Clinical Challenges and Management Strategies in Pulmonary Large-Cell Neuroendocrine Carcinoma.

Pulmonary large-cell neuroendocrine carcinoma (LCNEC) is a rare but aggressive malignancy of the lung. Its nonspecific presentation and propensity for severe disease at the time of diagnosis create challenges in treatment. We report a case of an asymptomatic 61-year-old female who was incidentally found to have a pulmonary nodule after a fall. Upon further workup, she was found to have an aggressive LCNEC. The patient underwent a robotic-assisted left upper lobectomy, which was complicated by left lower lobe bronchus kinking and post-obstructive atelectasis, warranting further management by thoracic surgery. The patient additionally underwent video-assisted thoracoscopic surgery (VATS), open thoracotomy, pneumopexy, and bronchial stenting. This case highlights the need for strategies for early detection in at-risk populations. A multidisciplinary approach, which may involve both medical and surgical subspecialties, is essential in the management of this complex disease from the time of diagnosis to follow-up postoperatively to achieve the best clinical outcome.

An Unusual Presentation of Acute Severe Mitral Regurgitation.

Mitral regurgitation (MR) results from retrograde blood flow from the left ventricle to the left atrium. Common etiologies of acute severe MR include papillary muscle rupture from myocardial infarction, leaflet perforation in infective endocarditis, chordal rupture (pop) in myxomatous valve disease, acute rheumatic fever with carditis, or functional MR due to cardiomyopathies, myocarditis or Takotsubo cardiomyopathy. Here, we present an unusual case of acute severe MR due to ruptured chordae tendineae likely secondary to degenerative valve disease. A 59-year-old male with a past medical history of hypertension and renal calculi was evaluated in the outpatient office for a urologic procedure. He was sent to the emergency room with left-sided chest pain, 6/10 in intensity, burning in nature, and non-radiating with no aggravating and relieving factors. He had nausea and vomiting for the past three days. He reported similar chest pain at rest and on exertion multiple times over the past year. He also had a chronic cough with no recent changes. His examination was unremarkable. Chest X-ray showed interstitial lung markings. Electrocardiography revealed an old right bundle branch block, but no ST/T-wave changes. He was admitted and treated for atypical pneumonia with ceftriaxone and azithromycin. The following morning, he complained of persistent chest pain 9/10 in intensity which improved with nitroglycerin. His examination revealed a new onset holosystolic murmur heard over the precordium. A two-dimensional echocardiogram showed a preserved ejection fraction of 55-60%, severe MR with eccentric jet, concerning for partially flail leaflet of the mitral valve. He was transferred to the university hospital for mitral valve replacement. Patients with acute rupture of chordae tendineae usually progress to severe mitral valve regurgitation. These patients usually present with pulmonary edema, signs of heart failure, and cardiogenic shock. Papillary muscle dysfunction, as well as partial or complete rupture of the mitral chordae can be detected as a new-onset holosystolic murmur and can be a crucial sign for early recognition. In our case, the patient developed a new holosystolic murmur on day two of admission which was recognized early, and prompt surgical intervention was performed.

A Rare Case of Trichilemmal Carcinoma of the Scalp.

