Management of bilateral Wilms tumor over three decades: The perspective of a single center.

INTRODUCTION: Outcomes of Wilms tumor improved in last 50 years and excellent survival rates can be achieved especially in case of non-metastatic disease and favorable histology. Nevertheless, bilateral cases still stand as a therapeutic challenge. Prognosis of bilateral Wilms tumor (BWT) is not as good as the unilateral tumors of similar stage and histology in terms of survival and renal function. OBJECTIVE: Management of BWT is constantly evolving and still stands as a therapeutic challenge. This study is designed to review and share our experiences on this topic from a surgical standpoint. STUDY DESIGN: The records of patients treated in our clinic between 1980 and 2013 according to Turkish Pediatric Group of Oncology protocol were analyzed retrospectively and clinical data, surgical details, pathology results, long term outcomes were analyzed. RESULTS: Thirteen girls and 7 boys with a mean age of 2,5 years were treated. There were 2 patients with Wilms tumor-Aniridia-Growth Retardation complex and one with isolated hemihypertrophy. Metastasis were detected in lungs of 4 patients; liver of 2 and in the cranium of one. All patients except one with the presumptive diagnosis of unilateral Wilms tumor were given preoperative chemotherapy. To sum up; 19 nephroureterectomies, 8 partial nephrectomies and 13 enucleations were performed to 36 kidneys without any major early or late postoperative complications. Pathologic results revealed positive surgical margins in 2 lesions with enucleation and in 2 with partial nephrectomies and anaplasia in 4 patients. Two patients were not operated due to parental disapproval. Two patients had the need of dialysis; one was anephric and the others' renal functions recovered over a year. Seven patients received radiotherapy for pulmonary metastasis, positive surgical margins or local recurrences. Overall, 13 patients survived and 7 died due to metastasis, recurrences, and complication of dialysis and refusal of surgical treatment. Survival among all patients was 65% and 72.2% among operated ones. Of the 7 patients with the partial nephrectomy, 2 died and 5 survived. Among enucleation group, 8 out of 10 survived and 2 died. Survival was slightly higher among enucleation group (80% vs 71.4%). Median time of follow-up for survivors of disease is 5.8 years (min: 6 months and max: 14 years). DISCUSSION: Outcomes of BWT management have changed dramatically during the last few decades from only survival, to a long life expectancy without the need of renal replacement therapy owing to improvements in treatment options. We argue that positive surgical margins do not necessarily lead to local recurrence. For this reason it may be wiser to favor on more nephron sparing surgery than to achieve negative surgical margins. Adjuvant chemotherapy and radiotherapy may be adequate to prevent local recurrence. Also, survival did not differ significantly between different ways of nephron sparing surgeries, so it may be wiser to choose enucleation over partial nephrectomy which preserves more nephrons. Nephron-sparing surgery should have utmost importance despite the risk of positive margins. On the other hand, there is not enough data to interpret if positive surgical margins have role on distant metastases or not. Presence of metastasis and recurrence seems to be an important determinant of prognosis given the fact that none of the survivors had any metastasis or recurrence. CONCLUSION: Nephron preservation should be the aim while taking positive surgical margin risk on nephron sparing surgery side relying on postoperative chemotherapy and carefully planned radiotherapy to avoid recurrence. However, there is significant diversity on the management BWT in different centers and a certain validated guideline or protocol to provide the optimal treatment is still lacking.

Clinical presentation and management of gastroesophageal reflux disease in a referral center in Turkey.

