RESUMEN
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease with a poor prognosis. It frequently affects the skin; indeed, dermal lesions may be the first clinical manifestation. We report three cases of BPDCN where the patients presented with skin lesions and describe the clinical, histopathological and immunohistochemical findings, its molecular characteristics and metastatic work-up. One of the patients remains in a clinical trial with IMGN632, a molecule directed against CD123, while the other two patients died after different therapeutic regimens. BPDCN is a complex diagnostic challenge which, together with its poor prognosis, requires close clinical-pathological cooperation in order to accelerate its diagnosis and offer early therapeutic alternatives with drugs directed against specific molecular targets.
Asunto(s)
Patología Clínica , Neoplasias Cutáneas , Humanos , Enfermedades Raras , Células DendríticasRESUMEN
Inflammatory myofibroblastic tumor is a neoplasm with uncertain behavior. We describe a case in a 66-year-old female who underwent resection of a left atrial tumor suspected to be a cardiac myxoma which was subsequently diagnosed as an inflammatory myofibroblastic tumor. After three years' follow-up the patient underwent a second operation to remove tumoral occurrence in the right atrium, diagnosed as an intimal sarcoma. It cannot be confirmed whether the tumoral recurrence with a different diagnosis (intimal sarcoma) was a progression from the primary tumor or the metachronous appearance of a spontaneous sarcoma.
Asunto(s)
Apéndice Atrial , Neoplasias Cardíacas , Sarcoma , Femenino , Humanos , Anciano , Sarcoma/diagnóstico , Sarcoma/patología , Sarcoma/cirugía , Diagnóstico Diferencial , Atrios Cardíacos/patología , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugíaRESUMEN
The majority of gastric neoplasms are of epithelial type. Stromal tumours are the next most frequent and are most commonly gastrointestinal stromal tumours, followed by leiomyoma and schwannoma. We present an exceptional case of a patient with a gastrointestinal stromal tumour with suspicion of residual gastric disease, which was diagnosed post-operatively as a schwannoma.