Methylphenidate causes chronic eosinophilic pneumonia.

A man who is 38 years old and diagnosed with attention-deficit hyperactivity disorder was prescribed methylphenidate. Three weeks later, he began experiencing progressive shortness of breath and coughing. Imaging of his chest showed patchy bilateral ground-glass opacities, and bronchoscopy revealed a 15% eosinophil count in his bronchoalveolar lavage. A transbronchial biopsy confirmed a diagnosis of eosinophilic pneumonia. The patient's condition improved when he was given steroids and stopped taking methylphenidate. However, he developed the same symptoms again a few days after restarting the medication, along with a skin rash. This strongly suggests that methylphenidate was the cause of his eosinophilic pneumonia.

Inverse Lichen Planus Associated With Nail Dystrophy.

Lichen planus is a chronic inflammatory disorder affecting skin and mucosal surfaces. There are multiple variants of lichen planus described in the literature. We report a case of inverse lichen planus in a healthy 50-year-old male who presented to our dermatology clinic with multiple violaceous to hyperpigmented patches affecting both axillae and groin for three months. A skin biopsy confirmed the diagnosis of lichen planus. The patient subsequently developed nail dystrophy affecting his fingernails consistent with nail lichen planus. Early recognition and treatment of nail lichen planus is important to prevent irreversible scarring.

Congenital pulmonary airway malformation complicated by aspergilloma: A rare adulthood presentation - Case report.

Congenital Pulmonary Airway Malformation (CPAM) is an uncommon condition in adults, which typically presents as acute fever and lung abscesses caused by bacterial infections. We present a case of a 39-year-old female with a CPAM in the upper lobe of the right lung, complicated by an aspergilloma, who presented with a history of hemoptysis. The patient underwent an upper lobectomy and is symptom-free in follow-up. 2012 Elsevier Ltd. All rights reserved.

Pathological Features and Clinical Characteristics of Kikuchi-Fujimoto Disease: A Tertiary Hospital Experience in Riyadh, Saudi Arabia.

Background Kikuchi-Fujimoto disease (KFD) - also known as necrotizing histiocytic lymphadenitis - is a benign histiocytic lymphadenitis known for its low incidence and misdiagnosis that occurs mostly in young Asian females more than males. This disease resolves spontaneously in a few months with a low risk of relapse (one in 30 patients) after resolution. Objectives The aim of this study is to share King Abdulaziz Medical City's (KAMC's) experience with KFD by determining its clinicopathological characteristics. Materials and methods In this study, we reviewed histopathological slides and pathological reports of all lymphadenopathy cases (683 cases) in the period between January 2008 and December 2018. Results Forty-four cases of KFD were found and their clinicopathological characteristics were recorded. There is a slight female predominance (59% females versus 41% males) with a wide age range from 10 months to 97 years (mean = 28.8). The majority of the cases (63.6%) are seen in young adults (between 21 and 40 years). Association with autoimmune diseases was shown by 20.5% of cases while viral infection association was shown by few cases. Most cases showed remission (59%) and no deaths were reported upon follow-up. Histopathologically, the majority of cases have proliferative type followed by the necrotic type and few cases showed xanthomatous type. Conclusions Our study has the largest number of KFD cases in this region. It is obvious that KFD has clinical, radiological, and pathological features that overlap with malignancy, especially lymphoma. Knowing this disease and careful diagnostic approach can help avoid misdiagnosis.

Primary Pulmonary Alveolar Rhabdomyosarcoma in a Pediatric Patient: A Case Report With Literature Review.

Rhabdomyosarcoma (RMS) is a rare soft tissue tumor originating from skeletal muscle that is mostly reported in children. The most common sites of involvement are the head, neck, and extremities. The 2020 WHO classification divide RMS into four types: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. Reports of RMS with primary lung origin are rare. We present a case of RMS in a 16-month-old boy who presented with a lung mass and microscopic examination with fluorescence in situ hybridization confirmed the diagnosis of alveolar RMS. In conclusion, RMS should be considered in the differential diagnosis of any lung mass with small round blue cell morphology in the microscopic evaluation and should be distinguished from metastatic RMS of other sites, pleuropulmonary blastoma, lymphoma, neuroblastoma, primitive neuroectodermal tumor (PNET)/EWING, and malignant peripheral nerve sheet tumors (MPNST).

