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1.
Mod Pathol ; 37(1): 100354, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37844870

RESUMO

Sclerosing pneumocytoma is a rare and distinct lung neoplasm whose histogenesis and molecular alterations are the subject of ongoing research. Our recent study revealed that AKT1 internal tandem duplications (ITD), point mutations, and short indels were present in almost all tested sclerosing pneumocytomas, suggesting that AKT1 mutations are a major driving oncogenic event in this tumor. Although the pathogenic role of AKT1 point mutations is well established, the significance of AKT1 ITD in oncogenesis remains largely unexplored. We conducted comprehensive genomic and transcriptomic analyses of sclerosing pneumocytoma to address this knowledge gap. RNA-sequencing data from 23 tumors and whole-exome sequencing data from 44 tumors were used to obtain insights into their genetic and transcriptomic profiles. Our analysis revealed a high degree of genetic and transcriptomic similarity between tumors carrying AKT1 ITD and those with AKT1 point mutations. Mutational signature analysis revealed COSMIC signatures 1 and 5 as the prevailing signatures of sclerosing pneumocytoma, associated with the spontaneous deamination of 5-methylcytosine and an unknown etiology, respectively. RNA-sequencing data analysis revealed that the sclerosing pneumocytoma gene expression profile is characterized by activation of the PI3K/AKT/mTOR pathway, which exhibits significant similarity between tumors harboring AKT1 ITD and those with AKT1 point mutations. Notably, an upregulation of SOX9, a transcription factor known for its involvement in fetal lung development, was observed in sclerosing pneumocytoma. Specifically, SOX9 expression was prominent in the round cell component, whereas it was relatively lower in the surface cell component of the tumor. To the best of our knowledge, this is the first comprehensive investigation of the genomic and transcriptomic characteristics of sclerosing pneumocytoma. Results of the present study provide insights into the molecular attributes of sclerosing pneumocytoma and a basis for future studies of this enigmatic tumor.


Assuntos
Fosfatidilinositol 3-Quinases , Hemangioma Esclerosante Pulmonar , Humanos , Fosfatidilinositol 3-Quinases/genética , Hemangioma Esclerosante Pulmonar/genética , Hemangioma Esclerosante Pulmonar/patologia , Genômica , Perfilação da Expressão Gênica , RNA
2.
Mod Pathol ; 37(9): 100560, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38972356

RESUMO

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.


Assuntos
Perfilação da Expressão Gênica , Neoplasias Pulmonares , Análise de Célula Única , Transcriptoma , Humanos , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/metabolismo , Hemangioma Esclerosante Pulmonar/patologia , Hemangioma Esclerosante Pulmonar/genética , Células Epiteliais Alveolares/patologia , Células Epiteliais Alveolares/metabolismo , Masculino , Feminino , Pessoa de Meia-Idade , Transição Epitelial-Mesenquimal/genética , Idoso , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/análise , Fatores de Transcrição/genética , Análise da Expressão Gênica de Célula Única
3.
Lab Invest ; 103(7): 100135, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36966952

RESUMO

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.


Assuntos
Neoplasias Pulmonares , Hemangioma Esclerosante Pulmonar , Neoplasias da Glândula Tireoide , Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Pulmão/patologia , Hemangioma Esclerosante Pulmonar/genética , Hemangioma Esclerosante Pulmonar/diagnóstico , Hemangioma Esclerosante Pulmonar/patologia , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/patologia , Metástase Linfática/patologia , Proteínas Proto-Oncogênicas B-raf/genética , Neoplasias da Glândula Tireoide/patologia
4.
BMC Med Imaging ; 22(1): 16, 2022 02 02.
Artigo em Inglês | MEDLINE | ID: mdl-35105314