PURPOSE: The aim of this study was to analyze the characteristics of the pathway from the onset of clinical findings related to gastroesophageal reflux disease (GERD) until the surgical therapy to achieve a better organization of the multiple disciplines and create the best management scheme in a referral center in Turkey. PATIENTS AND METHODS: All patients who underwent anti-reflux surgery for GERD in our unit between 2000 - 2006 were retrospectively reviewed. Information on their past medical follow-up, the clinical findings, diagnostic evaluation and the therapeutic approach was recorded. RESULTS: There were 24 girls and 40 boys with a median age of 36 months (3 - 192 months). Of the 64 cases, 36 (56.3 %) had a history of past medical follow-up. Of these 36 patients, 20 had received medical and/or surgical therapy for GERD without any standardization. The remaining 16 were treated supportively for neurological, respiratory and metabolic problems without having had a diagnosis of GERD. The median duration of symptoms in 28 patients without a past medical history was shorter than that in the remaining 36 patients (p = 0.03). Of the 64 patients at presentation, 35 had neurological, 4 had metabolic, and 3 had a respiratory pathology; the other 4 had a history of esophageal atresia and tracheoesophageal fistula operation; 5 had been operated in another center for GERD and only 13 cases had isolated GERD. The most commonly used diagnostic methods were contrast study (n = 52) and pH monitoring (n = 36). Forty-one of the 64 had anti-reflux surgery primarily after presentation, in addition to selection of medical therapies and/or other surgical interventions as a first step in the remaining 23. Reevaluation of those 23 patients after a median period of 7 months (1 - 36) finally led to anti-reflux surgery (ARS). Patients presenting with a stricture due to GERD (n = 13) underwent ARS, either as a primary procedure (n = 3) or after a course of dilatations (median period of time: 8.5 months) (n = 10). CONCLUSIONS: Extended nonresponsive medical therapy is as harmful as needless surgical therapy performed prior to appropriate medical management. Our experience emphasizes that guidelines on the use of a multidisciplinary approach is the first step for successful GERD treatment. ARS in early infancy should only be justified in the presence of severe neurological and/or respiratory pathologies. If there is any doubt about the diagnosis of GERD with preliminary methods, endoscopic and pathological confirmation of the disease is mandatory for a correct management.

The use of pH monitoring and esophageal manometry in the evaluation of results of surgical therapy for gastroesophageal reflux disease.

BACKGROUND/PURPOSE: Surgery is indicated for the treatment of gastroesophageal reflux disease (GERD) when medical treatment fails or complications are encountered in children. However, it has not been fully established how the results after surgery can be evaluated. A prospective study was performed to evaluate the results of surgical therapy for GERD by pH monitoring (PM) and esophageal manometry (EM) in children. METHODS: Patients who were candidates for anti-reflux surgery between 2003 and 2004 were evaluated for symptoms, growth and results of PM and EM both in the pre- and postoperative periods. RESULTS: Thirteen patients were included (mean age = 6.65 +/- 3.28 years, male/female ratio = 10/3). Most frequently occurring symptoms were recurrent respiratory infections (RRI) (n = 11) and vomiting (n = 8). Nissen fundoplication was performed because of unresponsiveness to treatment (n = 10), RRI (n = 9), failure to thrive (n = 7) and esophagitis (n = 2) after medical treatment (2 - 36 months). Symptoms were resolved in 83.9 % of patients and were not changed in 16.1 % following surgery. Weight percentiles had significantly improved (pre: 12.38, post: 25.4, p < 0.05) during a short follow-up period (1 - 4 months). Mean reflux index (pre: 24.73 +/- 21.07 %, post: 0.93 %, min: 0 - max: 3.6, p < 0.05), reflux time (pre: 368 +/- 313 min, post: 17.1 +/- 15.9 min, p < 0.05), number of episodes (pre: 344.7 +/- 343.6, post: 19.53 +/- 11.13, p < 0.05) and number of reflux episodes longer than 5 minutes (pre: 4.3, min: 0 - max: 58, post: 0.61, min: 0 - max: 3, p < 0.05) were found to be reduced after surgery by PM. Lower esophageal sphincter pressure (pre: 55 +/- 27 cmH (2)O, post: 64.46 +/- 30.85 cmH (2)O), contraction amplitude (pre: 141.92 +/- 69.11 cmH (2)O, post: 130.69 +/- 45 cmH (2)O) and contraction velocity (pre: 1.94 cm/s, min: 0.1 - max: 7.5, post: 4.29 cm/s, min: 0.2 - max: 10) did not differ postoperatively (p > 0.05). However, contraction times were decreased postoperatively (pre: 73.6 +/- 52.9 s, post: 27.67 +/- 20.1 s, p < 0.05) and were found to be correlated with reflux time and the number of reflux episodes longer than 5 minutes. CONCLUSION: Nissen fundoplication is effective for the treatment of GERD. It supports the anti-reflux mechanism without affecting esophageal motility except for contraction times. The decrease in contraction time after surgery can be explained by the decreases in reflux time and in the number of reflux episodes longer than 5 minutes. PM and EM confirmed the clinical improvement and can be used for the evaluation of results of NF.

Sigmoid colon vaginoplasty in children.

