Renal cell carcinoma in children and adolescents: a retrospective study of a French-Italian series of 93 cases.

AIMS: Renal cell carcinomas (RCCs) represent 2-5% of kidney malignancies in children and adolescents. Appropriate diagnostic and classification are crucial for the correct management of the patients and in order to avoid inappropriate pre-operative chemotherapy, which is usually recommended if a Wilms' tumour is suspected. METHODS AND RESULTS: A French-Italian series of 93 renal cell carcinomas collected from 1990 to 2019 in patients aged less than 18 years was reclassified according to the 2016 World Health Organization (WHO) classification and the latest literature. TFE3 and TFEB fluorescence in-situ hybridisation (FISH) analyses and a panel of immunohistochemical stains were applied. The median age at diagnosis was 11 years (range = 9 months-17 years). MiT family (MiTF) translocation RCCs accounted for 52% of the tumours, followed by papillary (20%) and unclassified RCCs (13%). Other subtypes, such as SDHB-deficient and fumarate hydratase-deficient RCCs, represented 1-3% of the cases. We also described a case of ALK-rearranged RCC with a metanephric adenoma-like morphology. CONCLUSION: A precise histological diagnosis is mandatory, as targeted therapy could be applied for some RCC subtypes, i.e. MiTF-translocation and ALK-translocation RCC. Moreover, some RCC subtypes may be associated with a predisposition syndrome that will impact patients' and family's management and genetic counselling. A precise RCC subtype is also mandatory for the clinical management of the patients and inclusion in new prospective clinical trials.

Postoperative Bronchopleural Fistula: A Conservative Way of Treatment in Selected Cases.

BACKGROUND: Postoperative bronchopleural fistula represents a challenging issue for thoracic surgeons. The treatment options reported include bronchoscopic or surgical procedures but the method yielding the best results remains unclear. METHODS: In our thoracic surgery department, between January 2011 and June 2020, 11 patients treated conservatively for early bronchopleural fistula after lobectomy or bilobectomy were reviewed. The fistula size ranged between 2 and 3 mm and complete suture dehiscence. RESULTS: In all 11 patients favorable conditions such as clinical stability, complete expansion of the remaining lung, and resolution of the pleural infection allowed a successful conservative treatment with chest tube drainage. CONCLUSION: In selected cases, conservative management of early bronchopleural fistula after lobectomy or bilobectomy may be an alternative therapeutic option to bronchoscopic or surgical procedures, regardless of the fistula size.

Circumferential esophageal leiomyoma: Management by combined robotic surgery and intraoperative endoscopy.

Leiomyoma is the most common benign tumor of the esophagus. Open thoracotomy, the traditional approach adopted for the enucleation of the esophageal leiomyoma, over the years, has been gradually replaced by video-assisted thoracoscopic surgery. However, this minimally invasive approach has limitations, such as two-dimensional vision and reduced range of motion, which have recently been overcome by technical advantages of robot-assisted surgery. In the surgical management of circumferential esophageal leiomyoma, a combined use of robotic surgery and intraoperative endoscopy may be helpful to facilitate tumor enucleation and to prevent esophageal mucosal injury during the surgical procedure.

Diagnostic Accuracy of Ultrasound Guided Percutaneous Pleural Needle Biopsy for Malignant Pleural Mesothelioma.

Background/Objectives: Ultrasound (US) has been progressively spreading as the most useful technique for guiding biopsies and fine-needle aspirations that are performed percutaneously. Malignant pleural mesothelioma (MPM) represents the most common malignant pleural tumour. Thoracoscopy represents the gold standard for diagnosis, although conditions hampering such diagnostic approach often coexist. The Objective was to determine whether ultrasound-guided percutaneous needle biopsy (US-PPNB) has a high diagnostic accuracy and represents a safe option for diagnosis of MPM. Methods: US-PPNB of pleural lesions suspected for MPM in patients admitted from January 2021 to June 2023 have been retrospectively analyzed. An 18-gauge semi-automatic spring-loaded biopsy system (Medax Velox 2®) was used by experienced pneumologists. The obtained specimens were histologically evaluated and defined as adequate or non-adequate for diagnosis according to whether the material was considered appropriate or not for immunohistochemistry (IHC) analysis. The primary objective of the study was the diagnostic yield for a tissue diagnosis. Results: US-PPNB was diagnostic of MPM in 15 out of 18 patients (sensitivity: 83.39%; specificity: 100%; PPV: 100%). Three patients with non-adequate US-PPNB underwent thoracoscopy for diagnosis. We found significant differences in terms of mean pleural lesion thickness between patients with adequate and not-adequate biopsy (15.4 mm (SD: 9.19 mm) and 3.77 mm (SD: 0.60 mm), p < 0.0010. In addition, a significant positive correlation has been observed between diagnostic accuracy and FDG-PET avidity value. Conclusions: US-PPNB performed by a pneumologist represents a valid procedure with a high diagnostic yield and accuracy for the diagnosis of MPM, and may be considered as an alternative option in patients who are not suitable for thoracoscopy.

Incidental synchronous bronchial tumour: an unusual bronchoscopic finding.

We describe a patient with incidental endobronchial synchronous hamartoma and typical carcinoid with different management strategy.

Mediastinal malignant mesothelioma discovered in a patient with dysphagia.

A mediastinal mass in patients with a history of asbestos exposure should raise the suspicion of malignant mesothelioma.

Left lower lobectomy and partial preservation of segmental arteries of left upper lobe: A strategy to avoid pneumonectomy in selected cases.

In this article we report two cases of left lower lobe lung cancer undergoing a surgical procedure that allowed the preservation of lung parenchyma and avoided pneumonectomy. The first case concerned a left lower lobe non-small cell lung cancer with extracapsular spread in a metastatic interlobar lymph node and the second a left lower lobe lung cancer with invasion of the pulmonary artery at the origin of lobar branches to the lower lobe. In both cases, a lung-sparing surgical treatment was preferred and a left lower lobectomy was performed with division of lingular arteries and the interlobar artery, preserving the remaining arterial branches to the upper lobe.

Pembrolizumab as Neoadjuvant Therapy Before Radical Cystectomy in Patients With Muscle-Invasive Urothelial Bladder Carcinoma (PURE-01): An Open-Label, Single-Arm, Phase II Study.

PURPOSE: To determine the activity of pembrolizumab as neoadjuvant immunotherapy before radical cystectomy (RC) for muscle-invasive bladder carcinoma (MIBC) for which standard cisplatin-based chemotherapy is poorly used. PATIENTS AND METHODS: In the PURE-01 study, patients had a predominant urothelial carcinoma histology and clinical (c)T≤3bN0 stage tumor. They received three cycles of pembrolizumab 200 mg every 3 weeks before RC. The primary end point in the intention-to-treat population was pathologic complete response (pT0). Biomarker analyses included programmed death-ligand 1 (PD-L1) expression using the combined positive score (CPS; Dako 22C3 pharmDx assay), genomic sequencing (FoundationONE assay), and an immune gene expression assay. RESULTS: Fifty patients were enrolled from February 2017 to March 2018. Twenty-seven patients (54%) had cT3 tumor, 21 (42%) cT2 tumor, and two (4%) cT2-3N1 tumor. One patient (2%) experienced a grade 3 transaminase increase and discontinued pembrolizumab. All patients underwent RC; there were 21 patients with pT0 (42%; 95% CI, 28.2% to 56.8%). As a secondary end point, downstaging to pT<2 was achieved in 27 patients (54%; 95% CI, 39.3% to 68.2%). In 54.3% of patients with PD-L1 CPS ≥ 10% (n = 35), RC indicated pT0, whereas RC indicated pT0 in only 13.3% of those with CPS < 10% (n = 15). A significant nonlinear association between tumor mutation burden (TMB) and pT0 was observed, with a cutoff at 15 mutations/Mb. Expression of several genes in pretherapy lesions was significantly different between pT0 and non-pT0 cohorts. Significant post-therapy changes in the TMB and evidence of adaptive mechanisms of immune resistance were observed in residual tumors. CONCLUSION: Neoadjuvant pembrolizumab resulted in 42% of patients with pT0 and was safely administered in patients with MIBC. This study indicates that pembrolizumab could be a worthwhile neoadjuvant therapy for the treatment of MIBC when limited to patients with PD-L1-positive or high-TMB tumors.

Unclassified Renal Cell Carcinoma With Medullary Phenotype Versus Renal Medullary Carcinoma: Lessons From Diagnosis in an Italian Man Found to Harbor Sickle Cell Trait.

Medullary carcinoma is a rare malignant tumor of the kidney. It affects individuals of African descent and all cases reported show evidence of sickle cell trait. We reviewed an unusual carcinoma arising in a white man, the ninth in the literature. The tumor demonstrated features associated with renal medullary carcinoma, or unclassified renal cell carcinoma, medullary phenotype as recently described; the presence of sickle cell trait confirmed the diagnosis of medullary carcinoma. This case is helpful in the differential diagnosis with non-sickle cell associated "renal cell carcinoma, unclassified with medullary phenotype," and study of this spectrum of tumors is ongoing.