reports of pain during ejaculation and voiding were 45% and 43%, respectively. European patients had a significantly higher number of pain localizations and symptoms compared with North American patients (p < 0.001). Severity of pain correlated well with frequency of pain (r = 0.645). No specific pain localization/type was associated with more severe pain. Correlation of pain domain with quality of life (QoL) (r = 0.678) was higher than the urinary domain (r = 0.320). Individually, pain severity (r = 0.627) and pain frequency (r = 0.594) correlated better with QoL than pain localization (r = 0.354). Pain severity categories results for NIH-CPSI item 4 (0–10 numerical rating scale for average pain) were mild, 0–3; moderate, 4–6; severe, 7–10; CPSI pain domain (0–21): mild, 0–7; moderate, 8–13; and severe, 14–21.

Conclusions

Pain has more impact on QoL than urinary symptoms. Pain severity and frequency are more important than pain localization/type. Cut-off levels for disease severity categories have been identified that will prove valuable in symptom assessment and the development of therapeutic strategies.     

Solid pseudopapillary neoplasm of the pancreas in children: A 15-year experience and the identification of a unique immunohistochemical marker

Purpose

To review our 15-year experience in the management of children with solid pseudopapillary neoplasm (SPPN) of the pancreas at a single pediatric institution, to delineate a unique immunohistochemical marker for SPPN, and to analyze cumulative data on this rare entity in the literature.

Methods

We did a retrospective analysis of the demographic data, clinical presentation, immunohistochemical characteristics, surgical approach, and long-term outcomes of all patients diagnosed with SPPN between 1997 and 2012.

Results

There were 6 patients in the series, 5 females and 1 male. Median age at presentation and at surgery was 15 years (11–18 years). Abdominal pain was the presenting symptom in 5 cases and jaundice in 1 case. Two patients had a pancreatic head tumor and underwent a pylorus-preserving pancreaticoduodenectomy. Two patients had the tumor in the pancreatic tail and underwent a distal pancreatectomy with splenectomy. Two patients had the tumor in the pancreatic body and underwent a distal pancreatectomy with splenectomy in one case and with preservation of the spleen in the other. All tumors were completely resected with pathologic margins free of disease. The median maximum diameter was 6.8 cm (3 to 15 cm). On immunohistochemistry the tumors exhibited different combinations of non-specific markers like chromogranin, vimentin and neuron-specific enolase, but all tumors showed the highly SPPN-specific paranuclear dot-like immunoreactivity pattern for CD99 in the solid as well as in the pseudopapillary areas. No patient had metastasis at presentation. Median follow-up was 6.5 years (6 months to 15 years). There were no recurrences, no long-term metastasis, and all patients are disease-free.

Conclusion

Our series supports the concept that complete resection is necessary to achieve the best possible long-term results. Additionally, we demonstrate that SPPN exhibits a very unique immunostaining pattern for CD99 that is present in all cases.     

Gastric emptying in children with gastroesophageal reflux and in healthy children

Purpose

The aim of this report is to examine whether children with gastroesophageal reflux (GER) have delayed gastric emptying compared to healthy children.

Methods

All patients had GER verified by 24-hour pH monitoring. Gastric emptying of cow’s milk was examined by radionuclide scintigraphy in 51 patients with GER and in 24 controls. Gastric emptying rate was expressed as exponential half time (T_1/2).

Results

Median age was 4.4 years [range 0.1–15.4] in patients and 6.1 years [range 2.5–10.0] in controls (p = .10). A wide range of gastric emptying rates was observed both in GER patients [range 16–121] and controls [range 29–94]. One GER patient (2%) had slower gastric emptying (T_1/2 = 121 min) than the healthy child with the longest T_1/2 (94 min). Mean T_1/2 was 49 minutes (SD 20.1) and 46 minutes (SD 14.2) in GER patients and controls, respectively (p = .51).

Conclusions

Gastric emptying rate of milk was not significantly different between children with GER and healthy children. A wide range of gastric emptying rates was observed in both groups.     

Synchronous splenectomy during cholecystectomy for hereditary spherocytosis: Is it really necessary?