BACKGROUND: Vaginal construction is necessary for the patients with aplasia of Mullerian ducts, testicular feminisation and androgen insensitivity syndromes. Many methods of vaginal construction have been described. We report here the outcomes of six adolescent patients who underwent sigmoid colon vaginoplasty with special emphasis on the surgical technique and outcomes. PATIENTS AND METHODS: Between 1990 and 2003, six patients underwent sigmoid vaginoplasty after a diagnosis of 5alpha-reductase deficiency (n = 3), testicular feminisation (n = 2) or vaginal atresia (n = 1). The mean age was 16 years (13 to 18). Wide spectrum antibiotics and whole-gut preparation were used in all cases. A 15-20 cm segment of sigmoid colon was pulled through the retrovesical tunnel. The proximal end was closed in two layers in patients with 5alpha-reductase deficiency and with testicular feminisation. A distal anastomosis was carried out to the opening made on the vaginal plate (5alpha-reductase deficiency) or on the tip of the shallow rudimentary vagina (testicular feminisation). The sigmoid segment was interposed between the blind end of the atretic vagina and the perineum in the patient with vaginal atresia. Patients were instructed to perform daily vaginal irrigation. The neovagina was examined and calibrated under anaesthesia. No routine vaginal dilatation was recommended. RESULTS: All but one patient had an uneventful postoperative period and were discharged within 7-8 days. All patients had an excellent cosmetic result with an appropriate vaginal length. One of the patients experienced late stenosis of the introitus which responded to dilatations. Mucus discharge was not a significant problem. The patient with vaginal atresia (Bardet-Biedl syndrome) experienced deep vein thrombosis, renal failure and sepsis, resulting in death. CONCLUSION: Sigmoid colon vaginoplasty is a special procedure which appears appropriate for the construction of a new vagina in children. A sigmoid colon neovagina meets all necessary criteria after a vaginoplasty. It provides an adequate diameter and length, and produces less scar tissue in the perineum. It is self-moistening, easily adaptable to the uterus, cervix and rudimentary atretic vaginal segments and does not require routine dilatation. Mild stenosis of the introitus can be treated by dilatations and revision can be easily performed in severely stenotic cases. On the other hand, the patient may face morbidity after laparotomy and other serious complications may occur due to accompanying diseases.

Clinical predictors for differential diagnosis of acute scrotum.

Accurate and early diagnosis of acute scrotum is of the utmost importance to avoid testicular loss and/or needless surgery. The aim of this study is to analyze the clinical presentation and physical examination parameters together with the results of imaging studies in order to find out predictors for the differential diagnosis of acute scrotum with special emphasize on testicular torsion (TT). Patients who were treated for acute scrotum in our unit from 1970 up to and including 2000 were reviewed retrospectively. The study group was subdivided into three groups: "testicular torsion (TT)", "torsion of testicular appendage (TTA)", and "epididymoorchitis (EO)" group according to the diagnosis confirmed by radiologic and surgical findings. Presence and/or absence of various parameters related to clinical presentation, physical examination, and results of imaging studies were analyzed by statistical means in each group. One hundred and sixty children with a mean age of 12.2 years formed the study group. The mean duration of symptoms was 15 hours. No significant difference was noted between the three groups with regard to mean age and duration of symptoms. Nuclear scintigraphy and Doppler ultrasound were used as diagnostic aids in 50 and 66 children, respectively. Clinical presentation parameters including the presence of a) previous trauma, b) pain attacks, and c) nausea and vomiting, had the highest sensitivity, specificity, positive and negative predictive values in the TT group and the lowest values in the EO group. In contrast, the EO group had the highest statistical values with regard to presence of dysuria and micturition disorders. Physical examination parameters consisting of a) elevation, b) transverse location of testis, c) anterior rotation of epididymis, and d) absence of cremasteric reflex had the highest statistical values in the TT group. Contrary to this, imaging studies had the highest specificity and positive predictive values in the TTA group and the lowest values in the TT group. Previous history of trauma and pain attacks, presence of nausea and vomiting, and absence of urinary complaints are the main predictors of TT. Elevation and transverse location of testis with an anteriorly rotated epididymis associated with loss of ipsilateral cremasteric reflex strongly indicate TT. Although accuracy of imaging studies is higher for the differential diagnosis of TTA and EO, there is a considerable risk of misdiagnosis. Therefore, differential diagnosis of acute scrotum, particularly TT, still remains a clinical diagnosis, and clinical parameters deserve more importance in surgical decision making.

Evaluation of pelvic contractility in ureteropelvic junction obstruction: an experimental study.

