We describe a case of intramedullary melanocytic schwannoma (MS) studied by means of fine-needle aspiration cytology (FNAC). The main cytologic features were (1) large three-dimensional clusters overshadowed by heavy pigment deposits which tested positive for Fontana Masson (and bleached with potassium permanganate), HMB-45, Vimentin, and S-100 protein; (2) loose syncytial monolayered sheets with very little pigment deposit; and (3) isolated spindle cells with polarized nucleus and tapered, thin cytoplasmic ends. Both the cytologic features and the immunophenotypic profile were nonspecific and on their own were insufficient to allow the diagnosis of MS to be reached. Clinical data and the magnetic resonance image (MRI) in conjunction with the cytologic findings did suggest the diagnosis of intramedullary MS.
Pleuropulmonary blastoma (PPB) is a rare malignancy of childhood. It often represents a manifestation of a hereditary tumor predisposition syndrome (DICER1 syndrome). Because of its malignant potential, surgical resection of cystic lung lesions is recommended in germline DICER1 mutation carriers. We present a case of a 3-year-old male child with type III PPB successfully managed with ifosfamide, vincristine, actinomycin-D, and doxorubicin (IVADo) chemotherapy and surgery. A heterozygous germline pR688X mutation of DICER1 gene was demonstrated. Six years after primary diagnosis, several small lung cysts remained stable without further therapy. The management of residual asymptomatic lung cysts represents a clinical challenge in these patients.
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