RESUMO

BACKGROUND: As a rare benign lung tumour, pulmonary sclerosing pneumocytoma (PSP) is often misdiagnosed as atypical peripheral lung cancer (APLC) on routine imaging examinations. This study explored the value of enhanced CT combined with texture analysis to differentiate between PSP and APLC. METHODS: Forty-eight patients with PSP and fifty patients with APLC were retrospectively enrolled. The CT image features of the two groups of lesions were analysed, and MaZda software was used to evaluate the texture of CT venous phase thin-layer images. Independent sample t-test, Mann-Whitney U tests or χ2 tests were used to compare between groups. The intra-class correlation coefficient (ICC) was used to analyse the consistency of the selected texture parameters. Spearman correlation analysis was used to evaluate the differences in texture parameters between the two groups. Based on the statistically significant CT image features and CT texture parameters, the independent influencing factors between PSP and APLC were analysed by multivariate logistic regression. Extremely randomized trees (ERT) was used as the classifier to build models, and the models were evaluated by the five-fold cross-validation method. RESULTS: Logistic regression analysis based on CT image features showed that calcification and arterial phase CT values were independent factors for distinguishing PSP from APLC. The results of logistic regression analysis based on CT texture parameters showed that WavEnHL_s-1 and Perc.01% were independent influencing factors to distinguish the two. Compared with the single-factor model (models A and B), the classification accuracy of the model based on image features combined with texture parameters was 0.84 ± 0.04, the AUC was 0.84 ± 0.03, and the sensitivity and specificity were 0.82 ± 0.13 and 0.87 ± 0.12, respectively. CONCLUSION: Enhanced CT combined with texture analysis showed good diagnostic value for distinguishing PSP and APLC, which may contribute to clinical decision-making and prognosis evaluation.


Assuntos
Processamento de Imagem Assistida por Computador/métodos , Neoplasias Pulmonares/diagnóstico por imagem , Hemangioma Esclerosante Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Diagnóstico Diferencial , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sensibilidade e Especificidade , Adulto Jovem
5.
BMC Pulm Med ; 22(1): 404, 2022 Nov 07.
Artigo em Inglês | MEDLINE | ID: mdl-36344955

RESUMO

BACKGROUND: Surgical 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. METHODS: Participants 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. RESULTS: This 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. CONCLUSIONS: All-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.


Assuntos
Hemangioma Esclerosante Pulmonar , Humanos , Adolescente , Adulto , Hemangioma Esclerosante Pulmonar/cirurgia , Hemangioma Esclerosante Pulmonar/diagnóstico , Hemangioma Esclerosante Pulmonar/patologia , Pulmão/patologia , Estimativa de Kaplan-Meier , Modelos de Riscos Proporcionais , Tomografia Computadorizada por Raios X , Estudos Retrospectivos
6.
World J Surg Oncol ; 20(1): 140, 2022 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-35490241

RESUMO

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.


Assuntos
Neoplasias Pulmonares , Hemangioma Esclerosante Pulmonar , Feminino , Humanos , Pulmão/patologia , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Masculino , Pessoa de Meia-Idade , Prognóstico , Hemangioma Esclerosante Pulmonar/patologia , Hemangioma Esclerosante Pulmonar/cirurgia , Estudos Retrospectivos
7.
Acta Chir Belg ; 122(2): 123-126, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32397805

RESUMO

INTRODUCTION: Pulmonary Sclerosing Pneumocytoma (PSP) is a rare type of benign lung tumor usually encountered in middle-aged Asian women. The lesion is mostly found on routine chest x-rays. Though surgery is recognized as the recommended treatment, there is no consensus on the standard operative procedure for this tumor. CASE PRESENTATION: We report a case of a 48 year-old Caucasian woman who presented with a right para-hilar mass mimicking a hydatid cyst. After an unsuccessful initial treatment with oral Albendazole, and a steady growth over 10 years, the patient was programmed for surgical resection by video-thoracoscopic (VATS) approach. We were able to completely resect the tumor by VATS. Histopathological analysis suggested the diagnosis of Pulmonary Sclerosing Pneumocytoma. No further treatment was required and the patient was rapidly discharged. CONCLUSIONS: Pulmonary sclerosing pneumocytoma is a rare form of benign tumor that should be part of the differential diagnosis of lung lesions of unknown origin. Because of its well-defined encapsulated structure allowing total enucleation, VATS can be proposed as a less invasive alternative to classic thoracotomy.


