Résumé
@#Primitive neuroectodermal tumours (PNETs) occurring directly from the lung parenchyma without involvement of the chest wall or pleura are particularly unusual. We describe a 16-year-old male who presented with severe chest and back pain following a fall during high jump competition. Incidental finding of an opacity at the right lung upper lobe was seen on his chest radiograph. Computed tomography of the thorax demonstrated a lobulated hypodense lesion in the anterior segment of right upper lobe and subsequent biopsy confirmed the diagnosis of Ewing Sarcoma/ PNET following histologic and immunohistochemical examination. Whole-body positron emission computed tomography/computed tomography (PET/CT) revealed mild, homogenous F18-fluorodeoxyglucose (FDG) uptake in the lung lesion with no evidence of metastasis. He eventually underwent right thoracotomy and right upper lobectomy. Indeed, F18-FDG PET/CT is a valuable imaging method in demonstrating active focus of glucose metabolism in lung parenchymal lesion, for preoperative evaluation and as baseline scan for patient’s monitoring.
Résumé
We hereby report a rare case of primary primitive neuroectodermal tumour (PNET)/Ewing's tumour of the urinary bladder. Primary PNETs of the bladder are very aggressive neoplasms. They are extremely rare and only 12, as on July 2014, have been reported in the literature. A young 22-year old male patient, agricultural labourer by occupation, presented with complaints of urinary obstruction and haematuria of 1-month duration. A provisional diagnosis of small round cell tumour was considered on examination of the biopsy specimen. Subsequently, the panel of immunohistochemical markers CD-99, NSE, LCA, Desmin and vimentin were performed and finally reported as urinary bladder – PNET/Ewing's tumour – CD-99+.
Résumé
Askin tumour, a primitive neuroectodermal tumour of the thoracopulmonary region, is a rare tumour presenting in childhood. Its presentation in adults is rare. We report a case of an Askin tumour in an adult patient who presented to us with worsening breathlessness and vague chest pain. Investigations including immunohistochemistry confirmed the diagnosis of Askin tumour.
Sujets)
Adulte , Protocoles de polychimiothérapie antinéoplasique/administration et posologie , Biopsie , Tumeurs osseuses/traitement médicamenteux , Tumeurs osseuses/anatomopathologie , Tumeurs osseuses/physiopathologie , Évolution de la maladie , Humains , Poumon/anatomopathologie , Tumeurs du poumon/anatomopathologie , Métastase lymphatique , Mâle , Tumeurs neuroectodermiques primitives périphériques/anatomopathologie , /secondaire , Plèvre/anatomopathologie , Tumeurs de la plèvre/anatomopathologie , Tumeurs de la plèvre/secondaire , Sarcome d'Ewing/traitement médicamenteux , Sarcome d'Ewing/anatomopathologie , Sarcome d'Ewing/physiopathologie , Paroi thoracique/anatomopathologie , Tomodensitométrie/méthodes , Résultat thérapeutiqueRésumé
The concept of Primitive Neuroectodermal Tumor (PNET) has been evolving for many years, since its nomenclature has been done. A 3 years old boy presented with unable to stand and walk, vertigo, vomiting and visual disturbances. MRI of brain revealed suggestive of malignant tumour with intratumoral hemorrhage involving the frontotemporo- parietal region. A gross total removal of tumor was achieved through craniotomy. The pathological finding was consistent with PNET. This case is an exclusive one as it was huge size. Supratentorial PNETs are rare tumour and carry poor prognosis. Newer modalities of treatment should be tried to improve survival.