Patterns of abdominal relapse and role of sonography in Wilms tumor

This study characterizes the patterns of abdominal recurrence of Wilms tumor and describes the role of sonography in its detection. Twelve patients who had initial tumor recurrence in the abdomen were evaluated. Five patients had recurrence in the kidney; all had nephrogenic rests detected by computed tomography (CT) or magnetic resonance (MR) imaging but not by sonography. The remaining 7 patients had recurrence in the peritoneum (4), the nephrectomy site (2), or the regional lymph nodes (1); tumor spillage had occurred in five of these patients. Four recurrences were detected during therapy, and eight within 3 years after completion of therapy. Seven of the 12 recurrences were first detected by sonography. All 11 sonograms obtained at the time of relapse showed tumor recurrence. Nine patients died a median of 10 months after relapse. The results suggest that regular sonographic surveillance for 3 years after therapy is likely to reveal most abdominal recurrences. Supplementation with CT or MR imaging is indicated for detection of nephrogenic rests.     

Unenhanced helical CT in the evaluation of the urinary tract in children and young adults following urinary tract reconstruction: comparison with sonography

Purpose. To compare the accuracy of unenhanced, helical CT with sonography for the detection of complications of urinary tract reconstruction. Materials and methods. Forty-six kidneys in 24 patients were examined with CT and sonography. All scans were assessed for ease of renal visualization, presence of renal, ureteral, and bladder calculi, renal scars, hydronephrosis, and abdominal wall hernia. The results of both imaging modalities were independently reported. Results. CT provided excellent visualization of all 46 kidneys, while sonography provided poor visualization of 8 kidneys (17 %) (P < 0.001). CT detected calculi in 10 kidneys, 1 ureter, and 7 bladders. Sonography detected calculi in only 2 kidneys, and 2 bladders. Overall, CT detected significantly more calculi than US (18 vs 4, P = 0.01). CT detected scarring in 15 kidneys, while sonography detected scarring in 10. Hydronephrosis was detected in 6 kidneys by CT and in 8 kidneys by sonography. Three abdominal wall hernias were seen at CT that were not seen at sonography. Conclusion. CT is superior to sonography for the detection of urinary tract calculi and renal scarring. CT will demonstrate abdominal wall hernias that are unsuspected. Received: 18 April 2000 Accepted: 14 August 2000     

Very late recurrence of childhood acute lymphoblastic leukemia treated with chemoimmunotherapy: a report of three cases occurring 19, 11, and 9 years after discontinuation of chemotherapy

Current therapeutic modalities for childhood acute lymphoblastic leukemia (ALL) are associated with a high cure rate, and recurrences more than 4 years after therapy cessation are very unusual. We report three cases of exceptionally late recurrences of childhood ALL after cessation of chemotherapy (CT) given for respective periods of 8, 7, and 24 months. CT was followed by maintenance immunotherapy (IMT) with Bacillus Calmette-Guérin (BCG) and allogeneic leukemic lymphoblasts pretreated with formaldehyde or irradiated in vitro. Leukemic recurrences were observed 19, 11, and 9 years after cessation of CT and appeared morphologically similar to the original blasts. A second complete remission was easily achieved in all three patients, but two went on to repeated relapse (one has died following the fourth recurrence). We speculate that some residual leukemic cells, remaining after the inadequate, short-term CT, were responsible for these unusual evolutions, and we question a possible delaying role of IMT in prolonging remission. Other possible etiologies are discussed.     

Relapse surveillance in AFP-positive hepatoblastoma: re-evaluating the role of imaging

Background

Children with hepatoblastoma routinely undergo repetitive surveillance imaging, with CT scans for several years after therapy, increasing the risk of radiation-induced cancer.

Objective

The purpose of this study was to determine the utility of surveillance CT scans compared to serum alpha-fetoprotein (AFP) levels for the detection of hepatoblastoma relapse.

Materials and methods

This was a retrospective study of all children diagnosed with AFP-positive hepatoblastoma from 2001 to 2011 at a single institution.