Assuntos
Neoplasias Pulmonares , Hemangioma Esclerosante Pulmonar , Feminino , Humanos , Pulmão , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Pessoa de Meia-Idade , Hemangioma Esclerosante Pulmonar/diagnóstico por imagem , Hemangioma Esclerosante Pulmonar/cirurgia , Cirurgia Torácica Vídeoassistida , Toracotomia
8.
Medicina (Kaunas) ; 57(6)2021 May 23.
Artigo em Inglês | MEDLINE | ID: mdl-34071040

RESUMO

Pulmonary sclerosing pneumocytoma is a rare benign pulmonary tumor of primitive epithelial origin. Because of the unspecific radiological features mimicking malignancies and its histological heterogeneity, the differential diagnosis with adenocarcinoma and carcinoid tumors is still challenging. We report our experience of two cases of sclerosing pneumocytoma, as well as a review of the literature. Immunohistochemical findings showed intense staining of the cuboidal epithelial cells for cytokeratin-pool and TTF-1, with focal positivity for progesterone receptors. Round and spindle cells expressed positivity for vimentin, TTF-1 and focally for the progesterone receptor. Cytologic diagnosis of pulmonary pneumocytoma requires the identification of its dual cell population, made up of abundant stromal cells and fewer surface cells. Since the pre- and intraoperative diagnosis should guide surgical decision making, obtaining a sufficient specimen size to find representative material in the cell block is of paramount importance.


Assuntos
Adenocarcinoma , Neoplasias Pulmonares , Hemangioma Esclerosante Pulmonar , Diagnóstico Diferencial , Humanos , Pulmão , Neoplasias Pulmonares/diagnóstico por imagem , Hemangioma Esclerosante Pulmonar/diagnóstico por imagem , Hemangioma Esclerosante Pulmonar/cirurgia
9.
Mod Pathol ; 33(3): 391-403, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31527710

RESUMO

Sclerosing pneumocytoma is a unique benign neoplasm of the lungs. The molecular alterations in sclerosing pneumocytoma are not well understood. In a previous whole-exome sequencing study, recurrent AKT1 point mutation was observed in about half of the cases of sclerosing pneumocytoma. However, in the remaining half, cancer-related mutations have still not been identified. In this study, we first analyzed the raw sequence data from the previous whole-exome sequencing study (PRJNA297066 cohort). Using Genomon-ITDetector, a special software for detection of internal tandem duplications, we identified recurrent internal tandem duplications in the AKT1 gene in 22 of the 44 tumor samples (50%). All the cases positive for AKT1 internal tandem duplications lacked AKT1 point mutations. Next, we performed targeted next-generation sequencing in an independent cohort of sclerosing pneumocytoma from our hospital (VGH-TPE cohort), and again identified recurrent AKT1 internal tandem duplications in 20 of the 40 (50%) tumor samples analyzed. The internal tandem duplications resulted in duplications of 7 to 16 amino acids in a narrow region of the Pleckstrin homology domain of the AKT1 protein. This region contains the interaction interface between the Pleckstrin homology and kinase domains, which is known to play a critical role in the activation of the AKT1 protein. Moreover, we found that AKT1 internal tandem duplications were mutually exclusive of other forms of AKT1 mutations, including point mutations and short indels. Taking all forms of AKT1 mutations together, we detected AKT1 mutations in almost all the sclerosing pneumocytomas in our study (PRJNA297066 cohort: 41 out of 44 cases, 93%; VGH-TPE cohort: 40 out of 40 cases, 100%). Our results suggest that AKT1 mutation is the genetic hallmark of sclerosing pneumocytoma. These results would help in better understanding of the pathogenesis of sclerosing pneumocytoma.


