关键词: Mullerian type borderline epithelial neoplasm bullous emphysema child hydrocele placental transmogrification

Mesh : Child Male Female Humans Pregnancy Adolescent Placenta / pathology Pulmonary Emphysema / diagnosis diagnostic imaging Lung / pathology Emphysema / pathology Neoplasms, Glandular and Epithelial / pathology

来  源:   DOI:10.24953/turkjped.2021.5515

Abstract:
BACKGROUND: 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.
METHODS: 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.
CONCLUSIONS: 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.
摘要:
背景:胎盘肺部移位(PTL)是一种从无症状到严重肺部损害的临床频谱;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,很少能出现在胸部影像学上的结节或囊肿形成。患有巨大大疱性肺气肿的PTL有男性偏好,更常见的是单侧的,主要影响一个肺叶,但很少涉及一个以上的叶。
方法:这里我们报道一个13岁的男孩,表现为大疱性肺气肿,并伴有一个交界性睾丸肿瘤。他没有咳嗽的抱怨,痰,或者呼吸急促.五年前他有肺炎病史。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.因为两个睾丸都有鞘膜积液,所以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“苗勒型交界性上皮性肿瘤”,是卵巢浆液性交界性肿瘤的类似物。
结论:结论:我们报道了文献中最年轻的PTL病例,一种病理生理学未知的罕见疾病,表现为大疱性肺气肿和偶发苗勒型交界性上皮性肿瘤。诊断患有大疱性肺气肿的儿童的胎盘肺部转移非常重要,因为与其他囊性肺疾病相比,它是一种良性疾病,如果不存在其他恶性肿瘤,肺叶切除术或全肺切除术是治疗这种疾病的方法。
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