Background/Purpose

Expert guidelines recommend performing synchronous splenectomy in patients with mild hereditary spherocytosis (HS) and symptoms of gallstone disease. This recommendation has not been widely explored in the literature. The aim of this study is to determine if our data support expert opinion and if different practice patterns should exist.

Methods

This is an IRB-approved retrospective study. All HS patients under 18 years of age who underwent cholecystectomy for symptomatic gallstones at a single institution between 1981 and 2009 were identified. Patients who underwent cholecystectomy without concurrent splenectomy were reviewed retrospectively for future need for splenectomy and evidence of recurrent gallstone disease.

Results

Of the 32 patients identified, 27 underwent synchronous splenectomy. The remaining 5 patients underwent cholecystectomy without splenectomy and had a mean age of 9.4 years. One of the 5 patients eventually required splenectomy for left upper quadrant pain. None of the remaining 4 required hospitalization for symptoms related to hemolysis or hepatobiliary disease. Median follow-up is 15.6 years.

Conclusion

The need for splenectomy in patients with mild HS and symptomatic cholelithiasis should be assessed on a case by case basis. Our recommendation is to not perform synchronous splenectomy in conjunction with cholecystectomy for these patients if no indication for splenectomy exists.     

Operative Details and Oncological and Functional Outcome of Robotic-Assisted Laparoscopic Radical Prostatectomy: 400 Cases with a Minimum of 12 Months Follow-up

Background

Robotic-assisted laparoscopic radical prostatectomy (RALP) using the da Vinci^® surgical system (Intuitive Surgical, Sunnyvale, CA) is increasingly used for the management of localised prostate cancer.

Objective

We report the operative details and short-term oncological and functional outcome of the first 400 RALPs performed at our unit.

Design, setting and participants

From December 2003 to August 2006, 400 consecutive patients underwent RALP at our institution. A prospective database was established to record the relevant details of all RALP cases.

Surgical procedure

A six port transperitoneal approach using a 4-arm da Vinci^® system was used to perform RALP. This database was reviewed to establish the operative details and oncological and functional outcome of all patients with a minimum of 12 months follow-up.

Measurements

Perioperative characteristics and outcomes are reported. Functional outcome was assessed using continence and erectile function questionnaires. Biochemical recurrence (prostate-specific antigen (PSA) ≥0.2 ng/mL) is used as a surrogate for cancer control.

Results and limitations

The mean age ± standard deviation (SD) was 60.2 ± 6 years. Median PSA level was 7.0 (interquartile range (IQR) 5.3–9.6) ng/mL. The mean operating time ± SD was 186 ± 49 mins. The complication rate was 15.75% comprising Clavien grade I-II and Clavien grade III complications in 10.5% and 5.25% of patients respectively.The overall positive surgical margin rate was 19.2% with T2 and T3 positive margin rates of 9.6% and 42.3% respectively. The biochemical recurrence-free survival was 86.6% at a median follow-up of 22 (IQR = 15–30) months. At 12 months follow-up, 91.4% of patients were pad-free or used a security liner. Of those men previously potent (defined as Sexual Health Inventory for Men [SHIM] score ≥21) who underwent nerve-sparing RALP, 62% were potent at 12 months.

Conclusions

The safety and feasibility of RALP has already been established. Our initial experience with this procedure shows promising short-term outcomes.     

Complications and Early Postoperative Outcome in 1080 Patients After Thulium Vapoenucleation of the Prostate: Results at a Single Institution

Background

Thulium vapoenucleation of the prostate (ThuVEP) has been introduced as a minimally invasive treatment for benign prostatic obstruction (BPO).

Objective

To analyze immediate outcomes and the institutional learning curve of ThuVEP, and to report its standardized complication rates, using the modified Clavien classification system (CCS) to grade perioperative complication rates.

Design, settings, and participants

A prospective evaluation of 1080 patients undergoing ThuVEP from January 2007 until May 2012 at our institution.

Intervention

ThuVEP was performed using the 2-μm, continuous-wave, thulium:yttrium-aluminum-garnet laser.

Outcome measurements and statistical analysis

Preoperative status, surgical details, and immediate outcome were recorded for each patient. Perioperative complications were classified according to the modified CCS.

