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.