RESUMEN
Plexiform neurofibroma is a rare benign tumor developed from cells of the sheath of Schwann peripheral nervous system often associated with neurofibromatosis type 1 or Von Recklinghausen disease. Laryngeal involvement in neurofibromatosis is rare and usually presents with obstructive respiratory symptoms. Imaging including CT and MRI provides the diagnosis as well as the staging of this lesion, whose treatment is primarily surgical. We report the case of a plexiform neurofibroma of the larynx found in a 4-year-old child revealing a mass completely obstructing the laryngeal vestibule.
Asunto(s)
Disnea/etiología , Neoplasias Laríngeas/complicaciones , Neurofibroma Plexiforme/complicaciones , Obstrucción de las Vías Aéreas/etiología , Preescolar , Femenino , Humanos , Laringoscopía , Imagen por Resonancia Magnética , Estadificación de Neoplasias , Ruidos Respiratorios/etiología , Tomografía Computarizada por Rayos X , TraqueotomíaRESUMEN
We report the case of a 66-year-old patient with a pilomatrix carcinoma from the right submandibular region with pulmonary and cerebral metastases. The histological confirmation of the diagnosis has been based on the biopsy of the submandibular tumefaction which was rapidly growing. We tried at first a doxorubicine and cisplatine chemotherapy because of the considerable locoregional extension and the existence of pulmonary metastases. The patient response to three cures of chemotherapy was spectacular with a partial clinical response (75%) and a partially cleaned-up chest observed in the radiological evaluation. In the 5th cycle of chemotherapy following the same protocol, the patient presented a relapse with cerebral metastases. The patient received hypofractionated radiotherapy on the brain followed by etoposide and cisplatine chemotherapy, then oral vinorelbine. The patient died of progressive disease after 32 weeks.
Asunto(s)
Neoplasias Faciales/patología , Pilomatrixoma/secundario , Neoplasias Cutáneas/patología , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/secundario , Cisplatino/administración & dosificación , Terapia Combinada , Doxorrubicina/administración & dosificación , Etopósido/administración & dosificación , Neoplasias Faciales/diagnóstico , Neoplasias Faciales/tratamiento farmacológico , Resultado Fatal , Humanos , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Pulmonares/secundario , Masculino , Pilomatrixoma/diagnóstico , Pilomatrixoma/tratamiento farmacológico , Pilomatrixoma/radioterapia , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/tratamiento farmacológico , Vinblastina/administración & dosificación , Vinblastina/análogos & derivados , VinorelbinaRESUMEN
We report 4 cases of tuberculous osteitis of the ribs in patients aged 25 to 60 years. All patients had thoracic CT scan. Two cases presented as multifocal tuberculosis, involving nodes and lung in one case, a costo-verebral joint and lung in the second case. In two patients, the aspect was a misleading pseudo-tumor form with a primitive malignant bone tumor and infiltration of perivertebral and endocanalar soft tissue in one case of costo-transverse joint tuberculosis with neurologial disorder; in the other case, the rib affection presented as a breast tumor due to the involvement of anterior thoracic wall in a 50-year-old woman. In all cases, the radiological aspect was quite extensive osteolysis of the rib with involvement of endo- and/or extrathoracic soft tissue noted as cystic or tissue density. We underline the value of CT scan for a complete investigation of rib lesions, which enables characterization of soft tissues involvement, guiding biopsies and search for associated vertebral lesions.