Results and limitations

Median prostate size was 51 ml (interquartile range [IQR]: 36–78.7). Median operation time was 56 min (IQR: 40–80), and median enucleation time was 32.5 min (IQR: 22-50). Median catheterization time was 2 d (IQR: 2–2); median length of hospital stay was 4 d (IQR: 3–5). Median resected tissue weight was 30 g (IQR: 16.00–51.25). Incidental carcinoma of the prostate was detected in 59 (5.5%) patients. Median maximum urinary flow rate (8.9 vs 18.4 ml/s) and postvoid residual urine volume (120 vs 20 ml) changed significantly (p < 0.001). Minor complications occurred in 24.6% of the patients (Clavien 1: 20.8%; Clavien 2: 3.8%). Early reinterventions were necessary in 6.6% of the patients (Clavien 3a: 0.6%; Clavien 3b: 6%). One Clavien 4 complication occurred (0.09%). The overall complication rates decreased significantly over time due to decreasing Clavien 1, 2, and 3b events. The major limitations of the study are the prospective, unicentric study design, the lack of a control group, and that only short-term data were documented on morbidity and efficacy of the ThuVEP procedure.

Conclusions

ThuVEP is a safe and effective procedure for the treatment of symptomatic BPO, with low perioperative morbidity.     

Pleural effusion following blunt splenic injury in the pediatric trauma population

Background

Pleural effusion is a potential complication following blunt splenic injury. The incidence, risk factors, and clinical management are not well described in children.

Methods

Ten-year retrospective review (January 2000–December 2010) of an institutional pediatric trauma registry identified 318 children with blunt splenic injury.

Results

Of 274 evaluable nonoperatively managed pediatric blunt splenic injures, 12 patients (4.4%) developed left-sided pleural effusions. Seven (58%) of 12 patients required left-sided tube thoracostomy for worsening pleural effusion and respiratory insufficiency. Median time from injury to diagnosis of pleural effusion was 1.5 days. Median time from diagnosis to tube thoracostomy was 2 days. Median length of stay was 4 days for those without and 7.5 days for those with pleural effusions (p < 0.001) and 6 and 8 days for those pleural effusions managed medically or with tube thoracostomy (p = 0.006), respectively. In multivariate analysis, high-grade splenic injury (IV–V) (OR 16.5, p = 0.001) was associated with higher odds of developing a pleural effusion compared to low-grade splenic injury (I–III).

Conclusions

Pleural effusion following pediatric blunt splenic injury has an incidence of 4.4% and is associated with high-grade splenic injuries and longer lengths of stay. While some symptomatic patients may be successfully managed medically, many require tube thoracostomy for progressive respiratory symptoms.     

Intra-abdominal venous thrombosis after colectomy in pediatric patients with chronic ulcerative colitis: Incidence,treatment, and outcomes

Purpose

Children with chronic ulcerative colitis (CUC) are at increased risk for venous thromboembolism, especially after colectomy procedures. We aim to review our patients with CUC who underwent a colectomy and suffered intra-abdominal thrombosis; moreover we wanted to define thrombotic incidence and outcomes

Methods

In this is IRB approved retrospective study, we reviewed our patients who underwent colectomy for CUC from January 1999 to December 2011 for development of intra-abdominal thrombosis.

Results

Of 366 patients with CUC who underwent colectomy, 15 (4%) were diagnosed with a venous thromboembolism. All patients presented with acute abdominal pain. The locations of thrombus formation varied: 13 (87%) developed thrombi in the portal vein, 4 (27%) in the splenic vein, 2 (13%) in the superior mesenteric vein, 1 (7%) in the hepatic vein, and 1 (7%) in the hepatic artery. The mean number of post-operative days at diagnosis of thrombus was 38.7 days (range 3–180 days). Fourteen patients (93%) underwent anticoagulation for treatment. The mean number of days of anticoagulant therapy until documented resolution of thrombus on imaging was 96.3 days (range 14–364 days). All thrombi resolved with therapy. There was no mortality during follow-up.