Results

Twenty-six children with hepatoblastoma were identified, with a mean age at diagnosis of 2 years 4 months (range 3 months to 11 years). Mean AFP level at diagnosis was 132,732 ng/ml (range 172.8–572,613 ng/ml). Five of the 26 children had hepatoblastoma relapse. A total of 105 imaging exams were performed following completion of therapy; 88 (84%) CT, 8 (8%) MRI, 5 (5%) US and 4 (4%) FDG PET/CT exams. A total of 288 alpha-fetoprotein levels were drawn, with a mean of 11 per child. The AFP level was elevated in all recurrences and no relapses were detected by imaging before AFP elevation. Two false-positive AFP levels and 15 false-positive imaging exams were detected. AFP elevation was found to be significantly more specific than PET/CT and CT imaging at detecting relapse.

Conclusion

We recommend using serial serum AFP levels as the preferred method of surveillance in children with AFP-positive hepatoblastoma, reserving imaging for the early postoperative period, for children at high risk of relapse, and for determination of the anatomical site of clinically suspected recurrence. Given the small size of this preliminary study, validation in a larger patient population is warranted.     

Teratoma of the stomach in a 4-day-old newborn infant

Teratoma of the stomach is a very rare tumor. Nearly all previously reported patients were males. We report a 4 day-old male infant, who presented with a palpable abdominal mass. A preliminary diagnosis of teratoma was made by abdominal x-ray, sonography and MR imaging. Surgery was performed and diagnosis of teratoma of the stomach was confirmed by histological examination of the tumor. Because teratoma of the stomach is always a benign neoplasm surgery is curative and no further therapy is needed.     

Pediatric Burkitt's Lymphoma and Diffuse B-Cell Lymphoma: Are Surveillance Scans Required?

Outcomes in pediatric B-Non-Hodgkin Lymphoma (B NHL) have improved with intensive chemotherapy protocols, with long-term survival now over 80%. However, long-term adverse effects of therapy and poor outcomes for patients who relapse remain challenges. In this study, we aimed to evaluate the potential risks and benefits of routine relapse surveillance imaging after the completion of therapy. We reviewed 44 B NHL patients diagnosed and treated at Texas Children's Cancer Center in the period between 2000 to 2011. All cross-sectional diagnostic imaging examinations performed for disease assessment after completion of chemotherapy were reviewed and cumulative radiation dosage from these examinations and the frequency of relapse detection by these examinations were recorded. Only 3 patients of the 44 relapsed (6.8%), though none of the relapses were initially diagnosed by computed tomography (CT) or fludeoxyglucose positron emission tomography (FDG-PET) scans. Median effective dose of ionizing radiation per patient was 40.3 mSv with an average of 49.1 mSv (range 0–276 mSv). This single-institution study highlights the low relapse rate in pediatric B-NHL with complete response at the end of therapy, the low sensitivity of early detection of relapse with surveillance CT or FDG-PET imaging, and the costs and potential increased risk of secondary malignancies from cumulative radiation exposure from surveillance imaging. We propose that routine surveillance CT or FDG-PET scans for these patients may not be necessary.     

Intra-abdominal metastasis in osteosarcoma: survey and literature review

Extrapulmonary metastasis, particularly abdominal metastasis from osteogenic sarcoma, are rare and generally appear as a solid mass of calcification as the primary tumor. The aim of this case report is to document the incidence, characteristics, treatment, and prognosis of abdominal metastasis in osteosarcomas in a single institution and to review the literature. From September 1989 to December 2002, 94 children ≤16 years of age with osteosarcomas were diagnosed and treated in the Division of Pediatric Oncology, Oncology Institute, Istanbul University. Patients with abdominal metastasis were assessed. Two girls of 94 patients (2.1%) with osteosarcoma developed abdominal metastasis. One had pulmonary metastasis at diagnosis and the other had developed lung metastasis 15 months after diagnosis. They developed abdominal metastasis 4 and 3 years after diagnosis during therapy or relapse at a median duration of 16 months (1-70 months) from initial diagnosis. All patients had metastasis to various sites, mostly lung, at the time the abdominal metastasis were detected. Treatment included surgery, chemotherapy, and radiotherapy in one and only surgery in the other patient. Both patients died at a median time of 4 months (2-6 months) from the time of abdominal metastasis with progressive disease. Abdominal metastasis in osteosarcoma is a rare event, but abdomen should be investigated in case of recurrence from osteosarcoma. The outcome for these patients is dismal in this series and in the literature.     

