Pulmonary Sclerosing Pneumocytoma Research Articles

Histopathological diagnosis of pulmonary sclerosing pneumocytoma in needle biopsy specimens

Objective: To investigate the diagnostic features of pulmonary sclerosing pneumocytoma (PSP) in needle biopsy specimens so as to improve the preoperative diagnostic accuracy and to prevent misdiagnoses. Methods: A total of 79 needle biopsy cases confirmed as PSP in surgical resection specimens were collected in the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China from January 2015 to January 2023. A retrospective analysis was conducted to investigate the clinical, pathological, and immunohistochemical characteristics of PSP. Results: Among the 79 cases, there were 8 males and 71 females, with an age range of 14 to 67 years (median 47 years). Among the 79 needle biopsy cases of PSP, 5 cases were initially misdiagnosed as adenocarcinoma and 1 as carcinoid preoperatively, while the remaining 73 cases were correctly diagnosed. 84.8% (67/79) of the PSP presented with well-defined, homogeneous, solitary solid tumors on chest imaging. Morphologically, 26.6% (21/79) of the PSP mainly showed a single histological component, 67.1% (53/79) contained two histological components, and 6.3% (5/79) contained three histological components. There were no cases containing all four histological components simultaneously. The tumor was composed of cuboidal cells on the surface and round cells in the stroma and lacked significant cytological atypia and mitotic figures. Some cases exhibited variations in histology and cellular morphology, such as glandular spaces (58.2%, 46/79), sclerotic papillae (46.8%, 37/79), hypercellularity (16.5%, 13/79), and cytological atypia (24.1%, 19/79). Immunophenotyping indicated that both tumor cell types expressed TTF1, EMA and β-catenin, while surface cells expressed pan-cytokeratin and Napsin A, and stromal cells expressed vimentin. In some cases, ER and PR were also expressed. Conclusions: When diagnosing PSP in needle biopsy specimens, the key to avoiding misdiagnosis is recognizing the presence of dual-cell populations within the tumor. The useful clues include presence of cellular papillae, mild cellular atypia, morphological diversity, interstitial foam-like cell aggregates, and prominent background hemorrhage and sclerosis. The characteristic immunophenotype and middle-aged female predilection are also helpful for the diagnosis of PSP.

Multiple Pulmonary Sclerosing Pneumocytomas (PSPs): A Comprehensive Analysis of Clinicopathological Characteristics and Whole-exome Sequencing (WES) Results.

Pulmonary sclerosing pneumocytoma (PSP) is a rare neoplasm with indolent clinical behavior and usually presents as a solitary nodule, while only a few cases involving multiple nodules. Recent studies have revealed frequent AKT1 mutations in PSP; however, the molecular genetics of multiple PSPs remain unclear. To better understand the genetic background, eleven patients (4.2%, 11/260) with multiple PSP nodules were identified, and whole-exome sequencing (WES) was performed on 6 patients. Among 5 patients with 2 or 3 PSP nodules, AKT1 alterations were the most common (50%, 7/14), and the predominant alteration was p.E17K (21.4%, 3/14). Novel ARID1A mutations were the second most common driver (14.3%, 2/14), and we first identified these mutations cooccurred with AKT1 p.E17K mutation. Moreover, we observed limited concordance in the mutation spectra and few comutated genes among different lesions from these 5 patients, indicating that PSP with 2 or 3 nodules were independent arising tumors. No AKT1 mutations were identified in 3 PSP samples from a patient with multiple diffuse nodules. However, there were 17 shared genetic alterations among the 3 lesions, but none were typical driver mutations. The findings on multiple diffuse PSP nodules may also have independent origins, but the potential that some of these nodules are metastatic nodules cannot be excluded. In conclusion, this retrospective study is the largest series of multiple PSP cases and provides new insights into the genomic underpinning of PSP. This work has a potential to broaden our understanding of the pathogenesis and development of these lesions and warrants analysis in larger cohorts.