Assuntos
Biomarcadores Tumorais/genética , Mutação Puntual , Proteínas Proto-Oncogênicas c-akt/genética , Hemangioma Esclerosante Pulmonar/genética , Hemangioma Esclerosante Pulmonar/patologia , Sequências de Repetição em Tandem , Adulto , Idoso , Análise Mutacional de DNA , Bases de Dados Genéticas , Feminino , Predisposição Genética para Doença , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Sequenciamento do Exoma
10.
Histopathology ; 77(5): 718-727, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32441345

RESUMO

AIMS: To determine the clinicopathological features of pulmonary sclerosing pneumocytoma (PSP) with spindle cells and in cases with positive detection of PSP cells in the lymph nodes. METHODS AND RESULTS: This article report the clinical, histological and immunohistochemical features of PSP with dense spindle stromal cells in five patients (including one case with lymph node metastasis) and PSP accompanied by positive nodes in two patients out of 239 cases diagnosed at our institution between 2007 and 2019. The literature on PSP was also reviewed in detail. Six patients were female, and one (with a positive node) was male; their average age was 53 years. Thoracic imaging revealed solid tumours with clear borders and a uniform texture in six patients, but one patient had a lobulated tumour with uneven densities. All tumours were unifocal, and they had an average size of 31 mm. Tumours from five cases were mainly composed of solid regions of diffuse spindle cells rather than polygonal cells. Immunohistochemical staining demonstrated that thyroid transcription factor-1, vimentin, epithelial membrane antigen (weak) and oestrogen receptor (partial) were expressed in spindle cells. The average follow-up time was 31 months. Two of the 234 PSP cases for which adequate data were available had positive nodes (metastasis rate: 0.8%), and one of the five patients with PSP with spindle cells showed lymph node metastasis (metastasis rate: 20%). In addition, stromal cells were found to be predominant at metastatic sites. CONCLUSIONS: Spindle cells are present among the stromal cells of PSP, and not all of them are round cells. PSP patients with spindle cells or male patients may be more prone to metastasis than others.


Assuntos
Metástase Linfática/patologia , Hemangioma Esclerosante Pulmonar/patologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
11.
BMC Cancer ; 19(1): 1154, 2019 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-31775674

RESUMO

BACKGROUND: Pulmonary sclerosing pneumocytoma (PSP) is a rare benign tumor. Although lymph node metastasis has been reported, it is still considered benign. No malignant transformation has been reported. This is the first case of malignant transformation of both cuboidal surface cells and stromal round cells. CASE PRESENTATION: A 64-year-old male had been complaining of intermittent hemoptysis several times per day for eight months. Chest computed tomography scan showed parenchymal infiltration with cystic lesion in the right lower lobe accompanied by enlarged right hilar lymph nodes. Lobectomy and systemic lymph node dissection was performed. On grossly pathological examination, the lesion was 50 mm from the bronchial stump. It was a mixture of both cystic and solid components and 30 mm * 20 mm in size with unclear border. Microscopically, the cuboidal surface cells transformed to adenocarcinoma. The stromal round cells also had a malignant transformation. The Ki-67 proliferation index in malignant cuboidal surface cells and stromal round cells were 70 and 55%, respectively. Furthermore, E-cadherin was negative in primary tumor but positive in metastatic lymph node, which suggested that the mesenchymal to epithelial transition may play an important role in lymph node metastasis. CONCLUSIONS: To our knowledge, we present the first case of malignant transformation of both cuboidal surface cells and stromal round cells in PSP. The process of mesenchymal to epithelial transition may play an important role in lymph node metastasis.


Assuntos
Células Epiteliais Alveolares/patologia , Transformação Celular Neoplásica/patologia , Hemangioma Esclerosante Pulmonar/diagnóstico , Células Estromais/fisiologia , Idoso , Biomarcadores Tumorais , Biópsia , Humanos , Imuno-Histoquímica , Linfonodos/patologia , Masculino , Células Estromais/patologia , Tomografia Computadorizada por Raios X
12.
Medicina (Kaunas) ; 55(2)2019 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-30691016