Secondary Spontaneous Pneumothorax Caused by Pulmonary Schistosomiasis.

Schistosomiasis is an endemic disease commonly found in areas of Africa and the Middle East. It is a prevalent parasitic infection in the southern region of Saudi Arabia and Yemen. The most common species found are Schistosoma mansoni and Schistosoma haematobium. Schistosomiasis can manifest in the urinary bladder, liver, and gastrointestinal system. The occurrence of the infection in the lungs is very rare and usually appears after years of initial infection. We report a case of a 23-year-old Yemeni male who presented to the emergency department complaining of sudden, right-sided chest pain with shortness of breath of one day. Examination and chest X-ray revealed the presence of pneumothorax, and a chest tube was inserted accordingly. As the pneumothorax did not resolve and a continuous air leak was present, the patient was taken to the operation theatre on suspicion of a fistula. The diagnostic procedure found the presence of bullae and patterns of inflammatory infection. A resected lung wedge revealed the presence of Schistosoma eggs, and schistosomiasis was diagnosed. In conclusion, spontaneous pneumothorax secondary to infection can present in young healthy males. Meanwhile, schistosomiasis infection must be kept in mind when dealing with patients coming from endemic areas even if they present with no recent visit to endemic areas.

Expression of Immune Response Markers in Arab Patients With Lung Cancer.

PURPOSE: Programmed death-ligand 1 (PD-L1) is a marker for checkpoint inhibitor use in the management of solid tumors, especially in non-small-cell lung cancer (NSCLC). Our study was aimed at determining the patterns of PD-L1 expression and cluster of differentiation 8 (CD8) immunostains in patients with NSCLC in the Arab population. METHODS: Archival tumor tissue from patients with a confirmed diagnosis of NSCLC were obtained and stained for PD-L1 with antibody 22C3, using immunohistochemistry staining and giving the tumor proportion score (TPS) as a percentage from 0%-100% of stained tumor cells. Tumors were categorized into negative expressers (TPS < 1%), low positive (TPS, 1%-49%), and high positive (TPS, 50%-100%). Correlation of expression with clinical and pathologic features, including CD8-positive (CD8+) lymphocyte density, was also analyzed. RESULTS: Two hundred patients with NSCLC were included in the study from 6 centers in Saudi Arabia and Algeria. Median age was 65 years (28-93 years), and the majority were men (75%) with stage 4 NSCLC (64%). The TPS was high in 37 patients (18%), low in 60 patients (30%), and negative in 103 patients (52%). In a univariate analysis, the following were significant predictors of any PD-L1 expression (> 1%): male sex, being Saudi national patients, high expression of CD8+, and presence of tumor-infiltrating lymphocytes. In the multivariate analysis, only high expression of CD8+ cells (≥ 2+) was significant, with an odds ratio of 4.4 (95% CI, 1.5 to 12.9; P = .003). CONCLUSION: PD-L1 expression in our population is similar to the published literature and correlated with the density of CD8+ cells. Validation of the predictive value of this marker in our population and identifying easier and reliable methods to test for it are warranted.

Clinicopathological Characteristics of Hirschsprung's Disease With Emphasis on Diagnosis and Management: A Single-Center Study in the Kingdom of Saudi Arabia.

Introduction: Hirschsprung's Disease (HD) is a motor disorder of the gut caused by the failure of neural crest cells to migrate craniocaudally into the bowel during intestinal development, resulting in a functional obstruction. The majority of patients with HD are diagnosed in the neonatal period when they present with symptoms of distal intestinal obstruction. Aim: This study aims to identify the clinic-pathological characteristic of HD patients in our institution in KSA and comparing it with local and international data. Materials and Methods: This retrospective cohort study was conducted in King Abdulaziz Medical City (KAMC), a tertiary care center in Riyadh, Kingdome of Saudi Arabia (KSA). Results: A total of 54 patients (72% male) were diagnosed with HD. Forty-eight patients (89%) were born at term, and 6 were pre-term. Sixty-three percent of the patients presented in the neonatal period. Twenty-two patients (41%) underwent one-stage endorectal pull-through procedure, 23 patients (43%) two-stage endorectal pull-through, and 9 patients (16%) had three-stage endorectal pull-through. Five out of 54 patients had ganglion cells seen on FS but were absent in the permanent section. Therefore, the concordance rate was 90.8%. Conclusion: FS biopsy is a necessary method to determine the level of aganglionosis intraoperatively in HD, but the definitive diagnosis should be with permanent section. Also, the choice of surgical operation type (single-stage or multi-stage pull-through) depends on the patient's clinical condition.

