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

Abstract

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.

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

“…He suggested that BRAF V600E mutation and AKT1-E17K pathway activation may be related to PSP carcinogenesis. Zhang et al 8 and Fan et al 53 through somatic mutation sequencing and pathway analysis also showed that the activation of AKT1 may be involved in the occurrence and development of PSP. Many researchers have discovered the activation of the AKT1-p.E17K pathway in their patients and believe that this activation may be related to the occurrence and metastasis of PSP.…”

“…Six of the 8 PSP with growth patients were observed only, and the increase was found after 6–492 months (median [IQR]: 42 months (34.75–120)), and then surgical resection was performed again. One of the patients died after gradual enlargement of the tumor during observation, 8 and one patient who was not followed up in a timely manner underwent pneumonectomy because the tumor size increased. 46 Some patients even had extrapulmonary metastases.…”

“…Recently, we found a case of PSP with lymph node metastasis, extrapulmonary metastasis, multiple and growing. 8 This patient died of respiratory and circulatory failure caused by PSP. Therefore, by searching the literature of various countries, the authors found that PSP is not a simple benign and harmless nodule, but it may be accompanied by lymph node metastasis, multiple tumors, growth, recurrence, and eventual mortality.…”

“…In this study, we searched the literature on PSP patients with malignant tumor characteristics (metastasis, recurrence, and growth). There was a total of 40 PSP patients with lymph node or extrapulmonary metastasis, 8–48 8 PSP patients with gradually increasing tumor size at follow up, and 6 PSP patients who had relapse at the original location or other locations after the original lesions were removed. We retrieved the medical records of patients with benign PSP who had undergone surgery for PSP in our hospital in the past 5 years for comparative analysis of the clinical characteristics of the these patients and those reported in the literature with malignant tumor characteristics with the goal of to find more appropriate methods of diagnosis and treatment.…”

Pulmonary sclerosing pneumocytoma and mortality risk

“…Surgical resection is usually recommended for the treatment of PSP [6] considering that cases with lymph node metastasis [3,[8][9][10][11][12][13][14], distant or pleural metastasis [15][16][17], recurrence [18], malignant transformation [19] and death [20] have been reported. However, as most patients with PSP show a benign indolent course, not all patients with PSP undergo surgical resection clinically [21].…”