Methods
A 9-year-old caucasian boy was admitted to our Oncology Department with a history of seizures, asthenia and dizziness from two weeks. No abdominal and lung involvement was detected on a total body CT scan. Brain MRI showed a wide right parieto-occipital edema and two isointense lesions on the right side of the cerebral falx, and in the upper surface of the ipsilateral tentorium (Fig. 1A-D). Radiological signs oriented for malignant meningiomas. Electroencephalogram showed slow cerebral electrical activity on the right temporal-occipital regions. CSF and bone marrow cytopathological examinations detected no tumor infiltration. Thus, the multidisciplinary neuro-oncological pediatric board discussed the case and decided for surgical approach. A complete surgical excision of the falx-tentorial lesions was performed with the aid of neuronavigation system, fluorophores and intraoperative neurophysiological monitoring. Histologically, the tumor showed a diffuse proliferation of large cells, with markedly atypical nuclei and abundant eosinophilic cytoplasm (Fig. 2A). There were many neoplastic cells characterized by bean-shaped nuclei and an eosinophilic peri-nuclear area (Fig. 2B). The tumor cells extensively infiltrated the surrounding brain tissue and focally the meninges. Tumor cells were positive for CD30 (Fig. 2C), EMA (Fig. 2D) and ALK, also showing positivity for T-cell markers (CD3, CD4 and CD5). One week after surgery, the boy became febrile with no signs of infection. A18F-FDG PET/CT scan showed a pathological uptake in supra- retro clavicular, mediastinal, ilar, peribronchial, paraesophageal, paraaortic and mesenteric fields, and multiple enlarged lymph nodes. T (2;5) (p23; q35) NPM-ALK RT-PCR was negative both in bone marrow and peripheral blood. Thus, we decided to treat the patient with 6 chemotherapy cycles (including corticosteroids, methotrexate, cytarabine, cyclophosphamide, daunomycin, ifosfamide, etoposide, vindesine, vincristine), plus CNS prophylaxis with intrathecal injections of methotrexate, cytarabine and prednisolone, according to the AIEOP LNH-97 protocol, high risk group. The disease MRI reassessment demonstrated no signs of residual disease. Chest and abdomen CT scan detected a lymph nodes shrinkage, and PET-CT scan showed no pathological enhancement. Complete disease remission was achieved at the end of chemotherapy, and no evidence of disease recurrence emerged at one-year follow-up.
We’ve conducted a systematic review following the ENTREQ framework. This methodology was chosen as it better describes qualitative research according to the guidelines for reporting systemic rewiews at: http://www.cochrane.de/de/LeitlinienForschungsberichte. A case-based literature search about pediatric cases of CNS ALCLs was performed. The main search was conducted through PubMed and MeSH Database and the terms used were “ALCL”, “ALCL” AND “brain” OR “ALCL” AND “brain” AND “pediatrics”, “ALCL” AND “CNS”. Only full text, published between 2002 and 2022 in peer-reviewed journals, were included. A systematic approach to the collected data and a collegial discussion between the authors led to a final version of the manuscript.