Conclusions

Four percent of our pediatric patients with chronic ulcerative colitis who underwent colectomy developed symptomatic intra-abdominal venous thromboembolism. 3 to 6 months of anticoagulant therapy is adequate treatment in almost all patients. Practitioners should have a high index of suspicion for intra-abdominal venous thrombus when these patients complain of abdominal pain postoperatively. Based on our experience, prophylactic anticoagulation should be strongly considered peri-operatively in this population.     

Treatment of esthesioneuroblastomas

Objective

To analyze the clinical features, treatment outcomes, pattern of failures, and course of the disease of a cohort of patients treated for esthesioneuroblastoma (ENB) with craniofacial resection (CFR) at a single institution during a 12-year period.

Material and methods

Retrospective analysis of 11 patients with ENB treated with CFR in a tertiary care academic medical center from 1998 to 2009.

Results

Median age at diagnosis was 51 years (range 41–67 years). The most common presenting symptom was nasal obstruction (91%). Four patients (36%) presented with Kadish stage B, six patients (55%) with Kadish stage C, and one patient (9%) with Kadish stage D. The initial treatment was craniofacial resection (CFR) alone for three patients (23%), CFR followed by postoperative radiation therapy (RT) in seven patients (64%), while one patient (9%) received both neoadjuvant and adjuvant RT in addition to surgery. The mean and median follow-up times were 66 and 58 months, respectively (range 23–158 months). Seven patients are currently alive with no evidence of disease (64%), while two patients are alive with disease (18%). Overall survival was 100% at one year postoperatively and 80% five years after the primary treatment. The progression free survival was calculated to 73% at one year and 64% at five years.

Conclusions

ENB is an uncommon diagnosis with an incidence of 0.037/100,000 persons/year in the catchment area of our institution. Treatment can be challenging, especially with advanced disease. CFR with RT offers good oncologic disease control with minimal morbidity.     

Identification du risque de douleur chronique après hépatectomie pour don de foie par les trajectoires de douleur

Objectives

To assess pain trajectories in predicting risk of chronic postoperative pain (CPP) after liver resection for living donor transplantation.

Study design

Retrospective analysis of patients undergoing liver resection for living donor transplantation during 3 years.

Patients and methods

After recording perioperative data, patients presenting CPP at 3 months were separated from patients without postsurgical pain problem in order to build a pain trajectory for liver donor patients without CPP. Postoperative course of liver donors with CPP was then compared to that standard pain trajectory.

Results

Sixty-five patients (30 females, 35 males) were included. Epidural analgesia was used in 66%; others received autocontrolled analgesia by morphine. Severe acute pain was expressed by 11% and 37% at rest and movement respectively on the first day. Chronic pain involved six patients without any link with gender or type of analgesia. Analysis of pain trajectories shown that these patients presented either higher initial pain at day 1 or positive slopes with worsening of pain.

Conclusion

Acute postoperative pain is a risk factor of developing CPP. Identification of those people by pain trajectories can be useful to treat them early.     

Douleur et troubles comportementaux après adénoïdectomie et pose d’aérateurs transtympaniques chez l’enfant

Objectives

The purpose of this study was to evaluate postoperative pain in the hospital and at home as well as behavioural changes at home following outpatient adenoidectomy (VG) and ear tube (ATT) surgery.

Study design

Prospective cohort study.

Patients and methods

Sixty-four children (mean age 4.3 ± 2.4 years): 28 VG, 16 (ATT), 20 dual surgeries (VG-ATT). Postoperative pain was evaluated (arrival in recovery room, departure from wake-up room, departure from hospital) using the Objective Pain Scale (OPS). Parents evaluated their child's pain at home over a period of seven days using a numeric pain scale. Behavioural changes were measured with the Post-Hospital Behaviour Questionnaire (PHBQ).

Results

At arrival in the recovery room, OPS = 3.5 [0–6]. A statistically significant difference (p < 0.05) was shown between the VG group (OPS = 5 [2.25–7.75]), and the ATT (OPS = 0 [0–5.5]) and VG-ATT (OPS = 2 [0–5.75]) groups. OPS was 1.0 [0–2] when leaving the recovery room, and OPS was 0 [0–1] when leaving the hospital. Numeric pain scale scores recorded at home were extremely low. Postoperatively, 75% of parents at Day 1 and 40.6% at Day 7 reported at least one postoperative behavioural change.