Pediatric Hodgkin lymphoma: are we over-scanning our patients?

Despite the favorable outcome of most pediatric patients with Hodgkin lymphoma (HL), there is rising concern about risks of carcinogenesis from both diagnostic and therapeutic radiation exposure for patients treated on study protocols. Although previous studies have investigated radiation exposure during treatment, radiation from post-treatment surveillance imaging may also increase the likelihood of secondary malignancies. All diagnostic imaging examinations involving ionizing radiation exposure performed for surveillance following completion of therapy were recorded for 99 consecutive pediatric patients diagnosed with HL from 2000 to 2010. Cumulative radiation dosage from these examinations and the frequency of relapse detection by these examinations were recorded. In the first 2 years following completion of therapy, patients in remission received a median of 11 examinations (range 0-26). Only 13 of 99 patients relapsed, 11 within 5 months of treatment completion. No relapse was detected by 1- or 2-view chest radiographs (n = 38 and 296, respectively), abdomen/pelvis computed tomography (CT) scans (n = 211), or positron emission tomography (PET) scans alone (n = 11). However, 10/391 (2.6%) of chest CT scans, 4/364 (1.1%) of neck CT scans, and 3/47 (6.4%) of PET/CT scans detected relapsed disease. Thus, only 17 scans (1.3%) detected relapse in a total of 1358 scans. Mean radiation dosages were 31.97 mSv for Stage 1, 37.76 mSv for Stage 2, 48.08 mSv for Stage 3, and 51.35 mSv for Stage 4 HL. Approximately 1% of surveillance imaging examinations identified relapsed disease. Given the very low rate of relapse detection by surveillance imaging stipulated by current protocols for pediatric HL patients, the financial burden of the tests themselves, the high cure rate, and risks of second malignancy from ionizing radiation exposure, modification of the surveillance strategy is recommended.     

Survival after recurrence of osteosarcoma: a 20-year experience at a single institution

BACKGROUND: Approximately one-third of patients with osteosarcoma who have a complete response to their initial treatment can be expected to relapse. It is important to define what host, tumor, or treatment characteristics determine outcome after relapse. We present findings in 59 patients treated at our institution from 1974 to 1996 who have relapsed one or more times after their initial response. METHODS: Host and tumor characteristics at diagnosis and relapse, therapeutic interventions and survival outcomes were determined from examination of medical records and a follow-up questionnaire. RESULTS: Of the 59 patients, 37 initially presented with localized disease of the extremity, 11 with localized non-extremity disease, and 11 with metastatic disease. This report focuses on those with localized disease of the extremity. For these patients, median time from original diagnosis to first recurrence was 14 months. Median survival after first recurrence was 31 months. The median post initial relapse survival was the same for patients whose first relapse occurred before or after 14 months from original diagnosis. Seventeen of 29 patients with systemic metastasis at first recurrence had complete removal of their disease and had a median post-op survival of 2.5 years, while the remaining 12 patients with no surgery, had a median survival of 2 years. Of the 37 patients who presented with primary disease only in the extremities and relapsed: 31 died (2 more than 6 years from first recurrence) and 6 are alive from 6 to 24 years from first recurrence (5 without disease and 1 with disease). Three of the five disease-free survivors had three or more relapses. CONCLUSION: With a long follow-up time, we found 15% of patients with relapsed osteosarcoma who originally presented with localized disease in the extremity are alive with no evidence of disease at 10 years from first recurrence (Kaplan-Meier estimate). Even patients with multiple relapses may have long-term disease-free survival after salvage therapy. Chemotherapy and time to first recurrence were unrelated to survival after relapse in this study. Complete surgical removal of metastatic disease may be important for long-term survival.     

