Fetal presentation of congenital fibrosarcoma of the meninges: case report and literature review.

AIM: Congenital infantile fibrosarcoma (CIFS) exceptionally occurs in the meninges, with no cases reported before or at birth. We report herein a meningeal CIFS diagnosed in a fetus at 40 weeks of gestation (WG). CASE REPORT: A 24-year-old pregnant woman was referred to the obstetrics department for vaginal bleeding. A severe right hydrocephalus due to a large tumor invading the left hemisphere and ventricles was discovered in the fetus, and medical termination of the pregnancy was achieved. RESULTS: Histological examination revealed a highly cellular spindle or ovoid shaped cell proliferation organized in interlacing bundles; it was diffusely positive for vimentin, and scarcely for SMA (smooth muscle actin). NFs (neurofilaments), NeuN, S100 protein, desmin, GFAP (glial fibrillary acidic protein), Olig2, chromogranin, synaptophysin, CD31, CD34, BCL2, and EMA (epithelial membrane antigen) antibodies were negative. Ki67 antibody labeled 20% of the nuclei. A reverse transcription polymerase chain reaction assay was performed to detect the gene fusion ETV6-NTRK3 transcript. Despite negative results, it was concluded to be a CIFS of the meninges. DISCUSSION: CIFS of the meninges during the fetal period has never been reported. Its diagnosis is based on immunohistochemistry, and, whenever possible, on the detection of the reciprocal translocation t (12;15) resulting in the gene fusion ETV6-NTRK3. Its prognosis depends on rapid growth and local invasiveness leading to cerebral structure damage.

Workhorse Free Functional Muscle Transfer Techniques for Smile Reanimation in Children with Congenital Facial Palsy: Case Report and Systematic Review of the Literature.

BACKGROUND: Pediatric facial palsy represents a rare multifactorial entity. Facial reanimation restores smiling, thus boosting self-confidence and social integration of the affected children. The purpose of this paper is to present a systematic review of microsurgical workhorse free functional muscle transfer procedures with emphasis on the long-term functional, aesthetic, and psychosocial outcomes. MATERIALS AND METHODS: We performed a literature search of the PubMed database from 1995 to 2019 using the following search strategy: "facial paralysis"[Title/Abstract] OR "facial palsy"[Title]. We used as limits: full text, English language, age younger than 18 years, and humans. Two independent reviewers performed the online screening process using Covidence. Forty articles met the inclusion criteria. The protocol was aligned with the PRISMA statement (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and was registered at the International Prospective Register of Systematic Reviews (PROSPERO, CRD42019150112) of the National Institute for Health Research. RESULTS: Free functional muscle transfer procedures include mainly segmental gracilis, latissimus dorsi, and pectoralis minor muscle transfer. Facial reanimation procedures with the use of the cross-face nerve graft (CFNG) or masseteric nerve result in almost symmetric smiles. The transplanted muscle grows harmoniously along with the craniofacial skeleton. Muscle function and aesthetic outcomes improve over time. All children presented improved self-esteem, oral commissure opening, facial animation, and speech. CONCLUSIONS: A two-stage CFNG plus an FFMT may restore a spontaneous emotive smile in pediatric facial palsy patients. Superior results of children FFMT compared to adults FFMT are probably attributed to greater brain plasticity.

[Congenital melanocytic nevi--a multifaceted problem]. / Kongenitale melanozytäre Nävi. Früh exzidieren oder abwarten?

Congenital melanocytic nevi are specific pigmented lesions that differ from acquired melanocytic nevi in terms of the age at which they develop and, usually, also in their clinical and histological features. Their potential for malignant transformation and their association with leptomeningeal seeding of nevomelanocytic cells have long been known, at least in the case of large melanocytic nevi. In addition, significant psychosocial stigmatization may affect patients with larger congenital nevi, in particular when they appear at exposed sites. Together, these aspects, in combination with the individual reaction of the patient and his/her parents, must be considered before a therapeutic recommendation is made. In the present review, our current knowledge of this condition is summarized with the aim of helping to determine the most appropriate management for the individual affected.

Coexisting intracranial meningeal melanocytoma, dermoid tumor, and Dandy-Walker cyst in a patient with neurocutaneous melanosis. Case report.

Neurocutaneous melanosis (NCM) associated with Dandy-Walker malformation is a very rare congenital neurodysplasia with the same origin. Primary intracranial melanocytic and dermoid tumors are also benign congenital lesions that usually arise from the leptomeninges and are formed by the inclusion of cutaneous ectoderm at the time of neural tube closure. The authors describe a patient with coexisting intracranial meningeal melanocytoma, NCM with Dandy-Walker malformation, and intraventricular dermoid tumor.

