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PURPOSE: Anaplastic lymphoma kinase (ALK) mutations occurs in approximately 3-5% of patients with non-small cell lung cancer (NSCLC). Pleural involvement/effusion is common in ALK-positive patients with NSCLC at baseline. The aim of the study was to evaluate the characteristics of ALK-positive patients who have Ple-I/E. METHODS: In this multicenter study, patients with ALK-positive NSCLC who have Ple-I/E were retrospectively analyzed. Clinical and demographic characteristics of the disease, response rates, median progression-free survival (PFS), and overall survival (OS) were evaluated in 362 ALK-positive patients with NSCLC. RESULTS: Of the patients, 198 (54.7%) were male. The median age at the time of diagnosis was 54 (range 21-85) years. All patients' histology was adenocarcinoma (100%). At baseline, 57 (15.7%) patients had Ple-I/E. There was no association between Ple-I/E and gender, lung metastasis, or distant lymphadenopathy (LAP) metastasis. The frequencies of liver, brain, and bone metastases were significantly higher in ALK-positive patients without Ple-I/E compared to those with Ple-I/E (respectively 18.2% vs 4.8%, p = 0.008; 19.1% vs 4.8%, p = 0.001; 20.6% vs 8.9%, p = 0.002). The median PFS was longer in ALK-positive patients who had Ple-I/E (18.7 vs 10.6 months, p = 0.017). Similarly, the median OS was longer in ALK-positive patients who had Ple-I/E (44.6 vs 22.6 months, p = 0.051). CONCLUSION: Brain, liver, and bone metastases were lower in ALK-positive patients with Ple-I/E. Patients presented with Ple-I/E were prone to have better PFS and OS.

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International Federation of Gynecology and Obstetrics (FIGO) staging classification for stage IV epithelial ovarian cancer (EOC) separates stages IVA (pleural effusion) and IVB (parenchymal and/or extra-abdominal lymph node metastases). We aimed to evaluate its prognostic impact and to compare survival according to the initial metastatic location. We conducted a multicenter study between 2000 and 2020, including patients with a FIGO stage IV EOC. Primary endpoint was overall survival (OS). The secondary endpoints were progression-free survival (PFS) and recurrence rates. We included 307 patients: 98 (32%) had FIGO stage IVA and 209 (68%) had FIGO stage IVB. The median OS and PFS of stage IVA patients were significantly lower than those of stage IVB patients (31 versus 45 months (p = 0.02) and 18 versus 25 months (p = 0.01), respectively). Recurrence rate was higher in stage IVA than IVB patients (65% versus 47% (p = 0.004)). Initial pleural involvement was a poor prognostic factor with a median OS of 35 months versus 49 months for patients without initial pleural involvement (p = 0.024). Patients with FIGO stage IVA had a worse prognosis than patients with FIGO stage IVB EOC. Pleural involvement appears to be relevant for predicting survival. We suggest a modification of the current FIGO staging classification.

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Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition known to affect multiple organ systems. While its manifestations are diverse, pulmonary involvement, especially of the pleura, remains less common. We report the case of a 99-year-old Saudi male with a medical history of diabetes mellitus, chronic kidney disease, and hypertension. He presented with dyspnea and syncope, with radiological findings revealing pleural effusion and a mass in the right hemidiaphragm. Laboratory investigations highlighted elevated serum IgG4 levels, and histopathological evaluation confirmed the diagnosis of IgG4-RD. Notably, the patient's thoracic histopathology differed from typical IgG4-RD presentations, emphasizing the variability of the disease. This case underscores the significance of recognizing IgG4-RD as a potential cause of unexplained pleural effusion. It also highlights the need for a comprehensive diagnostic approach, integrating laboratory values, histopathological findings, and clinical context. Given the potential variability in thoracic IgG4-RD histopathology, clinicians should maintain a heightened awareness of this condition to avoid missed diagnoses.

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INTRODUCTION: Stage IVa thymoma is a rare disease without a standard of care. Subtotal pleurectomy and HITHOC introduced in highly selected patients may provide interesting oncologic results. The purpose of this study was to distinguish de novo stage IVa tumors (DNT) from distant relapse (DR) with respect to post-operative and long-term outcomes to provide the procedure efficacy. METHODS: From July 1997-December 2021, 40 patients with IVa pleural involvement were retrospectively analyzed. The surgical procedure was subtotal pleurectomy and HITHOC (cisplatin 50 mg/m2, mitomycin 25 mg/m2, 42 °C, 90 min). The post-operative outcome, disease-free interval (DFI) and overall survival (OS) were analyzed. RESULTS: Mean age was 52 ± 12 years. B2 and B3 thymomas were preponderant (27; 67.5%). The median number of pleural nodes were nine (4-81) vs. five (1-36); p = 0.004 * in DNT and DR, respectively. Hospital mortality rate was 2.5%. There were four specific HITHOC complications (10%). DFI were 49 and 85 months (p = 0.02 *), OS were 94 and 118 months (NS), in DNT and DR, respectively. CONCLUSIONS: Subtotal pleurectomy with HITHOC in IVa offers satisfying results in highly selected patients, for both DNT and DR. Due to the disease rarity, multicentric studies are needed to define HITHOC as a standard of care.

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BACKGROUND: To explore if chest high-resolution computed tomography (HRCT) can make higher accurate stages for thoracic sarcoidosis stage than X-ray (CRX) only. METHODS: Clinical data from medical records of consecutive patients with a confirmed diagnosis of pulmonary sarcoidosis at Shanghai Pulmonary Hospital from January 1 2012 to December 31 2016 and consecutive patients treated at the Sarcoidosis Center of University of Cincinnati Medical Center, Ohio, USA from January 1 2010 to December 31 2015 were reviewed. The clinical records of 227 patients diagnosed with sarcoidosis (140 Chinese and 87 American) were reviewed. Their sarcoidosis stage was determined by three thoracic radiologists based on CXR and HRCT presentations, respectively. The stage determined from CXR was compared with that determined from HRCT. RESULTS: Overall, 50.2% patients showed discordant sarcoidosis stage between CXR and HRCT (52.9% in Chinese and 44.8% in American, respectively). The primary reason for inconsistent stage between CXR and HRCT was failure to detect mediastinal lymph node enlargement in the shadow of the heart in CXR (22.1%) and small nodules because of the limited resolution of CXR (56.6%). Stage determined from HRCT negatively correlated with carbon monoxide diffusing capacity (DLCO) significantly (P < .01) but stage determined from CXR did not. Pleural involvement was detected by HRCT in 58 (25.6%) patients but only in 17 patients (7.5%) by CXR. Patients with pleural involvement had significantly lower forced vital capacity and DLCO than patients without it (both P < .05). CONCLUSION: Revised staging criteria based on HRCT presentations included 5 stages with subtypes in the presence of pleural involvement were proposed. Thoracic sarcoidosis can be staged more accurately based on chest HRCT presentations than based on CXR presentations. Pleural involvement can be detected more accurately by HRCT.

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INTRODUCTION: Malignant mesothelioma is an aggressive neoplasm arising from serosal lining and has a poor prognosis. Definite diagnosis requires confirmation through a biopsy; however, it is sometimes difficult on microscopic evaluation alone and requires the use of a wide panel of immunohistochemical markers. So, immunohistochemistry (IHC) is of paramount importance and must be routinely used for a definite diagnosis. Till date, very few studies on morphology and detailed IHC markers of mesothelioma have been reported from India. AIMS: To analyze the histomorphological findings of malignant mesothelioma, study the utility and role of the various immunohistochemical markers. MATERIAL AND METHODS: A total of 76 cases of mesotheliomas diagnosed at a tertiary cancer center in Udaipur were analyzed retrospectively from January 2015 to January 2020. Comprehensive data were analyzed including demographic, clinical, radiological, histopathological features along with a wide panel of IHC markers. RESULTS: Mesothelioma occurs over a wide age range from 40 to 70 years. It most commonly involved pleura in 68 cases (89.47%) with very few cases from the peritoneum. On computed tomography (CT) scan, nodular pleural or peritoneal thickening was present. On microscopy, the most common histopathological type was epithelioid mesothelioma (58 cases, 74.3%) followed by sarcomatous (9 cases, 12.8%), deciduoid (6 cases, 8.6%), and 3 cases of biphasic (4.3%). On IHC, WT1, mesothelin, and calretinin markers were positive in 85.91%, 80%, and 93.33% cases of mesothelioma, respectively. Other markers were helpful to rule out differential diagnosis in difficult scenarios. CONCLUSION: Therefore, the correlation of histopathology with clinico-radiological findings and judicious use of a panel of IHC markers is required for routine evaluation and definite diagnosis. IHC is also useful in situations with similar morphological spectrum in specific locations.

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OBJECTIVES: Understanding the clinicopathological features of patients with skip N2 metastases (SN2) in clinical early stage lung cancer is important for surgical planning and other treatment considerations; however, the factors associated with SN2 are unclear. This study aimed to investigate the clinicopathological features associated with SN2 in patients with clinical stage IA (cIA) non-small-cell lung cancer (NSCLC). METHODS: We retrospectively studied patients with cIA NSCLC who underwent pulmonary resection (at least lobectomy) and extensive lymphadenectomy (more than ND2a-1) at our institution between January 2004 and December 2010. We investigated the following factors for their association with SN2: age; sex; tumour marker (carcinoembryonic antigen); tumour size on computed tomography (CT), evaluated with a lung-window (LW) and a mediastinal-window (MW) setting; pathology, with or without adenocarcinoma; differentiation; visceral pleural invasion (VPI) and vascular/lymphatic invasion. RESULTS: In total, 422 patients were enrolled, with the following pathological node (pN) statuses: 331 pN0 (78.4%), 39 pN1 (9.3%) and 52 pN2 (12.3%). There were 21 (23.1%) SN2 cases among the patients with nodal metastases. When the cut-off level was defined as a receiver operating characteristic curve with MW (11.5 mm), the sensitivity and specificity of SN2 was 95.2% and 42.9%, respectively. VPI was a statistically independent relevant factor for SN2 in both the patients with cIA and in those with nodal involvement. The VPI classification comprised 59 PL-0 (64.8%), 12 PL-1 (13.2%) and 20 PL-2 (22.0%) with nodal metastases, and there was a significant difference between the three groups (P = 0.03) according to SN2 frequency. There was no difference between VPI 1 and 2 (P = 0.27). CONCLUSIONS: In conclusion, our study suggests that the incidence of SN2 is significantly associated with VPI in patients with cIA NSCLC. Although MW (>11.5 mm) had a low specificity in the assessment of SN2, it had a high sensitivity, suggesting the possibility of a superior benefit compared with LW. Standard hilar and mediastinal lymph node dissection should be required in patients with suspicious VPI and MW (>11.5 mm) on preoperative CT.

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Sarcoidosis is a multi-system granulomatous disorder of an unknown etiology and affects individuals worldwide. It is characterized pathologically by the presence of non-caseating granulomas in more than one involved organ. However, pleural involvement of sarcoidosis is rare and there are no reported cases in Korea. Traditionally, sarcoidosis has often been treated with systemic corticosteroids or cytotoxic agents. In particular, chylothorax with sarcoidosis is usually treated with corticosteroid for approximately 3~6 months, followed by repeated therapeutic thoracentesis, talc pleurodesis, dietary treatment, or thoracic duct ligation where needed. We encountered a 46 years old female patient presenting with cough, dyspnea and both hilar lymphadenopathy (stage I) on chest radiograph. The patient was diagnosed with a non-caseating granuloma, sarcoidosis by a mediastinoscopic biopsy. For one month, she had suffered from dyspnea due to right side pleural effusion, which was clearly identified as a chylothorax on thoracentesis. Corticosteroid therapy with dietary adjustment was ineffective. She was treated successfully with a subcutaneous injection of octreotide for 3 weeks and oral corticosteroid. We report a case of successful and rapid treatment of chylothorax associated with sarcoidosis using octreotide and oral corticosteroid.

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Waldenstr m's macroglobulinemia (WM) is a rare lymphoproliferative disorder characterized by lymphocytic tumor infiltration of the bone marrow and monoclonal IgM gammopathy. There had been anecdotal reports of pleural involvement in WM. We experienced a case of WM with pleural involvement and reported here for the first time in Korea with review of literature. A 73-year-old male patient was admitted to our hospital due to dizziness and general weakness. Serum protein electrophoresis showed M-peak in the gamma-globulin region, which was revealed as IgM kappa macroglobulin by serum and urine immunoelectrophoresis. He complained headache, visual disturbance and epistaxis associated with hyperviscosity syndrome and plasma filtration and combination chemotherapy was performed immediately. Symptoms and laboratory parameters such as serum IgM level and globulin fraction were markedly improved thereafter. But during the treatment, insidiously progressive exertional dyspnea was developed and the chest X-ray showed bilateral pleural effusion. The pleural fluid contained abundant plasmacytoid lymphocytes with reactive mesothelial cells. His dyspnea was completely resolved with clearing of the radiographic pleural effusion after continued steroid therapy.

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Chronic myelogenous leukemia (CML) has been reported to show extra-medullary involvement in 10% of cases and affect mostly lymph nodes and spleen. Extensive pleura involvement of leukemic cells is very rare and no cases of CML infiltrating pleura have been reported in Koreans. Authors recently experienced a case of CML presenting pleural effusion with malignant leukemic cells. A 76-year-old woman was admitted at Inha University Hospital because of dyspnea and rectal bleeding 3 months ago. The patient had a past history of treatment for massive pleural effusion in local clinic 4 month ago. In biochemical test, LD (lactate dehydrogenase) was 620 IU/dL, and serum uric acid was 8.1 ug/dL. Peripheral blood smear showed 3% of blasts and 65% of basophils and bone marrow biopsy revealed marked increase of cellularity. Total cell number of pleural fluid was 14,100/uL and cytological examination of the pleural fluid revealed cells with the morphological features of myeloblasts and occasional megakaryocytes. The bcr-abl gene rearrangement was detected in cells isolated from pleural fluid and bone marrow aspirates. The patient refused treatment with chemotherapeutic for CML and was discharged.

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