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Surgical treatment of primary pulmonary sarcomas.

OBJECTIVE: We sought to identify the long-term prognosis after surgical treatment for primary pulmonary sarcoma. METHODS: Twenty-three patients were retrospectively identified as having been treated surgically for primary pulmonary sarcoma between 1981 and 1996. The records of all patients were reviewed, and the histopathology reexamined by a pathologist. RESULTS: Fifteen patients were male and eight female; their ages ranged from 20 to 78 (mean 51) years. Tumors measured between 0.9 and 12.0 (mean 5.2) cm across the greatest diameter. The histologic diagnoses were malignant fibrous histiocytoma (8, three grade 1 or 2, two grade 3), synovial sarcoma (4), malignant schwannoma (3), leiomyosarcoma (3), and one case each of angiosarcoma, intimal sarcoma, epitheloid hemangioendothelioma, fibrosarcoma and primitive neuroectodermal tumor. Three patients were found to be unresectable. All three underwent radiation and chemotherapy. Lobectomies or bilobectomies were performed in 13 patients including two sleeve resections, one carinal resection, and one chest wall resection. Four patients underwent radical pneumonectomies. Three patients with invasion of the pulmonary artery, pulmonary veins or atrial wall underwent extended resections with the use of cardiopulmonary bypass. In two, a homograft was used to reconstruct the right ventricular outflow tract. Of the resected patients, six had a positive resection margin, and four had at least one positive lymph node in the specimen. Three patients underwent repeat pulmonary resections for recurrences. Eleven patients received postoperative chemotherapy and eight had radiation therapy. Follow-up was available on 22 patients, and ranged from 2 to 183 (mean 48) months; 14 patients are disease free, six died of disease, one died of surgical complications (operative mortality 5%), and two are alive with disease. Actuarial 3- and 5-year survival of the resected patients was 69%. Size and grade were not found to be correlated with significantly increased survival, but completeness of resection was (P<0.05). CONCLUSIONS: Resection of primary pulmonary sarcomas can produce an acceptable survival rate if the resection is complete. Cardiopulmonary bypass can be a useful adjunct when tumors involve a resectable area of the heart or great vessels.

Adult↗

The specificity of ultrasound in the detection of fetal intracranial tumors.

AIMS: To evaluate the specificity of ultrasound in identifying fetal brain neoplasms and its accuracy in the diagnosis of the tumor's histological type. METHODS: A retrospective evaluation of 7 cases of fetal brain tumors occurred at our unit in the period between January 92 and June 98 has been performed. All prenatal ultrasonographic diagnoses were compared with the postnatal findings on the aborted fetuses (post-mortem examination) or on the newborns (MRI or post-mortem examination). RESULTS: In 6 out of 7 cases the antenatal ultrasonographic diagnosis of brain tumor was confirmed postnatally, in one case a supratentorial arachnoid cyst was mistaken for a teratoma with cystic components (86% specificity in the diagnosis of congenital brain neoplasms). Out of the 6 cases of suspected teratomas, one revealed to be a glioblastoma, one an arachnoid cyst and one a primitive neuroectodermal tumor. The prenatal diagnosis was confirmed in the case of choroid plexus papilloma. Prenatal ultrasonography was accurate in identifying the tumor's histological type in the 57% of the cases. The diagnoses were not modified by the transvaginal scans performed in 2 cases. CONCLUSIONS: Prenatal ultrasonography is a useful tool to identify any intracranial space-occupying lesion larger than 10 mm. In the present study it has shown an 86% specificity in the diagnosis of brain neoplasms. However the accuracy of ultrasound in diagnosing the tumor's histological type was limited (57%).

Brain Neoplasms↗

Increasing incidence of childhood cancer: report of 20 years experience from the greater Delaware Valley Pediatric Tumor Registry.