Neurocutaneous melanomatosis with a rapidly deteriorating course.

Neurocutaneous melanosis is a rare congenital syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant pigment cell tumors of the leptomeninges. The prognosis is extremely poor for symptomatic patients, even in the absence of malignant melanoma. We present serial MR imaging findings in the brain and spine of a child with congenital giant hairy nevi who developed progressive leptomeningeal melanomatosis and whose neurologic condition rapidly deteriorated.

Congenital central nervous system tumors.

Congenital central nervous system (CNS) tumors are a rare and diverse group of tumors with variable biological behavior depending on location and histology. This review summarizes the published literature and describes the definition, epidemiology, diagnosis and evaluation, and treatment of congenital central nervous system tumors.

Clinicopathologic studies on leptomeningeal glioneuronal heterotopia in congenital anomalies.

Leptomeningeal glioneuronal heterotopia was observed in 40 of 129 autopsied infants (31%). It was present in 49% of patients who had congenital anomalies in general and in 65% of patients who had central nervous system malformations. Most of the leptomeningeal glioneuronal heterotopias appeared in the base of the brain (62.5%), midbrain (40%), frontal lobe (37%), and pons (35%). Leptomeningeal glioneuronal heterotopia is closely related to migration disorders on the basis of frequent association with polymicrogyria or neuronal heterotopias.

Meningioma in a neonate: case report.

A 10-day-old female with a parasagittal meningioma presenting as a subcutaneous tumor is reported. Meningiomas within the 1st month of life are rare. The clinical and pathological characteristics of congenital meningioma are reviewed.

Type 1-primary cutaneous meningioma of the scalp.

Type 1-primary cutaneous meningioma is a rare and often clinically unsuspected lesion of the scalp, forehead or paravertebral region which occurs at birth or during childhood. The pathogenesis of these lesions still remains uncertain. Several authors have emphasized that type 1-cutaneous meningiomas are not real tumors but sequestrated meningoceles or heterotopic meningeal nodules of the skin. Nevertheless, the search for an intracranial or intravertebral connection should be carried out. We describe the clinical and pathological features of a congenital type 1-meningioma of the vertex. No cranial defect or intracranial tumor was found. However, the cutaneous lesion was directly linked to a large cranial vein. The purpose of this study was to illustrate this rare lesion and to indicate the possible surgical risks and the pathological characteristics.

[Intracranial lipomas in paediatrics: a retrospective study of 20 patients]. / Lipomas intracraneales en pediatría: estudio retrospectivo de 20 pacientes.

INTRODUCTION: Intracranial lipomas (ICL) are congenital malformations that are due to an anomalous differentiation of the primitive meninges. AIMS: The purpose of this study is to determine the most frequent symptoms related to ICL in the paediatric age, as well as to evaluate whether they are directly related to their location and to their association with other brain malformations. PATIENTS AND METHODS: A retrospective clinico-radiological study was conducted involving 20 paediatric patients diagnosed as suffering from ICL between 1985 and 2003 at three hospitals in the Valencian Community. RESULTS: 70% of the cases were females and the average age when ICL was diagnosed was 5.3 years (interval from 2 months to 13 years). The most common initial clinical justification for the neuroradiological study was psychomotor retardation in eight patients (40%) and persistent, predominantly migrainous, headaches in six of them (30%). Only one patient was examined because of epilepsy. The prevailing site was the interhemispheric fissure in 10 patients (50%), followed by the quadrigeminal cistern in five (25%) and the mamillary body infundibulum axis in three of them (15%). One case was located in the cerebellum and another was found in the lateral ventricle, with involvement of the choroid fissure. Associated anomalies were observed in eight patients (40%). Alterations were seen in the development of the corpus callosum in six patients (30%), five in an isolated way and in one case there was partial absence of the associated septum pellucidum. Another patient was found to have an isolated partial agenesis of the septum and Goldenhar syndrome was detected in another case. The radiological follow up, which varied according to the initial clinical features, did not show any differences in the morphology or the size of the lipoma. CONCLUSIONS: 1) ICL are more frequently located in the interhemispheric fissure and in the quadrigeminal cistern; 2) In these locations they can be associated with other brain malformations, the most important being defects in the callosa and septa; 3) Most ICL were found incidentally during the course of a neuroimaging examination. Only in the case of three patients (15%) was there any presumed relation between the location of the ICL (pericallosal), the symptomatology (overall psychomotor retardation) and the associated malformation (hypoplasia of the corpus callosum); 4) The incidence of epilepsy was much lower than that claimed in earlier reports, and the predominant associated neurological symptomatology was found to be psychomotor retardation and headache (70%); 5) ICL is a developmental anomaly that does not require neurosurgical treatment and which does not usually change with the passage of time, although it is liable to increase because of adipose hypertrophy.

