Tumor > Malignant > Pulmblastoma

Pulmonary Blastoma:

Clinical:

An exceedingly rare tumor with a biphasic distribution (1st and 7th decades), although patients may present at any age. Men are affected more than women (2.5:1). It is typically peripheral and often large by the time of presentation. Histologically the lesion consists of both an epithelial and mesenchymal components and two subtypes have been described- biphasic and well differentiate fetal adenocarcinoma. Metastases are frequently found in patients with biphasic tumors- typically intrathoracic. Prognosis depends upon the histologic subtype- patients with the well-differentiated fetal adenocarcinoma subtype have a significantly better prognosis with a 10 year survival of 78% (compared to 8-14% ten year survival for the biphasic tumor).

X-ray:

The tumor usually presents as a large peripheral mass (typically well circumscribed) with abundant central necrosis and hemorrhage. The average size at presentation is 6 cm.

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