Departments of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Türkiye.
Departments of Pathology, Hacettepe University Faculty of Medicine, Ankara, Türkiye.
Turk J Pediatr. 2022;64(5):964-969. doi: 10.24953/turkjped.2021.5515.
Placental transmogrification of the lung (PTL) is a clinical spectrum varying from asymptomatic to severe pulmonary impairment; such as recurrent pneumothorax, bronchopneumonia, respiratory distress syndrome and chronic obstructive airway disease. PTL usually presents as a bullous lesion, and rarely can appear in nodule or cyst formation on chest imaging. PTL with giant bullous emphysema has a male preference, is more commonly unilateral and mostly affects one lobe, but can rarely involve more than one lobe.
Here we report a 13-year-old boy presenting with bullous emphysema and coexisting with a borderline testicular tumor. He had no complaints of cough, sputum, or shortness of breath. He had a past medical history of pneumonia five years ago. In order to elucidate the underlying lung pathology, a wedge lung biopsy was performed and the patient was diagnosed with PTL. Scrotum ultrasonography was performed because of hydrocele in both testes, and bilateral epididymal cysts with papillary solid projections were reported. Pathological examination of the epididymal tumor revealed a Mullerian type borderline epithelial neoplasm which is an analogue of the ovarian serous borderline tumor.
In conclusion, we reported the youngest PTL case in the literature, a rare disease with unknown pathophysiology, presenting as bullous emphysema and coincidental Mullerian type borderline epithelial neoplasm. It is important to diagnose placental transmogrification of the lung in a child with bullous emphysema because compared to other cystic lung diseases it is a benign disease and if no additional malignity exists, lobectomy or pneumonectomy is the cure for the disease.
肺胎盘转化（PTL）是一种临床谱，从无症状到严重的肺损伤不等；例如复发性气胸、支气管肺炎、呼吸窘迫综合征和慢性阻塞性气道疾病。PTL 通常表现为大疱性病变，在胸部影像学上很少出现结节或囊肿形成。伴有巨大大疱性肺气肿的 PTL 具有男性偏好，更常见于单侧，主要累及一叶肺，但很少累及一叶以上。
这里我们报告了一个 13 岁男孩，表现为大疱性肺气肿，并伴有交界性睾丸肿瘤。他没有咳嗽、咳痰或呼吸困难的症状。他有五年前肺炎的既往病史。为了阐明潜在的肺部病理学，进行了楔形肺活检，患者被诊断为 PTL。由于双侧睾丸积水，进行了阴囊超声检查，报告双侧附睾囊肿伴乳头状实性突起。附睾肿瘤的病理检查显示为“Mullerian 型交界性上皮性肿瘤”，这是卵巢浆液性交界性肿瘤的类似物。
总之，我们报告了文献中最年轻的 PTL 病例，这是一种罕见的疾病，其发病机制尚不清楚，表现为大疱性肺气肿和偶然的 Mullerian 型交界性上皮性肿瘤。对于有大疱性肺气肿的儿童，诊断肺胎盘转化非常重要，因为与其他囊性肺部疾病相比，它是一种良性疾病，如果没有其他恶性肿瘤存在，肺叶切除术或全肺切除术是治愈该病的方法。
