ABSTRACT
Pleuropulmonary blastoma (PPB) is a rare embrionary mesenchymal neoplasm but is recognized as the most common pulmonary malignancy of childhood. It may present metastasis to the brain and also be indicative of other neoplasms in affected individuals or in their relatives. Being such a rare disease, it is considered a difficult diagnosis to be made. A 3-year-old female presented with fever and respiratory distress. At first, she had been treated for pneumonia with antibiotics for 14 days in another hospital with no response. Computed tomography (CT) scan showed a right lung/mediastinal mass. The patient was referred to our institution and a new CT-scan evidenced a complex mass of irregular borders, cystic areas and solid projections, along with a right pneumotorax. The mass was biopsied and hematoxylin-and-eosin (HE) stained histological sections showed a neoplasm composed of small and round cells with hyperchromatic nuclei and scant cytoplasm. The immunohistochemical profile demonstrated positivity for desmin, myogenin and Myo-D1, suggesting the diagnosis of rhabdomyosarcoma. After two weeks of hospitalization, the patient was clinically stable and initiated the first chemotherapy cycle. Surgical resection of the mass was performed and the HE slides demonstrated a neoplasm composed of anaplastic and condrossarcomatous cells with extensive necrosis. The correlation of clinical data, radiological and morphological features were conclusive of a PPB type II. The recognition and diagnosis of this entity is of great importance due to its clinical and prognostic particularities.
Subject(s)
Humans , Pulmonary Blastoma/diagnosis , Pleural Neoplasms/diagnosis , Lung Neoplasms/diagnosis , ImmunohistochemistryABSTRACT
Plasmocitoma extramedular é uma doença imunoproliferativa e monoclonal das células B. Trata-se de uma entidade rara, sendo que a maioria ocorre na região da cabeça e pescoço, mais freqüentemente na nasofaringe e seios paranasais. A localização na orofaringe é muito rara. É descrito um caso raro de uma paciente do gênero feminino, de 44 anos de idade, com plasmocitoma extramedular solitário localizado em base de língua, diagnosticado por biópsia e tratado com radioterapia exclusiva.
Extramedular plasmocytoma is a B cell monoclonal immunoproliferative disease. It is a rare entity which usually occurs in the head and neck area, commonly on rhinopharynx and paranasal sinuses. The ocurrence in oropharynx is very rare. It is presented a case of 44-year-old woman with solitary extramedular plasmocytoma loacated on tongue base, diagnosed by biopsy, who underwent exclusive radiotherapy treatment.