Introduction: Ependymoma is a slow-growing tumor that arises from the ependymal cells that line the ventricles, the passages in the brain and the center of the spinal cord. It affects children and young adults. Case presentation: female patient, 10 years old, black and urban origin. He presents with a history of intense holocranial headache, visual analog scale 6/10 since September 2021 in the right hemicranium and suboccipital, and also associates dizziness plus instability in walking with lateropulsion on the right side and general malaise. Studies are carried out by different specialties where it is diagnosed by clinical and tomography imaging and its first surgery with excision of the lesion with a pathological diagnosis of anaplastic ependymoma of the posterior fossa (grade III). She is treated at the provincial pediatric hospital for radiotherapy planning, a simple computed axial tomography of the skull is performed and she undergoes microscopic surgery (approximately 95%). Complete treatment with radiotherapy. Then she is admitted to the pediatric oncotherapy service; no oncological therapeutic alternative can be offered since the patient's general condition does not allow it and she dies. Conclusions: Anaplastic ependymomas are rare tumors in pediatric patients, which require a detailed physical examination and clinical interpretation to obtain an early outcome of the disease. Complementary ones such as computerized axial tomography and magnetic resonance establish the diagnosis.
Ependymoma, Case report, Brain neoplasms, Neurosurgery, Pediatrics
Brain tumors are a heterogeneous group due to the different cell lines that originate from the central nervous system (CNS) itself or from metastases established in the brain. The first printed report on intracerebral tumor surgery in the world was that of Bennett and Godle, in 1884, in London, on a glioma. Interventions on this type of surgery were published at the same time in France, with Chipault, in Italy with Durante, in the United States with Keen, in Germany with Krause and Oppenheim, among others. In Mexico, Rafael Lavista performed the first surgery on an intracerebral neoplasm in 1891 [1], [2] .
CNS tumors in children represent the second most frequent cause of malignant tumors, which is an important cause of morbidity and mortality despite the advances achieved in their diagnosis, treatment and follow-up [3] .
Every year, between 1,300 and 1,500 new cases of childhood cancer are reported worldwide , accounting for 20%. In 2021, a total of 26,791 new cases of malignant tumors were diagnosed in Cuba, 141 of them in children under 19 years of age (²,4) .
Ependymoma is a slow-growing tumor arising from the ependymal cells lining the ventricles and passageways in the brain and central spinal cord . It affects children and young adults. Histologically, it corresponds to grade II of the World Health Organization (WHO) classification. The anaplastic variant corresponds to grade III. Ependymomas are the third most common brain tumor in children, after astrocytomas and primitive neuroectodermal tumors [5] .
The anaplastic form is even rarer, representing only 4 to 7% of all ependymomas and 26 to 30% of intracranial ependymomas. They have been reported to be more frequent in children around 5 years of age, and there is no gender prevalence . They usually present as solid-cystic or cystic lesions with thin walls that enhance after contrast administration. They may show heterogeneous foci with respect to hemorrhage (methemoglobin and hemosiderin), necrosis, and calcifications [6] .
The treatment of choice for ependymoma is surgery , and when this is not possible, treatment is completed with local conformal radiotherapy (linear accelerator) whether high or low grade, depending on the histological grade and the existence of postoperative lesion remnant . Chemotherapy has not been shown to be effective in either type of ependymoma. Survival is better and the chances of cure are much higher in a supratentorial anaplastic ependymoma with total resection than in a posterior fossa anaplastic ependymoma, with benign histology, with subtotal resection [7] .
