Journal of Tumour Research & Reports

Pulmonary Blastoma: Understanding the Challenges of Diagnosis and Treatment

Min Fang^* Department of Radiation Oncology, Chang Gung University, Kaohsiung, Taiwan
^*Correspondence: Min Fang, Department of Radiation Oncology, Chang Gung University, Kaohsiung, Taiwan, Email:

Received: 24-May-2023, Manuscript No. JTRR-23-22042; Editor assigned: 26-May-2023, Pre QC No. JTRR-23-22042 (PQ); Reviewed: 12-Jun-2023, QC No. JTRR-23-22042; Revised: 20-Jun-2023, Manuscript No. JTRR-23-22042 (R); Published: 28-Jun-2023, DOI: 10.35248/2684-1614.23.8.195

Description

Pulmonary blastoma is a rare and aggressive form of primary lung cancer. It primarily affects adults, with a slight predilection for males, and presents significant diagnostic and therapeutic challenges due to its rarity and diverse histological features.

Clinical presentation

Pulmonary blastoma can manifest with a wide range of symptoms or may be incidentally detected on routine imaging studies. The most common presenting symptoms include cough, hemoptysis (coughing up blood), chest pain, dyspnea (shortness of breath), and recurrent respiratory infections. However, some patients may remain asymptomatic until the disease reaches an advanced stage. Given its non-specific symptoms and rarity, pulmonary blastoma is often misdiagnosed as other lung malignancies or benign conditions.

Pathological features

Pulmonary blastoma is classified into three subtypes based on its histology. The subtypes are biphasic, monophasic, and well differentiated fetal adenocarcinoma. The biphasic variant, the most common form, consists of both mesenchymal and epithelial components. The mesenchymal component often exhibits immature blastematous cells, while the epithelial component can resemble various lung tumor types such as adenocarcinoma or squamous cell carcinoma. The monophasic variant predominantly displays a sarcomatoid or carcinomatoid appearance. The well-differentiated fetal adenocarcinoma subtype, less common than the biphasic variant, resembles fetal lung tissue with primitive epithelial structures.

Diagnostic approaches

The diagnosis of pulmonary blastoma involves a combination of imaging studies, histopathological examination, and immunohistochemistry. Chest X-ray and Computed Tomography (CT) scans may reveal a solitary mass lesion with irregular borders or infiltrative growth. However, these findings are nonspecific and can resemble other lung malignancies. A definitive diagnosis requires a histopathological examination of a tissue sample obtained through a biopsy or surgical resection. Immunohistochemical stains can aid in distinguishing pulmonary blastoma from other lung tumours, as they often express specific markers such as TTF-1 (Thyroid Transcription Factor 1), WT1 (Wilms Tumour 1), and p63.

Prognosis and staging

The prognosis of pulmonary blastoma is generally poor, primarily due to its aggressive nature and a propensity for metastasis. The tumour frequently invades nearby structures and may spread to regional lymph nodes, liver, brain, bones, and distant organs. Staging is based on the tumour size, extent of invasion, lymph node involvement, and presence of distant metastasis. The most commonly used staging system is the TNM (Tumour, Node, and Metastasis) classification. Due to the rarity of pulmonary blastoma, there is limited data regarding its specific staging and prognostic factors, making individualized treatment challenging.

Treatment options

Due to the rarity of pulmonary blastoma, there is no established consensus regarding the optimal treatment approach. Treatment strategies are often extrapolated from those used for other lung cancers, such as surgery, chemotherapy, and radiation therapy. Surgical resection is considered the primary treatment modality for localized disease, with the aim of achieving complete tumour removal. Adjuvant chemotherapy and/or radiation therapy may be considered to reduce the risk of recurrence or for unresectable tumours. The effectiveness of targeted therapies and immunotherapies in the management of pulmonary blastoma is currently under investigation.

Pulmonary blastoma is an exceedingly rare primary lung malignancy with complex histopathological features and an aggressive clinical course. Its rarity poses significant challenges in diagnosis, treatment, and prognosis. The biphasic subtype is the most common, while the monophasic and well-differentiated fetal adenocarcinoma subtypes are less frequently encountered.

Accurate diagnosis requires a multidisciplinary approach involving radiological imaging, histopathological examination, and immunohistochemistry. Further research is needed to enhance our understanding of this rare malignancy and to develop standardized treatment guidelines to improve patient outcomes.