US, CT and MR imaging characteristics of nephroblastomatosis

Objectives. To describe the imaging features of nephroblastomatosis with US, CT and MR, to point out characteristics of differentiation between nephrogenic rests (NR) and Wilms' tumour (WT) and to determine the most appropriate imaging modality. Materials and methods. We reviewed the US, CT and MR images of 29 cases of histopathologically confirmed nephroblastomatosis sent to our department for reference evaluation (German nephroblastoma study). The series included 17 kidneys with NR, 6 kidneys with WT and 32 kidneys with both NR and WT. Results. NR presented as multinodular, peripheral, cortical lesions, the diffuse form of distribution being less common. Foci were homogeneous and of low echogenicity, density or signal intensity. The lesions were most clearly depicted with contrast-enhanced CT and T1-weighted (T1-W) MR images. Lesions smaller than 1 cm were rarely identified by US. The most reliable criterion to differentiate NR from WT was their homogeneity. Conclusions. Contrast-enhanced CT and T1-W MR images are of similar potential and superior to US in the diagnosis of nephroblastomatosis. Due to the significant radiation dose of serial CT, MR imaging should be the method of choice wherever it is available. The cost-effectiveness and availability of US makes it ideal for serial follow-up of known lesions. Received: 6 June 1997 Accepted: 9 January 1998     

Management and algorithm for focal nodular hyperplasia of the liver in children.

PURPOSE: The aim of this study was to determine an appropriate management plan for childhood and adolescent FNH, in particular to establish an algorithm for preoperative diagnosis and treatment. PATIENTS AND METHODS: Between 1985 and 2003, 4 children with FNH were diagnosed. Of these 4 patients, 3 (Group A) underwent tumor resection, and 1 (Group B) was treated by conservative management. Clinical data, pathological findings and follow-up were evaluated retrospectively. RESULTS: The 3 patients in Group A were symptomatic, while the 1 patient in Group B was asymptomatic. In 3 of 4 patients, a homogeneous tumor with a central stellate area was noted on abdominal ultrasonography, CT scan and MR imaging. In case 2, SPIO-enhanced MR imaging was useful for differentiating FNH from hepatocellular carcinoma. Though percutaneous needle biopsy was performed in case 3, a pathologically definitive diagnosis was impossible. An open biopsy was performed in case 4 and FNH was diagnosed. In case 4 treated by conservative management, the tumor size did not change during the 7 years after the diagnosis of FNH. CONCLUSION: FNH is usually treated conservatively because of the good evolutionary outcome of the lesion. Surgery is indicated in cases of complications, compressed adjacent organs, lesion progression, or for symptomatic patients. We advocate the use of less invasive SPIO-enhanced MR imaging instead of open biopsy when the diagnosis of focal liver lesions is not clear after contrast-enhanced CT scan and non-enhanced MR imaging.     

Bilaterally multicentric synchronous Wilms' tumor: Successful conservative treatment despite persistence of nephrogenic rests

We report a case of synchronous bilateral Wilms' tumor in a girl with incomplete Beckwith-Wiedemann syndrome and hemihypertrophy. Multiple small tumors were present in both kidneys. The initial diagnostic biopsy showed stage I monophasic blastematous Wilms' tumor of favorable histology, with multiple perilobar nephrogenic rests (nephroblastomatosis). By flow cytometry, tumor was diploid, with an S-phase fraction of 13.9%. Dactinomycin and vincristine were begun as per the National Wilms' Tumor Study IV (18 week course). After 1 month, only a single small lesion was evident, which persisted unchanged. Excisional biopsy 5 months after beginning chemotherapy showed entirely necrotic tumor but apparently unaltered nephrogenic rests. After completing chemotherapy, the child has done well, with normal renal function and no evidence of disease 3 years after diagnosis. Management of stage V Wilms' tumor is tailored to the individual case, being as conservative as possible to spare renal parenchyma. Given the high incidence of coexisting nephrogenic rests in bilateral Wilms' tumor, careful follow-up is required, as these potentially pre-malignant rests may resist chemotherapy. Med. Pediatr. Oncol. 28:420–423, 1997. © 1997 Wiley-Liss, Inc.     