RESUMO

Background and objective: Sclerosing pneumocytoma is a rare, benign tumor of the lung that represents a diagnostic challenge due to the diversity of pathohistological findings. The aim of this study was to present a 10-year experience with sclerosing pneumocytoma of a large center 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 six patients diagnosed and treated with sclerosing pneumocytoma in the 10-year period. The study analyzed various parameters, which are: Sex, age, symptoms, size and localization of the tumor, and its gross and histological features. Results: Sclerosing pneumocytoma was more frequently diagnosed in females (83.34%). The patients ranged in age from 38 to 61. Most of the patients (66.66%) were asymptomatic. Two patients underwent a video-assisted thoracoscopic surgery, two patients had a video-assisted minithoracotomy, and two patients underwent a thoracotomy in order to remove the tumor. The tumor was localized in the left lower lobe, in the right upper lobe, and in the right lower lobe in 50%, 33.34%, and 16.66% of patients, respectively. The tumor size ranged from 1 to 2.5 cm. A pathohistological examination of all six cases reported that all four major histological patterns were found in tissue sections: solid, papillary, sclerosing, and hemorrhagic. In all six cases, an immunohistochemical analysis showed positive expression of TTF-1 and panCK in surface epithelial cells, and TTF-1 positivity and panCK negativity in round stromal cells. Conclusions: Sclerosing pneumocytoma is a strictly histological diagnosis supported by clinical and radiological findings and corresponding immunohistochemical methods. Lung pathologists should always keep this tumor in mind, since its spectrum of differential diagnosis is wide, and therefore it can be an important diagnostic pitfall.


Assuntos
Hemangioma Esclerosante Pulmonar/diagnóstico , Hemangioma Esclerosante Pulmonar/cirurgia , Doenças Raras/diagnóstico , Doenças Raras/cirurgia , Adulto , Doenças Assintomáticas , Península Balcânica , Proteínas de Ligação a DNA/análise , Diagnóstico Diferencial , Erros de Diagnóstico , Feminino , Hospitais Universitários , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias de Células Epitelioides Perivasculares/diagnóstico , Hemangioma Esclerosante Pulmonar/patologia , Doenças Raras/patologia , Estudos Retrospectivos , Fatores Sexuais , Cirurgia Torácica Vídeoassistida , Toracotomia , Fatores de Transcrição/análise
13.
Histopathology ; 72(3): 500-508, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28881050

RESUMO

AIMS: Using intraoperative frozen sections to diagnose pulmonary sclerosing pneumocytoma is always challenging. However, an accurate diagnosis is needed to guide surgical management and prevent unnecessary treatment. The aim of this study was to investigate the most frequently misdiagnosed histological patterns and evaluate the potential diagnostic pitfalls of using frozen sections. METHODS AND RESULTS: We reviewed retrospectively 59 cases of sclerosing pneumocytoma that underwent an intraoperative frozen section examination. All original frozen section slides and permanent section slides were reviewed. The rate of accurate diagnosis using frozen sections was 44.1%, the deferral rate was 15.3% and 10 cases (16.9%) were misdiagnosed as malignancy. A solid-predominant pattern is misdiagnosed more frequently than other growth patterns. We also summarised the five major diagnostic pitfalls, including hypercellularity, glandular spaces, desmoplasia-like sclerosis, cellular atypia and coagulative necrosis. CONCLUSIONS: In addition to evaluating the tumour circumscription and identifying the various growth patterns, we propose that the key to avoiding a misdiagnosis is to recognise the dual-cell populations in a tumour, i.e. cuboidal surface cells and stromal round cells.


Assuntos
Secções Congeladas , Hemangioma Esclerosante Pulmonar/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
14.
Pathol Int ; 68(7): 425-430, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29696743

RESUMO

Both glandular papilloma (GP) and sclerosing pneumocytoma (SP) are rare tumors in the lung. We herein report an extremely rare case of coexistence of these two uncommon tumors. The patient was a 40-year-old Japanese woman with no chief complaint. A solitary nodule of the lung was detected using chest computed tomography. The transbronchial biopsy revealed that the tumor histologically corresponded to GP. The patient subsequently underwent partial resection of the right upper lobe. Histological examination of the resected specimens further revealed that the mass contained two different and independent elements and displayed typically histological features of GP and SP. Molecular analysis further revealed the presence of BRAF V600E and AKT1 E17K mutations in GP, whereas only AKT1 mutation was detected in SP. To our knowledge, this is the first case of coexistence of GP and SP in the bronchiole harboring common AKT1 mutation and different BRAF V600E mutational status.


Assuntos
Bronquíolos/patologia , Neoplasias Pulmonares/patologia , Neoplasias Primárias Múltiplas/patologia , Papiloma/patologia , Hemangioma Esclerosante Pulmonar/patologia , Adulto , Feminino , Humanos
15.
Arkh Patol ; 80(1): 52-57, 2018.
Artigo em Russo | MEDLINE | ID: mdl-29460895

RESUMO

The paper presents the data available in the literature on the pathogenesis, clinical and morphological, histological and immunohistochemical features of pulmonary sclerosing pneumocytoma (SP). The paper gives the detailed histological and immunohistochemical characteristics of 6 SP cases. The need for the differential diagnosis of SP is determined by the features and complexity of their histo- and morphogenesis within a single tumor and a complex diagnostic study.


Assuntos
Hemangioma Esclerosante Pulmonar , Diagnóstico Diferencial , Humanos , Morfogênese , Hemangioma Esclerosante Pulmonar/diagnóstico , Hemangioma Esclerosante Pulmonar/patologia
18.
World J Surg Oncol ; 12: 209, 2014 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-25022617

RESUMO

We encountered an extremely rare case of multiple pulmonary sclerosing hemangiomas (PSH) with extensive neuroendocrine lesions, including pulmonary neuroendocrine cell (PNC) hyperplasia, multiple carcinoid tumorlets and typical carcinoid tumors within one pulmonary lobe. To the best of our knowledge, this is the first reported case in the English medical literature of PSH combined and admixed with carcinoid tumors and extensive neuroendocrine proliferation. This case is noteworthy for several reasons. First, the lesion is multi-nodular and unusually large for a typical PSH, which may mimic malignancy on imaging studies and cause diagnostic difficulties. Second, sampling bias may lead to diagnostic errors for a lesion containing two different types of neoplasms. Third, our case displays a rare mixed and mosaic pattern of PSH with a full spectrum of pulmonary neuroendocrine lesions, which may imply a potential intrinsic association in pathogenesis between PSH and concomitant neuroendocrine neoplasms. The clinical implication of multiple PSHs is also discussed.


Assuntos
Tumor Carcinoide/diagnóstico , Proliferação de Células , Hiperplasia/diagnóstico , Neoplasias Pulmonares/diagnóstico , Neoplasias Primárias Múltiplas/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Hemangioma Esclerosante Pulmonar/diagnóstico , Tumor Carcinoide/cirurgia , Feminino , Humanos , Hiperplasia/cirurgia , Neoplasias Pulmonares/cirurgia , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/cirurgia , Tumores Neuroendócrinos/cirurgia , Prognóstico , Hemangioma Esclerosante Pulmonar/cirurgia
19.
Sci Rep ; 14(1): 12242, 2024 05 28.
Artigo em Inglês | MEDLINE | ID: mdl-38806602

RESUMO

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.


Assuntos
Hemangioma Esclerosante Pulmonar , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Hemangioma Esclerosante Pulmonar/patologia , Hemangioma Esclerosante Pulmonar/diagnóstico , Hemangioma Esclerosante Pulmonar/epidemiologia , Hemangioma Esclerosante Pulmonar/cirurgia , Idoso , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/terapia , Neoplasias Pulmonares/epidemiologia , Prognóstico
20.
Med Phys ; 51(6): 4219-4230, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38507783

RESUMO

BACKGROUND: 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. PURPOSE: We aimed to investigate the feasibility of radiomics in the differentiation between PSP and PC. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.


Assuntos
Tumor Carcinoide , Neoplasias Pulmonares , Hemangioma Esclerosante Pulmonar , Tomografia Computadorizada por Raios X , Humanos , Diagnóstico Diferencial , Masculino , Pessoa de Meia-Idade , Feminino , Neoplasias Pulmonares/diagnóstico por imagem , Tumor Carcinoide/diagnóstico por imagem , Hemangioma Esclerosante Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Processamento de Imagem Assistida por Computador/métodos , Adulto , Idoso , Radiômica
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