Trichilemmal carcinoma (TC) is a rare, low-grade, malignant adnexal tumor. It is usually less than 3 cm long and arises from the external root sheath of the hair follicle, most commonly in sun-exposed areas of the body. The treatment of choice is wide local excision with tumor-free margins. We present an 88-year-old male patient who presented with an incidental large, dry, fumigating mass on his scalp for a one-year duration requiring surgical excision. The mass, initially thought to be a benign sebaceous cyst, was a 12-cm trichilemmal carcinoma diagnosed based on the histopathologic features of the mass. The specimen was composed of keratinaceous material and necrotic debris. The viable tumor was consistent with atypical squamous proliferation. The mass was fully excised down to the scalp on the first encounter, leaving no further tissue to excise. The patient's scalp site remained clean and without bleeding or recurrence. Currently, there is an increasing incidence of trichilemmal carcinoma. The pathophysiology of this disease is still unclear. The radiation from the sun is one of the factors that causes the growth of the lesions due to its location and distribution. Trichilemmal cysts can also transform into malignant trichilemmal carcinomas due to the p53 deletion. TC has a non-aggressive course despite its aggressive histology. The prognosis is generally good as it has low metastatic potential, like cutaneous squamous cell carcinoma. However, TC with metastasis has a poor prognosis, and there is no consensus yet on treatment. For non-metastatic TC, simple surgical excision with adequate (0.5-1 cm) margins is an effective treatment. Different studies use different margins, and there is no consensus on the measurement for margin excision. Regular follow-up is recommended, but further studies regarding follow-up schedules are needed. Furthermore, despite the common use of chemotherapy in cases of malignant TC, only a limited number of studies have explored this treatment approach. Given the increasing incidence of the disease, we highly recommend more research to address this knowledge gap.

Cryptococcal Meningitis in an Immunocompetent Patient With Underlying Risk Factors.

Cryptococcal meningitis, a severe fungal infection of the central nervous system, is usually found in immunocompromised patients, especially those with human immunodeficiency virus/acquired immunodeficiency syndrome. Its occurrence in immunocompetent individuals is rare and the presentation can be nonspecific. We present a case of cryptococcal meningitis in a young, immunocompetent male with a known history of intravenous drug abuse who was also found to have hepatitis C during admission. Induction therapy with amphotericin B and flucytosine was completed for 14 days. This shorter duration was considered as he had a good clinical response with rapid improvement in mental status and intracranial pressure with an extraventricular drain and negative repeat cerebrospinal fluid (CSF) culture. However, during the consolidation phase with fluconazole, the patient developed new neurologic symptoms and the induction phase had to be re-initiated for a total of 28 days. The patient likewise required the re-placement of an extraventricular drain and the creation of a ventriculoperitoneal shunt due to persistent CSF accumulation and increased intracranial pressure. He was eventually discharged on fluconazole for a planned consolidation phase of eight weeks, followed by a prolonged maintenance phase, but the patient was lost to follow-up.

Cardiac Tamponade: An Initial Manifestation of Diffuse Large B Cell Lymphoma.

Pericardial effusion, a clinical condition characterized by an abnormal accumulation of fluid in the pericardial cavity, has multiple etiological factors. One of the prominent causes is malignant effusion. The patient is a 69-year-old female with a past medical history of Crohn's disease, melanoma status post-resection, and osteoarthritis. She presented with complaints of abdominal discomfort, shortness of breath on exertion, and lower extremity swelling for the past 2-3 days. She was recently discharged four days before this visit after being treated for a viral infection. A physical examination was significant for tachycardia, muffled heart sounds, abdominal distention, and bilateral lower extremity swelling. Labs were in the normal range except for elevated liver enzymes and sodium of 130 mmol/L. A chest X-ray revealed a small bilateral effusion; a bedside echocardiogram showed an ejection fraction greater than 70% and a large pericardial effusion >2 cm, consistent with cardiac tamponade. Emergent pericardiocentesis was performed with the drainage of 250 milliliters of hemorrhagic fluid, which was sent for diagnostic studies. Post-procedure echo on the next day showed an EF of 35-40% and no recurrent pericardial effusion. The workup for connective tissue disease was negative except for elevated antinuclear antibodies (ANA). CT of the abdomen and pelvis revealed gastric wall thickening with no solid organ mass. Her pericardial fluid studies were consistent with exudative etiology and positive for atypical lymphoid cells, leading to the diagnosis of diffuse large B-cell lymphoma. Diffuse large B-cell lymphoma is the most common type of non-Hodgkin lymphoma. Malignant pericardial effusion is common due to solid organ malignancy; however, it is rare with diffuse large B cell lymphoma (DLBCL). We present an intriguing case where pericardial effusion was the precursor to the eventual diagnosis of DLBCL, highlighting the complexity and diverse manifestations of this lymphoma subtype.

