Identification of the BRAF V600E mutation in a patient with sclerosing pneumocytoma: A case report

Jiang, Guanming; Zhang, Min; Tan, Qinquan; Lin, Shunhuan; Zeng, Yihong; Liu, Chun; Chen, Rongrong; Zhou, Jianping

doi:10.1016/j.lungcan.2019.09.004

Cited by 14 publications

(7 citation statements)

References 11 publications

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“…Beta-catenin was the secondly most common gene mutation in the sclerosing pneumocytoma [ 11 , 41 ], which might also play a role in producing a benign tumor but not a malignant one. Mutations in other tumor-related genes were also identified in the sclerosing pneumocytoma, like PTEN , BRAF V600E , BLM , KMT2D , but with relatively smaller incidence than AKT1 and β-catenin [ 11 , 41 , 43 ].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary sclerosing pneumocytoma: clinical features and prognosis

Zheng

Zhou

et al. 2022

World J Surg Onc

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Background Pulmonary sclerosing pneumocytoma is a kind of rare benign pulmonary tumor with potential malignancy. The clinical features, risk factors for prognosis, and optimal treatment have not been identified yet. This study aimed to investigate the clinical features and prognosis of pulmonary sclerosing pneumocytoma. Methods We retrospectively performed a review of pulmonary sclerosing pneumocytoma patients in West China Hospital from 2009 to 2019. The basic characteristics, treatment regimens, operation detail, postoperative variables, and follow-up time were recorded for each case. Differences in features between patients undergoing lobectomy and segmentectomy were compared. We also performed a case review and summarized reported clinical features in former studies. Results Altogether 61 pulmonary sclerosing pneumocytoma patients were retrospectively reviewed. Fifty-six patients were female and 5 were male. The patients’ median age was 51 (23-73). Seven (11.48%) patients had smoking history. Twenty tumors were located in the right lung [upper lobe (n = 7), middle (n = 2), and lower (n = 11)] and 41 in the left [upper (n = 12) and lower (n = 29)]. The median tumor size was 2 (0.9-7) cm. Thirty-six (59.02%) patients underwent sublobectomy (segmentectomy or wedge resection) whereas 25 (40.98%) underwent lobectomy. All patients recovered uneventfully, and no perioperative mortality was identified. Sublobectomy showed a trend towards reduced chest tube duration and shorter postoperative hospital stays compared with lobectomy. Conclusions The findings showed good prognosis of pulmonary sclerosing pneumocytoma and proved its benign characteristics. Sublobectomy showed advanced efficacy regarding chest tube duration and postoperative hospital stay compared with lobectomy.

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Section: Discussionmentioning

confidence: 99%

Pulmonary sclerosing pneumocytoma: clinical features and prognosis

Zheng

Zhou

et al. 2022

World J Surg Onc

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“…Some researchers have even studied malignant PSP at the genetic level. Jiang et al 52 performed Sanger sequencing and next-generation sequencing (NGS) in a patient with multiple PSP and found BRAF V600E mutation and AKT1-E17K pathway activation. He suggested that BRAF V600E mutation and AKT1-E17K pathway activation may be related to PSP carcinogenesis.…”

Section: Resultsmentioning

confidence: 99%

Clinical Characteristics of Malignant Pulmonary Sclerosing Pneumocytoma Based on a Study of 46 Cases Worldwide

Zhang¹,

Dong²,

Liu³

et al. 2022

CMAR

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Objective To analyze the clinical characteristics of patients with malignant pulmonary sclerosing pneumocytoma (PSP) with metastasis, recurrence, and growth and to improve clinicians’ understanding of PSP in patients with malignant tumor characteristics. Methods A total of 46 PSP patients with malignant tumor characteristics were identified in the literature search and compared with 38 patients with benign PSP diagnosed and treated in our hospital in the past 5 years. We explored the pathogenesis, clinical symptoms, diagnostic methods, treatment strategies and prognosis of PSP patients with malignant tumor. Results The characteristics of young age (≤41 years old), larger tumor (≥36mm), lymph node metastasis and distribution in East Asians are indicative of PSP with malignant potential. Such patients should undergo segmental resection or lobectomy, combined with necessary lymph node dissection or biopsy. All patients with PSP should have an entire course of follow-up management, because they may have an adverse prognosis such as recurrence, growth, metastasis, and even death. Conclusion PSP has the potential for malignancy. Anatomical lobectomy or segmental resection combined with lymph node dissection should be performed in PSP with some specific characteristics. Inappropriate diagnosis and treatment may lead to poor prognosis in PSP patients.

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“…On the other hand, Wang et al (21) and Teng et al (22) suggested that round cells may originate from Epithelialmesenchymal Transformation (EMT) of surface cells and may be closely related to tumor metastasis. Additionally, Jiang et al (23) conducted Sanger sequencing and Next-generation Sequencing (NGS) on samples from a patient with multiple PSP and found a BRAF V600E mutation and the activation of the AKT1-E17K pathway, which was speculated to be related to the malignant transformation of PSP. Moreover, Fan et al (24) assessed somatic mutations through sequencing and also conducted pathway analysis.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Rare Pulmonary Sclerosing Pneumocytoma: Large, Multiple, Metastatic, and Fatal

et al. 2021

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Pulmonary sclerosing pneumocytoma (PSP) is a rare benign or low-grade malignant tumor, but it has the potential to present with multiple lesions, lymph node metastasis, extra-pulmonary metastasis, recurrence and even cause death. Herein, a case of PSP that was huge, presented with multiple lesions and had lymph node as well as extrapulmonary metastases (liver, abdominal cavity, bones) is reported for the first time. This patient was also the first one to die of respiratory and circulatory failure caused by the PSP tumor and its metastases which compressed the mediastinal tissue.

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Identification of the BRAF V600E mutation in a patient with sclerosing pneumocytoma: A case report

Cited by 14 publications

References 11 publications

Pulmonary sclerosing pneumocytoma: clinical features and prognosis

Pulmonary sclerosing pneumocytoma: clinical features and prognosis

Clinical Characteristics of Malignant Pulmonary Sclerosing Pneumocytoma Based on a Study of 46 Cases Worldwide

Case Report: Rare Pulmonary Sclerosing Pneumocytoma: Large, Multiple, Metastatic, and Fatal

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