As little is known about the aetiology of cancer in children, analysis of time trends may be useful. Recent data on time trends for paediatric cancers are very limited. We report here on trends in the incidence of 15 categories of cancer in children under 15 years of age from 1970 to 1989, using data from the Greater Delaware Valley Pediatric Tumor Registry in the US. Total cancer incidence increased 1% per year (P < 0.001). Neither acute lymphocytic leukaemia, acute myelocytic leukaemia, nor total leukaemia incidence changed significantly. In contrast, the incidence of central nervous system (CNS) tumours rose 2.7% per year (P < 0.001). All three subgroups of this category, glioma, primitive neuroectodermal tumor (PNET)/medulloblastoma, and other CNS tumours, showed increases. For glioma and PNET/medulloblastoma, trends differed by age, race, and/or gender. Among the other childhood cancers, significant increases were observed for non-Hodgkin lymphoma and neuroblastoma. For osteosarcoma and retinoblastoma, no overall change in incidence was observed, although decreases were observed in some age and race subgroups. The rise in CNS tumour incidence confirms previous reports from the US and Great Britain. The lack of change for acute lymphocytic leukaemia conflicts with other data from the US, but diagnostic changes appear to explain at least part of the discrepancy. The increase in neuroblastoma has also been observed in Great Britain. In contrast to our finding, investigators in the US and Great Britain have reported no rise in non-Hodgkin lymphoma. Analyses for more of the childhood cancers from other registries would aid in detecting and interpreting incidence trends in recent years.

Adolescent↗

Studies of childhood brain tumors using immunohistochemistry and microwave technology: methodological considerations.

The immunohistochemical detection of antigens in archival tissue sections has been hampered by the poor reactivity of certain polypeptides in conventional formalin-fixed, paraffin-embedded material. For example, the poor reactivity of neurofilament proteins (NFPs) in surgical and autopsy specimens has been a major drawback of previous large, retrospective, clinicopathologic studies of pediatric primitive neuroectodermal tumors (PNETs), also known as medulloblastomas. We report our experience with a method of antigen retrieval which greatly enhanced the immunohistochemical detection of neuronal and glial intermediate filament proteins, retinal S-antigen (RSA), and the proliferating cell nuclear antigen (PCNA) in archival, paraffin-embedded, formalin or Bouin's-fixed, pediatric brain tumors, particularly PNETs. The technique involves adding a single brief step to an established avidin-biotin complex (ABC) immunohistochemical protocol (Vectastain Elite Kit). This step involves boiling tissue sections in distilled water for 5 min in a microwave oven. The specificity of staining was consistent with known cell and tissue specificities of the well-characterized monoclonal antibodies used and there was minimal background. Synaptophysin (SYN) staining was unaffected by heating and immunoreactivity of the low affinity nerve growth factor (p75NGFR) and the neural cell adhesion molecule (NCAM) were lost. The enhanced detection of neuronal and glial antigens in routinely prepared, formalin-fixed, archival material should facilitate large retrospective clinicopathologic studies designed to assess the prognostic implications of differentiation in PNETs and to better understand the biology of these tumors.

Adult↗

Oncogene complementation in fetal brain transplants.

Using a neural transplantation model and retrovirus-mediated gene transfer, we have introduced the oncogenes v-Ha-ras and v-myc into the developing rat brain. Upon insertion of a construct encoding v-Ha-ras and the Escherichia coli beta-galactosidase marker gene, the retroviral vector was found to be expressed in neurons, astrocytes, and endothelial cells of the graft. After latency periods of several months, fascicular neoplasms with expression of S-100 protein were observed in 50% of the transplants. The foreign genes were shown to be highly expressed in the tumors and in intact donor cells, by 5-bromo-4-chloro-3-indolyl-beta-D-galactopyranoside histochemistry, indicating that an activated Ha-ras oncogene has the potential to initiate neoplastic transformation of glial cells. Introduction of the v-myc oncogene into 15 grafts resulted in only a single primitive neuroectodermal tumor. However, simultaneous expression of the v-Ha-ras and v-myc genes yielded highly malignant, polyclonal neoplasms in all recipient animals, as early as 13 days after transplantation, from which cell lines could be easily derived. In addition, neoplastic transformation was also observed in vitro following introduction of ras and myc into embryonic forebrain cultures and into newborn cerebellar cultures. These data indicate a powerful complementary transforming effect of ras and myc on neural progenitors in vivo and in vitro. Coexpression of ras and myc may, therefore, provide a highly efficient tool for transforming neural precursor cells in distinct segments of the central nervous system at different stages of development.

Animals↗

Primary CNS malignant rhabdoid tumor (MRT): report of two cases and review of literature.