BACKGROUND/PURPOSE: Ureteropelvic junction (UPJ) obstruction causes adaptive and/or compensatory alterations in renal pelvic contractility. As these alterations directly affect the outcome after renal damage, definition of these alterations is of the utmost importance from a clinical point of view. Thus, an experimental study was designed to determine the alterations of renal pelvic contractility in response to partial and complete UPJ obstruction. METHODS: Fifteen adult female New Zealand white rabbits were randomly assigned into three groups (each containing 5 rabbits) according to the degree of unilateral UPJ obstruction. Group I: sham operation was performed and served as the control group; group II: partial UPJ obstruction was made; group III: complete UPJ obstruction was made. The animals in groups I and II were sacrificed after three weeks and the rabbits in group III were sacrificed after two weeks. Muscle strips from the renal pelvis were prepared. Spontaneous mechanical activity and contractile responses to phenylephrine (PE), serotonin (5-HT), and KCl were recorded isometrically and compared in all groups. RESULTS: Both the frequency and amplitude of spontaneous mechanical contractions were significantly (p < 0.05) increased in partial (group II) and complete UPJ obstruction (group III) groups. PE and 5-HT-induced tonic contractions, which were more prominent in the complete and partial obstruction groups when compared with the control group (p < 0.05). PE and 5-HT also increased the frequency of spontaneous contractions in both partial and complete obstruction groups. KCl induced long lasting tonic contractions in the control muscles. The duration of contraction to reach the maximum amplitude was shortened in the obstruction groups and the amplitudes of the contractions were significantly augmented when compared to control preparations. CONCLUSION: UPJ obstruction alters the contractile properties of renal pelvis smooth muscle. Increased frequency of spontaneous mechanical activity suggests that pacemaker cells of the renal pelvis change their activities in response to UPJ obstruction. Increase in tonic contraction amplitudes in response to PE and 5-HT suggests an increased sensitivity of smooth muscle cells to these agents. Potentiation of the contractile response to KCl suggests that adaptive changes take place at the level of excitation-contraction coupling in the smooth muscle of the renal pelvis following UPJ obstruction.

Thymic teratoma or thymic remnant attached to mediastinal teratoma? The cellular origin of mediastinal teratomas revisited.

Thymic tissue can be encountered in about 20 % of mediastinal teratomas. It is found located at the periphery of the teratoma mass. Although rare, mediastinal teratoma arising within the thymus can be seen. It has not been defined yet whether the thymus is an organ simply attached to the teratoma or whether the teratoma arises from the thymus. The authors present here two illustrative cases of mediastinal teratoma, one raised within the thymus and the other closely attached to the thymic remnant, to discuss the cellular origin of mediastinal teratomas.

An experimental study of choleretic effect and histopathologic alterations in the gastrointestinal system after whole-bowel irrigation.

BACKGROUND/PURPOSE: Whole-bowel irrigation (WBI) has been used for different purposes. It has been the preferred method of bowel cleansing before large bowel surgery and colonoscopy. There are some studies about histopathologic alterations in the gastrointestinal system caused by WBI. It was reported that large quantities of bile-stained intraluminal fluid were seen after WBI with lactated Ringer's solution. In light of all these studies, the authors have decided to inspect whether WBI is choleretic and whether the histopathologic findings in the gastrointestinal system are caused by this choleretic effect. METHODS: During the experimental procedures, animals at first were divided into 2 major groups of A and B; A consisted of the animals whose bile was not collected, and B consisted of animals whose bile was collected. Later, these 2 groups were divided into 4 subgroups consisting of 6 animals each. Sham operations were performed on the animals in group SH-A and SH-B. The animals in group saline-A and saline-B were given 0.9% of sodium chloride (isotonic sodium chloride). The animals in group LR-A and LR-B received lactated Ringer's solution. The last group (PEG-A and PEG-B) underwent WBI by using polyethylene glycol solution (PEG). After completing WBI, animals were rested for 4 hours, and at the end of resting time, laparotomy was performed to take biopsy specimens from stomach, small bowel, and large bowel. All of the specimens were evaluated and graded for congestion, edema, and inflammation by the individual blinded pathologist. RESULTS: According to the results, WBI has been shown to affect biliary secretion and to have a choleretic effect (P <.05, Tuckey-Kramer). The choleretic effect has also been shown to be responsible for histopathologic alterations in some groups (P <.05, Tuckey-Kramer). The most severe changes of congestion, edema, and inflammation have been detected in isotonic sodium chloride solutions in all groups. The least alterations have been seen in lactated Ringer's and PEG solutions. CONCLUSIONS: In light of these findings, it might be concluded that the use of WBI can cause different degrees of histopathologic changes in gastrointestinal system depending on the type of solutions. WBI has been shown to have choleretic effect, and it was found that there is a connection between histopathologic changes in gastrointestinal system and choleretic effect of WBI.