A case report on incidentally detected pulmonary sclerosing pneumocytoma: a diagnostic challenge

Introduction and importance: Pulmonary sclerosing pneumocytoma (PSP) is a rare non-cancerous lung tumor that is usually asymptomatic, but may cause respiratory distress if it becomes large. PSPs are often detected incidentally because of their slow growth, lack of symptoms, characteristic radiographic features, and increased use of imaging studies. Although it is not a malignant tumor, it can mimic malignancy on imaging and histology, leading to misdiagnosis and unnecessary surgery. Case presentation: A 23-year-old asymptomatic female was incidentally diagnosed with PSP during evaluation for a breast fibroadenoma. A chest CT revealed a 3 cm lobulated mass in the left upper lobe. Cytology showed malignant cells with necrotic debris. Immunohistochemistry was positive for TTF-1 and EMA, negative for p63 and AE1/AE3. Histopathology confirmed a well-circumscribed benign neoplasm, consistent with pulmonary sclerosing pneumocytoma. There was no mediastinal lymph node invasion, and the post-surgery prognosis was good. Clinical discussion: PSP is a slow-growing tumor that is often asymptomatic until it reaches a significant size. Owing to their well-circumscribed margins and the presence of calcifications, they are often detected incidentally during imaging studies, such as routine chest radiography or CT scans for unrelated conditions. Although these tumors are often incidental, it is important to diagnose and treat them appropriately to prevent potential complications and malignant transformation. Conclusion: The findings of this study contribute to the existing literature, increase awareness of this rare tumor, and provide insights into its diagnosis, treatment, and follow-up.

Revisiting Pulmonary Sclerosing Pneumocytoma.

Pulmonary sclerosing pneumocytoma (PSP) is a quite rare tumor outside Eastern countries. This rarity, together with a wide histological appearance, makes its correct identification a diagnostic challenge for pathologists under the microscope. Historically, PSP was considered a vascular-derived neoplasm (sclerosing hemangioma), but its immunohistochemical profile clearly supports its epithelial origin. No specific molecular fingerprint has been detected so far. This short narrative revisits the clinical, histological, immunohistochemical, and molecular aspects of this tumor, paying special attention to some controversial points still not well clarified, i.e., clinical aggressiveness and metastatic spread, multifocality, the supposed development of sarcomatoid change in a subset of cases, and tumor associations with lung adenocarcinoma and/or well-differentiated neuroendocrine hyperplasia/tumors. The specific diagnostic difficulties on fine-needle aspiration cytology/biopsy and perioperative frozen sections are also highlighted. Finally, a teaching case of tumor concurrence of lung adenocarcinoma, neuroendocrine lesions, and PSP, paradigmatic of tumor association in this context, is also presented.

Single-Cell Transcriptome Analysis Reveals 2 Subtypes of Tumor Cells of Sclerosing Pneumocytoma With Distinct Molecular Features and Clinical Implications

Pulmonary sclerosing pneumocytoma (PSP) is a rare, distinctive benign lung adenoma of pneumocyte origin. Despite its rarity, the tumor’s unique cellular morphology has sparked ongoing debates regarding the origin of its constituent cells. This study aimed to elucidate the molecular features of PSP tumor cells and enhance our understanding of the cellular processes contributing to PSP formation and biological behavior. Tissue samples from PSP and corresponding normal lung tissues (n = 4) were collected. We employed single-cell RNA sequencing and microarray-based spatial transcriptomic analyses to identify cell types and investigate their transcriptomes, with a focus on transcription factors, enriched gene expression, and single-cell trajectory evaluations. Our analysis identified 2 types of tumor cells: mesenchymal-epithelial dual-phenotype (MEDP) cells and a distinct subpopulation of type II alveolar epithelial cells exhibiting characteristics slightly reminiscent of type I alveolar epithelial cells (AT2Cs) corresponding to histologic round stromal cells and surface cuboidal cells, respectively. MEDP cells displayed weak alveolar epithelial differentiation but strong collagen production capabilities, as indicated by the expression of both TTF-1 and vimentin. These cells played a pivotal role in forming the solid and sclerotic areas of PSP. Moreover, MEDP cells exhibited a pronounced propensity for epithelial-mesenchymal transition, suggesting a greater potential for metastasis compared with AT2Cs. The capillary endothelial cells of PSP displayed notable diversity. Overall, this study provides, for the first time, a comprehensive mapping of the single-cell transcriptome profile of PSP. Our findings delineate 2 distinct subtypes of tumor cells, MEDP cells and AT2Cs, each with its own biological characteristics and spatial distribution. A deeper understanding of these cell types promises insights into the histology and biological behaviors of this rare tumor.

