Modern Pediatrics. Ukraine. (2026).3(155): 159-164. doi: 10.15574/SP.2026.3(155).159164
Kalinchuk O. O.¹, Kulyk О. М.^1,2, Yakhnyk O. F.¹, Korol Y. I.³, Miskiv I. P.¹, Konoplitskiy D. V.¹, Karpiv A. I.²
¹CC «First Lviv Territorial Medical Union» Subdivision «St. Nicholas Hospital», Ukraine
²Danylo Halytsky Lviv National Medical University, Ukraine
³CC «First Lviv Territorial Medical Union» Subdivision «St. Pantelemon Hospital», Ukraine

 For citation: Kalinchuk OO, Kulyk ОМ, Yakhnyk OF, Korol YI, Miskiv IP, Konoplitskiy DV, Karpiv AI. (2026). A clinical case of single-moment removal of a left lung and left atrium lumbar in a child. Modern Pediatrics. Ukraine. 3(155): 159-164. doi: 10.15574/SP.2026.3(155).159164.
Article received: Dec 15, 2025. Accepted for publication: Apr 13, 2026.

Aim – to present a rare clinical case of successful one-stage surgical management of type III pleuropulmonary blastoma (PPB) with tumor extension through the pulmonary veins into the left atrium and to highlight the role of a multidisciplinary surgical approach.
Clinical case. A 5-year-old girl with a history of cystic nephroma of the right kidney, suggestive of a possible DICER1-associated tumor predisposition syndrome, was diagnosed with a large mass in the lower lobe of the left lung during follow-up evaluation. Histopathological examination confirmed type III PPB. Despite multimodal chemotherapy, the disease progressed with invasion of the pulmonary veins and formation of a tumor thrombus extending into the left atrium, creating a high risk of mitral valve obstruction and sudden hemodynamic compromise. Following multidisciplinary team discussion, the patient underwent one-stage radical surgery via a clamshell thoracotomy. The procedure included left pneumonectomy and complete removal of the intracardiac tumor thrombus under cardiopulmonary bypass with cardioplegic cardiac arrest. Histopathological analysis confirmed type III PPB with metastatic involvement of regional lymph nodes. The postoperative course was uneventful. After completion of protocol-based chemotherapy, the patient remained alive and free of local recurrence or distant disease progression during a two-year follow-up.
Conclusions. PPB with intracardiac extension is an exceptionally rare and life-threatening condition requiring prompt multidisciplinary management. Simultaneous pneumonectomy and removal of the left atrial tumor thrombus under cardiopulmonary bypass may provide safe radical treatment in carefully selected patients. This case also emphasizes the importance of considering DICER1 syndrome in children presenting with both cystic nephroma and PPB, as early genetic evaluation may facilitate surveillance and management of associated neoplasms.
The study was conducted in accordance with the principles of the Declaration of Helsinki. Informed consent was obtained from the patients.
The authors declare the absence of a conflict of interest.
Keywords: pleuropulmonary blastoma, children, DICER1 syndrome, left atrium, tumor thrombus, pneumonectomy, cardiopulmonary bypass.

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