Female patient, 10 years old, black schoolgirl, urban origin, product of vaginal delivery, Apgar 9-10, fully vaccinated, with no significant personal pathological history. She comes with a history of intense holocranial headache, visual analogue scale (VAS) 6/10 since September 2021 in the right and suboccipital hemicranium, which subsided with conventional analgesic, also associated dizziness plus instability when walking with lateral pulsion on the right side and general malaise, which is why she is taken for studies by different specialties where a space-occupying lesion in the right hemisphere is diagnosed by clinic and CT imaging. The first surgery is performed on November 25, 2021, with excision of the lesion and a pathological diagnosis of anaplastic ependymoma of the posterior fossa (grade III). After that, with a complication of surgical site infection, it is corrected. She is seen at the Provincial Pediatric Hospital for radiotherapy planning, starting in June, she receives two radiotherapy sessions and begins with headache and vomiting, a simple cranial computed tomography (CT) scan is performed, which shows a space-occupying lesion in the right posterior fossa, which collapses in the fourth ventricle, with dilation in addition to temporal horn and mild hydrocephalus, there is a poorly defined area at the level of the posterior cerebellum, with hypodense areas, in its periphery, which erases the gyri and sulci, it measures approximately 63 mm, a surgical resolution is decided which proceeds quickly, on July 7, 2022, she is operated on by microscopic surgery (approximately 95%), not everything is removed due to invasion of the lesion into the sinuses, which makes total excision difficult, with the above it is determined: anaplastic ependymoma of the right hemisphere posterior fossa postoperative recurrence. She concludes radiotherapy treatment.
Skin and mucous membranes: Normally colored and moist. There is evidence of an occipital keloid scar from the 2021 procedure; none currently present.
Respiratory system: audible breath sounds and vocal vibrations are preserved. No rales are heard. Respiratory rate: 20 minutes.
Cardiovascular system: Rhythmic heart sounds of good pitch and intensity. No murmurs are audible. Peripheral pulses are present and synchronous. Heart rate: 99/minute.
Abdomen: soft, depressible, non-tender to superficial and deep palpation. Air sounds present.
Subcutaneous cellular tissue: not infiltrated.
Central Nervous System on admission: Glasgow Coma Scale (GCS): 1/15 points, cerebellar ataxia, adiadochokinezi dysmetria, cerebellar gait, hypotonia, no motor or sensory defect, no meningeal signs, fundus without alterations.
Central Nervous System upon discharge: ECG: 1/15 points, dysmetria, hypotonia, apathy, abulia, emotional lability, no motor or sensory defect, no meningeal signs, fundus without alterations.
A simple cranial CT scan was performed. No epicranial collections were seen. The bone window revealed a loss of continuity at the level of the right occipital concha. No juxtadural collections were seen, and the subarachnoid space was present. There was no displacement of midline structures. A longitudinal hyperdense image extending to the frontal horn of the right lateral ventricle was present, consistent with external shunting. The ventricles and cisterns were present. At the infratentorial level, a well-defined, rounded, hypointense image measuring 36 x 34 mm was seen in the right hemisphere, consistent with the surgical bed ( FIGURE 1 ).
FIGURE 1 : Simple cranial computed tomography scan
-Contrasted nuclear magnetic resonance (NMR)
Sequences: T1 axial sagittal, T2 axial coronal sagittal, axial flair
Axial sagittal coronal contrast-enhanced T1-weighted image. At the intracranial, intraaxial infratentorial level, a rounded image with well-defined edges measuring 38 x 35 mm is observed, which is hypointense on T1, hyperintense on T2, hypointense on flair, with a peripheral hyperintense halo on flair and in the contrast-enhanced study, leptomeningeal enhancement is seen, and a rounded hypointense image measuring 9.2 x 9.6 mm, all located in the right cerebellar hemisphere ( FIGURE 2 A, FIGURE 2 B).
FIGURE 2 A: Magnetic resonance imaging. Axial view
FIGURE 2 B: Magnetic resonance imaging. Sagittal view.
Prior to surgery, the corresponding complementary examinations and informed consent were performed, then anesthesia induction was carried out, prophylactic antibiotics were given, the patient is transferred to the room in the supine position, general orotracheal anesthesia is administered with a modified parachute tube, after which, in the prone position, the Mayfield headrest is placed, marking of the two surgical sites for external derivation and the surgical approach to the posterior fossa, placement of shoulder, hip and support site wedges, profuse washing, asepsis and antisepsis, placement of sterile fields.
