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Cerebellar mutism in children: report of six cases and potential mechanisms.

Cerebellar mutism is a rare finding associated with resection of posterior fossa tumors or cerebellar hemorrhages. We reviewed the medical records of six children, aged 6 to 12 years, who developed cerebellar mutism after resection of a posterior fossa mass or as a result of posterior fossa trauma. From 1989 to 1994, 210 children underwent posterior fossa resection at our institution, and four developed mutism (an incidence of 1.6%). All four patients had primitive neuroectodermal tumors. The fifth patient experienced trauma, and another patient had an arteriovenous malformation (AVM). In four children, hydrocephalus developed as a result of their tumor or AVM. Four developed cerebellar mutism 24 to 48 hours after surgery or trauma, and one developed cerebellar mutism 5 days after surgery, coincident with hydrocephalus. In one, mutism occurred after a second resection was performed for a recurrence of his posterior fossa tumor. Cerebellar mutism lasted 10 days in one patient and 2 to 8 weeks in the other four. Dysarthria was apparent in four patients during the recovery phase. We suggest trauma to the dentate nucleus and/or its outflow tract, the superior cerebellar peduncle, as a cause of reversible mutism. Because posterior fossa tumors are common in children, mutism should be recognized as an important side effect of surgery.

Brain Concussion↗

Outcome for children <4 years of age with malignant central nervous system tumors treated with high-dose chemotherapy and autologous stem cell rescue.

BACKGROUND: Children <4 years of age (yo) with malignant central nervous system (CNS) tumors have a dismal prognosis. In an attempt to delay or obviate radiation therapy (XRT) and improve outcome, our institution has treated children <4 yo with newly diagnosed malignant CNS tumors with high-dose chemotherapy (HDC) and autologous stem cell rescue (ASCR) followed by selective XRT. PROCEDURE: Fifteen children (age 4-38 months) with malignant CNS tumors have completed treatment with HDC/ASCR. All patients received three cycles of induction chemotherapy (cisplatin 3.5 mg/kg- day 0, cyclophosphamide 60 mg/kg- day 1 and 2, etoposide 2.5 mg/kg- day 0-2, vincristine 0.05 mg/kg, day 0, 7, 14) followed by three cycles of HDC (carboplatin 17 mg/kg and thiotepa 6 mg/kg, day 0 and 1) with ASCR. Histology included five medulloblastomas, four primitive neuroectodermal tumors (PNET), five malignant gliomas, and one ependymoma. Outcome and treatment toxicities were evaluated by retrospective chart review. RESULTS: Median follow-up time of the 15 patients is 22 months (range 8-82 months). The 1- and 2-year progression-free survival (PFS) is 86.1% and 52.2% and overall survival (OS) 91.6% and 72.1%, respectively. Ten patients are alive and disease free 3-77 months (median 18 months) after having completed HDC/ASCR, thereoff five received XRT. Toxicity was primarily myelosuppression. There was no treatment mortality. CONCLUSIONS: We are encouraged by the outcome of 15 children <4 yo with malignant CNS tumors treated with tandem cycles of HDC and ASCR at our institution. The treatment regimen is relatively well tolerated.

Antineoplastic Combined Chemotherapy Protocols↗

Association of a suprasellar mass and intraocular retinoblastoma: a variant of pineal trilateral retinoblastoma?

OBJECTIVE AND IMPORTANCE: 'Trilateral retinoblastoma' designates the association of bilateral retinoblastomas with an ectopic (non-metastatic) intracranial primitive neuroectodermal tumor (PNET). Although the intracranial tumor, usually located in the pineal region, is seen 2-3 years after discovery of the bilateral ocular tumors, variant presentations do occur. The intraocular tumor can be unilateral, and the intracranial tumor can be suprasellar, presenting before the retinal lesions. CLINICAL PRESENTATION: A 4-month-old boy presented with irritability, vomiting and nystagmus. Head CT revealed a large calcified sellar-suprasellar mass. Ocular examination disclosed white macular lesions in the right fundus. He was taken to surgery where subtotal resection of the sellar-suprasellar mass was achieved. Microscopic examination showed a primitive, small, round, blue cell tumor consistent with retinoblastoma. The right ocular lesions, also consistent with retinoblastoma, were treated by cryosurgery. CONCLUSION: The appearance of a suprasellar PNET can precede the appearance of retinal masses in retinoblastoma. The literature suggests that patients with 'sellar' trilateral retinoblastoma have characteristics that differ from patients with a pineal region trilateral retinoblastoma: the intracranial mass more often presents initially; it occurs at a younger age; it is predominant in females, and is more often associated with unilateral ocular lesions. Retinal screening of patients with suprasellar PNET and other atypically located PNETs may disclose more cases of sellar 'trilateral retinoblastoma' and hereditary retinoblastomas. This will have implications on genetic counseling as siblings harboring the disease may be diagnosed and treated at an earlier stage.