Discussion

In all three groups, parents reported frequent postoperative behaviour changes despite adequate analgesia.

Conclusion

The relatively high frequency of postoperative behaviour changes in this population demonstrates the need to systematically evaluate those changes in order to improve overall paediatric care.     

Infiltration peropératoire de lévobupivacaïne après césariennes : étude randomisée en double insu contre placebo

Objectives

The efficacy of single-dose intraincisional infiltration with levobupivacaine in postoperative analgesia and chronic pain after caesarean sections is unknown.

Study

A placebo-controlled double-blind randomized trial.

Patients and methods

After ethical approval, and written inform consent, 140 women scheduled for a caesarean section were randomly assigned and received 30 mL of levobupivacaine 0.5% (L group) or saline (placebo-P group) into their wound. The primary endpoint was morphine consumption (using intravenous morphine patient-controlled analgesia) for the first 24 h after surgery. At 1 h to 48 h, side effects, pain at rest and pain 2 months later were recorded.

Results

All included patients had similar demographic and surgical characteristics. The morphine consumption was significantly lower in the L group at h6, h8 and h12 (considering both total intake and each request). At h4, the mean total morphine consumption was 25 (12) mg in the L group versus 31 (14) mg in the P group (P = 0.05). Time until discharge and side effects including nausea-vomiting (14 vs 20%), wound scar complications (6 vs 8%) and chronic pain after 2 months (25% in both groups complained of small pain, and 75% no pain) were similar between the two groups (P > 0.05).

Conclusion

Single-dose local infiltration of levobupivacaine 0.5% reduced opioid requirement at 12 h, with no difference after 24 h. www.clinicaltrials.com, number: NCT00621907.     

Lung biopsy for chronic pulmonary disease in children

Introduction and objectives

Lung biopsy is frequently used in the management of children with chronic pulmonary disease to obtain a histological diagnosis. We further evaluate the role of lung biopsy by reviewing our experience of this procedure.

Methods

A retrospective case-note review was carried out of all patients in our regional service under 16 years who underwent a lung biopsy from 1998 to 2011.

Results

Thirty-three children (12 boys) (median 5 years 5 months, range 2 months to 16 years) underwent lung biopsy in the period studied. Following the procedure, 17 patients required ventilation on the intensive care unit for a median of two days (range 1–56 days). Complication rate was 30% (10/33); seven simple pneumothoraces, one tension pneumothorax, and one pneumonia (one child experienced more than one complication). The operative mortality was 12% (4/33). Three children (9%) died within 28 days of surgery. Twenty-six (79%) biopsies provided a definitive histological diagnosis. In 16 (48%) children, the working diagnosis and treatment were changed following lung biopsy.

Conclusion

Lung biopsy has an important role in the management of children with chronic pulmonary disease. However, it carries significant risks which must be considered when assessing the need for histological diagnosis.     

Biological characteristics of pediatric renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions

Purpose

To investigate the clinical features of pediatric Xp11.2 translocation renal cell carcinoma (RCC).

Methods

A retrospective review of 22 cases over 35 years.

Results

Xp11.2 translocation RCCs were identified in 13 boys and 9 girls with a median age of 10.5 years (range: 2.5–16 years). RCC presented with hematuria in 17, abdominal mass in 1, abdominal masses with hematuria in 2, abdominal pain with hematuria in 1, and as an incidental finding in 1 patient. Ten patients were classified stage I, 10 were stage III, and two were stage IV. Of the 10 patients with stage I RCCs, 3 patients with tumor measuring less than 7 cm had nephron-sparing surgery (NSS) and 17 patients underwent simple nephrectomy. A 15-cm tumor was incompletely removed in one patient and another patient with a 25-cm × 18-cm × 15-cm tumor had gross residual. Of the 15 patients followed up between 6 months and 35 years, 13 were still living and 2 had died after surgery.

Conclusions

Xp11.2 translocation RCC is the predominant form of pediatric RCC, associated with advanced stage at presentation. Nephrectomy is the usual treatment for RCC but NSS is an option for patients with tumors measuring < 7 cm. Patients with N + M0 maintained a favorable prognosis following surgery alone.