Value of surveillance imaging in the management of medulloblastoma

BACKGROUND: To investigate the value of surveillance scanning for the detection of recurrences in medulloblastoma. PROCEDURE: The charts of 95 patients with medulloblastoma were retrospectively reviewed. Information regarding the patient characteristics, treatment modalities, dates, types and results of CT and MRI studies, the frequency with which recurrences were identified on surveillance images, changes in patient management, outcome of the patients following recurrences, and survival data were analyzed. RESULTS: Thirty-one patients had a recurrence of tumor in the central nervous system; none experienced extraneural relapses. Of all recurrences, 21 were symptomatic and 10 were discovered by surveillance scans asymptomatically. None of the patients with a recurrence survived. For all 95 patients, 5-year overall and event-free survival rates were 47.1 and 49.8%, respectively. In patients with symptomatic and asymptomatic recurrences, the mean time to recurrence since initial diagnosis, the mean duration of survival post-recurrence, and the mean duration of overall follow-up were 19.2 and 26.1 months, 3.6 and 8.0 months, and 22.8 and 34.1 months, respectively. For 95 patients, 468 surveillance and 38 symptomatic images were reviewed as 313 CTs and 193 MRIs. Rate of diagnosis of recurrence per surveillance image was 2.1% (10/468). CONCLUSIONS: In our study, surveillance scanning brought no survival advantage since it detected a minority of recurrences. Longer survival achieved by early detection of recurrences might be a reflection of lead-time and length biases. Surveillance procedures will gain more importance as new effective therapeutic options are developed for recurrent medulloblastoma.     

Bilateral disease and new trends in Wilms tumour

Wilms tumour is a great therapeutic success story within paediatric oncology; its prognosis is excellent. Although mainly sporadic, occurring in otherwise well children, it occurs in a small number of genetically predisposed children. Thus regular surveillance imaging is performed in predisposed children in parts of the USA and Europe. The risks and benefits of surveillance are unclear, as the existing ad-hoc surveillance protocols are lacking in consistency of practice and equity of provision. We present guidelines for Wilms tumour surveillance based on a review of current practice and available evidence, outlined by a multidisciplinary working group in the UK. Wilms tumours are bilateral in 4–13% of affected children. Bilateral synchronous nephroblastomas are observed in 5% of affected children and are usually associated with the presence of nephrogenic rests, congenital malformations and predisposing syndromes. The major challenge in bilateral disease is to achieve a cure and at the same time to preserve sufficient functional renal tissue for normal growth and development. The association among Wilms tumour, nephrogenic rests and nephroblastomatosis makes detection and characterization of renal lesions with imaging extremely important. We discuss the relative strengths and weaknesses of the different modalities used for diagnosis and follow-up in bilateral renal disease. We also discuss newly emerging diagnostic imaging tests such as ¹⁸F-fluorodeoxyglucose positron emission tomography (FDG-PET). This technique, when fused with CT (PET-CT), allows accelerated metabolic activity to be accurately anatomically localised and so is potentially useful for staging, assessment of treatment response, and for surgical and radiotherapy planning. In addition, quantitative MRI techniques have been proved to be valuable in intracranial tumours, but no such role has been validated in abdominal disease. Diffusion-weighted imaging with calculation of ADC maps is feasible in abdominal tumours, and our own preliminary data suggest that tissue cellularity is an important determinant of ADC value, which might help in terms of early prediction of therapy response.     