Multiple pulmonary sclerosing pneumocytoma, based on a study of 36 cases worldwide

To analyze the clinical characteristics and to improve clinicians’ understanding of multiple pulmonary sclerosing pneumocytoma (PSP) patients. A total of 36 PSP patients with multiple tumor characteristics were identified from the literature search. They were compared with 43 solitary PSP patients diagnosed and treated in our hospital in the past 5 years. Thus, the pathogenesis, clinical symptoms, diagnosis methods, treatment strategies, and prognosis of pulmonary sclerosing pneumocytoma (PSP) patients with multiple tumors were explored. Patients with multiple PSP are mostly distributed in Asia (88.89%) and are females (83.33%). PSP can be located in any one lobe (19.44%), or grow across ipsilateral lobes (44.44%), or even, bilateral lobes (36.11%). It can be accompanied by metastasis (9.09%) and is prone to misdiagnosis (27.78%). Compared with solitary PSP, the occurrence age of multiple PSP was younger (mean ± standard deviation [SD]: 40.36 ± 18.12: 51.28 ± 12.74 years), but there was no significant difference in sex, tumor size (mean ± SD: 43.54 ± 46.18: 30.56 ± 17.62 mm), or symptoms. Individualized surgical resection is required for treatment, including pneumonectomy (17.65%), lobectomy (23.53%), subpulmonary lobectomy (38.24%), or combined lobectomy (5.88%). Multiple PSP is relatively rare. Surgical resection within a limited time should be the main treatment for such patients. The prognosis of patients with multiple PSP is generally good, but inappropriate diagnosis and treatment plans may lead to poor prognosis.

CT-based radiomics for differentiating peripherally located pulmonary sclerosing pneumocytoma from carcinoid.

Pulmonary sclerosing pneumocytoma (PSP) and pulmonary carcinoid (PC) are difficult to distinguish based on conventional imaging examinations. In recent years, radiomics has been used to discriminate benign from malignant pulmonary lesions. However, the value of radiomics based on computed tomography (CT) images to differentiate PSP from PC has not been well explored. We aimed to investigate the feasibility of radiomics in the differentiation between PSP and PC. Fifty-three PSP and fifty-five PC were retrospectively enrolled and then were randomly divided into the training and test sets. Univariate and multivariable logistic analyses were carried to select clinical predictor related to differential diagnosis of PSP and PC. A total of 1316 radiomics features were extracted from the unenhanced CT (UECT) and contrast-enhanced CT (CECT) images, respectively. The minimum redundancy maximum relevance and the least absolute shrinkage and selection operator were used to select the most significant radiomics features to construct radiomics models. The clinical predictor and radiomics features were integrated to develop combined models. Two senior radiologists independently categorized each patient into PSP or PC group based on traditional CT method. The performances of clinical, radiomics, and combined models in differentiating PSP from PC were investigated by the receiver operating characteristic (ROC) curve. The diagnostic performance was also compared between the combined models and radiologists. In regard to differentiating PSP from PC, the area under the curves (AUCs) of the clinical, radiomics, and combined models were 0.87, 0.96, and 0.99 in the training set UECT, and were 0.87, 0.97, and 0.98 in the training set CECT, respectively. The AUCs of the clinical, radiomics, and combined models were 0.84, 0.92, and 0.97 in the test set UECT, and were 0.84, 0.93, and 0.98 in the test set CECT, respectively. In regard to the differentiation between PSP and PC, the combined model was comparable to the radiomics model, but outperformed the clinical model and the two radiologists, whether in the test set UECT or CECT. Radiomics approaches show promise in distinguishing between PSP and PC. Moreover, the integration of clinical predictor (gender) has the potential to enhance the diagnostic performance even further.

Pulmonary Sclerosing Pneumocytoma - A Comprehensive Exploration of Clinical, Radiological, and Surgical Dimensions By IJISRT

Objectives: The study findings have important Clinical implications, especially in the preoperative evaluation of Lung Nodules and aid in the radiological diagnosis based on CT features. Pulmonary Sclerosing pneumocytoma is a Benign, rare tumor of the lung that represents a diagnostic challenge due to the Non-specific CT findings. The Aim of this study was to present a 10-year experience with sclerosing pneumocytoma of a large center of CHINA for the diagnosis and treatment of pulmonary diseases, and to emphasize differential diagnostic dilemmas as a potential source of errors.  Material and Methods: This represents a retrospective study of 31 patients diagnosed and treated with sclerosing pneumocytoma in the 10-year period. The study analyzed various Variables, including Gender, Age, Smoking history, Reason for CT, Nodule location, Nodule shape, Clinical symptoms, Calcifications, and Surgical Resections.  Results: Sclerosing pneumocytoma was more frequently diagnosed in females (93.5%). The patients ranged in age from 28 to 68. Most of the patients (77.4%) were asymptomatic. 30 patients had no history of smoking (96.8%). Mean Nodule size was 14.4mm. Most nodules have Round, oval and smooth margin, with majority of Nodules have location in the lower lobes of both lungs. Most nodules were peripherally situated (54.83%). VATS with lobectomy performed in 25 (80.64%) patients while VATS with wedge resection performed in 6(19.64%) patients, without post-surgical complications and Normal follow up.

Pulmonary Sclerosing Pneumocytoma: A Case Revealed During 8-Year of Follow-up with CT imaging.

Pulmonary sclerosing pneumocytoma (PSP) is a rare, benign tumor. Given the challenges of a bronchoscopic diagnosis, surgery is performed during the early stages of the disease. Therefore, little is known about the growth pattern of PSP. This case of PSP was not diagnosed despite bronchoscopy, resulting in lung resection eight years after the anomaly was first identified on computed tomography (CT). This report compares the long-term follow-up of CT and pathological findings and discusses the difficulty in making a diagnosis using a bronchoscopic forceps biopsy to aid in future PSP diagnoses and treatment planning.

Transformer-Based Weakly Supervised Learning for Whole Slide Lung Cancer Image Classification.

Image analysis can play an important role in supporting histopathological diagnoses of lung cancer, with deep learning methods already achieving remarkable results. However, due to the large scale of whole-slide images (WSIs), creating manual pixel-wise annotations from expert pathologists is expensive and time-consuming. In addition, the heterogeneity of tumors and similarities in the morphological phenotype of tumor subtypes have caused inter-observer variability in annotations, which limits optimal performance. Effective use of weak labels could potentially alleviate these issues. In this paper, we propose a two-stage transformer-based weakly supervised learning framework called Simple Shuffle-Remix Vision Transformer (SSRViT). Firstly, we introduce a Shuffle-Remix Vision Transformer (SRViT) to retrieve discriminative local tokens and extract effective representative features. Then, the token features are selected and aggregated to generate sparse representations of WSIs, which are fed into a simple transformer-based classifier (SViT) for slide-level prediction. Experimental results demonstrate that the performance of our proposed SSRViT is significantly improved compared with other state-of-the-art methods in discriminating between adenocarcinoma, pulmonary sclerosing pneumocytoma and normal lung tissue (accuracy of 96.9% and AUC of 99.6%).

Bronchiolar adenoma with squamous metaplasia: a distinct phenotype.

Pulmonary bronchiolar adenoma is a benign lung tumour characterised by nodular proliferation of bilayered bronchiolar-type epithelium with a continuous layer of basal cells. The aim of this study was to describe a distinct and rare histological type of pulmonary bronchiolar adenoma: bronchiolar adenoma with squamous metaplasia. We examined the clinicopathological, immunohistochemical, and molecular characteristics of five cases (two cases from the same patient). The samples were histopathologically characterised by bilayered bronchiolar-type cells with sheets like spindle-oval and polygonal cells. Immunohistochemistry analysis revealed that columnar surface cells of the tumour were diffusely positive for TTF-1 and Napsin A, while basal cells were positive for P40 and P63. Moreover, the squamous metaplastic cells in the stroma were positive for P40, and P63, while being negative for TTF-1, Napsin A, S100, and SMA. Genomic analyses uncovered that all five samples had BRAF V600E mutations. Notably, both squamous metaplastic and basal cells were positive for BRAF V600E staining. We discovered a distinct subtype of pulmonary bronchiolar adenoma termed bronchiolar adenoma with squamous metaplasia. It is composed of columnar surface cells, basal cells, and sheet-like spindle-oval cells with squamous metaplasia in the stroma. All five samples harboured the BRAF V600E mutation. Importantly, BASM may be misdiagnosed as pulmonary sclerosing pneumocytoma upon frozen sections analysis. It may need further immunohistochemistry staining.

Characteristics of Metastatic and Nonmetastatic Pulmonary Sclerosing Pneumocytomas: A Clinicopathological Study of 68 Cases and 15 Reported Metastatic Cases

To characterize the clinicopathologic features of pulmonary sclerosing pneumocytoma (PSP) and compare these features between the tumors with and without metastasis, 68 cases of PSP (1/68 [1.47%] with metastasis) diagnosed from 2009-2022 in our hospital and 15 previously reported metastasizing cases were studied. There were 54 female patients and 14 male patients, with age ranging from 17 to 72 years and tumor size ranging from 0.1 to 5.5 cm (mean, 1.75 cm). In all, 85.4% of the cases presented with ≥2 patterns, including papillary, sclerotic, solid, and hemorrhagic. Thyroid transcription factor 1, epithelial membrane antigen, CKpan, and CK7 were expressed in surface cells in 100% of the cases and napsin A was expressed in 90% of the cases. Stromal cell expression of these markers occurred in 100%, 93.9%, 13.5%, 13.8%, and 0% of the cases, respectively. Of the 16 PSP cases with metastasis, 8 were female patients and 7 were male patients, with age ranging from 14 to 73 years. The tumor size ranged from 2.5 to 12 cm (mean, 4.85 cm). Forty-five of the cases were negative for BRAF V600E immunostaining and 6 were focally weak positive, in which fluorescent PCR tests showed no detectable mutations. There were significant differences in gender, age, and tumor size between PSP cases with and without metastasis. No BRAF V600E mutation was found in patients with PSP. AKT1 p.E17K mutations were detected in both the primary lung tumor and the lymph node metastatic tumor of our PSP case with lymph node metastasis. In conclusion, PSP is an uncommon pulmonary neoplasm with significant female predilection and has distinct morphologic and immunohistochemical characteristics. The BRAFV600E mutation was not detectable in patients with PSP and thus may not involve in its tumorigenesis. Most PSP tumors are benign, with a minority exhibiting potential for metastasis and malignant behavior.

Pulmonary sclerosing pneumocytoma containing spindle cells with sarcomatoid features: a case report with literature review

BackgroundPulmonary sclerosing pneumocytoma (PSP) is an uncommon benign neoplasm originated from pneumocyte and PSP with malignant transformation is extremely rare.Case presentationWe report a case of PSP of a 65-year-old male patient presented as a lobulated mass in the upper lobe of the left lung, in which part of the stromal round cells transformed to spindle cells with sarcomatoid features and showed no specific differentiation. The patient underwent partial lobectomy without further treatment. No recurrence and metastasis was found after eight month’s follow up.ConclusionsTo our knowledge, this is the first case of PSP with sarcomatoid malignant transformation devoid of differentiation. Our case adds the evidence in that a subset of PSP bear malignant potential and more studies are needed in order to determine the treatment and prognosis to such patients.

Treatment and post-operative follow-up of pulmonary sclerosing pneumocytoma: A case report

Pulmonary sclerosing pneumocytoma (PSP) is a rare tumor thought to originate from respiratory epithelial cells. It is usually benign, but may rarely metastasize to lymph nodes. Surgeons face unique challenges in diagnosis and management of this condition, and ideal surgical management is yet to be established. 48-year-old woman with a 7×7 mm pulmonary lesion discovered incidentally on computerized tomography (CT) imaging, which grew to 9 mm over the following year. Seven years later, follow-up imaging revealed that the mass had grown to 1.3 cm in largest dimension. Surgery was recommended and the mass was resected via a right video-assisted thoracic surgery (VATS) middle lobectomy with mediastinal lymph node dissection. All lymph nodes were negative and the patient's postoperative course was unremarkable. There are few evidence-based guidelines available on the treatment and postoperative surveillance of PSP. Research has shown comparable recurrence-free survival rates for sublobar resection and lobectomy, though recurrence can occur, especially following sublobar resection in larger or more centrally-located tumors. In absence of established guidelines, it was decided to follow this patient according to NCCN guidelines for surveillance of early-stage non-small cell lung cancer due to potential risk of recurrence. This case report adds to the limited literature on PSP and depicts a possible treatment and postoperative follow-up plan. Right VATS middle lobectomy can effectively treat some cases of central PSP. In absence of established guidelines for postoperative follow-up of PSP, NCCN guidelines may outline one possible strategy for postoperative management.