[Congenital brain tumors in children under 1 year old]. / Vrozhdennye opukholi golovnogo mozga u detei pervogo goda zhizni.

Secondary, paraventricular and, less frequently, primary tumors of the lateral and third ventricles usually of supratentorial localization prevailed among congenital tumors of the brain. They impair the drainage of cerebrospinal fluid and cause internal hydrocephalus. The clinical picture of congenital cerebral tumors is characterized by a manifest hypertensive-hydrocephalic syndrome and a severe general condition. The neurological symptomatics, the EEG and the Echo-EG usually yielded exact information on internal hydrocephalus but rarely helped in determining the localization and character of the tumor. Pneumoventriculography, angiography, computer tomography, and radioisotope diagnosis were methods which provided the most information. Pneumoventriculography, however, causes many complications and the method of isotope diagnosis and computer tomography is therefore preferable because it is sparing and safe for the use in children of the first year of life.

Current management approaches for congenital melanocytic nevi.

Lack of information and misinformation abounds regarding the potential risks of malignancy, management approaches, benefits of surgical intervention, follow-up strategies, and overall prognosis for individuals with congenital melanocytic nevi (CMN). This review is intended to provide answers to questions that frequently arise shortly after the birth of individuals with CMN, especially of larger types.

Giant supratentorial meningeal haemangiopericytoma in a newborn.

A brain tumour in a newborn is rare. The incidence of congenital tumours is 0.34 per one million births. We report a case of a huge right hemispheric tumour operated upon successfully in a child 5 days old, who had a total tumoural excision and an uneventful outcome with 5 months follow up. This tumour appears to be the first meningeal haemangiopericytoma described in a newborn. Adequate treatment and histological findings are discussed. meningeal haemangiopericytomas are now considered as originating in pericytes, in the light of recent immunopathological results, and are to be separated from true meningiomas.

Congenital meningioma.

The clinical, operative and autopsy findings associated with a huge meningioma in an 8-month-old boy are described. Especially noteworthy are the patient's age, the size of the tumour and the poorness of neurological symptoms.

Meningiomas in a male fetus from a hormonally dysequilibrated mother.

Congenital multiple meningiomas in a male fetus of 37 cm length are reported. The tumor was histologically a highly vascular meningioma. An unbalanced level of hormones before and at the beginning of the mother's pregnancy could be ascertained, which resulted in gestational abnormality.

Congenital meningeal sarcoma--a case report.

A 30-year-old multigravida woman was admitted to the obstetrical unit in the third trimester of gestation, because of a large-for-date uterus. Repeated ultrasonic examinations over a 2-week period revealed an increase in biparietal diameter far too large to be normal. This progressive asymmetrical hydrocephalus was diagnosed as caused by an intracranial space-occupying lesion in the middle cranial fossa. Because of the only minimal thickness of the cerebral cortex prognosis was judged so poor, that labor was induced. A stillborn female infant was delivered vaginally after an ultrasonically guided transabdominal and later transcervical encephalocentesis. Autopsy revealed hydrocephalus and section through the fixed brain showed that the mentioned mass was an undifferentiated meningeal sarcoma that filled the left middle cranial fossa. Microscopically the sarcoma was invading the brain tissue. Definitions of the term "congenital brain-tumors" vary among authors. "Congenital" can be interpreted as "derived from embryonal tissue" or as "originating in utero". Congenital intracranial neoplasms are very rare. Antenatally intracranial tumors have been reviewed by Wells, Solitare and Farwell. Only a few cases of congenital brain tumors arising in the meninges have been reported in these reviews. The genesis of the tumor is explained as derived from multipotential cells. The undifferentiated type of a meningeal sarcoma has a poor prognosis. Most meningeal sarcoma present with progressive hydrocephalus.(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital extracranial meningioma.

The authors report a case of congenital meningioma in a newborn. This tumour is extremely rare and only six cases have been reported in the literature. Those reported cases were mainly intracranial. This is the first case of a neonatal extracranial meningioma that was evaluated preoperatively by computed tomography and magnetic resonance imaging.

Sex hormone receptors in a congenital meningioma.

The authors report a case of a congenital meningioma in a male infant in whom receptor binding sites for progesterone were significantly elevated.

Giant congenital meningioma in a newborn.

The authors report the case of a giant meningioma in a 5-day-old newborn. Congenital meningiomas are extremely rare. Five newborns with meningiomas have been reported in the literature to date. This is the first case of a newborn with a giant meningioma with supra- and infratentorial and intraorbital components.