Inflammatory myofibroblastic tumor of the lung involving the central nervous system in pediatric patients.

INTRODUCTION: The inflammatory myofibroblastic tumor (IMT) is a very rare lesion with an incidence of less than 0.1% of total neoplasms and with main affection in the lungs. Involvement in the central nervous system is extremely rare, but with a much more aggressive course than IMT diagnosed in the rest of the body. We report the 2 cases presented in our neurosurgery department to date; both were treated satisfactorily without intercurrences in 10 years of follow-up. HISTORICAL BACKGROUND: The World Health Organization described the IMT as a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. CLINICAL PRESENTATION: Clinical manifestations of patients with CNS IMT vary and may consist of headache, vomiting, seizures, and blindness. Seizures are the most common symptom in patients with focal lesions. DIAGNOSIS: The true origin of this entity remains to be elucidated, but to date, etiologies ranging from chromosomal alterations to autoimmune or postinfectious mechanisms have been described. Due to its rarity and non-specificity in imaging, the final diagnosis of IMT in the brain parenchyma relies on pathological examination. MANAGEMENT: Treatment options are controversial and include total or subtotal removal, high-dose steroids, and radiation therapy. In the last decade, the development of ALK Tyrosine Kinase Inhibitors allows the possibility of chemotherapy in those patients harboring ALK mutations. CONCLUSION: IMT is a rare tumor that can exceptionally be found in the CNS. The cause is still unknown although the different studies focus on a neoplastic origin. The diagnosis is based in the use of different modalities of imaging and with histological confirmation. Optimal management is gross total resection whenever possible, is the only established curative treatment. Further research with longer follow-up is needed to clarify the natural history of this rare tumor.

Hemorrhagic cerebral proliferative angiopathy in two pediatric patients: case reports.

Cerebral proliferative angiopathy (CPA) is an infrequent vascular malformation. It is composed of a nidus, arterial feeders, and venous drainage. Some special features differentiate it from the common arteriovenous malformations (AVM). The nidus has normal cerebral tissue intermingled and occupies a large portion of the brain. There is a frequent transdural arterial supply. Arterial feeders and draining veins are of small caliber concerning the nidus size. Ischemic strokes are the most common clinical event. Intracranial bleeding when occurs has a worse prognosis due to its high recurrence rate. We have presented two cases of CPA in pediatric patients who required surgical resolution of their acute episode.

Neuroendoscopic lavage for the treatment of pyogenic ventriculitis in children: personal series and review of the literature.

INTRODUCTION: Pyogenic ventriculitis is a severe infection of the central nervous system with serious and often irreversible consequences in the quality of life of patients. Its treatment is difficult due to the impossibility of achieving sterility of cerebrospinal fluid (CSF) and the physiological characteristics promptly. Several treatment options have been described, from prolonged antibiotic treatments to placement of ventricular drains with continuous irrigation and puncture reservoirs. We propose an aggressive and minimally invasive treatment with neuroendoscopic lavage (NEL). METHODS: Retrospective and descriptive study. We analyzed the NEL performed in our hospital for pyogenic ventriculitis between 2011 and 2020. A total of 16 patients were found; 2 of them lost follow-up, so they were not included. All patients had a diagnosis of pyogenic ventriculitis, either due to the macroscopic characteristics of the CSF or due to imaging criteria. Between 1 and 3 NEL were performed per patient until obtaining sterility and normalization of protein and cell counts of CSF. RESULTS: The average age was 38 months (2 months to 16 years). Ten patients were female and 4 were male. Sixty-four percent of germs in cultures corresponded to gram-negative and polymicrobial flora. The average number of days until the first sterile CSF post-NEL was 3.8 days (0 to 10 days). The NEL produced a significant improvement in the characteristics of the CSF compared to the pre-NEL. The mean pre-NEL of CSF protein levels was 907 mg/dl (123-4510 mg/dl) compared with the post-NEL of 292 mg/dl (38-892 mg/dl) with a p-value = 0.0076. Regarding cellularity, statistically significant results were also achieved (p-value = 0.0011) with a pre-surgical cellularity of 665 elements/mm3 (4-3090 elements/mm3) compared with 57 elements/mm3 (0-390 elements/mm3) post-NEL. Of the patients, 85.7% had a shunt prior to the onset of ventriculitis and the average number of days until the new shunt was 36.56 days (17-79 days), with a total hospitalization days ranging from 22 to 170. CONCLUSIONS: NEL allows rapid sterilization of CSF, decreasing the deleterious effect of infection in the CNS more rapidly compared to other types of conventional treatment.

Ruptured dermoid cyst in the Meckel's cave presenting with trigeminal neuralgia in a pediatric patient: a case report.

Intracranial dermoid cysts are benign congenital slow growing masses. They account for less than 1% of all primary intracranial lesions. They can be asymptomatic and appear incidentally on brain images. However, there are some dermoid cysts that, depending on the location, can cause a variety of symptoms, especially if they compress vital structures. Rupture of intracranial dermoid cysts is relatively uncommon; the incidence is 0.18%. Dermoid cyst in children appears in the posterior fossa, while in adults is more common to find them in the sellar, temporal, and frontobasal region. The Meckel's cave is not a frequent location for these lesions and there are only a few cases in the adult population reported in the literature. We presented a 12-year-old patient with a dermoid cyst in the Meckel's cave who first presented with symptoms of a ruptured cyst but eventually evolved with a trigeminal neuralgia. We decided to do a transzygomatic approach and a middle fossa pealing to locate and excise the lesion. After surgery, the patient resolved the symptoms. Surgery is recommended in symptomatic lesions although surgical decision-making should consider the region where the cyst is located to achieve a safe, maximal resection without adding any further damage.