Xanthogranulomatous pyelonephritis in children: diagnostic and therapeutic aspects.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is an uncommon chronic, inflammatory disease of the kidney. Etio-pathogenesis, diagnosis and management of pediatric XGP is still obscure due to the limited number of cases. Therefore, a retrospective clinical study was carried out to present an updated picture of the entire spectrum of pediatric XGP based on our 30 years' experience covering one of the largest non-collected series treated in a single medical center. METHODS: Records of children who were treated for XGP in our unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures,treatment methods, histopathologic findings and outcome. RESULTS: 17 children with a mean age of 6.6 +/- 0.8 years, consisting of 15 males and 2 females, were treated for XGP. Eight patients had a urological disease history and 4 of them underwent surgery for urinary calculi and exstrophia vesica repair. The most common presenting symptoms were abdominal pain, fever,weight loss and anorexia. Palpable flank mass was the most common physical examination finding. Left and right kidneys were involved in 10 and 7 patients, respectively. Diagnostic procedures were intravenous pyelography, retrograde pyelography, ultrasound scan and computerized tomography (CT). Renal calculi and/or calcifications and non-functioning kidney were the most frequent findings in radiologic investigations. By combining all these investigations, the preoperative diagnoses were XGP (n = 2), renal and/or perirenal abscess (n = 5), psoas abscess(n = 2), non-functioning kidney due to recurrent pyelonephritis(n = 4), Wilms' tumor and/or renal clear-cell carcinoma (n = 4). XGP was correctly diagnosed in only two patients based on cr findings. Drainage of the renal or psoas abscess was performed in 8 patients as an initial procedure (surgical drainage in 6 and ultrasound-guided percutaneous drainage in 2 patients). Surgical procedures included nephrectomy (n= 13), partial nephrectomy (n=2), nephrectomy and diversion of reno-colic fistula(n= 1), and renal biopsy and nephrostomy (n= 1). Operative and postoperative complications were colonic perforation (n= 3) and wound infection (n = 3). Complications were noted only in patients who underwent nephrectomy without initial drainage procedures. Histopathologic examinations showed diffuse and focal XGP in 14 and 3 patients, respectively. CONCLUSION: XGP should be included in the differential diagnosis of all children presenting with perirenal or psoas abscess, renal mass and/or non-functioning kidney associated with/or without urolithiasis. Clinical awareness and a high index of suspicion is required to achieve the correct preoperative diagnosis and appropriate management. CT seems to be the most valuable imaging method for the diagnosis. We strongly recommend percutaneous drainage of the abscess and adjunctive antibiotic therapy prior to nephrectomy to avoid complications. Complete nephrectomy is the proper treatment for the diffuse form whereas frozen section biopsies followed by partial nephrectomy are mandatory for the proper treatment of focal disease.

Colonic atresia: surgical management and outcome.

Colonic atresia (CA) is a very rare cause of intestinal obstruction, and little information has been available about the management and predictors of outcome. A retrospective clinical trial was performed to delineate the clinical characteristics of CA with special emphasis on surgical treatment and factors affecting outcome. Children with CA who were treated in our department between 1977 and 1998 were reviewed: 14 boys and 4 girls aged 1 day to 5 months. All but 2 referred patients and 1 with prenatal diagnosis presented with intestinal obstruction. Plain abdominal X-ray films showed findings of intestinal obstruction in 14 cases; a barium enema demonstrated a distal atretic segment and microcolon in 4. The types of atresia were IIIa (n=9), I (n=6), and II (n=3). Type IIIa atresias were located proximal to the splenic flexure (n=8) and in the sigmoid colon (n=1), type I atresias were encountered throughout the colon; and all type II atresias were proximal to the hepatic flexure. Associated anomalies were multiple small-intestinal atresias (MSIA) (n=4), gastroschisis (GS) (n=2), pyloric atresia (n=1), Hirschsprung's disease (n=1), and complex urologic abnormalities (n=1). The initial management was an enterostomy in 15 patients (83%), including 2 referred and 2 with GS, and primary anastomosis in the remaining 3 (17%). Secondary procedures were the Santulli operation (n=2), colostomy closure and recolostomy followed by a Swenson operation (n=1), sacroabdominoperineal pull-through (n=1), and colostomy closure (n=1). Leakage was encountered in all primarily anastomosed patients. The overall mortality was 61%. Deaths occurred in patients with associated major anomalies (GS 2, MSIA 3, pyloric atresia 1) (55%) and in 3 patients who were initially managed by primary anastomosis (27%). Two additional patients died of sudden infant death syndrome (18%). Type I atresia was more common than in previously reported series and was associated with proximal multiple atresias. The initial management of CA should be prompt decompression of the intestine by an ostomy procedure, preferably end- or double-barrel. The type of surgery (primary anastomosis without prior colostomy) and associated abnormalities are the major determinants of poor outcome.

