Ghosh Moupali, Islam Nelofar, Ghosh Arindam, Chaudhuri Priyanka Maity, Saha Koushik, Chatterjee Uttara
a Dept. of Pathology , Institute of Post Graduate Medical Education and Research and SSKM Hospital (IPGME&R & SSKM) , Kolkata , India.
b Department of Pediatric Surgery , Nil Ratan Sircar Medical College and Hospital (NRSMCH) , Kolkata , India.
Fetal Pediatr Pathol. 2018 Oct;37(5):377-386. doi: 10.1080/15513815.2018.1520943. Epub 2018 Oct 25.
Pleuropulmonary blastoma (PPB) is a childhood malignancy known to be associated with congenital pulmonary airway malformation (CPAM).
An 18 months boy presented with respiratory distress. Computed tomography (CT) scans revealed a large right-sided lung mass. Fine needle aspiration cytology (FNAC) showed sheets and clusters of small round to oval cells with scanty cytoplasm. The possibility of PPB was suggested. Trucut biopsy from the mass confirmed the diagnosis of PPB, of at least type II. The child had earlier been diagnosed as CPAM for which he had undergone lobectomy at six months, which on review was diagnosed as PPB I.
We describe the cytological and histological findings of a case of PPBII/III evolving from a PPB I originally thought to be a CPAM type IV. This supports the theory that PPB I may progress to a more aggressive type II with time, and highlights the importance of the adequately treating the PPB I to prevent this transformation.
肺胚细胞瘤(PPB)是一种已知与先天性肺气道畸形(CPAM)相关的儿童恶性肿瘤。
一名18个月大的男孩出现呼吸窘迫。计算机断层扫描(CT)显示右侧肺部有一个大肿块。细针穿刺细胞学检查(FNAC)显示有片状和簇状的小圆形至椭圆形细胞,胞质稀少。提示可能为PPB。对肿块进行粗针活检确诊为PPB,至少为II型。该患儿早些时候被诊断为CPAM,并在6个月时接受了肺叶切除术,经复查诊断为PPB I型。
我们描述了一例最初被认为是CPAM IV型的PPB I型演变为PPB II/III型病例的细胞学和组织学表现。这支持了PPB I型可能随时间进展为更具侵袭性的II型的理论,并强调了充分治疗PPB I型以防止这种转变的重要性。