Erdheim-Chester disease as complex clinical presentation and diagnosis: A case report and concise review of literature.

RATIONALE: Erdheim-Chester disease (ECD) is a rare multisystemic disease characterized by the infiltration of multiple organs by foamy CD68 + CD1a-histiocytes. The genetic background consists of gain-of-function somatic mutations in the mitogen-activated protein kinase pathway. The purpose of the present paper is to make a contribution to the scientific literature on ECD by reporting our experience with a complex clinical case report, along with a concise review of the literature. We discussed the unusual clinical presentation, the complex diagnostic process and the comparison with other published cases. PATIENT CONCERNS: A 70-year-old man presented with arthralgia due to multiple bone areas of sclerosis, first diagnosed with metastases of a prostatic neoplasm. Sequential thorax-abdomen, femoral and homer contrast-enhanced computed tomography (CT) showed pericardial effusion, pulmonary fibrosis, and perirenal fibrous tissue as "hairy kidneys." He underwent. Three bone biopsies were unsuccessful to reach diagnosis. DIAGNOSES: A xanthelasma biopsy showed histopathological signs compatible with ECD; genetic analysis showed the mutation BRAFV600E. INTERVENTIONS: The patient underwent targeted therapy with vemurafenib (BRAF-inhibitor), discontinued 2 weeks later due to the onset of a diffuse erythematous papular rash on the trunk and limbs. OUTCOMES: At the 1-year follow-up, there was only progression of chronic kidney disease (CKD). LESSONS: The present case report describes how ECD diagnosis could represent a challenge for clinicians, owing to its heterogeneous clinical presentation. Early diagnosis followed by prompt therapy is essential for modifying the natural history of the disease.

Postoperative outcomes, lymph node dissection and effects on costs among thoracotomy, video-assisted and robotic-assisted lobectomy for clinical stage I non-small cell lung cancer.

BACKGROUND: Thoracotomy, video-assisted thoracoscopic surgery (VATS) and robotic assisted thoracoscopic surgery (RATS)-lobectomy are widely accepted procedures for the surgical treatment of clinical (c)stage I non- small cell lung cancer (NSCLC). In the current literature which procedure gives more benefits is still debated. We present a comparison between these three procedures in term of advantages and postoperative outcomes. METHODS: A multicentric study about 259 lobectomies from 2013 to 2019: 128 patients underwent TL, 96 VATS and 35 RATS. Different variables were retrospectively analyzed among these three cohorts of patients with diagnosis of cStage I NSCLC. RESULTS: Rate of major complications comparable in VATS, RATS and TL; Advantages for RATS in minor complications (TL 34.4% vs. VATS 18.75% vs. RATS 8.57%. P=0.0015), postoperative days in Intensive Care Unit, days to chest tube removal, length of postoperative hospitalization (P<0.0001) and number of lymph nodes dissected (P=0.0257). Operating times are shorter in VATS than RATS (P<0.05). Pain (NRS Scale) is comparable. CONCLUSIONS: TL remains the conventional approach for stage II-IIIA(N2) NSCLC. RATS showed great advantages, but its higher operating time and costs, mostly, today don't justify its adoption as gold standard for the surgical treatment of cStage I NSCLC, instead of VATS.