Testicular tumors in children.

PURPOSE: The aim of this study was to present an updated picture of surgical management of pediatric testicular tumors based on our 30 years' experience, which consisted of one of the largest noncollected series treated in a single medical center. METHODS: Records of children who were treated for testicular tumor in our unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures, treatment methods, histopathologic findings, and outcome. RESULTS: Fifty-one patients with a mean age of 3.8 +/- 0.5 years were treated for testicular tumors. Of these, 35 (69%) had germ cell testis tumor (GCT) and 16 (31%) had non-germ cell testis tumor (NGCT). Endodermal sinus tumor and paratesticular rhabdomyosarcoma were the dominant histologic subtypes in each group, respectively. The most common mode of presentation was painless scrotal mass. At initial presentation, retroperitoneal (n = 5), both retroperitoneal and lung (n = 2), and retroperitoneal and liver (n = 3) metastases were recorded in 10 (19%) patients. Initial operative procedures were radical inguinal orchiectomy (RIO) (n = 29), scrotal orchiectomy (SO; n = 9), bilateral RIO (n = 2), both RIO and unilateral retroperitoneal lymph node (RPLN) excision (n = 6), testis-sparing enucleation of the tumor (n = 5). SOs were performed elsewhere, and these patients underwent high ligation (n = 4) and both high ligation plus RPLN excision (n = 5) in our unit. Histopathologically, spermatic cord invasion and RPLN involvement were present in 10 patients. Scrotal recurrences were encountered in 2 patients who had scrotal orchiectomy initially. Retroperitoneal recurrences were noted in a patient presenting with stage I embryonal carcinoma and in 2 patients presenting with group IV paratesticular rhabdomyosarcoma. The mean follow-up period was 89 +/- 10 months. Four patients with stage IV embryonal carcinoma (n = 2) and group IV paratesticular rhabdomyosarcoma (n = 2) died of progression of the disease. All remaining patients were alive and disease free at their last outpatient appointment. No significant difference was noted with regard to 5-year survival rates between (1) malignant GCT and paratesticular rhabdomyosarcoma patients (91% v 80%) and (2) patients treated by RIO (88%), SO plus high ligation (87%), and RIO plus RPLN excision (80%). Five-year survival rates were 100% for stage I, II, III patients and 33.3% for stage IV and group IV patients presenting with malignant testicular tumors (P <.05). CONCLUSIONS: Childhood testicular tumors deserve special attention from the therapeutic point of the view. A solid scrotal mass should be considered malignant until proved otherwise. Any suspicion of the testicular tumor warrants an inguinal approach to prevent scrotal violation by the tumor. Current trends emphasize that testis-sparing surgery should be performed for benign lesions such as teratoma, leydig cell tumor, and epidermoid cyst based on frozen biopsy findings. Literature findings and our experience suggest that RIO is the accurate treatment for stage I malignant GCT and group I and IIa paratesticular rhabdomyosarcoma. RPLN excision is not of benefit either as a staging or therapeutic procedure in stage I and group I and IIa diseases of these tumors. RPLN excision should be reserved for (1) malignant GCT patients who have persistent elevation of alpha-fetoprotein after orchiectomy in the presence of normal total body CT scan, and for patients presenting with stage II and III disease with definitive abnormality on CT scans, and (2) group IIb, IIc, and III paratesticular rhabdomyosarcoma patients with radiologic evidence of retroperitoneal involvement on CT scans. High ligation should be done as a complementary procedure after SO to increase the survival rates. J Pediatr Surg 36:1796-1801.

Pneumatosis intestinalis in an infant undergoing bone marrow transplantation for Wiskott-Aldrich syndrome.

A 7-month-old patient with Wiskott-Aldrich syndrome (WAS) developed pneumatosis intestinalis (PI) in the immediate post-transplant period after receiving paternal human leucocyte antigen (HLA) phenotypically matched bone marrow (BM). PI has been described in patients with congenital or acquired immunodeficiency states and after bone marrow transplantation (BMT). To our knowledge, the condition has not been described in WAS. The underlying bowel mucosa damage as a result of the history of massive rectal bleeding, the effects of the conditioning regimen, immunosuppression, neutropenia, and infection, may all have contributed to the development of PI. Although the condition resolved by conservative management alone, the patient developed Klebsiella pneumonia sepsis, interstitial pneumonitis, failed to engraft, and died on day +66 following a second infusion of stem cells mobilized from his father's peripheral blood.