Brain Neoplasms↗

Foreign body reaction to hemostatic materials mimicking recurrent brain tumor. Report of three cases.

Chemical agents routinely used in neurosurgery to achieve intraoperative hemostasis can cause a foreign body reaction, which appears on magnetic resonance (MR) images to be indistinguishable from recurrent tumor. Clinical and/or imaging evidence of progression of disease early after surgical resection or during aggressive treatment may actually be distinct features of granuloma in these circumstances. A series of three cases was retrospectively analyzed for clinical, imaging, surgical, and pathological findings, and the consequences they held for further disease management. All patients were boys (3, 3, and 6 years of age, respectively) and all harbored primitive neuroectodermal tumors. Two tumors were located in the posterior fossa and one was located in the right parietal lobe. Two boys exhibited clinical symptoms, which were unexpected under the circumstances and prompted new imaging studies. One patient was asymptomatic and imaging was performed at planned routine time intervals. The MR images revealed circumscribed, streaky enhancement in the resection cavity that was suggestive of recurrent disease. This occurred 2 to 7 months after the first surgery. At repeated surgery, the resected material had the macroscopic appearance of gelatin sponge in one case and firm scar tissue in the other cases. Histological analysis revealed foreign body granulomas in the resected material, with Gelfoam or Surgicel as the underlying cause. No recurrent tumor was found and the second surgery resulted in imaging-confirmed complete resection in all three patients. Because recurrent disease was absent, the patients continued to participate in their original treatment protocols. All patients remain free from disease 34, 32, and 19 months after the first operation, respectively. During or after treatment for a central nervous system neoplasm, if unexpected clinical or imaging evidence of recurrence is found, a second-look operation may be necessary to determine the true nature of the findings. If the resection yields recurrent tumor, additional appropriate oncological treatment is warranted, but if a foreign body reaction is found, potentially harmful therapy can be withheld or postponed.

Brain↗

Peripheral neuroepithelioma with ganglion cells: report of two cases and review of the literature.

Peripheral neuroepitheliomas, also known as peripheral primitive neuroectodermal tumors, are by definition primitive embryonal lesions generally composed of poorly differentiated neuroectodermal elements. We have examined two cases that paradoxically contain extensive foci of ganglionic differentiation similar to that of ganglioneuroblastoma, in addition to primitive elements. One tumor arose from the chest wall of an 8-year-old male and the other from the abdominal wall of a 15-year-old male. Differentiation into a mature ganglionic phenotype was confirmed by immunohistochemistry in one case. Rare peripheral neuroepitheliomas have a capacity for maturation that is not generally appreciated. These lesions should not be confused with ganglioneuroblastomas, which are genotypically unrelated neoplasms.

Adolescent↗

Prolonged cerebral salt wasting syndrome associated with the intraventricular dissemination of brain tumors. Report of two cases and review of the literature.

Hyponatremia is a frequent event in neurosurgery practice and is usually associated with subarachnoid hemorrhage, head trauma, infections and neoplasms. The two common clinical manifestations are the inappropriate secretion of antidiuretic hormone (SIADH) and the cerebral salt wasting syndrome (CSWS), which were usually attributed to each other due to identical clinical presentation. In contrast to the better-recognized SIADH, there has not been a uniform consensus over the humoral and neural mechanisms of CSWS and functional aspects of renal response. In this article, we report on 2 cases of a primitive neuroectodermal tumor with prolonged CSWS manifested during the intraventricular dissemination of primary disease and the high catabolic stage.

Brain Diseases, Metabolic↗

Molecular analysis of PinX1 in medulloblastomas.

