Intracranial tumors during the 1st year of life

Twenty-eight patients with intracranial tumors symptomatic during the 1st year of life were managed from January 1970 to March 1988. Supratentorial location (19 cases) was dominant over the infratentorial. The most common histological type was astrocytoma; choroid plexus papilloma and ependymal tumors followed in frequency. Twenty-two infants (78.6%) had associated hydrocephalus. Macrocrania, vomiting, delayed milestones, and behavioral disturbances were the chief clinical manifestations. Four patients were admitted in extremely deteriorated condition and died preoperatively. Twenty cases underwent surgery for tumor removal with a 1-month mortality rate of 20%. Tumor excision provided permanent relief of hydrocephalus in the majority of the surviving cases. Five patients received conventional radiotherapy. Stereotactic brachytherapy yielded an excellent result in 1 case. Overall 13 cases are currently alive; 6 of them have survived longer than 6 years. Fourteen of the 15 patients who were dead at follow-up succumbed within 1 year after diagnosis.     

Primary intramedullary spinal cord primitive neuroectodermal tumor with intracranial seeding in an infant

Primary spinal cord primitive neuroectodermal tumor (PNET) is a rare entity. In all, 13 cases have been reported in the literature, including 3 with intracranial seeding. A 3-month-old girl with involvement of the spinal cord below the mid-thoracic level is described. The brain MRI revealed findings indicative of seeding along the intracranial subarachnoid space. Biopsy, duraplasty and removal of laminotomy flap were done. In spite of a good response to the first cycle of postoperative 8-drugs-in-a-day chemotherapy, further treatment was refused. She died 21 days after the onset of leg weakness, which reveals the rapid progression of untreated cases. To our knowledge, this is the first case of spinal cord PNET with parenchymal involvement that has been described in an infant.     

Clinicopathologic correlations in epithelial choroid plexus neoplasms: a study of 52 cases

Summary Sixty-seven tumor specimens of epithelial choroid plexus neoplasms obtained by 60 biopsies and 7 autopsies from 52 patients were investigated. Diagnoses of the first operations were choroid plexus papilloma (PP; 32 cases), choroid plexus papilloma with histological atypies (atypical PP; 6 cases), and choroid plexus carcinoma (PC; 14 cases). Carcinoembryonic antigen was expressed by 2 of the 3 biopsies autoptically recognized as metastatic carcinomas and by 2 autopsy cases of PC, while it was absent in all biopsies of true choroid plexus tumors. Tumor cells positive for transthyretin (TTR, prealbumin), S-100 protein (S100), and glial fibrillary acidic protein (GFAP) were detected in 39, 46 and 13, respectively, of the 49 cases of true choroid plexus tumors. Fourth ventricle tumors expressed more S100 (number of positive tumor cells) than lateral ventricle tumors, PP more S100 and TTR than atypical PP/PC. Tumors from patients 20 years of age and older expressed more GFAP and TTR than tumors from younger patients. Of the 30 patients with complete follow-up 19 were alive 2 to 11 years after surgery, including 7 recurrencies. Eleven died from the tumor 4 months to 7 years after surgery. The following histopathologic features (in order of decreasing significance) were correlated with poor prognosis (recurrency or fatal outcome): less than 50% of the tumor cells heavily positive for S100, presence of mitoses, absence of TTR-positive cells, brain invasion by cell nests, absence of marked stromal edema, and presence of necrotic areas. Our results indicate that some histologic features correlate significantly with poor prognosis and that immunohistochemical results correlate with tumor localization, age, and malignancy.     

