Pial invasion is an important predictor of tumor recurrence in benign and atypical meningiomas. In atypical meningiomas bone involvement and large peritumoral brain edema are associated with increased tumor progression.
Chordomas are rare midline axial skeletal neoplasms that typically present in adults. They are infrequent in childhood with typical localization in the spheno-occipital skull base. They are derived from remnants of the embryonic notochord. We present the case of 4 months old girl, who was born with „blueberry muffin” syndrome and was first negatively diagnosed for neuroblastoma and leukemia (two negative skin biopsies were performed) was admitted with axial laxity. In imaging testes there was a tumor of the scull base, metastases in the lungs and kidneys (that were not seen at previous assessments) and a small lesion in the heart. The third biopsy of skin lesion was performed and pathological examination revealed a neoplasm composed of cords, clusters, and chains of multivacuolated cells embedded within a myxoid matrix and separated by fibrous septa. No atypical and dedifferentiated features were present. Mitotic activity was not observed. Neoplastic cells showed the typical cytoplasmic immunostaining for EMA, S100 and cytokeratin AE1/AE3, strong nuclear brachyury expression, and retention of nuclear INI-1 expression. The diagnosis of chordoma was established. Neoplastic tissue and blood samples were obtained for molecular analysis using next generation sequencing, including germline mutations assessment (are ongoing). Chemotherapy as for soft tissue sarcomas was undertaken. Currently a patient is on treatment with improvement of neurological status.
Kikuchi-Fujimoto disease (KFD) is an extremely rare, self-limiting disorder of unknown etiology, with most cases occurring in Asia. The treatment of KFD is mainly supportive with NSAIDs or steroids in isolated cases. Its clinical presentation and histopathological findings may overlap with more common disorders, including malignancies such as Hodgkin Lymphoma (HL). It's essential to thoroughly reevaluate such diagnoses before initiating the treatment. Case presentation:A 6-year-old boy was admitted to the regional hospital's pediatric ward with a high fever, cervical lymphadenopathy, enlarged liver and spleen. Before admission, he was treated with antibiotics (clarithromycin and clindamycin) with no response. After exclusion of infectious origin in laboratory tests, the imaging studies and biopsies of bone marrow and cervical lymph nodes were performed. Based on the histopathological examination, the diagnosis of Kikuchi-Fujimoto disease was established. There were no further consultations prior to treatment with methylprednisolone. Three months later, on admission to Pediatric Oncology Department, the patient presented with generalized lymphadenopathy, jaundice, hepatosplenomegaly and ascites. Laboratory tests showed anemia, thrombocytopenia, hyperbilirubinemia, elevation of inflammatory markers and features of liver insufficiency. The histopathological specimens were reviewed in a reference center where diagnosis of Anaplastic Large Cell Lymphoma (ALCL) and HL was considered. The chemotherapy regimen for ALCL was administered. Soon after, the definitive diagnosis of HL was confirmed. Chemotherapy was modified resulting in significant clinical improvement. Conclusion:Every extremely rare diagnosis should be carefully reconsidered and confirmed in a reference center. Our case shows that misdiagnosis that entails inappropriate treatment may result in worsening of prognosis and life-threatening compilations for the patient.
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