Our group has previously demonstrated a high frequency of allelic loss on the short arm of chromosome 8 and identified a region of homozygous deletion of 1.41 Mb, flanked by D8S520 and D8S1130, on 8p23.1 in medulloblastomas, suggesting the presence of a tumor suppressor gene in this critical deletion region. The aim of our study was to investigate whether PinX1, a newly identified gene whose product is a potent inhibitor of telomerase, is the target gene in the homozygous deletion region identified in medulloblastomas. We assessed for alterations in gene sequence and transcript expression of PinX1, as well as the correlation between PinX1 expression and telomerase activity in a series of 52 medulloblastomas, 3 medulloblastoma cell lines (D283, D341 and Daoy) and 4 primitive neuroectodermal tumors (PNETs). Direct sequence analysis of all 7 exons and splice junctions of the PinX1 gene revealed no somatic mutations but 11 genetic polymorphisms. Transcript expression of PinX1, as evaluated by reverse transcription-polymerase chain reaction, in microdissected tumors and normal cerebellum showed 2 transcript variants, corresponding to the full-length form and an alternative spliced variant lacking exon 6, in all samples. This result indicated that PinX1 expression was not suppressed in medulloblastomas. Using the telomeric repeat amplification protocol (TRAP) assay, 13 of 19 (68%) medulloblastomas, 1 of 2 PNETs and all 3 cell lines showed telomerase activity. There is no significant correlation between PinX1 transcript expression and telomerase activity, but our results showed that telomerase activation is involved in medulloblastomas. Taken together, our results suggest that PinX1 does not play a major role in the oncogenesis of medulloblastomas.

Adolescent↗

Immunohistology, cytogenetics, and molecular studies of small round cell tumors of childhood. A review.

Malignancies of childhood include a well-defined spectrum of hematolymphoid, organ specific (adrenal, kidney, liver), soft tissue, bone, and nervous system (central and peripheral) neoplasms with variable biology. Small round cell neoplasms, a subset of childhood malignancies, are histologically similar but differ markedly in their histogenesis, therapy, and prognosis. Traditionally, clinical information and light microscopy, with the aid of histochemistry and ultrastructural evaluation, establish a diagnosis or at least narrow the differential diagnosis. Additionally, immunohistology, cytogenetics, and molecular studies have become important in diagnosis and in defining phenotype/genotype, patient treatment modalities, and prognosis in specific cases. The 11;22 chromosomal translocation typifies Ewing's sarcoma, primitive neuroectodermal tumor, and Askin's tumor, as does the resultant chimeric transcript, while expression and amplification of N-myc oncogene are predictive of the prognosis in neuroblastoma. Furthermore, studies of genes and gene products are elucidating mechanisms of oncogenesis and tumor progression.

Child↗

Pediatric neuroblastic brain tumors containing abundant neuropil and true rosettes.

We have encountered a series of seven unusual neuroblastic pediatric central nervous system (CNS) neoplasms with a unique constellation of histologic, immunohistochemical, and ultrastructural features. The tumors presented in five girls and two boys, ages 1 to 3 years. In six cases the lesions involved the frontoparietal region, in one case the tectal plate. The tumors consisted of small to medium-sized, round to oval, hyperchromatic cells with poorly defined cytoplasmic borders. Cells were found in clusters and cords set in a paucicellular fibrillar neuropil matrix. Distinctive, virtually anuclear regions of neuropil were scattered throughout the lesions. True rosettes with well-formed central lumens often filled with granular debris were present, along with perivascular pseudorosettes and occasional Homer-Wright rosettes. Mitoses and apoptosis were frequent, but large regions of confluent necrosis were absent. Immunohistochemically, the neuropil-like areas as well as the perinuclear cytoplasm of many embryonal tumor cells were positive for synaptophysin and neurofilament protein. Ultrastructurally, the tumor cells showed microtubule-containing neuronal processes, some with neurosecretory granules. While the lesions were largely glial fibrillary acidic protein (GFAP) negative, there was focal GFAP positivity consistent with divergent differentiation in one case. The clinical outcome was poor, with five patients dead from their disease 5 to 14 months after initial presentation and one patient with recurrent disease 7 months after resection and chemotherapy. The final patient is alive without recurrent disease 30 months after initial presentation. These lesions present distinctive histological features within the group of primitive neuroectodermal tumors.

Apoptosis↗

Parental occupations and childhood brain tumors: results of an international case-control study.