脉络丛肿瘤临床特点分析

目的总结脉络丛肿瘤（CPT）的临床特点和诊治经验。方法本组31例CPT中，28例为脉络丛乳头状瘤，3例为脉络丛癌．全部行显微手术治疗。结果肿瘤全切24例，次全切除6例，大部分切除1例，无手术死亡病例。术后9例患者复查CT示脑积水改善不明显。结论脑积水是CPT的突出表现，与肿瘤大小及性质不一致，手术缓解率不高。虽然脉络丛肿瘤大多为慢性起病．但仍有少数表现为急症。     

Immunohistochemical evidence of endothelin-1 in human choroid plexus

Summary An immunohistochemical investigation was carried out on 17 specimens of human choroid plexus obtained post mortem, 1 biopsy of normal choroid plexus including part of the lateral ventricle and 1 papilloma of the choroid plexus removed surgically. The material was fixed in formalin. Paraffin and cryostat sections were used. A polyclonal antiserum to endothelin-1 served as a primary antibody. The avidin-biotin-peroxidase method was applied to demonstrate the immunoreaction. The epithelial cells of the choroid plexuses, the choroid papilloma and most ependymal cells of the lateral ventricle showed a distinct brown reaction product in their cytoplasm indicating antigenic sites to endothelin-1. The reaction was of lesser intensity in the ependymal cells. The connective tissue in choroid plexus was unstained. A positive immunoreaction was present in the walls of some vessels in the choroid plexus in cryostat sections. This is the first report on the presence of antigenic sites to endothelin-1 in the epithelial cells of the human choroid plexus. The role of endothelin in these cells should be investigated to ascertain if the cells synthesize this biologically active peptide or if it is merely bound to receptors in them.Supported by grants from Swedish Medical Research Council, project 03020, 1987 Års stiftelse för strokeforskning, Selanders stiftelse, Åhlen-stiftelsen and Stiftelsen Gamla Tjänarinnor, Stockholm Sweden     

Intracranial tumors of the first year of life

The introduction of new diagnostic tools for neuroimaging has resulted in the early recognition of congenital brain tumors. In the present report the authors describe an International Multicenter Series of 886 children with brain tumor, in whom the diagnosis was obtained during the first 12 months of life. Most of the tumors were located within the supratentorial compartement. In spite of a relatively high operative mortality, surgery still appears to be the more effective therapy. Radiotherapy in this age group is of little value due to the vulnerability of the infantile brain. At the present time, the use of chemotherapy is still controversial.     

First report of occurrence of choroid plexus papilloma and medulloblastoma in the same patient

Introduction Choroid plexus papilloma is a benign epithelial brain tumour showing a striking predilection for infants and occurring most frequently in the lateral and fourth ventricles. Medulloblastoma, on the other hand, is a primitive neuroectodermal tumour and is the most frequent malignant brain tumour of the posterior fossa in children. In this study, we report a metachronous occurrence of choroid plexus papilloma and medulloblastoma in the same patient, which has not been reported before to the best of our knowledge. Case report The authors describe the case of a girl who presented with an atypical choroid plexus papilloma on the posterior wall of the left lateral ventricle at 3 months of age that was resected completely. She was followed up regularly after surgery and made good progress with normal development. At 8 years of age, she presented with right cerebellar medulloblastoma. Discussion The authors review literature for incidence and aetiology of the two tumours.     

Primary intracranial neoplasms of infancy and early childhood

We investigated the age-related location, gender distribution, and histology of 107 brain tumors in children under 4 years of age seen in our department between 1984 and 1997. The male-to-female ratio was 1.4 (62/45 cases) with a prevalence of supratentorial tumors (60/47=1.3); the main histological entity was astrocytoma (33.6%), followed by ependymoma (14.0%). In the 1st year of life 22 cerebral neoplasms became clinically apparent. A higher ratio for supratentorial tumors was revealed (17/5=3.4), but without gender preference, and primitive neuroectodermal tumors (PNET) were the most frequent (5/22). In the 2nd year 25 tumors were found. The male-to-female ratio was 1.5 (15/10) and the supratentorial-to-infratentorial ratio, 1.1 (13/12). The two most common entities were astrocytoma and ependymoma (6 cases each). In addition, a survey of previously published investigations into this subject was performed and a compilation of data on 1960, 545 and 1084 tumors in children below the age of 1, 2 and 4 years, respectively, was prepared, which makes it the most extensive review of brain tumors of infancy and early childhood yet undertaken. Received: 14 July 1997     

