Pulmonary Sclerosing Pneumocytoma: Did Lightening Just Strike Twice?

Alshelli, I.A.; Seeley, E. Scott

doi:10.1053/j.semtcvs.2017.11.009

Cited by 1 publication

(2 citation statements)

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“…Pulmonary sclerosing pneumocytoma (PSP), formerly known as pulmonary sclerosing hemangioma, is a rare primary lung tumor initially described by Liebow et al, in 1956 as a tumor with prominent sclerosis and vascularization [1,2]. It mainly affects adults > 50 years of age, with a female to male ratio of 5:1 [3,4].…”

Section: Introductionmentioning

confidence: 99%

“…Immunohistochemistry (IHC) markers clari ed the epithelial origin of PSP and suggested that the condition originated from incompletely differentiated type II pneumocytes. Therefore, the term "pulmonary sclerosing hemangioma" was abandoned in the WHO 2016 classi cation for lung and pleura tumors, and PSP was adopted [2,5,6]. Although PSP has some clinical and imaging characteristics for differentiation from similar lesions, these are not speci c for diagnosis [7,8].…”

Section: Introductionmentioning

confidence: 99%

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Clinicopathological and Molecular Characteristics of Atypical Pulmonary Sclerosing Pneumocytoma With Multiple Metastases

Wang¹,

Yang²,

Jiang³

et al. 2020

Preprint

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Background: Few patients with pulmonary sclerosing pneumocytoma (PSP) may suffer from recurrence and oligometastasis as it is a benign tumor or has a low malignancy potential. Herein, an in-depth study, based on an extremely rare PSP case with atypical features, was carried out to elaborate the potential mechanism underlying the rapid malignant progression. Methods: The clinicopathological data of this atypical PSP (AP) case were obtained. Formalin‑fixed and paraffin‑embedded tissues from all the lesions of AP and five classic PSP cases (as control) were used for whole-exome sequencing (WES) and immunohistochemistry. Results: A 23-year-old male showed a 6.5-cm pulmonary nodule in the right middle lobe (RML) and enlarged mediastinal lymph nodes (LNs). He underwent thoracoscopic RML lobectomy, systematic LNs dissection, and mediastinal lymphadenectomy. The metastases to the cervical LNs and liver were detected in a short period and then resected. Postoperative pathological examination confirmed the diagnosis of PSP in all the lesions, based on the typical histological characteristics and immunophenotypes. Furthermore, WES identified both AKT1 E17K somatic mutation and TP53 C176Y germline mutation in this AP case. The genomic evolution analysis showed different evolutionary branches in the metastatic tumors distinct from the primary lesion. Moreover, compared to the control group, the AP case showed high copy number variations (CNVs) and significantly high copy number instability (CNI). Conclusions: We speculated that the atypical histopathological features and malignant behaviors may be due to the co-mutations of somatic AKT1 E17K and germline TP53 C176Y, combined with the high CNVs and CNI.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%