OBJECTIVE: To evaluate the role of parental occupations in the etiology of childhood brain tumors (CBT). METHODS: Population-based case-control studies were conducted concurrently in seven countries under the coordination of the International Agency for Research on Cancer, gathering 1,218 cases and 2,223 controls. We report here the findings related to parental occupations during the 5-year period before the child's birth. Risk estimates related to a number of paternal and maternal occupations were obtained by unconditional logistic regression adjusted for age, sex, year of birth, and center, for all types of CBT combined and for the subgroups of astroglial, primitive neuroectodermal tumors (PNET), and other glial tumors. RESULTS: An increased risk in relation with agricultural work was seen for all CBT combined and for other glial tumors. Increased risks for all tumors and PNET were seen for paternal occupation as an electrician; the same pattern held for maternal occupation when children under 5 were selected. Paternal occupation as a driver or mechanic, and maternal work in an environment related to motor-vehicles were associated with an increased risk for all CBT and astroglial tumors. More case mothers compared to control mothers were employed in the textile industry. CONCLUSION: Our study reinforces previous findings relative to the role of parental work in agriculture, electricity, or motor-vehicle related occupations and maternal work in the textile industry. It does not confirm previous associations with work environments including aerospace, the chemical industry, or the food industry, or with maternal occupation as a hairdresser, a nurse, or a sewing machinist, and paternal occupation as a welder.

Adolescent↗

Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?

Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.

Adult↗

Nasal CD56 positive small round cell tumors. Differential diagnosis of hematological, neurogenic, and myogenic neoplasms.

CD56-positive nasal and nasal-type natural killer (NK)/T-cell lymphoma is now a well-defined disease entity. Rare cases of blastic NK-cell lymphoma positive for CD56 have been recently reported. However, CD56 expression is also identified in several types of non-hematopoietic small round cell tumors in which lymphoma is included as a differential consideration. Here, we present nine cases of CD56+ small round cell tumors of histological origin unrelated to nasal NK/T-cell lymphoma. Eight of the nine cases presented as solid tumors of the sinonasal region. Clinical, histological, ultrastructural, and immunohistochemical examination and gene analysis for T-cell receptor (TcR) and immunoglobulin heavy chain (IgH) genes and in situ hybridization (ISH) for Epstein-Barr virus (EBV) were performed. Two cases presented with features consistent with blastic NK-cell lymphoma or lymphoblastic lymphoma of NK-cell phenotype. These cases showed features of lymphoblastic lymphoma, phenotypes of sCD3-, cCD3+, CD45+, CD56+, TdT+, and human leukocyte antigen (HLA)-DR+, germline of IgH and TcR genes, and EBV negative reactivity. One case had myeloid/NK-precursor acute leukemia/lymphoma with a phenotype of CD13+, CD33+, CD34+, CD56+, and MPO-. Three cases were neurogenic, including one case of olfactory neuroblastoma and two of primitive neuroectodermal tumors (PNET). It was difficult to differentiate CD56+ PNET from blastic NK-cell lymphoma, especially when only paraffin-embedded sections were available. Myogenic markers, such as HHF35, alpha-sarcomeric actin, and desmin, were positive in three cases of rhabdomyosarcomas. Our findings suggest that as CD56 is used more routinely as a marker in immunohistochemical staining, the differential diagnosis of extranodal lymphohematological malignancies and small round cell tumors will become more complicated.

Adolescent↗

Risk of brain tumors in children and susceptibility to organophosphorus insecticides: the potential role of paraoxonase (PON1).

Prior research suggests that childhood brain tumors (CBTs) may be associated with exposure to pesticides. Organophosphorus insecticides (OPs) target the developing nervous system, and until recently, the most common residential insecticides were chlorpyrifos and diazinon, two OPs metabolized in the body through the cytochrome P450/paraoxonase 1 (PON1) pathway. To investigate whether two common PON1 polymorphisms, C-108T and Q192R, are associated with CBT occurrence, we conducted a population-based study of 66 cases and 236 controls using DNA from neonatal screening archive specimens in Washington State, linked to interview data. The risk of CBT was nonsignificantly increased in relation to the inefficient PON1 promoter allele [per PON1(-108T) allele, relative to PON1(-108CC): odds ratio (OR) = 1.4; 95% confidence interval (CI), 1.0-2.2; p-value for trend = 0.07]. Notably, this association was strongest and statistically significant among children whose mothers reported chemical treatment of the home for pests during pregnancy or childhood (per PON1(-108T) allele: among exposed, OR = 2.6; 95% CI, 1.2-5.5; among unexposed, OR = 0.9; 95% CI, 0.5-1.6) and for primitive neuroectodermal tumors (per PON1(-108T) allele: OR = 2.4; 95% CI, 1.1-5.4). The Q192R polymorphism, which alters the structure of PON1 and influences enzyme activity in a substrate-dependent manner, was not associated with CBT risk, nor was the PON1(C-108T/Q192R) haplotype. These results are consistent with an inverse association between PON1 levels and CBT occurrence, perhaps because of PON1's ability to detoxify OPs common in children's environments. Larger studies that measure plasma PON1 levels and incorporate more accurate estimates of pesticide exposure will be required to confirm these observations.