The role of endoscopic choroid plexus coagulation in the surgical management of bilateral choroid plexuses hyperplasia

Background Bilateral choroid plexus hyperplasia is a rare condition often associated with cerebrospinal fluid (CSF) overproduction. CSF overproduction is usually so high that the placement of a CSF ventriculoperitoneal shunt almost always results in progressive ascites leading to the necessity of removing the inserted shunt device. A direct surgical treatment of the hyperplastic choroid plexuses is then mandatory. Endoscopic coagulation of the choroid plexuses has been recently proposed as an alternative to open surgical plexectomy. However, the effectiveness of the procedure in controlling CSF overproduction is still debated.Technique We report a case of bilateral choroid plexus hyperplasia in which an extensive bilateral endoscopic coagulation of the choroid plexuses failed to reduce the CSF formation rate sufficiently. A one-stage bilateral open surgical plexectomy was performed.Results The procedure succeeded to control CSF overproduction. Intraoperative blood loss during the surgical removal of the choroid plexuses was significantly reduced due to the previous coagulation of their surface.Conclusion On these grounds, we suggest that endoscopic choroid plexuses coagulation, even when failing to normalize CSF production, may still be considered as a valid adjuvant procedure in the management of this condition.     

Disseminated choroid plexus papillomas in adults: A case series and review of the literature

Choroid plexus papillomas (CPPs) are uncommon, usually intraventricular, low-grade tumors, accounting for less than 1% of all intracranial neoplasms and 2–4% of brain tumors in children. Dissemination of CPPs to multiple levels of the neuraxis has been seldom observed. Thus far, only 26 adult patients have been reported in the English language literature. With some exceptions, disseminated CPPs have been observed in adults and involved multiple sites along the cerebrospinal fluid pathways. Occasionally, intraparenchymal extension has been documented, and secondary involvement of the suprasellar region has been reported in only five patients. Postoperative treatment of CPPs has not been standardized. Most recommended therapies have been extrapolated from a series of atypical papillomas or carcinomas of the choroid plexus in children. We herein report a series of three patients of disseminated choroid plexus papillomas providing additional insights into this relatively rare entity.     

An immunohistochemical study of cytokeratin and glial fibrillary acidic protein in chroid plexus papilloma

Summary Cellular localization of cytokeratin and glial fibrillary acidic protein (GFAP) was examined in two normal choroid plexuses and five choroid plexus papillomas by the peroxidase-antiperoxidase (PAP) method and double immunofluorescence (IFL) microscopy. Cytokeratin was observed in the majority of epithelial cells in all samples of normal and neoplastic choroid plexuses. On the other hand, GFAP was observed in some of the constituent epithelial cells in two cases of papilloma. Most of these GFAP-positive papilloma cells were simultaneously positive for cytokeratin, as could be seen by the PAP stainings of serial sections and by the double IFL stainings of the same sections. From these findings, it was suggested that normal and neoplastic choroid plexus epithel cells usually express cytokeratin and that some of the neoplastic cells can simultaneously express boty cytokeratin and GFAP.     