Primary CNS rhabdoid tumor is an enigmatic and extremely rare malignant tumor of early childhood, probably embryonal, that has often been histopathologically classified together with rhabdoid tumor of kidneys or other organs in infants. Only four previously documented cases of primary CNS malignant rhabdoid tumor (MRT) were found in the literature. We report two cases of primary CNS MRT with biopsy and complete autopsy findings that share close clinicopathologic, immunohistochemical and ultrastructural similarities between the two. It is concluded that the primary CNS MRT is an entity which is extremely malignant, easily mistaken as a primitive neuroectodermal tumor, and potentially derives from a meningothelial precursor cell which is embryonally equal to the serosal mesothelial precursor cells which surround the kidneys and other organs. Hence, it tends to anatomically occur at the location of abundant meningeal infoldings such as the cerebellar cortex and may be diffuse or multicentric in its meningeal involvement. Furthermore, it may concurrently or multicentrically occur in association with MRT originating from the serosal membrane of other organs, such as the kidney.

Biomarkers, Tumor↗

In vitro and in vivo cytotoxicity of gossypol against central nervous system tumor cell lines.

Gossypol is a lipid soluble polyphenolic compound isolated from cotton seed oil which has been previously shown to have antiproliferative activity in vitro against a variety of human solid tumor cell lines. It has been extensively tested in clinical trials as a male contraceptive agent and found to be well tolerated. Its mechanism of action is thought to be inhibition of cellular energy metabolism. It inhibits glycolysis through inhibition of LDH isoenzyme type 5, and it inhibits mitochondrial oxidative phosphorylation and electron transport. We tested the in vitro antiproliferative effect of gossypol against four well characterized human glioma cell lines, HS 683, U373, U87 and U138, and one rat glioma cell line, C6, using the colorimetric Microculture Tetrazolium Assay (MTT). Cytotoxicity was found to be concentration and time dependent and increased with incubation times up to 8 days. The relative sensitivity of the glioma cell lines to gossypol at 48 hour incubation correlated with their respective LDH isoenzyme profiles, with the more sensitive cell lines expressing increased cathodal LDH isoenzymes (LDH5). The in vitro cytotoxicity of gossypol to these CNS tumor lines was compared to the other non central nervous system solid tumor cell lines which had been previously reported as being sensitive to gossypol, including SW-13 (adrenal), MCF-7 (breast), T47-D (breast), and HeLa (cervical). Additional lines tested included SK-MEL-3 (melanoma), Colo 201 (colon) and BRW, a line established in our laboratory from a patient with a Primitive Neuroectodermal tumor. C6, HS 683, and BRW had similar IC50s as the sensitive solid tumor cell lines. U373, U87 and U138 had significantly less sensitivity at 48 hours. There was greater cytotoxicity and no significant differences in the IC50s between any of cell lines at 8 day incubations. Additionally, we tested the cytotoxicity of gossypol against BRW in vivo, using the nude mouse xenograft model. Gossypol, given at a dose of 30 mg/kg per day five days a week for four weeks orally via gavage, was found to decrease the mean tumor weight of treated xenografts by more than 50% as compared to untreated xenografts. These findings suggest that gossypol has potential for further study as an agent for the treatment of primary CNS malignancies.

Animals↗

High-dose chemotherapy in childhood brain tumors.

Early attempts to use high-dose chemotherapy technology in order to improve the effect of nitrosourea on high-grade gliomas resulted in minimal benefit as well as in severe toxicity. Since then, other drugs have been applied in conjunction with either autologous bone marrow or peripheral blood stem cells, including thiotepa, etoposide, melphalan, cyclophosphamide, and busulfan. The data suggest benefit in recurrent primitive neuroectodermal tumors (PNET), in newly diagnosed young children with PNET and possibly in young children with newly diagnosed ependymoma, as a strategy not only to improve tumor-free survival but also to avoid exposure of the young brain to irradiation. In other tumors such as recurrent ependymoma and newly diagnosed or recurrent brain stem glioma, high-dose chemotherapy remains ineffective. New protocols under evaluation include new agents, multiple cycles of high-dose chemotherapy and allogeneic transplantation as immunotherapeutic approach.

Antineoplastic Agents↗

A modern analysis of intracranial tumors of infancy.