Aryldialkylphosphatase↗

Expression of pericyte, mesangium and muscle markers in malignant rhabdoid tumor cell lines: differentiation-induction using 5-azacytidine.

Malignant rhabdoid tumor (MRT) has been considered to have multiphenotypic diversity characteristics. Some MRTs exhibit a neural phenotype. However, it is still unclear whether MRT cells can display a skeletal muscle, smooth muscle or smooth muscle-like cell phenotype, like those of pericytes and mesangial cells. To determine if MRTs exhibit skeletal muscle cell or smooth muscle-like cell phenotypes, six MRT cell lines (TM87-16, STM91-01, TTC549, TTC642, YAM-RTK1 and TTC1240) were examined for markers of skeletal muscle (MyoD, myogenin, myf-5, myf-6, acetylcholine receptor-alpha, -beta and -gamma), smooth muscle (alpha-smooth muscle actin, SM-1 and SM22), and smooth muscle-like cells, such as pericytes (angiopoietin-1 and -2) and mesangial cells (megsin), using conventional RT-PCR, semi-quantitative PCR, western blotting and immunocytochemistry before and after differentiation-induction with 5-azacytidine. alpha-Smooth muscle actin and SM22 were detected in all six MRT cell lines, while MyoD and myf-5, crucial markers for skeletal myogenic determination, were not. The TM87-16 cell line expressed SM-1 and angiopoietin-1. TTC1240 also expressed angiopoietin-1. Interestingly, STM91-01 expressed megsin, a novel marker for mesangial cells, in addition to angiopoietin-1. Our results indicated that some MRTs exhibited smooth muscle and/or smooth muscle-like cell phenotypes and some renal MRTs might be of mesangial origin. Recently, smooth muscle and also smooth muscle-like cells have been considered to be of neuroectodermal origin. MRT can thus considered to belong to the category of primitive neuroectodermal tumors (PNETs) in the broad sense.

Antimetabolites, Antineoplastic↗

Italian Cooperative Study for the treatment of children and young adults with localized Ewing sarcoma of bone: a preliminary report of 6 years of experience.

BACKGROUND: In 1991, the Italian Association for Pediatric Hematology-Oncology and the National Council of Research (CNR) initiated an Italian Cooperative Study (SE 91-CNR Protocol) with the main objective of improving the overall survival (SUR) and the event free survival (EFS) of children and young adults with localized Ewing sarcoma and primitive neuroectodermal tumors of bone compared with a previous study (IOR/Ew2 Protocol). METHODS: Between November 1991 and November 1997, 165 patients were enrolled in this study, 160 of whom were evaluable. The patients were treated with a multimodal approach characterized by intensified chemotherapy, hyperfractionated and accelerated radiation therapy, and the addition of ifosfamide and etoposide to standard chemotherapy with vincristine, actinomycin-D, doxorubicin, and cyclophosphamide. RESULTS: After a median follow-up of 37 months, 126 of the 160 evaluable patients remained free of disease recurrence. Thirty-one patients developed a disease recurrence (20 with disseminated disease). CONCLUSIONS: The 3-year SUR and EFS rates found in the current study (83.6% and 77.8%, respectively) may be considered satisfactory. Only age at diagnosis < or =14 years and a good histologic response appeared to affect the outcome of patients with localized Ewing sarcoma positively. These results appear to demonstrate the efficacy of the addition of ifosfamide in induction chemotherapy to four-drug standard combination chemotherapy, as confirmed by the improved outcome in terms of 3-year EFS reported in the SE 91-CNR Protocol compared with the IOR/Ew2 Protocol (77.8% vs. 60.7%). In addition, the better outcome also could be explained by the change in treatment strategy with a trend toward the use of more surgery than radiation therapy compared with the authors' previous protocol.

Adolescent↗

Peripheral neuroepithelioma: a light and electron microscopic study.