Inflammatory myofibroblastic tumor in children: diagnosis and treatment.

BACKGROUND/PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm. Although it is commonly seen in children, the number of childhood cases in the current literature is limited. Furthermore, malignant degeneration or transformation to lymphoma in the recurrent or residual IMT have directed attention to this interesting entity. Herein, the authors present their experience with IMT with special emphasis on diagnosis and treatment. METHODS: All records of children treated with diagnosis of IMT between 1977 and 1999 inclusive were evaluated retrospectively. RESULTS: Seven children were treated for IMT with the mean age of 9.14 +/- 2 years (range, 6 to 12 years). Male to female ratio was 5:2. Respiratory symptoms and clubbing were present in a patient with pulmonary IMT (n = 1). Abdominal pain (n = 3), fever (n = 2), and weight loss (n = 4) were encountered in intraabdominal IMTs. The most frequent physical finding was palpable intraabdominal mass (n = 4). Plain films showed nonspecific findings such as radiodense area in the hemithorax (n = 1), displacement of bowel segments (n = 2), air-fluid levels (n = 1), and amorphous calcification (n = 4). Ultrasonography and CT showed calcified masses in 4 cases. Except the case with intrathoracic IMT, all the tumors were located in the abdomen at various sites such as cardioesophageal junction (n = 1), left hepatic lobe (n = 1), mesentery of the small bowel (n = 2), and antimesenteric wall of the descending colon (n = 1), gastrosplenic region and porta hepatis (n = 1). Tumor sizes ranged from 3 x 2 x 2 cm to 15 x 15 x 13 cm. The masses were excised totally in all but one case. Infiltrated organs (esophagogastric junction, a segment of jejunum, and spleen, stomach wall, and renal capsule) were resected in 3 cases. Total surgical excision of IMT was considered adequate for treatment in 6 cases. One patient with aggressive IMT required further treatments such as immunomodulation and chemotherapy and died of neutropenic sepsis. CONCLUSIONS: IMT is a benign neoplasm rarely presented with malignant features such as local invasiveness, recurrence, distant metastasis, or malignant transformation. IMT can be suspected preoperatively through some hematologic abnormalities and radiologic findings, but precise diagnosis should be made on the basis of histologic findings. Complete surgical resection and close follow-up are all necessary for appropriate treatment to avoid recurrences as well as unnecessary and potentially harmful therapy. The optimal management of locally aggressive and recurrent forms should be decided individually for each patient. J Pediatr Surg 36:908-912.

Adrenocortical tumors in children.

BACKGROUND/PURPOSE: Etiopathogenesis and management of pediatric adrenocortical tumors (ACTs) is still obscure because of the limited number of cases. The aim of this study is to present a clear picture of the entire spectrum of pediatric ACTs by reviewing one of the largest noncollected pediatric series treated in a single medical center. METHODS: Records of children treated for ACTs in our unit between 1970 and 1999, inclusive, were reviewed. Information recorded for each patient included age, sex, clinical characteristics, diagnostic methods, stage of disease, treatment, pathologic findings, and outcome. The patients were subdivided into 2 groups: group I, patients with adrenocortical carcinoma (ACC) and group II, patients with adrenocortical adenoma (ACA). These groups were analyzed with regard to parameters mentioned above. RESULTS: There were 30 children treated for ACTs in the study period with a mean age of 6.7 +/- 4.2 years (range, 2.5 to 13 years). Of these, 20 had ACC, and 10 had ACA. The tumors were right sided in 22 patients, left sided in 6 and bilateral in 2. Analysis of each group with regard to age and site of tumor showed no significant difference. Endocrine dysfunction was noted in 83% of the patients and virilization was the most common presentation followed by Cushing's syndrome. The most striking difference between 2 groups was the prepondarance of virilization in group II and Cushing's syndrome in group I. In the latter, 14 patients presented with palpable abdominal mass and 3 patients with distant metastases. The mean time from initial symptoms to diagnosis was 8.1 +/- 0.2 months, and this interval was similar in 2 groups, in functional and nonfunctional tumors, and in both sexes. Ultrasound scan, computerized tomography, magnetic resonance imaging, intravenous pyelography, and angiography were used for the diagnosis. All patients with ACA had localized disease, whereas 80% of the patients with ACC had regional or metastatic disease. Total excision was done in all patients with ACA, but only in 13 patients with ACCs. Of the latter, 2 patients underwent ipsilateral nephrectomy, and 1 patient had right hepatic lobectomy plus nephrectomy. Adjuvant chemotherapy consisting of mitotane (n = 12), mitotane plus cisplatin and etoposide (n = 2) was commenced. Seven patients with ACC had distant metastases postoperatively. The presence of regional disease at presentation was associated with a significantly shorter disease-free interval. All patients presenting with nonfunctional ACC (n = 4), functional ACC that have been totally resected (n = 4), and partially resected (n = 3) died of disease within the first 2.5 years after diagnosis. There was no significant difference between the functional and nonfunctional ACCs with regard to survival rate. All patients who had distant metastases postoperatively and who had partial excision died. Of the surviving 9 patients with ACC, there are 6 known long-term survivors who are still alive. CONCLUSIONS: ACAs are treated by total excision satisfactorily without any complication. For the time being, the most important aspect of therapy for ACCs is early diagnosis and total excision. Partial excision and advanced-stage disease are the major determinants of poor outcome. None of the clinical, laboratory, or pathologic features are reliable predictors for recurrence and discrimination of malignancy in ACTs. Because of the steadily increasing incidence of precancerous genetic syndromes of adrenal glands and poor prognosis of ACCs, childhood patients of endocrine disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults. Patients with ACTs should be entered into multi-institutional trials to adequately assess effective chemotherapy and radiotherapy protocols and molecular mechanisms of oncogenesis. J Pediatr Surg 36:549-554.