OBJECTIVE: The management results of pediatric brain tumors should have improved with progress in neurosurgery, intra- and perioperative care, and adjunctive therapy. We assessed the outcomes of 88 consecutive children under 2 years of age with brain tumors managed in the modern era, primarily by two neurosurgeons, and compared the results within the study group over time and to historical controls. METHODS: Medical records were reviewed for diagnosis, location, surgery, surgical morbidity and mortality, adjunctive therapy, and long-term results. Outcomes were available on all 88 patients. RESULTS: Primitive neuroectodermal tumors, astrocytomas, ependymoma, and choroid plexus papillomas in descending order of frequency accounted for two thirds of tumors. Supratentorial location predominated (60%), although in the 1- to 2-year group, there was a slight majority of infratentorial tumors. Surgical mortality and immediate morbidity were 9 and 26%, respectively, with substantial improvements in the last half of the series (2 and 16% with long-term morbidity of 11%). 53 of 88 (60%) of our patients are alive, and all children treated since January 1, 1994, except for 1 operative death, remain alive. CONCLUSION: Children less than 2 years of age remain a multidisciplinary challenge. Improved neuroimaging, surgical and pediatric intensive care management, and neuro-oncological care seems to have improved outcome both with respect to tumor control and neurological function. Aggressive surgery is possible with a good outcome generally expected. Hopefully this will set the stage for either surgical cure in children with benign tumors or combined surgical and adjunctive cure in patients with malignant tumors.

Brain Neoplasms↗

Rapid diagnosis of germline p53 mutation using the enzyme mismatch cleavage method.

The p53 tumor suppressor gene is the most commonly altered gene in human cancers. Germline mutations in p53 are the genetic alteration underlying predisposition to multiple cancers in Li-Fraumeni syndrome and Li-Fraumeni-like syndrome. We describe a patient who presented with developed adrenocortical carcinoma at age 19 months and a cerebral primitive neuroectodermal tumor at age 5 years. The patient did not have a family history of cancer. We used the enzyme mismatch cleavage (EMC) method to screen for mutations in the p53 gene and found a germline mutation in exon 7 (codon 248). Loss of heterozygosity analysis in one tumor revealed loss of the wild-type p53 allele. In our report we demonstrate the EMC method to be a rapid and sensitive method for mutation detection.

Adrenocortical Carcinoma↗

Expression pattern of galectin-3 in neural tumor cell lines.

Galectin-3 is a member of the galectin family of beta-galactoside-specific animal lectins. Here we show that galectin-3 is constitutively expressed in 15 out of 16 glioma cell lines tested, but not by normal or reactive astrocytes, oligodendrocytes, glial O-2A progenitor cells and the oligodendrocyte precursor cell line Oli-neu. Galectin-3 is also expressed by one oligodendroglioma cell line, but not by primitive neuroectodermal tumor and 4 neuroblastoma cell lines tested so far. In all galectin-3 expressing cell lines, the lectin is predominantly, if not exclusively, localized intracellularly and carries an active carbohydrate recognition domain (shown for C6 rat glioma cells). Moreover, in contrast to primary astrocytes, glioma cells do not or only weakly adhere to substratum-bound galectin-3, probably reflecting an unusual glycosylation pattern. Our findings indicate that the expression of galectin-3 selectively correlates with glial cell transformation in the central nervous system and could thus serve as a marker for glial tumor cell lines and glial tumors.

Animals↗

Phase II study of daily oral etoposide in children with recurrent brain tumors and other solid tumors.

Pre-clinical data and adult experience suggests that topoisomerase targeted anti-cancer agents may be highly schedule dependent, and efficacy may improve with prolonged exposure. To investigate this hypothesis, 28 children with recurrent brain and solid tumors were enrolled in a phase II study of oral etoposide (ETP). Patients were prescribed ETP at 50 mg/m2/ day for 21 consecutive days. Courses were repeated every 28 days pending bone marrow recovery. Evaluation of response was initially performed after 8 weeks and then every 12 weeks either by CT or MRI. Three of 4 patients with PNET (primitive neuroectodermal tumor)/medulloblastora achieved a partial response (PR). Two of 5 with ependymoma responded, one with a complete response and one with a PR. Toxicity was manageable with only 1 admission for fever and neutropenia in 120 cycles of therapy. Five patients had grade 3 or 4 neutropenia. One had grade 4 thrombocytopenia and one grade 2 mucositis and withdrew as a result. One patient had grade 2 diarrhea. Two patients who achieved a PR had received ETP as part of prior combination chemotherapy regimens. Daily oral etoposide is active in recurrent PNET/medulloblastoma and ependymoma. Toxicity is manageable and rarely requires intervention. Daily oral etoposide in combination with crosslinking agents should be considered in future phase III trials. Determination of activity in glioma and solid tumors is not complete.