Pulmonary sclerosing pneumocytoma and mortality risk

BackgroundSurgical resection is usually recommended for the treatment of pulmonary sclerosing pneumocytoma (PSP). However, no comparative study has demonstrated that surgical resection leads to improved outcomes. We aimed to compare all-cause mortality between patients with PSP who underwent surgery or did not and those without PSP.MethodsParticipants aged ≥18 years who had pathologically diagnosed PSP between 2001 to 2018, at 3 hospitals were included. Randomly selected (up to 1:5) age-, sex-, and smoking status-matched controls without PSP who were randomly selected from those who underwent health checkups including chest CT were included. Mortality was compared using Kaplan–Meier estimates and Cox proportional hazards regression models. Literature review of studies reporting PSP was also conducted.ResultsThis study included 107 patients with PSP (surgery:non-surgery, 80:27) and 520 matched controls. There were no cases of lymph node or distant metastasis, recurrence, or mortality from PSP. No significant difference in all-cause mortality risk was observed between the PSP surgery, PSP non-surgery, and non-PSP groups (log rank test P = 0.78) (PSP surgery vs. non-PSP: adjusted hazards ratio [aHR], 1.80; 95% confidence interval [CI], 0.22–14.6; PSP non-surgery vs. non-PSP: aHR, 0.77; 95% CI, 0.15–3.86; PSP surgery vs. PSP non-surgery: aHR, 2.35; 95% CI, 0.20–28.2). In the literature review, we identified 3469 patients with PSP from 355 studies. Only 1.33% of these patients reported metastasis, recurrence, or death.ConclusionsAll-cause mortality did not differ between patients with PSP and those without, irrespective of undergoing surgery. Our study and the literature review suggest that PSP has less impact on increased mortality risk.

Large Symptomatic Sclerosing Pneumocytoma in a Young Male Smoker-A Rare and Deceptive Presentation.

Pulmonary sclerosing pneumocytoma (PSP) is a rare pulmonary tumor that behaves in a benign fashion and has excellent prognosis.[1] The tumor was historically termed as sclerosing hemangioma by Liebow and Hubbell in 1956.[2] The incidence peaks in sixth decade with a striking predilection for females (F:M = 5:1).[3] [4] Although it presents as a well-circumscribed solid nodular mass, the radiological features are nonspecific and can be misconstrued as malignant especially in large tumors.[5] Frozen sections and trucut biopsy interpretation of such tumors may also be misleading.

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

ObjectiveTo 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.MethodsA 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.ResultsThe 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.ConclusionPSP 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.

Coexistence of Multiple Pulmonary Sclerosing Pneumocytoma and Scleroderma-Rheumatoid Arthritis Overlap Syndrome: A Case Report.

Pulmonary sclerosing pneumocytoma is a rare, low-grade pulmonary tumor observed as unilateral or bilateral multiple nodules at a rate of 4%-5%. Among the autoimmune connective tissue disorders, those most commonly associated with lung malignancies are scleroderma and rheumatoid arthritis. In this study, we report a rare case of a 55-year-old middle-aged Asian woman with slow-growing bilateral multiple pulmonary sclerosing pneumocytoma and scleroderma–rheumatoid arthritis overlap syndrome. The autoimmune disorders and pulmonary fibrosis of this case might have led to the development of PSP.

Pulmonary sclerosing pneumocytoma: clinical features and prognosis

BackgroundPulmonary 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.MethodsWe 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.ResultsAltogether 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.ConclusionsThe 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.

A case of mediastinal lymph node metastasis positive for pulmonary sclerosing pneumocytoma that was difficult to diagnose preoperatively

A case of mediastinal lymph node metastasis positive for pulmonary sclerosing pneumocytoma that was difficult to diagnose preoperatively