A primitive neuroectodermal tumor of peripheral nerve origin was examined by light and electron microscopy. The lesion arose in association with the S1 nerve root of the lumbosacral plexus in a 22-year-old female. Following a surgical biopsy, the patient received systemic chemotherapy; however, approximately one-and-a-half years later, she suffered a clinical relapse with locally recurrent tumor. An excisional biopsy was performed, and pathologic examination revealed progressive neuroblastic differentiation. The ultrastructural features of neoplastic Homer-Wright rosettes will be illustrated, and the non-specificity of cytoplasmic glycogen in the evaluation of "small round cell neoplasms" will be emphasized.

Adult↗

Establishing a germ cell origin for metastatic tumors using OCT4 immunohistochemistry.

BACKGROUND: There are diverse morphologic manifestations of metastatic tumors. The determination of tumor origin is critical for patient management, and it is especially important when the differential diagnosis includes metastatic germ cell tumor, which is a highly treatable condition. OCT4 is a nuclear transcription factor that is expressed in pluripotent embryonic germ cells. In this study, the author sought to determine the usefulness of OCT4 immunohistochemistry in the diagnosis of metastatic germ cell tumors. METHODS: Sixty-two retroperitoneal lymph node dissection specimens from patients with histories of testicular germ cell tumors were stained using the antibodies against OCT4. In addition, 84 metastatic, nongerm cell lesions from men with known primary tumors were studied in parallel for OCT4 immunohistochemistry. RESULTS: All embryonal carcinoma components (n = 29 specimens) and seminoma components (n = 18 specimens) from retroperitoneal lymph node dissection specimens showed strong, intense, diffuse nuclear staining for OCT4. Yolk sac tumors (n = 12 tumors), choriocarcinomas (n = 4 tumors), mature teratomas (n = 16 tumors), and primitive neuroectodermal tumors (n = 5 tumors) were negative for OCT4 staining. Metastatic, nonsmall cell carcinomas from the lung (n = 14 tumors), colon (n = 12 tumors), stomach (n = 5 tumors), pancreas (n = 7 tumors), prostate (n = 12 tumors), kidney (n = 3 tumors), and urinary bladder (n = 15 tumors) all were found to be negative immunohistochemically for OCT4, as were metastatic small cell carcinomas (n = 4 tumors) and metastatic melanomas (n = 7 tumors). In addition, malignant lymphomas (n = 5 tumors) also were negative for OCT4. CONCLUSIONS: Immunohistochemical detection of OCT4 is highly sensitive and specific for the diagnosis of seminoma and embryonal carcinoma metastatic from the testis. Establishing germ cell origin for metastatic tumors has important implications for assessing patient prognosis and treatment options.

Biomarkers, Tumor↗

Role of spinal MRI in the follow-up of children treated for medulloblastoma.

BACKGROUND: The purpose of the current study was to describe the usefulness of spinal magnetic resonance imaging (MRI) in children with medulloblastoma or primitive neuroectodermal tumor (PNET) of the posterior fossa. METHODS: Children consecutively diagnosed with medulloblastoma/PNET and followed in the Hospital for Sick Children/Toronto were identified. A homogenous cohort of children treated with craniospinal irradiation as part of their initial treatment was considered. Contrast-enhanced spinal MRIs done concomitantly with cranial MRIs (doublets) were reviewed. Recurrence was defined as any new abnormal lesion (in the brain or in the spine) in symptomatic or asymptomatic patients. Doublets after the first recurrence were excluded in the final analysis. The utility of a spinal MRI in the presence of a negative cranial MRI was assessed. RESULTS: In all, 73 patients (21 females and 52 males; median age, 6.6 years, median follow-up time, 4.3 years) had at least 1 evaluable doublet during the follow-up period. Since concomitant cranial and spinal MRI was introduced as the standard evaluation for medulloblastoma/PNET in 1991, 286 doublets were evaluable. Fourteen spinal MRIs and 25 cranial MRIs showed new nodular or leptomeningeal lesions. In 2 patients, repeat MRIs ruled out recurrence (false-positive). All confirmed spinal recurrences were associated with intracranial recurrence. Of 261 doublets with negative cranial MRI, no new lesion was identified on spinal MRI. CONCLUSIONS: An absence of progression on cranial MRI is highly predictive of absence of progression on spinal MRI. There is little evidence that surveillance spinal MRI (in children who underwent craniospinal radiation as part of their initial treatment) improves the detection of recurrences in children with medulloblastoma.

Adolescent↗