Patterns of diagnostic procedures for lung cancer pathology in the Middle East and North Africa.

BACKGROUND: Accurate pathological diagnosis is the first critical step in the management of lung cancer. This step is important to determine the histological subtype of the cancer and to identify any actionable targets. Our study aimed at evaluating the patterns of procedures used to obtain pathological diagnosis of lung cancer in the Middle East and North Africa (MENA) Region. METHODS: Data of consecutive patients with the diagnosis of non-small cell lung cancer (NSCLC) were collected from participating centers from different countries in the MENA Region. Methods of obtaining tissue diagnosis and workup were analyzed to determine the practice patterns of obtaining tissue diagnosis of lung cancer. RESULTS: A total of 566 patients were recruited from 10 centers in 5 countries including Saudi Arabia, United Arab Emirates (UAE), Qatar, Lebanon and Algeria. Majority of patients were males (78.1%) with a median age of 61 years (range, 22-89 years). Obtaining tissue diagnosis was successful in the first attempt in 72.3% of patients, while 16.4% and 6.3% of patients required 2nd and 3rd attempt, respectively. The success in first attempt was as follows: image guided biopsy (91%), surgical biopsy (88%), endobronchial biopsy (79%) and cytology (30%). The success in the second attempt was as follows; surgical biopsy (100%), image guided biopsy (95%), endobronchial biopsy (65%), cytology (25%). CONCLUSIONS: More than quarter of the patients required repeated biopsy in the MENA Region. Image guided biopsy has the highest initial yield. Implementing clear process and multidisciplinary guidelines about the selection of diagnostic procedures is needed.

Saudi lung cancer management guidelines 2017.

BACKGROUND: Lung cancer management is getting more complex due to the rapid advances in all aspects of diagnostic and therapeutic options. Developing guidelines is critical to help practitioners provide standard of care. METHODS: The Saudi Lung Cancer Guidelines Committee (SLCGC) multidisciplinary members from different specialties and from various regions and healthcare sectors of the country reviewed and updated all lung cancer guidelines with appropriate labeling of level of evidence. Supporting documents to help healthcare professionals were developed. RESULTS: Detailed lung cancer management guidelines were finalized with appropriate resources for systemic therapy and short reviews highlighting important issues. Stage based disease management recommendation were included. A summary explanation for complex topics were included in addition to tables of approved systemic therapy. CONCLUSION: A multidisciplinary lung cancer guidelines was developed and will be disseminated across the country.

Interobserver Agreement of Usual Interstitial Pneumonia Diagnosis Correlated With Patient Outcome.

CONTEXT: - The histopathologic criteria for idiopathic pulmonary fibrosis were revised in the American Thoracic Society/European Respiratory Society/Japan Respiratory Society/Latin American Thoracic Association guidelines in 2011. However, the evidence of diagnosis based on the guidelines needs further investigation. OBJECTIVE: - To examine whether the revised histopathologic criteria for idiopathic pulmonary fibrosis improved interobserver agreement among pathologists and the predicted prognosis in patients with interstitial pneumonia. DESIGN: - Twenty, consecutive, surgical lung-biopsy specimens from cases of interstitial pneumonia were examined for histologic patterns by 11 pathologists without knowledge of clinical and radiologic data. Diagnosis was based on American Thoracic Society/European Respiratory Society guidelines of 2002 and 2011. Pathologists were grouped by cluster analysis, and interobserver agreement and association to the patient prognosis were compared with the diagnoses for each cluster. RESULTS: - The generalized κ coefficient of diagnosis for all pathologists was 0.23. If the diagnoses were divided into 2 groups: usual interstitial pneumonia (UIP)/probable UIP (the UIP group) or possible/not UIP (the non-UIP group), according to the 2011 guidelines, the κ improved to 0.37. The pathologists were subdivided into 2 clusters in which 1 showed an association between UIP group diagnosis and patient prognosis (P < .05). CONCLUSIONS: - Agreement about pathologic diagnosis of interstitial pneumonia is low; however, results after division into UIP and non-UIP groups provided favorable agreement. The cluster analysis revealed 1 of the 2 clusters providing high interobserver agreement and prediction of patient prognosis.

Patterns of epidermal growth factor receptor mutation in non-small-cell lung cancers in the Gulf region.

The aim of the present study was to determine the prevalence of epidermal growth factor receptor mutations (EGFRmut) in the Gulf region (GR) and its correlation with demographic and clinical characteristics. A multisite retrospective study was conducted, including institutions from Saudi Arabia, the United Arab Emirates and Qatar. All consecutive patients with non-small-cell lung cancer tested for EGFRmut were eligible. Data collected included demographic information, disease characteristics and EGFR test results. Data on 230 patients were obtained. The median age of the patients was 61 years (range, 26-87 years); 169 patients (69.83%) were male and 204 (88.7%) were Arab. The histological subtype was adenocarcinoma in 191 (83.4%) and squamous cell carcinoma in 21 cases (9.17%). Overall, EGFRmut were detected in 66 patients (28.7%), with a prevalence of 32.46% in adenocarcinoma. No squamous cell carcinomas were found to harbor EGFRmut. The univariate and multivariate analyses revealed that female gender, non-smoking status and adenocarcinoma subtype were significant predictors for EGFRmut. There was no difference between Arabs and non-Arabs. In conclusion, to the best of our knowledge, this is the first multisite study to report the prevalence of EGFRmut in the GR population, which was found to be higher compared with that in Western, but lower compared with that in Far Eastern populations. Studies evaluating the efficacy of targeted therapy in this population are underway.

Modification and implementation of NCCN guidelines on non-small cell lung cancer in the Middle East and North Africa region.

A lung cancer committee from the Middle East and North Africa (MENA) region was established to modify the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) on Non-Small Cell Lung Cancer to create a platform for standard care in the region. The committee comprised different experts in thoracic oncology from the region, including the disciplines of medical and clinical oncology, radiation oncology, thoracic surgery, pulmonary medicine, radiology, and pathology. The committee reviewed version 2 of the 2009 NCCN Guidelines on Non-Small Cell Lung Cancer and identified recommendations requiring modification for the region using published evidence and relevant experience. These suggested modifications were discussed among the group and with a United States-based NCCN expert for approval. The recommended modifications, with justification and references, were categorized based on the NCCN Guidelines flow. This article describes these recommended modifications. The process of adapting the first NCCN-based guidelines in the region is a step toward helping to improve lung cancer care in the region and encouraging networking and collaboration.

Bronchiolitis obliterans organizing pneumonia: pathogenesis, clinical features, imaging and therapy review.

Bronchiolitis obliterans organizing pneumonia (BOOP) was first described in the early 1980s as a clinicopathologic syndrome characterized symptomatically by subacute or chronic respiratory illness and histopathologically by the presence of granulation tissue in the bronchiolar lumen, alveolar ducts and some alveoli, associated with a variable degree of interstitial and airspace infiltration by mononuclear cells and foamy macrophages. Persons of all ages can be affected. Dry cough and shortness of breath of 2 weeks to 2 months in duration usually characterizes BOOP. Symptoms persist despite antibiotic therapy. On imaging, air space consolidation can be indistinguishable from chronic eosinophilic pneumonia (CEP), interstitial pneumonitis (acute, nonspecific and usual interstitial pneumonitis, neoplasm, inflammation and infection). The definitive diagnosis is achieved by tissue biopsy. Patients with BOOP respond favorably to treatment with steroids.

Intramuscular myxoma: a rare neck mass in a child.

Intramuscular myxoma (IM) is a true mesenchymal benign tumour which is rare in both adults and children. In children, it most commonly affects the heart, mandible or maxilla. We report a 5-year-old child with IM presenting as a posterolateral neck mass and review the literature. IM, although very rare, should be included in the differential diagnosis of neck masses in the paediatric age group.