Positron emission tomography/computed tomography with 18fluoro-deoxyglucose in the detection of local recurrence and distant metastases of pediatric sarcoma

BACKGROUND: Combined positron emission tomography with (18)fluoro-deoxyglucose and computed tomography (FDG-PET/CT) has been used in the diagnosis and staging of various malignancies, but their use in the management of pediatric sarcomas is less well defined. The potential role of FDG-PET/CT in the diagnosis of local recurrence and distant metastases of pediatric sarcomas was investigated. PROCEDURE: Nineteen children (aged 2-21) with sarcoma (9 Ewing sarcoma, 3 osteogenic sarcoma, 7 rhabdomyosarcoma) were evaluated between January 2000 and December 2005 by FDG-PET/CT for suspected local relapse or distant metastases. The results of 21 FDG-PET studies, 16 CT scans, 9 magnetic resonance imaging (MRI) studies, and 7 bone scans (BSs) were compared with surgical pathology or clinical follow-up for at least 3 months. RESULTS: FDG-PET detected local relapse in all seven patients and distant metastases in 10/13 (77%). FDG-PET/CT and CT/MRI/BS results were discordant in eight patients. FDG-PET/CT was the only modality that detected distant metastases in two patients. PET/CT was true negative and excluded disease in three patients with abnormal CT/BSs and was false negative in three patients with distant metastases. CONCLUSION: FDG-PET/CT may be useful and complementary to other imaging modalities for the detection of recurrent pediatric sarcomas, especially at the primary site. Its potential advantages and limitations compared with conventional imaging modalities need to be further investigated in larger homogenous patient groups.     

Primary malignant liver tumors in childhood: Assessment of resectability with high-field MR and comparison with CT

Nine children (mean age 20 months), with proven primary malignant hepatic tumors have been examined prospectively by high-field magnetic resonance (MR) imaging to assess tumor resectability. All patients had comparative ultrasonography (US), 8 patients had X-Ray computed tomography (CT), and surgical correlation was available in 8 patients. The hepatic and portal veins and the inferior vena cava were visualized in all patients on MR and in 4 of 8 patients on CT. MR accurately defined liver parenchymal involvement in all 8 patients who had surgical exploration. CT underestimated disease in 2 cases, and defined tumour margins less clearly than MR. MR identified abnormal extrahepatic tissue when present, but was unable to distinguish viable tumor from necrotic tumor or reactive nodes. High quality short TR/short TE spin echo images were obtained by combining cardiac triggering and signal averaging. Short TI inversion recovery images demonstrated tumor and lymphadenopathy most clearly. We conclude that MR is the imaging method of choice for the assessment of liver tumor resectability in children.Research Fellow supported by Action Research for the Crippled Child.Receives support from the Imperical Cancer Research Fund.     

Early recurrences of otitis media: reinfection or relapse?

In a prospective study, 36 (35%) of 103 patients had early recurrence of acute otitis media. We wished to identify risk factors for early recurrences (those recurring within 1 month of initial diagnosis) and to determine if the second episode was caused by the same pathogen (relapse) or a new organism (reinfection). When the same bacterial species was recovered in both episodes, Streptococcus pneumoniae were serotyped and Haemophilus influenzae were classified by biotypes and by electrophoretic pattern of the outer membrane proteins. Twenty-nine patients underwent tympanocentesis at the time of the recurrent episode. In 13, no pathogen was recovered either initially or at the time of recurrence. Twelve (75%) of the remaining 16 patients had reinfection; only four (25%) had relapse. Thus, early recurrences of acute otitis media were more often caused by a new organism. This finding suggests that underlying susceptibility to middle ear infection is important in the development of recurrent otitis media. Pediatricians should not assume that early recurrences are necessarily the result of failure of initial treatment. Tympanocentesis may be helpful in this setting to aid in choosing appropriate antibiotic therapy.     

Utility of 18-fluorodeoxyglucose positron emission tomography in children with relapsed/refractory leukemia

