Pleuropulmonary blastomas (PPBs) are very rare, highly aggressive, dysembryonic neoplasms of thoracopulmonary mesenchyme. These have been reported in the pediatric population and account for only 0.5%–1% of all primary malignant lung cancers. They normally arise from lung tissue, however rarely the parietal pleura may be the tissue of origin (extra pulmonary PPB) which are extremely rare. Common age of presentation is three to 4 years. The prognosis is poor with distant metastasis to central nervous system and bone with survival rate of approximately 42.9% at 5 years. They are managed by aggressive multimodal therapies including surgery and chemotherapy. We report a case of a 3-year-old male child with Type 2 PPB of the left hemithorax, managed by surgical excision of the mass and adjuvant chemotherapy.
Lungs are divided into lobes by the oblique and the transverse (horizontal) fissures. The fissures may be complete or incomplete and they may be absent. The fissures facilitate the movement of the lobes in relation to one another, which accommodates the greater distension and movement of the lower lobes during respiration. Knowledge of their position is necessary for the appreciation of lobar anatomy and thus for locating the bronchopulmonary segments which is significant both anatomically and clinically. Hence, the awareness of their variations is essential in performing lobectomy and in segmental resection. It could also be of significance in interpreting radiological images. Materials and Methods: Study on abnormal lobar pattern and position of fissures in human lungs was carried out in the department of anatomy SVS Medical College, Mahaboobnagar, India, With 50 human lungs (30 adult, 20 foetus) were examined by standard dissection method. Results: Present study, the right lungs have incomplete horizontal fissures 50%, complete horizontal fissure 31.25%, absent of horizontal fissure 18.75%. Complete oblique fissure 87.5%, incomplete oblique fissure 12.5% and in left lungs complete oblique fissure 57.14% , incomplete oblique fissure 42.86% and also specimen no 13 in adult Right lung , specimen no 6 in foetus Right lung were showed accessory fissure because of it the right lung divides in to 4 lobes and in these specimens the left lungs were normal. Discussion: Anatomical knowledge of anomalous lobar pattern of the lungs is important for identifying bronchopulmonary segments. Many times, radiologists may misinterpret an X-ray or CT scan. Anatomical knowledge of such variations is helpful for Thoracic surgeons performing lobectomy and clinicians for managing certain diseases that are limited to a single lobe or segment. Many a times the accessory fissures fail to be detected on CT scans, because of their incompleteness, thick sections and orientation in relation to a particular plane. Conclusion: knowledge of abnormal fissures and lobes are clinically important for surgeons performing lobectomy and also academic interest to all medical personnel.
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