First, Frazier incision to control hemostasis, single early hole, shunt placement plus patency verification. Second, on a keloid scar after a Naffziger incision to control hemostasis, detachment of fibrosis, extension of craniectomy to the limits. Incision on an infiltrated dura, approaching the lesion with an electron microscope (95% is not removed), all due to invasion of the lesion into the sinuses, making total excision difficult, obtaining an autologous galea graft for hermetic closure of the dura. Then, dural traction sutures, repair of incised muscles, hemostasis control, placement of a subdermal drain through a counter-opening, closure by layers.
She was admitted to the pediatric oncology service with quadriplegia, loss of strength and loss of sphincter control, with marked loss of appetite. The case was discussed collectively with neurosurgery and the best supportive treatment was decided upon since she presented with an unresectable tumor recurrence that infiltrated the spinal canal. No alternative oncological therapy could be offered since the patient's general condition did not allow it and she died.
tumors are one of the most common neoplasms in children and one of the most frequent reasons for consultation in neurosurgery departments. Ependymomas are well-circumscribed glial tumors composed of small, uniform cells with a round nucleus in a fibrillar matrix. They are characterized by perivascular anucleated zones (pseudorosettes) and, in some cases, ependymal rosettes [8], [9] .
Anaplastic ependymoma, also known as malignant ependymoma, is considered a malignant glioma of ependymal differentiation and, compared with Grade II ependymomas, shows increased cellularity and mitotic activity, which are often associated with microvascular proliferation and necrosis. They most frequently occur in the posterior fossa [10] .
The WHO classifies ependymal tumors by degree of malignancy [11] :
Grade I: subependymoma and myxopapillary ependymoma.
Grade II: cellular, papillary, tanycytic, clear cell, and mixed ependymoma.
Grade III: anaplastic ependymoma, ependioma positive for a RELA fusion (grade II or III).
Anaplastic ependymoma is six times more common in children and adolescents than in adults, whereas the reverse pattern is evident for subependymomas and myxopapillary ependymomas. For adults, the proportional distribution pattern for subependymoma increases with age, whereas decreases with age are observed for myxopapillary ependymoma [12] .
Kuai et al [12] report that previous studies have shown that the presence of cysts or necrosis are characteristic of anaplastic ependymomas, especially supratentorial ones. Furthermore, cysts larger than 1 cm in diameter are also frequently observed in supratentorial cysts, whereas infratentorial cysts tend to be associated with solid neoplasms.
Holocranial headaches are frequent as primary manifestations in 50 patients (43.8%) and vomiting, according to the study by García-Espinosa [13] , because they constitute the earliest warning signs of a brain injury such as that caused by tumor growth, which, in addition to increasing pressure in the brain, interferes with normal functions. Similar symptoms were present in the present case.
According to Sierra-Benítez et al [14] , the cornerstone of treatment is surgery followed by local radiotherapy, ensuring that there is no leptomeningeal seeding. Improved surgical techniques and irradiation therapies have resulted in improved 5-year disease-free survival rates (greater than 70%). The combination of surgery, radiotherapy, and chemotherapy was used in 59.09% of their cases. Chemotherapy was not used in the present case.
Magnetic resonance imaging (MRI) in particular has emerged as the most frequently used modality. It is the first-line diagnostic test for detecting brain tumors, as it allows for more precise images of the tumor, both in terms of its characteristics and location [15] . The patient underwent various sequences of this study to complement the diagnosis of the disease.
Anaplastic ependymomas are rare tumors in pediatric patients, requiring a detailed physical examination and clinical interpretation to obtain an early diagnosis. Complementary tests such as computed tomography and magnetic resonance imaging establish the diagnosis, and surgery, accompanied by radiotherapy and chemotherapy, allows for a favorable outcome.
DMH: conceptualization, resources, supervision, project administration, methodology design, writing-original draft, research.
EAHG: research, writing-original draft, formal analysis, visualization and editing.
AGPD: research, writing-original draft, formal analysis, methodological advice.
LLDM: resources , project management and research.
The authors declare that there are no conflicts of interest.
The authors did not receive funding for the development of this article.
Copyright 2024 Déborah Mitjans Hernández et al.
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