Abstract

Objective: Few data are available on the clinical significance of 18-fluorodeoxyglucose positron emission tomography (FDG-PET/CT) results in patients with leukemia. We investigated the utility of FDG-PET/CT at the time of relapsed/refractory disease in pediatric patients with leukemia. Methods: Medical records of 28 children with suspected leukemia progression or recurrence during/after chemotherapy or allogeneic stem cell transplantation (allo-SCT) were retrospectively reviewed to determine the utility of FDG-PET/CT. Results: Twenty-two of the 28 patients have documented abnormal imaging findings during clinical follow-up, while six had were interpreted as not demonstrating signal consistent with active leukemia. Of the 22 patients with abnormal FDG-PET/CT studies 14 were found to have FDG-PET/CT reported as consistent with active leukemia and increased leukemia blasts on bone marrow biopsy. Regarding the eight patients without positive FDG-PET/CT and proven leukemia relapse, four had discordant findings on FDG-PET/CT and biopsy, and four had FDG-PET/CT reported as infection. Mean maximum standardized uptake values (SUVmax) were significantly higher among patients whose FDG-PET/CT findings were positive for leukemia as opposed to infectious disease (p?<?.05). Mean SUVmax was also significantly higher among patients with multifocal lesions on FDG-PET/CT than among those with diffuse lesions (p?<?.05). Conclusions: The findings suggest that FDG-PET/CT may be a complementary imaging modality that could be combined with bone marrow examination to improve detection of subtle leukemic infiltration in children with suspected leukemia progression or recurrence after chemotherapy or allo-SCT.     

Pattern of recurrence in children with midline posterior fossa malignant neoplasms

BACKGROUND: Surveillance imaging of the brain and spinal neuraxis in patients with posterior fossa malignant tumors is commonly performed, with the assumption that early detection of tumor recurrence will improve outcome. However, the benefit of this imaging has not been proven. PURPOSE: To evaluate the usefulness of spinal surveillance imaging in children with nonmetastatic (at diagnosis, M0) posterior fossa ependymoma and medulloblastoma. MATERIALS AND METHODS: This retrospective study included 65 children (3 months to 16 years, mean 5.7 years) treated between 1985 and 1997 for ependymoma (22) and medulloblastoma (43). Medical records were reviewed for pathology and treatment data. Serial imaging of the head and spine was reviewed for evidence of tumor recurrence. RESULTS: Twenty-four patients (37 %) had tumor recurrence, including 13 with ependymoma and 11 with medulloblastoma. Of the 17/24 recurrent patients initially diagnosed as M0 (6 medulloblastoma and 11 ependymoma), 13 (76 %) had a cranial recurrence only, and 4 (24 %) presented with concomitant cranial and spinal recurrence. No M0 patient presented solely with spinal metastases at recurrence. CONCLUSION: This study suggests that spinal surveillance imaging in patients with posterior fossa ependymoma or medulloblastoma initially staged as M0 may not be useful, as these patients initially recur intracranially. Thus, until an intracranial recurrence is detected, these patients may be spared the time, expense and sedation risk necessary for spinal imaging.     

Late recurrence in children with Wilms' tumor

We aimed in this study to evaluate the clinical and radiological features of the late recurrence of Wilms' tumor in children. Among 553 children diagnosed with Wilms' tumor between 1972 and 2004, four cases were determined to be late recurrences. Clinical, histopathological parameters, treatment details, and outcomes of the patients were evaluated retrospectively. The ages of the patients at the time of diagnosis were 2, 5, 5, and 9 years and the male/female ratio was 1/3. Two patients had stage II disease and two had stage IV characteristics. Histopathological examination showed favorable histology in all of the patients. Initial treatment was surgery and chemotherapy, which included vincristine and actinomycin-D. Abdominal radiotherapy was performed in two patients. Recurrence times were 36, 41, 51, and 96 months. Local recurrence and lung metastasis were detected in two patients, local recurrence in one, and lung nodules in the fourth patient. At the time of relapse, the chemotherapy protocols were as follows: vincristine, actinomycin-D, adriamycin, and cyclophosphamide in two patients; vincristine, actinomycin-D, and epirubicin in one patient; and vincristine, actinomycin-D, and adriamycin in the last patient. In the cases with late local recurrence, one patient had a local spillage and one patient had regional lymph node involvement. Although the other patient had local spillage, regional lymph node involvement, and renal artery invasion, isolated lung recurrence was observed. Only one patient had progressive disease and is still under treatment, whereas the other patients died with disease. Major recurrence sites were both local and the lungs. All of the patients had regional features including spillage, regional lymph node involvement, and vascular or capsular involvement. Late recurrence in patients with Wilms' tumor is a poor prognostic factor and should be treated with an intensified regimen.