Primary high-grade B-cell lymphoma of the choroid plexus

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma arising from and confined to the neuraxis. It represents about 3-4% of all primitive brain neoplasms and is mainly located in deep supratentorial regions. A ventricular involvement is quite frequent, but in the vast majority of cases it is secondary to an extension from a subependymal location. Amongst the primary ventricular forms occasionally reported, a choroid plexus origin is extremely rare; to date, only three cases have been described in the English literature. We report the case of a 71-year-old left-handed woman admitted to our department after a progressive 2-week history of confusion, motor speech disturbances and left hemiparesis. A brain MRI scan displayed an intraventricular lesion located in the right atrium, about 2.5cm in its major axis, homogeneously enhancing after gadolinium administration, surrounded by edema in the homolateral deep hemispheric region; the main diagnostic hypothesis was meningioma. She underwent a parieto-occipital craniotomy and a navigation-assisted posterior parietal transcortical approach, achieving complete removal of the intraventricular lesion originating from the choroid plexus. The histological diagnosis was of high-grade diffuse large B-cell lymphoma. Both an MRI scan of the entire craniospinal axis and a thoracic and abdominal CT scan showed no other neoplastic lesions; a bone marrow biopsy was normal; a final diagnosis of primary central nervous system lymphoma was made. CSF cytological analysis did not show any signs of lymphoma dissemination. Adjuvant chemotherapy with methotrexate was administered, and at the last follow-up (26 months) our patient is clinically and radiologically disease-free. Primary choroid plexus lymphoma is a very rare tumor that should be included in the differential diagnosis of intraventricular neoplasms. It usually occurs in the lateral ventricles (with a predilection for the atrium) of adult people (>50 years of age), apparently with a male prevalence. Generally, the radiological picture mimics that of a meningioma, despite the fact that meningiomas usually reach a greater volume before clinical onset. Surgery is almost always necessary to obtain an adequate tissue sample and frequently leads to a radical removal of the lesion. Low-grade marginal zone B-cell, T-cell and high-grade diffuse large B-cell (present case) forms have been described. With a proper adjuvant therapy a relatively good outcome is observed.     

Primary malignant rhabdoid tumor of the cerebellum

Malignant rhabdoid tumor (MRT) is a recently described variety of childhood renal neoplasm. MRT arising primarily in the central nervous system (CNS) is still a rather unfamiliar pathological entity and is frequently misdiagnosed as medulloblastoma or primitive neuroectodermal tumor (PNET). We describe a 7-month-old boy who harbored a CNS-MRT that originated within the IV ventricle and invaded the brain stem and the cerebellar hemispheres. After an initial documented good response to chemotherapy the tumor recurred locally 9 months after operation. We discuss clinical and histopathological features distinguishing between PNET/medulloblastoma and MRTs. Current literature on MRT of the CNS is briefly reviewed. Received: 17 October 1996     

Infantile colic and transient developmental lag in the first year of life

This study investigates the impact of infantile colic on subsequent development. Infants with a history of colic scored significantly lower on the Mental and Psychomotor scales of the Bayley Scales of Infant Development at age 6 months but there was no significant effect of colic on test performance at later ages. The data suggest that this temporary delay in development may be due in part to less favorable patterns of caregiver-infant interaction as a result of the history of colic.Funding for this research was provided by the National Institute of Child Health and Human Development (HD-08945)     

Choroid plexus papillomas of the cerebellopontine angle in a child

An 11-year-old girl was admitted with a 3-year history of decreasing hearing, and headache, occasional vomiting and poor school performance of 8 months duration. Neurologically she showed signs of a cerebellopontine angle mass. This was confirmed by CT scan, which showed an enhancing lesion in the right cerebellopontine angle. The lesion was totally removed, with excellent results. Histologically the tumor appeared to be a choroid plexus papilloma. Choroid plexus papillomas of the cerebellopontine angle are extremely rare in children.

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Sommario Una bambina di 11 anni veniva ricoverata per una diminuzione dell'udito in AU dx, iniziata 3 anni prima ed accentuatasi da 8 mesi, quando erano comparsi altri sintomi quali cefalea, saltuari episodi di vomito e diminuzione del livello di rendimento scolastico. All'esame neurologico erano presenti segni di lesione espansiva dell'angolo ponto-cerebellare dx. Una TAC dimostrava una massa iperdensa, che assumeva marcatamente m.d.c., occupante l'angolo ponto-cerebellare dx. L'intervento chirurgico consentiva una rimozione totale della lesione, con eccellenti risultati. Il tumore risultava istologicamente essere un papilloma dei plessi coroidei, la cui localizzazione nell'angolo ponto-cerebellare è estremamente rara nell'infanzia.