Pheochromocytoma in children.

BACKGROUND/PURPOSE: Etiopathogenesis, management, and outcome of pediatric pheochromocytoma (PHEO) still is obscure because of limited number of cases. Therefore, a retrospective clinical study was performed to present an updated picture of the entire spectrum of pediatric PHEO based on the authors' 30 years' experience consisting of one of the largest noncollected series treated in a single medical center. METHODS: Records of patients treated for PHEO in the authors' unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical and family history, clinical characteristics, diagnostic methods, treatment, pathologic findings, and outcome. RESULTS: Sixteen children with a mean age of 10.7 +/- 2.9 years consisting of 12 boys and 4 girls were treated for PHEO. Most of the tumors were right sided (n = 6) and bilateral (n = 6). Sporadic cases of PHEO accounted for 14 patients (88%), whereas 2 children had von Hippel-Lindau (VHL) disease and multiple endocrine neoplasia type 2b (MEN2b). Hypertension was the most common symptom followed by headache and sweating. The diagnosis of PHEO was made by laboratory and radiologic studies. Preoperative medical therapy was done in all patients. Laparotomy confirmed that 11 patients had localized, 4 patients had regional, and 1 patient had metastatic disease. The localized tumors were excised totally by bilateral (n = 4) and unilateral (n = 6) adrenalectomy. Surgical procedures performed for regional disease were total excision (n = 2), incisional biopsy (n = 1) and partial excision (n = 1). Incisional biopsy could be taken only from a patient with metastatic disease at presentation. Two patients with localized disease and 2 patients with regional disease had benign recurrences in right (n = 2) and left (n = 2) adrenal glands within 3 to 7 years after operation. Total excision of the recurrent tumors was done in all patients. Pathologic examination found apparently malignant features in 3 patients who presented with regional (n = 2) or metastatic (n = 1) disease and underwent incisional biopsy (n = 2) or partial excision (n = 1). Pathologic features suggestive of malignancy were noted in 4 patients presenting with regional (n = 2) and localized disease (n = 2). Apparently benign pathologic features were noted in the remaining 9 patients. There was not any operative mortality in our series. Adjuvant chemotherapy was commenced postoperatively in all patients with malignant and suggestive of malignant pathologic features. During the long-term follow-up for 16 years, 3 patients died (19%). One patient with VHL disease died of astrositoma 5 years after her recurrent PHEO was excised. Of the 3 patients with malignant disease, 2 patients in whom only incisional biopsies were done had distant metastases and died of disease within 2 years. Another patient with malignancy who had MEN2b was lost to follow-up. CONCLUSIONS: Early diagnosis and total excision are the most important aspects of accurate treatment for childhood PHEO. Pre- intra- and postoperative medical management is as important as the surgical procedure. Our surgical treatment policy is mainly minimizing the risk of recurrence while preserving adequately functioning adrenal medullar tissue. Incomplete excision and advanced-stage disease are the major determinants of poor outcome. None of the clinical, laboratory, or pathologic features are reliable predictors for recurrence and discrimination of malignancy. Because of the steadily increasing incidence of precancerous genetic syndromes related to adrenal glands and poor prognosis of advanced-stage PHEO, childhood cases of hypertensive disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults.