II конгресс детских врачей РУз с международным участием
«Актуальные вопросы практической
педиатрии»
281
CLINICAL CASE: PLEUROPULMONARY BLASTOMA
Isabayeva A.S.
S.D. Asfendiyarov Kazakh National Medical University
Tulebayeva A.B.
S.D. Asfendiyarov Kazakh National Medical University
Tashenova G.T.
S.D. Asfendiyarov Kazakh National Medical University
Saduova A.T.
S.D. Asfendiyarov Kazakh National Medical University
Bulabaeva G.E.
Scientific Center of Pediatrics and Pediatric Surgery
Almaty, Kazakhstan
Keywords:
Pleuropulmonary blastoma, embryonal tumor, children
Introduction
Pleuropulmonary blastoma (PPB) is a rare, aggressive, and malignant
tumor arising from the lungs or pleura. It is typically diagnosed in children under
5 years of age and is characterized by an unfavorable prognosis. Pathologically,
PPB includes mesenchymal elements (undifferentiated cartilaginous, muscular
cells, fibroblasts of varying degrees of differentiation) and epithelial cells.
PPB classification proposed by L.P. Dehner, distinguishes three types of tumors:
Type I
–
with cystic areas and a favorable prognosis; Type II
–
mixed type,
including both cystic and solid areas; Type III
–
solid type, characterized by more
complex treatment.
Study Objective
To describe a clinical case of pleuropulmonary blastoma.
Materials and Methods
A 17-year-old patient, D., presented with complaints of cough, sweating,
difficulty breathing, dyspnea, oxygen dependency, subfebrile temperature for
2 weeks, weight loss up to 3 kg, fatigue, weakness, lethargy. These clinical
manifestations appeared since September 2023, following a respiratory viral
infection. He was hospitalized with suspicion of tuberculosis, but tuberculosis
tests were negative. Subsequently, the patient was referred to the Scientific
Center of Pediatrics and Pediatric Surgery for further examination. He underwent
a comprehensive examination, including assessment of laboratory parameters,
ECG, ultrasound of the pleural cavity, chest X-ray, computed tomography of the
chest (CT chest), computed tomography of the abdominal organs (CT abdomen),
transthoracic needle biopsy, histology, cytology, microscopy, as well as
immunohistochemical examination.
II конгресс детских врачей
РУз с международным участием
«Актуальные вопросы практической
педиатрии»
282
Results
CT chest revealed fibrous thickening of the pleural leaflets with
calcifications, as well as areas of consolidation in the projection of S3 on the right
and S2 on the left. Fluid was detected in both pleural cavities and pericarditis, as
well as mediastinal and retroperitoneal lymphadenopathy. CT abdomen revealed
calcified formations in the retroperitoneal space and punctate calcifications in the
projection of the adrenal glands. Cytological and histological examinations
showed a metastatic undifferentiated pleural tumor with solid-nested structure.
Immunohistochemical examination confirmed the diagnosis of PPB Type III.
Conclusion
In the everyday practice of oncologists-hematologists, general
practitioners, patients with pleuropulmonary blastoma are extremely rare. Given
the high degree of malignancy and low incidence rate of this tumor, it is necessary
to increase attention to the diagnosis and treatment of patients suspected of
having this condition. Implementation of molecular-genetic studies, such as
DICER1 mutation detection, is necessary to improve early diagnosis and
consequently treatment of PPB.