Administration, Oral↗

Immunohistochemical expression of inducible nitric oxide synthase (iNOS) in human brain tumors: relationships of iNOS to superoxide dismutase (SOD) proteins (SOD1 and SOD2), Ki-67 antigen (MIB-1) and p53 protein.

In this study, inducible nitric oxide synthase (iNOS) expression in a series of 158 human primary brain tumors was analyzed. To gain some insight into the biological significance of iNOS expression in tumor cells, comparative immunohistochemical analyses were employed to characterize the expression of iNOS, superoxide dismutase (SOD) proteins (SOD1 and SOD2), Ki-67 antigen (MIB-1) and p53 protein in these cells. Sixteen (39.0%) of the 41 glioblastoma multiforme (GBM) specimens showed iNOS immunoreactivity. Positive immunoreactions with iNOS were also detected in 2/8 anaplastic astrocytomas, 1/17 astrocytomas, 1/14 medulloblastomas and 1/11 primitive neuroectodermal tumors, but no positive reactions were observed in oligodendrogliomas (0/11), ependymomas (0/5), schwannomas (0/21), meningiomas (0/23) or pituitary adenomas (0/7). The MIB-1 labeling index of GBMs that expressed iNOS was significantly higher than that of GBMs that did not (0.025< P <0.05, Wilcoxon rank-sum test). Unlike iNOS-negative tumors, all iNOS-positive tumors coexpressed SOD1 or SOD2. In particular, there was a significant correlation between iNOS induction and SOD1 expression (P =1.65x10(-10), Fisher's exact test) in GBM specimens. There was no significant relationship between iNOS and p53 protein in any type of primary brain tumor (P >0.05, Fisher's exact test). No significant immunohistochemical reactions with iNOS, MIB-1 or p53 protein were observed in normal brain tissue sections. We conclude that primary brain tumors express iNOS, and that iNOS expression in brain tumor cells may depend, in part, on cellular proliferation potential. Based on the fact that SOD1 scavenges oxidative-stress species originating from large amounts of nitric oxide (NO) produced by iNOS, iNOS-expressing brain tumor cells may protect themselves against NO cytotoxicity by overinducing SOD1.

Blotting, Western↗

Cerebrospinal fluid pharmacokinetics of carboplatin in children with brain tumors.

The pharmacokinetics of carboplatin in cerebrospinal fluid (CSF) and plasma was studied in five children with brain tumors (four medulloblastomas and one ependimoblastoma) who underwent preirradiation treatment with carboplatin. Carboplatin pharmacokinetics was studied following the administration of 600 mg/m2 as a 1-h infusion. Four children were treated a few weeks after surgery, whereas one child with an unresectable tumor was treated prior to surgery. All patients had a ventricular-peritoneal CSF shunt connected to a subcutaneous reservoir. Total platinum and free carboplatin were measured. The mean AUC values for free carboplatin in CSF and plasma were 2.29 +/- 1.20 and 8.18 +/- 1.27 mg ml-1 min, respectively. The mean ratio of CSF AUC to plasma AUC was 0.28 (range, 0.17-0.46). Both plasma peak levels and AUC values showed limited interpatient variability. On the other hand, carboplatin levels in CSF showed substantial interpatient variability, with a greater than 5-fold difference in peak levels and a 3-fold difference in AUC values being recorded. The interpatient difference in CSF pharmacokinetics may have been related at least in part to the different anatomical alterations induced by the surgical procedures or by the presence of a large tumor mass. In the four evaluable patients exhibiting macroscopic residual tumor, we observed one complete remission (CR) and two partial remissions (PR) following two cycles that consisted of two doses of 600 mg/m2 carboplatin given on 2 consecutive days (total dose, 1200 mg/m2) and were separated by a 1-month interval. These results may give some indication as to the optimal dose and schedule for carboplatin administration in the treatment of primitive neuroectodermic tumors (PNET).

Brain Neoplasms↗

Central nervous system tumors among Koreans--a statistical study on 697 cases.

A total of 697 cases of intracranial and intraspinal tumors was obtained from the pathology file of Seoul National University Hospital and Children's Hospital during the period of 8 years from 1980 to 1987. These tumors were classified according to WHO classification. This study was performed to understand the recent trend of the relative frequency of the central nervous system tumors among Koreans and to compare it with the previous studies in Korea and other countries. There were 663 intracranial tumors and 34 intraspinal tumors. More common intracranial tumors were pituitary adenoma, meningioma, astrocytoma and medulloblastoma, each representing 23.4%, 20.8%, 11.8%, and 5.6%, respectively. In juvenile age group (under 15 years of age), medulloblastoma, astrocytoma, ependymoma and craniopharyngioma were more commonly encountered to be 25.6%, 21.6%, 13.6% and 12%, respectively. Both sexes were equally affected among adult group, but male preponderance was observed among juvenile group (1.49:1). Nine cases of primitive neuroectodermal tumor, a unique tumor which is not listed in WHO classification, were observed and all of them occurred before the age of 20. There were 27 metastatic tumors. Our previous study encompassing previous 17 years, 1963 to 1979, showed similar overall results except for intraspinal tumors that were more commonly encountered in previous series.

Adult↗

[Orbital tumors in children. Transcranial surgical approach].

From March 1980 to December 1995, at the Department of Neurosurgery of the Instituto Nacional de Pediatría (INP), 23 patients were treated for orbital tumors. In this communication, the authors report on the clinical presentation, age, radiological findings, visual-evoked potentials, type of surgery, histological diagnosis, and results. Two kinds of surgical techniques were employed: craniotomy with orbital roof removal (NAFZIGER) and fronto-orbitotomy. Eleven patients had optic nerve gliomas, 3 had fibrous osseous dysplasia, 2 meningiomas, neurofibromas, Langerhans cells histiocytosis, and 1 case of Anaplastic astrocytoma, Teratoma, primitive Neuroectodermal tumor, germinoma with areas of choriocarcinoma. The results were evaluated for the quality of life in regard to the Karnofsky scale, tumor recurrence and postoperative visual-evoked potentials. It is important to note the very variable histological kinds of lesions found in this series that can be approached surgically with cranio orbitotomy with good results.

Adolescent↗

Transient "cerebellar" mutism.

The term "cerebellar mutism" refers to a specific disorder in which a complete but transient loss of speech, followed by dysarthria, occurs following resection of intrinsic posterior cranial fossa tumors or cerebellar hemorrhages, or upon trauma. Although it is well known that the lack of long-tract findings and cranial nerve (CN) involvement is the rule, the pathophysiology of cerebellar mutism has not been clearly elucidated. A review of the relevant literature disclosed 93 patients with this condition, the majority of these being in the pediatric age group. The neuropathological findings were as follows: 57 primitive neuroectodermal tumors (PNET), 19 astrocytomas, 10 ependymomas, 5 vascular malformations, 1 metastatic tumor, and 1 traumatic injury. The interval before the onset of mutism ranged from 0 to 168 h (mean 40.9 h). The mutism lasted from 1 to 168 days (mean 37.6 days). Subsequent dysarthria was present in 75 (80%) of the 93 patients. In this article, some specific recent illustrative reports are presented, and the concept of the role of the cerebellum in language and cognition is discussed. With these data as our point of departure, various hypotheses that have been advanced to explain the pathogenesis of this transient speech disorder are analyzed. The findings of the study suggest that the cause of the cerebellar mutism is the ischemia caused by vasospasm, as it usually developed after a latent period.

Arteriovenous Malformations↗

Sacrococcygeal tumors in infancy and childhood; a retrospective histopathological review of 85 cases.

This study retrospectively examines the spectrum of sacrococcygeal tumors reported in a tertiary paediatric pathology department during a 15-year period. There were 85 sacrococcygeal tumors identified in total, including 79 (93%) sacrococcygeal germ cell tumors, of which 62 (78%) were benign, whereas 17 (22%) contained malignant yolk sac tumor elements. The median age at examination in cases with malignant elements present was significantly greater than in those with benign sacrococcygeal teratoma only (median 2 years, range birth--3 years versus median 1 week, range birth--10 years, respectively; p < .01). Of the 85 cases of total sacrococcygeal lesions 6 (7%) represented pathologies other than sacrococcygeal teratoma, including one case each of neuroblastoma, ganglioneuroma, myxopapillary ependymoma, primitive neuroectodermal tumor, lipomatous tumor, and unclassifiable inflammatory tumor. Of these 6 cases 3 were malignant (50%) compared with 17 of the 79 cases of sacrococcygeal germ cell tumors (22%; Z =1.59, p = .08). The median age in the group of non-germ-cell sacrococcygeal masses was 3 years (range 5 months to 13 years).

Adolescent↗