     

Choroid plexus papilloma of the III ventricle in an infant

A case of choroid plexus papilloma of the III ventricle associated with hydrocephalus in an 1-month-old boy is reported. The tumor was removed after placement of a ventriculoperitoneal shunt; however, the histologically mainly benign tumor grew very rapidly. The cause of this rapid growth may have been a drop in the intraventricular pressure after the shunting procedure.     

Intracranial tumors in the first year of life

Among 100 childhood brain tumors treated at Kobe Children's Hospital from May 1970 to June 1985, 18 of the children presented with symptoms during the first year of life. This paper analyzes these 18 cases. Supratentorial tumors (78%) were more common than infratentorial ones, and 67% of all the tumors were located in the central neural axis. Initial symptoms were cranial enlargement (56%), vomiting (17%), cranial deformity (11%), blepharoptosis, respiratory distress, and ataxia. Histological diagnosis of the tumors was as follows: teratoma (3 cases), medulloblastoma (3), glioblastoma (2), astrocytoma (2), ependymoma (2), craniopharyngioma (1), choroid plexus papilloma (1), hamartoma (1), lipoma (1), melanotic progonoma (1), and an undetermined type, probably medulloblastoma (1). Seventeen of the patients underwent craniotomy for tumor resection (4 total, 4 subtotal and 7 partial removal, and 2 biopsies). Additional therapeutic methods used separately and in various combinations included ventriculoperitoneal shunt, subduralperitoneal shunt, ventricular drainage, radiotherapy and chemotherapy. Nine patients died (average 98 days) after surgery. Of the 9 survivors, 6 are still alive after more than 5 years. Five of the 6 are mentally retarded and 4 are physically handicapped to some degree.Presented at the 13th Annual Meeting of the International Society for Pediatric Neurosurgery     

Behavioral manifestations of autism in the first year of life

In the interest of more systematically documenting the early signs of autism, and of testing specific hypotheses regarding their underlying neurodevelopmental substrates, we have initiated a longitudinal study of high-risk infants, all of whom have an older sibling diagnosed with an autistic spectrum disorder. Our sample currently includes 150 infant siblings, including 65 who have been followed to age 24 months, who are the focus of this paper. We have also followed a comparison group of low-risk infants. Our measures include a novel observational scale (the first, to our knowledge, that is designed to assess autism-specific behavior in infants), a computerized visual orienting task, and standardized measures of temperament, cognitive and language development. Our preliminary results indicate that by 12 months of age, siblings who are later diagnosed with autism may be distinguished from other siblings and low-risk controls on the basis of: (1) several specific behavioral markers, including atypicalities in eye contact, visual tracking, disengagement of visual attention, orienting to name, imitation, social smiling, reactivity, social interest and affect, and sensory-oriented behaviors; (2) prolonged latency to disengage visual attention; (3) a characteristic pattern of early temperament, with marked passivity and decreased activity level at 6 months, followed by extreme distress reactions, a tendency to fixate on particular objects in the environment, and decreased expression of positive affect by 12 months; and (4) delayed expressive and receptive language. We discuss these findings in the context of various neural networks thought to underlie neurodevelopmental abnormalities in autism, including poor visual orienting. Over time, as we are able to prospectively study larger numbers and to examine interrelationships among both early-developing behaviors and biological indices of interest, we hope this work will advance current understanding of the neurodevelopmental origins of autism.     

Intra-axial endophytic primitive neuroectodermal tumors in the pons: clinical,radiological, and immunohistochemical aspects in four children

We present the clinical findings, radiological aspects, operative results, and histopathological features of four typical primitive neuroectodermal tumors (PNET) located in the pontine region in children. All the tumors had an endophytic intra-axial growth pattern. All the children had a short history of severe neurological deficits with involvement of the cranial nerves and pyramidal tract. MRI did not reveal any common feature of malignancy. Compared to our successful experience in operations of intra-axial endophytic brainstem tumors in a total of 32 children, the outcome was poor: all 4 children died within 13 months. We conclude that PNET occurring in the pons is not as rare as was believed, and, compared to PNET in other areas the prognosis is worse.