Showing papers in "Respiratory medicine case reports in 2017"
TL;DR: A case of invasive pulmonary aspergillosis is reported in a patient taking ibrutinib, a Bruton's tyrosine kinase inhibitor used to treat refractory chronic lymphocytic leukemia, and it is hypothesize that the drug promoted this infection by suppressing innate immune responses against Aspergillus.
Abstract: We report a case of invasive pulmonary aspergillosis in a patient taking ibrutinib, a Bruton's tyrosine kinase inhibitor used to treat refractory chronic lymphocytic leukemia. We hypothesize that ibrutinib promoted this infection by suppressing innate immune responses against Aspergillus. Clinicians should be aware of potential Aspergillus infections in patients treated with this drug.
48 citations
TL;DR: The use of Synthetic Cannabinoids can cause respiratory depression in individuals without an underlying pulmonary disease and adds to the growing number of literature about the presentation and debilitating adverse events from its consumption.
Abstract: Synthetic Cannabinoids are a street drug that is widely attainable and cheap compared to natural cannabis, and has variable potency and unpredictable effects with no commercially available diagnostic test to confirm its presence. Similar to natural cannabis, Synthetic Cannabinoid intoxication can present in several ways with the most common emergency room presentations to be of neurologic and psychiatric manifestation. The respiratory depressive effect of Synthetic Cannabinoids has not been well documented in medical literature. We report four patients admitted in the Intensive Care Unit with acute respiratory failure necessitating endotracheal intubation after use of Synthetic Cannabinoid. All patients had a reversal of respiratory failure in less than 24 h, three patients had a complicated course due to aspiration pneumonia. All four patients exhibited aggressive behavior, with two of them diagnosed with Bipolar Disorder and Cocaine Use Disorder. The effect of Synthetic Cannabinoids in peripheral receptors such as chemoreceptors and baroreceptors can increase bronchial airway resistance. It is postulated that CB1 receptor stimulation could be one of the possible mechanisms of synthetic cannabinoid-induced respiratory depression. Chemical gases released after its inhalation may also cause damage to the bronchiolar epithelium and has the potential to disrupt the protective surfactant layer in the alveoli, which then could interfere with effective gas exchange leading to hypoxia and acidosis. The stimulation of CB1 receptors have a series of downstream signaling effects in the G protein-coupled pathway and mitogen-activated protein kinase (MAPK) pathway, causing suppression of both excitatory and inhibitory neuronal activity. The aforementioned molecular changes in the central nervous system after CB1 receptor stimulation could impact respiration. The use of Synthetic Cannabinoids can cause respiratory depression in individuals without an underlying pulmonary disease and adds to the growing number of literature about the presentation and debilitating adverse events from its consumption. Although there is no specific toxidrome associated with it, clinicians should have a high index of suspicion with its use especially in patients presenting with a history of drug overdose.
39 citations
TL;DR: It is concluded that in this case NSCLC had transformed to SCLC during immunotherapy with nivolumab in a rare case of transformation of non-small Cell lung cancer to small cell lung cancer (SCLC), without epidermal growth factor receptor (EGFR) gene mutation, during Immunotherapy treatment with nIVolumAB.
Abstract: We report a rare case of transformation of non-small cell lung cancer (NSCLC) to small cell lung cancer (SCLC), without epidermal growth factor receptor (EGFR) gene mutation, during immunotherapy treatment with nivolumab. A 75-year-old man was referred to our hospital following the observation of a 64 mm mass in a chest computed tomography (CT) scan. A transbronchial biopsy of the mass identified the pathological presence of poorly differentiated NSCLC, with no histological signs of SCLC. No mutations were identified in the EGFR gene. A clinical diagnosis of NSCLC (cT3N3M1a, stage IV) was made following a positron emission tomography (PET)-CT scan and enhanced brain magnetic resonance imaging. Docetaxel and bevacizumab were selected as the first-line chemotherapy regimen; however, after two cycles, the patient developed a gastrointestinal perforation, and discontinuation of cytotoxic chemotherapy was recommended. Owing to gradual disease progression, immunotherapy with nivolumab was selected as the second-line regimen. During the immunotherapy, the tumor continued to progress and some subcutaneous tumors emerged. Biopsy of a subcutaneous tumor revealed SCLC, with positive immunostaining for cluster of differentiation 56, synaptophysin, and thyroid transcription factor-1. Serum tumor markers of SCLC were also elevated. Based on these results, we concluded that in this case NSCLC had transformed to SCLC during immunotherapy with nivolumab.
28 citations
TL;DR: The clinical course of a patient with metastatic non-small-cell lung cancer who has undergone EGFR targeted therapy and been further challenged by TKI acquired resistance is reported.
Abstract: Specific oncogenes with driver mutations, such as the Epidermal Growth Factor Receptor (EGFR 1) gene can lead to non-small-cell lung cancer formation. Identification of these oncogenes, their driver mutations and downstream effects allow the targeting of these pathways by drugs. Such personalised therapy has become an important strategy in combating lung cancer and highlights the need to test for these mutations. Tyrosine Kinase Inhibitors (TKIs) against EGFR, such as Erlotinib, are able to halt these tumour promoting properties in non-small-cell lung cancers. Third generation EGFR TKIs, such as Osimertinib, are focussing on resulting acquired TKI resistance. Here we report the clinical course of a patient with metastatic non-small-cell lung cancer who has undergone EGFR targeted therapy and been further challenged by TKI acquired resistance. Her extended survival and maintained quality of life are a consequence of these modern, genotype-targeted, personalised metastatic non-small-cell lung cancer therapies.
24 citations
TL;DR: In the current case series, adverse effects from the authors' centers are presented and comment on the treatment and follow-up of the patients are commented on.
Abstract: In the past decade novel agents are on the market for non-small cell lung cancer adenocarcinoma based on pharmacogenomics. The epidermal growth factor receptor mutation, anaplastic lymphoma kinase and programmed death-ligand 1 investigation is necessary in the everyday clinical practice for the oncologic patient. Immunotherapy is nowadays the novel therapy for advanced stage non-small cell lung cancer with two agents nivolumab and pembrolizumab. In the current case series we will present adverse effects from our centers and comment on the treatment and follow-up of the patients.
22 citations
TL;DR: This paper will provide the experience with the 22G needle of the endobronchial convex-probe in several medical situations of the thorax.
Abstract: Endobronchial ultrasound endoscopy is a state of the art diagnostic endoscopic procedure for the thorax. Firstly it was designed mainly for the staging of lung cancer and of course for the diagnosis of suspicious findings in large central airways. The main limitation of the equipment is the diameter of the instrument and therefore it can only be guided through large airways. However; the diameter of the working channel also provides a large tissue sample nowadays with the 19G biopsy needle. We will provide our experience with the 22G needle of the endobronchial convex-probe in several medical situations of the thorax.
22 citations
TL;DR: New pathogens are identified with the evolution of the microbiota in cystic fibrosis respiratory tracts, and acquisition of Ralstonia spp.
Abstract: Background
Patients with Cystic Fibrosis are subject to repeated respiratory tract infections, with recent increasing isolation of unusual pathogens. Ralstonia species have lately been isolated at our institution, an organism historically frequently misidentified as Burkholderia or Pseudomonas. The prevalence of Ralstonia spp. in cystic fibrosis populations has yet to be determined, along with its clinical implications.
21 citations
TL;DR: It might be better to stop administration of JAK inhibitors, if possible, in patients being treated for pulmonary cryptococcosis in patients treated with ruxolitinib.
Abstract: We present the case of a 79-year-old man who showed multiple pulmonary nodules on chest computed tomography (CT) after being treated for 6 months with ruxolitinib, an inhibitor of Janus kinase (JAK) 1 and 2, to treat primary myelofibrosis. We examined the lesions by bronchoscopy, and the biopsy specimen revealed fungus bodies of Cryptococcus with granulomatous inflammation. As a result, the patient was diagnosed with pulmonary cryptococcosis. The patient was treated with fluconazole (200 mg daily for 2 weeks) with concomitant ruxolitinib administration, but the pulmonary lesions progressed. Subsequently, the patient was treated with voriconazole (300 mg daily for 3 weeks), but the lesions worsened further. The administration of ruxolitinib was therefore discontinued, and the dosage of voriconazole was increased to 400 mg daily. Three months later, the pulmonary lesions diminished in size. The present case of pulmonary cryptococcosis occurred in a patient treated with ruxolitinib. Treatment of pulmonary cryptococcosis with concomitant JAK inhibitor administration may result in poor treatment efficacy. It might be better to stop administration of JAK inhibitors, if possible, in patients being treated for pulmonary cryptococcosis.
21 citations
TL;DR: This is the first report of successful crizotinib single-agent therapy in EGFR-mutant NSCLC that acquired MET amplification during erlotinib therapy.
Abstract: MET is a driver oncogene in non-small-cell lung cancer (NSCLC), and its amplification is associated with acquired resistance to epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitors. A 56-year-old Japanese male with lung adenocarcinoma harboring an EGFR exon 21 L858R mutation received erlotinib to which he responded for 12 months. After disease progression, re-biopsy analyses revealed newly developed MET amplification. Neither EGFR exon 20 T790M mutation nor MET exon 14 mutations were detected. The MET inhibitor, crizotinib, showed a dramatic response. This is the first report of successful crizotinib single-agent therapy in EGFR-mutant NSCLC that acquired MET amplification during erlotinib therapy.
19 citations
TL;DR: A rare case of fentanyl-induced chest wall rigidity syndrome is described during a routine bronchoscopy with endobronchial ultrasound guided-transbronchia needle aspiration in a 55 year old male presenting with a lung mass and mediastinal lymphadenopathy.
Abstract: Combination of sedatives such as fentanyl and midazolam during bronchoscopy is recommended by American College of Chest Physician due to its favourable drug profile. It improves patient comfort and tolerance, and is commonly given unless contraindicated. We describe a rare case of fentanyl-induced chest wall rigidity syndrome during a routine bronchoscopy with endobronchial ultrasound guided-transbronchial needle aspiration (EBUS-TBNA) in a 55 year old male presenting with a lung mass and mediastinal lymphadenopathy. This was effectively managed with neuromuscular blockade, intubation and reversal agents including naloxone. This rare complication should be effectively managed by all bronchoscopist as it carries significant mortality and morbidity if not recognised early. We review the literature on the occurrence of fentanyl-induced chest wall rigidity and its predisposing risks factors.
18 citations
TL;DR: A rare case of a patient with lung cancer adenocarcinoma who developed vitiligo throughout his body after nivolumab administration is reported.
Abstract: Non-small cell lung cancer is still diagnosed at late stage due to the lack of early symptoms and methods of diagnostic prevention. In the past ten years several targeted therapies have been introduced or explored. Tyrosine kinase inhibitors and immunotherapy are currently considered the most effective and safe therapies in comparison to the non-specific cytotoxic agents. Regarding tyrosine kinase inhibitors the adverse effects have been fully explored, however; on the other hand for immunotherapy there are still several issues to be clarified. We report a rare case of a patient with lung cancer adenocarcinoma who developed vitiligo throughout his body after nivolumab administration.
TL;DR: The pediatric providers, particularly the emergency department physicians, intensivists and pulmonologists need to be aware of this rare albeit possible toxicity to the respiratory system caused by BAC used as a preservative in albuterol nebulizer solution.
Abstract: Nebulized bronchodilator solutions are available in the United States as both nonsterile and sterile-filled products. Sulfites, benzalkonium chloride (BAC), or chlorobutanol are added to nonsterile products to prevent bacterial growth. Bronchoconstriction from inhaled BAC is cumulative, prolonged, and correlates directly with basal airway responsiveness. The multi-dose dropper bottle of albuterol sulfate solution contains 50 μg BAC per/2.5 mg of albuterol, which may be below or at the lower limit of the threshold dose for bronchoconstriction. However, with repeated albuterol nebulization, the effect can be additive and cumulative, often exceeding the bronchoconstriction threshold. We report a case of a 17 years old patient, who received 32 mg of BAC via nebulization over a period of 3.5 days that probably caused persistent bronchospasm evidenced by failure to improve clinically and to increase peak expiratory flow rate (PEFR) from 125 L/min (27% of predicted value) to 300 L/min (68% of predicted value) within 2 hours of withdrawing BAC. The patient's respiratory status and PEFR improved dramatically once the nebulization solution was switched to BAC free lev-albuterol solution. The pediatric providers, particularly the emergency department physicians, intensivists and pulmonologists need to be aware of this rare albeit possible toxicity to the respiratory system caused by BAC used as a preservative in albuterol nebulizer solution.
TL;DR: A twenty two year old Caucasian male with ulcerative colitis on mesalamine who presented with dry cough, fever, malaise and dyspnea on exertion had peripheral eosinophilia and apical ground-glass opacities on computed tomography of the chest raising the suspicion of mesalamines induced eos inophilic pneumonia.
Abstract: We present the case of a twenty two year old Caucasian male with ulcerative colitis on mesalamine who presented with dry cough, fever, malaise and dyspnea on exertion. He had peripheral eosinophilia and apical ground-glass opacities on computed tomography of the chest. He had no new exposures and his infectious workup was non diagnostic. Bronchoalveolar lavage showed eosinophilia raising the suspicion of mesalamine induced eosinophilic pneumonia. His symptoms improved after discontinuing mesalamine with a tapered dose of steroids.
TL;DR: A case of granulomatous inflammation reactivation affecting the lungs in a patient previously exposed to Pembrolizumab is described and the importance of immunosuppression in controlling the dis-inhibited immune system is asserted.
Abstract: Sarcoid like reaction is a well-known entity that occurs as a consequence to several malignancies or their therapies. Immunotherapy has gained a lot of interest in the past few years and has recently gained approval as first line therapy in multiple advanced stage malignancies. Pneumonitis has been described as complication of such therapy. Granulomatous inflammation has been only rarely reported subsequent to immunotherapy. We describe a case of granulomatous inflammation reactivation affecting the lungs in a patient previously exposed to Pembrolizumab and have evidence of a distant granulomatous infection. We discuss potential mechanisms of the inflammation and assert the importance of immunosuppression in controlling the dis-inhibited immune system.
TL;DR: This is the first case to demonstrate the efficacy of PMX-DHP for rapidly progressive ILD with anti-MDA-5 antibody-positive CADM and a malignancy.
Abstract: The anti-melanoma differentiation-associated gene 5 (MDA-5) antibody is a marker of clinically amyopathic dermatomyositis (CADM) and rapidly progressive interstitial lung disease (ILD) with acute respiratory failure. A 35-year-old woman with cervical cancer showed Gottron's papules, severe hypoxemia, and diffuse ground-glass opacities on chest computed tomography. She was diagnosed with rapidly progressive ILD associated with CADM. Her serum was positive for the anti-MDA-5 antibody. Combination therapy with corticosteroids, immunosuppressants, and direct hemoperfusion using polymyxin B-immobilized fiber column (PMX-DHP) improved her respiratory dysfunction. Eventually, surgery for the cancer was performed successfully. This is the first case to demonstrate the efficacy of PMX-DHP for rapidly progressive ILD with anti-MDA-5 antibody-positive CADM and a malignancy.
TL;DR: The case of a patient with hemoptysis and chest pain, where computer tomography scan of the lung suggested a large, ruptured hydatid cyst, was reported, where it was concluded that the patient had a bacterial lung abscess.
Abstract: Cystic echinococcosis (CE) is an important helminthic zoonotic disease that commonly affects the liver and lungs. Imaging methods and serology establish the diagnosis in most cases. Chest x-ray can diagnose uncomplicated pulmonary hydatid cysts, whereas superinfection and/or rupture of the hydatid cyst (complicated cysts) may change the radiographic appearance and lead to delayed diagnosis and treatment. We report the case of a patient with hemoptysis and chest pain, where computer tomography scan of the lung suggested a large, ruptured hydatid cyst. However, serological tests with indirect hemagglutination (IHA)for Echinococcus granulosus antibodies were negative, and there was massive growth of Streptococcus pneumoniae in sputum. Based on this, we concluded that the patient had a bacterial lung abscess. The diagnosis of CE was only made after surgical removal of the cyst followed by microscopy and polymerase chain reaction.
TL;DR: The case of a 22-year-old female presented with SPM diagnosed by chest x-ray and chest CT scan who was treated conservatively with subsequent spontaneous resolution and the recurrence rate is very low.
Abstract: Spontaneous pneumomediastinum (SPM) is an uncommon finding in young adults presenting usually without any comorbidities or an underlying pathology. It is most commonly due to alveolar rupture in the setting of an inciting event such an underlying asthma, barotrauma, valsalva maneuver, or esophageal rupture. Individuals can have varying presentations, from chest pain, dyspnea and dysphagia, to anxiety, weakness, or facial and neck swelling. The majority of patients have subcutaneous emphysema on examination and can have abnormal laboratory findings such as an elevated C-reactive proteins and leukocytosis. Diagnostic modalities used include chest x-ray, CT scan, ultrasound and barium swallow or esophagram. Majority of individuals and treated conservatively with rest, analgesia and oxygen administration. The prognosis of SPM is usually good with resolution within several days in most cases and the recurrence rate is very low. We report the case of a 22-year-old female presented with SPM diagnosed by chest x-ray and chest CT scan who was treated conservatively with subsequent spontaneous resolution.
TL;DR: Four cases of SSc-ILD are presented that emphasize the importance of early screening and detection, close follow-up, and aggressive management and the need for well-conducted clinical trials designed to identify new and effective treatments.
Abstract: Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-up, and aggressive management. We also highlight the need for well-conducted clinical trials designed to identify new and effective treatments.
TL;DR: A second opinion is needed to confirm the diagnosis of Mesenchymal cystic hamartoma because it is essential to rule out other possibilities including malignancy.
Abstract: Mesenchymal cystic hamartoma, although first reported as early as 1980s, remains a very rare lung disease. There have been less than 20 cases reported to date. Mesenchymal cystic hamartoma usually has an indolent course, but it could potentially result in morbidity and mortality. Biopsy is needed to confirm the diagnosis because it is essential to rule out other possibilities including malignancy.
TL;DR: The first case of a patient with combined SLE, antiphospholipid syndrome (APS) and recurrent DAH who was successfully controlled by continued treatment with RTX is presented.
Abstract: Diffuse alveolar hemorrhage (DAH) is a rare but potentially fatal complication in systemic lupus erythematosus (SLE). DAH is typically characterized by hemoptysis, dyspnea, new infiltrates on chest x-rays or CT-scans and a drop in hemoglobin. DAH is seen in less than 2% of patients with SLE and carries a high acute mortality risk of up to 70-90%. The current treatment of DAH is high-dose intravenous corticosteroids, cyclophosphamide and extensive supportive care. Plasmapheresis is also often considered in the treatment. A few case reports have described patients with SLE and DAH in whom a single series of Rituximab (RTX), a specific anti-CD20-antigen B-cell antibody, successfully has been used to treat DAH. We here present the first case of a patient with combined SLE, antiphospholipid syndrome (APS) and recurrent DAH who was successfully controlled by continued treatment with RTX.
TL;DR: A rare case of acute pulmonary embolism with pulmonary infarction leading to cavitation and subsequent abscess formation requiring left lower lobe resection is described.
Abstract: Pulmonary infarction is an infrequent complication of pulmonary embolism due to the dual blood supply of the lung. Autopsy studies have reported cavitation to occur in only 4–5% of all pulmonary infarctions with an even smaller proportion of these cases becoming secondarily infected. Patients with infected cavitating pulmonary infarction classically present with fever, positive sputum culture, and leukocytosis days to weeks following acute pulmonary embolism. We describe a rare case of acute pulmonary embolism with pulmonary infarction leading to cavitation and subsequent abscess formation requiring left lower lobe resection.
TL;DR: An overview of the diagnostic and therapeutic procedures of the rare tumor is made and a long-term, systematic follow-up was recommended and performed.
Abstract: Solitary Fibrous Tumor of the Pleura (SFTP) is a rare tumor of the pleura. Worldwide about 800 patients diagnosed with this oncological entity have been described in the existing literature. We report our center's 13 year experience. During this time three patients suffering from this rare disease have been treated in our department. All patients were asymptomatic and their diagnosis was initially triggered by a random finding in a routine chest x-ray. The diagnosis was set preoperatively through a needle biopsy under computer tomography (CT) guidance. The tumors were resected surgically though video-assisted thoracoscopic surgery (VATS) or thoracotomy. Because of the lack of specific guidelines due to the rarity of the disease a long-term, systematic follow-up was recommended and performed. Parallel an overview of the diagnostic and therapeutic procedures of the rare tumor is made.
TL;DR: A case of an elderly man who presented to the emergency room with low-grade fever, cough and hemoptysis and was treated with antitubercular chemotherapy and was followed for more than a year without further recurrence of hemopTYsis is presented.
Abstract: Rasmussen's aneurysm, a very rare complication of active pulmonary tuberculosis (TB), is a pulmonary artery aneurysm adjacent to or within a tuberculous cavity. It may lead to rupture and life threatening massive hemoptysis, an uncommon but challenging medico-surgical emergency. This complication warrants attention in view of the resurgence of tuberculosis and increasing occurrence of multi-drug resistant TB, especially in resource-poor, high-TB burden countries like India. We present a case of an elderly man who presented to the emergency room with low-grade fever, cough and hemoptysis. Thoracic Multidetector row Computed Tomographic Angiography (MDCTA) showed left upper lobe consolidation with thick walled cavitary lesions and aneurysm along the apical segmental branch of left upper lobar pulmonary artery. Hemoptysis was successfully controlled with systemic artery embolization using polyvinyl alcohol (PVA) particles. He was treated with antitubercular chemotherapy and was followed for more than a year without further recurrence of hemoptysis.
TL;DR: A case of UAPA is described in an adult presenting with severe pulmonary hypertension and the importance of a detailed evaluation to determine the underlying aetiology is illustrated, particularly in rare causes.
Abstract: Unilateral absent pulmonary artery (UAPA) is a congenital abnormality rarely diagnosed in adults. UAPA has a myriad of clinical presentations and pulmonary hypertension is present in a quarter of all cases. Isolated UAPA commonly affects the right pulmonary artery and occurs as a result of abnormal development of the sixth aortic arch segment. Due to its rarity, it remains a diagnostic and therapeutic challenge. We describe a case of UAPA in an adult presenting with severe pulmonary hypertension. We describe the appropriate diagnostic approach to a patient with pulmonary hypertension and illustrate the importance of a detailed evaluation to determine the underlying aetiology, particularly in rare causes. Furthermore, we review the clinical presentation, diagnosis and management challenges of UAPA in adults.
TL;DR: The case of an 84-year-old man who was diagnosed with a mycotic aneurysm of the abdominal aorta and an adjacent soft tissue mass, after a 3- month history of fever is presented.
Abstract: The combination of miliary tuberculosis and tuberculous mycotic aneurysm has been described in the literature. We present the case of an 84-year-old man who was diagnosed with a mycotic aneurysm of the abdominal aorta and an adjacent soft tissue mass, after a 3- month history of fever. The patient underwent endovascular restoration of the aneurysm and was treated with broad-spectrum antibiotics. One and a half months later the fever relapsed and the chest CT scan revealed findings consistent with miliary tuberculosis and opacities of both upper lobes not present before, while the abdominal CT scan revealed an increase in the size of the para-aortic mass. Tuberculosis was documented by positive culture for M. tuberculosis of bronchial washing and by the CT-guided para-aortic mass biopsy. The patient received anti-TB treatment for 9 months leading to a spectacular improvement of his clinical condition and imaging findings. A review of the literature since 2008 revealed 28 more cases of tuberculous mycotic aneurysm. The treatment and outcome of all cases are described. Mycotic aneurysm of tuberculous etiology remains a reality and has a relatively good prognosis. Although miliary tuberculosis affects mortality even elderly patients may benefit from "aggressive" management and treatment.
TL;DR: A 61-year-old man with hypertension, diabetes mellitus and obesity admitted with severe acute respiratory distress syndrome is found to have Legionella pneumonia with associated diffuse alveolar hemorrhage diagnosed with bronchoscopic sequential bronchoalveolar lavage.
Abstract: Legionella pneumophila is a common, usually underreported and undiagnosed cause of community acquired pneumonia which can lead to significant morbidity and mortality. Diffuse alveolar hemorrhage rarely have been associated with legionella infection. We present a 61-year-old man with hypertension, diabetes mellitus and obesity admitted with severe acute respiratory distress syndrome. He was found to have Legionella pneumonia with associated diffuse alveolar hemorrhage diagnosed with bronchoscopic sequential bronchoalveolar lavage. He was successfully managed with antibiotics, lung protective strategies and intravenous pulse dose steroids. This patient highlights the unusual association of Legionella infection and diffuse alveolar hemorrhage. Additionally, the case re-enforces the need for early and aggressive evaluation and management of patients presenting with pneumonia and progressive hypoxia despite adequate treatment.
TL;DR: The present case is the first in the literature that associates PEComas with a TSC1 abnormality, and the pulmonary manifestations, including imaging appearance and pneumothorax, somewhat resembled those of lymphangioleiomyomatosis, a representative disease belonging to the PEComa family.
Abstract: Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with immunoreactivity for both melanocytic and smooth muscle markers. PEComas occur at multiple sites, and malignant PEComas can undergo metastasis, recurrence and aggressive clinical courses. Although the lung is a common metastatic site of PEComas, they usually appear as multiple nodules but rarely become cystic or cavitary. Here, we describe a female patient whose lungs manifested multiple cystic, cavity-like and nodular metastases 3 years after the resection of uterine tumors tentatively diagnosed as epithelioid smooth muscle tumors with uncertain malignant potential. This patient's subsequent pneumothorax necessitated video-assisted thoracoscopic surgery, and examination of her resected lung specimens eventually led to correcting the diagnosis, i.e., to a PEComa harboring tuberous sclerosis complex 1 (TSC1) loss-of-heterozygosity that originated in the uterus and then metastasized to the lungs. The administration of a gonadotropin-releasing hormone analogue later stabilized her clinical course. To the best of our knowledge, the present case is the first in the literature that associates PEComas with a TSC1 abnormality. Additionally, the pulmonary manifestations, including imaging appearance and pneumothorax, somewhat resembled those of lymphangioleiomyomatosis, a representative disease belonging to the PEComa family. Although PEComas are rare, clinicians, radiologists and pathologists should become aware of this disease entity, especially in the combined clinical setting of multiple cystic, cavity-like, nodular lesions on computed tomography of the chest and a past history of the tumor in the female reproductive system.
TL;DR: An interesting case of a 53-year-old male patient, who was referred to the authors' clinic for persistent cough, which revealed a left suprahilar density associated with plate like atelectasis, causing narrowing of the left upper lobe (LUL) bronchus with calcified bilateral hilar lymph nodes.
Abstract: Pulmonary hamartomas are rare benign tumors consisting of multiple mesenchymal cell lines like cartilage, bone and fat. We discuss an interesting case of a 53-year-old male patient, who was referred to our clinic for persistent cough. Chest X-ray revealed a left suprahilar density associated with plate like atelectasis, which on chest CT was found to be a densely calcified nodule, causing narrowing of the left upper lobe (LUL) bronchus with calcified bilateral hilar lymph nodes. A bronchoscopy revealed a smooth endobronchial mass with calcification, which was removed. Histopathology revealed pulmonary hamartoma.
TL;DR: Based on a case series, which of the three tyrosine kinase inhibitors is the best approach for T790M mutation is indicated.
Abstract: Guidelines for the treatment of non-small cell lung cancer adenocarcinoma positive in epidermal growth factor mutations indicate tyrosine kinase inhibitors. There are currently three tyrosine kinase inhibitors that can be used as first line treatment: gefitinib, erlotinib and afatinib. Regarding erlotinib and afatinib dosage can be modified in the case of severe adverse effects. In the case of disease relapse investigation for T790M mutation has to be made either with re-biopsy or liquid biopsy and osimertinib has to be administered when T790M is diagnosed. Based on a case series we indicate which is the best approach for T790M mutation.
TL;DR: A 38-year-old male with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics, highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eOSinophilic syndromes.
Abstract: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) and hypereosinophilic syndromes (HES). Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes.