Pulmonary blastomas

Abstract

Pulmonary blastoma is a rare lung tumor composed of immature mesenchyme and/or epithelium that morphologically mimics embryonal pulmonary structure. The prognosis of these tumors is poor, and the clinical course is not readily predicted from histologic appearance. In this report, the clinical, gross, microscopic, and immunopathologic features of 52 cases are described, and prognostically important correlates are determined. Twenty-eight patients were women, and 24 were men. There was a unimodal age peak in the fourth decade; only two patients were younger than 10 years old, and both had biphasic blastomas. Forty-one percent of patients were asymptomatic. Chest radiography typically showed a peripheral or midlung mass without predilection for any lobe. Microscopically, tumors could be divided into two classes: those composed solely of malignant glands of embryonal appearance (well-differentiated fetal adenocarcinomas [WDFA], 28 cases) and those with a biphasic appearance (24 cases). The malignant epithelium contained cytokeratin, carcinoembryonic antigen, milk fat globulin, and often chromogranin; vimentin, actin, and less frequently desmin and myoglobin were present in malignant stromal cells. More often WDFA was a smaller tumor (less than 5 cm) than biphasic tumors (P less than or equal to 0.001). It was more likely to be asymptomatic (P less than or equal to 0.001), and it was less likely to show pleural effusion by chest radiography (P less than or equal to 0.01) or giant or bizarre tumor cells (P less than or equal to 0.001) or frequent (greater than or equal to 30 mitoses/10 high-power fields) mitoses in the microscopic sections (P less than or equal to 0.01). Only 14% of patients with WDFA died of their tumor; 52% of patients with biphasic tumors died (mean follow-up, 97 months and 49 months, respectively). For patients with WDFA, the presence of thoracic adenopathy by chest radiography (P less than or equal to 0.001) and metastasis at initial presentation (P less than or equal to 0.001), followed by tumor recurrence (P less than or equal to 0.01), were the factors most highly correlated with poor prognosis. For patients with biphasic tumors, tumor recurrence (P less than or equal to 0.001) was the most significant indicator of poor prognosis, followed by metastasis at initial presentation (P less than or equal to 0.05) and gross size of the tumor (greater than or equal to 5 cm) (P less than or equal to 0.05). These findings support the idea that histologic class and gross and clinical findings can be of prognostic value in pulmonary blastoma.

Aberrant Nuclear Localization and Gene Mutation of β-catenin in Low-Grade Adenocarcinoma of Fetal Lung Type: Up-Regulation of the Wnt Signaling Pathway May Be a Common Denominator for the Development of Tumors that Form Morules

“…The clinically significant feature common to most of these tumors with morular formation is that they are either benign (11,13) or of low-grade malignancy with a relatively favorable prognosis (3,4,8,32,35). This is in sharp contrast to another group of tumors with frequent ␤-catenin or APC mutations and high-grade malignancy with a poor prognosis, such as hepatoblastoma (40,41) and anaplastic carcinoma of the thyroid (42).…”

“…Subsequent studies clarified that L-FLAC/ WDFA is a relatively indolent tumor most prevalent in the fourth decade of life with a mild female predominance and a death rate of about 10% (2)(3)(4). The salient histopathologic features of L-FLAC/ WDFA are complex glandular structures with cells rich in glycogen, resembling the fetal lung airway epithelium or endometrioid carcinoma and morular formation with optically clear nuclei (OCN) that are rich in biotin (4,5).…”

Adult pulmonary blastoma: Report of an unusual malignant lung tumor

“…It was described for the first time in 1952 by Barnard as an "embryoma" and in 1961 was regularly classified by Spencer who established that the tumor arises from pulmonary blastema as tumors developed from fetal tissues [5,10] . The neoplasm was divided by Koss et al [11] into three groups: biphasic pulmonary blastoma, monophasic pulmonary blastoma with prevalent epithelial expression, and pleuropulmonary blastoma with mesenchymal expression [2,5,10,11] . In adults, the tumor presents itself as a large chest mass causing pain, hemoptysis, cough and dyspnea; however, up to 40% of patients may be asymptomatic [4] .…”

Pulmonary Blastoma

“…Çekilen akciğer grafisindeki görünüm üzerine ileri tetkikler yapıldı. PB tedavisinde tümörün cerrahi rezeksiyonu esastır (4,7,10). Ama hastaların çoğunluğunda vital yapıların invazyonu ya da metastazlar nedeniyle cerrahi yapılama-maktadır.…”