Myxoma Involving the Pulmonary Valve - A Case Report of an Atypical Cardiac Tumor, Treated Using an Unusual Approach.

Background: Atypical myxoma has been reported in various locations in the heart, however, myxoma involving the pulmonary valve is rare. Here we present a case of pulmonic valve myxoma which was resected via a percutaneous approach. Case Report: A 66-year-old female with known metastatic adenocarcinoma of the lung, and chronic obstructive pulmonary disease presented with acute onset shortness of breath for two days. The patient experienced respiratory arrest en-route to the hospital and required intubation. Computed tomography angiography (CTA) of the chest revealed a new 1.4 × 1.6 cm intracardiac mass along the pulmonary valve. Further evaluation with cardiac magnetic resonance imaging revealed it to be a large vascular tumor on the ventricular side of the pulmonary valve, attached with a narrow stalk. Due to high surgical risk, the patient underwent transesophageal echocardiographic guided percutaneous removal of the mass. Pathology confirmed the mass to be a myxoma. Conclusion: Atypical myxoma should be considered in the differential diagnosis of valvular masses. Percutaneous resection of valvular masses may be feasible in high-risk surgical patients. LEARNING POINTS: Pulmonary valve myxoma is a rare condition and the literature on the characteristics and treatment options for pulmonary valve myxoma is limited.Our patient was treated with a minimally invasive treatment approach: removal of a tumor with intra operative transesophageal echocardiographic guidance using AngioVac and Flow Triever catheters.Percutaneous resection of valvular masses may be feasible in high surgical risk patients.

Parachute Mitral Valve and Mid-Aortic Syndrome - Unusual Associations of Alagille Syndrome.

Background: Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF). Coarctation of aorta (CoA), renal and intracranial arteries are commonly involved vessels in Alagille syndrome. We present two cases with rare cardiovascular manifestations of Alagille syndrome. Case description. Case 1: A 25-year-old female with a history of Alagille syndrome presented to the cardiologist office for progressive exertional dyspnoea, orthopnoea, and palpitations. She was tachycardiac on examination and had an apical diastolic rumble. A transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 60% and parachute mitral valve (PMV) with severe mitral stenosis. A transoesophageal echocardiogram (TOE) showed insertion of chordae into the anterolateral papillary muscle, severe mitral stenosis with a valve area of 0.7 cm. She was referred to a congenital heart disease specialist and underwent robotic mitral valve replacement with improvement in her symptoms. Case 2: A 27-year-old female with known Alagille syndrome and resistant hypertension presented to the cardiologist office due to progressive exertional dyspnoea for a year. She was hypertensive and had a new 2/6 systolic ejection murmur along the left upper sternal border. TTE revealed an LVEF of 60% and pulmonary artery pressure of 19 mmHg. A CoA was suspected distal to the left subclavian artery due to a peak gradient of 38 mmHg. Cardiac magnetic resonance (CMR) imaging ruled out CoA, and diffuse narrowing of the descending thoracic aorta measuring 13-14 mm in diameter was noted. The patient was referred to a congenital heart disease specialist for further management. Conclusion: PMV presenting as mitral stenosis and mid-aortic syndrome are not commonly described anomalies in association with Alagille syndrome. TTE, TOE and CMR played a key role in diagnosis and management of these patients. LEARNING POINTS: Alagille syndrome (ALGS) is a complex multisystem disorder involving the liver, heart, skeleton, face, and eyes. Cardiovascular involvement occurs in up to 95% of the patients.Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF).A parachute mitral valve (PMV) presenting as mitral stenosis and mid-aortic syndrome is not commonly described anomalies in association with ALGS. Here, we present such rare cases.

Left Anterior Descending Coronary T-wave Inversion Pattern (Wellens' Syndrome) Associated with Myopericarditis and a Normal Left Coronary Artery.

Background: Wellens' syndrome is characterised by a history of chest pain with an abnormal electrocardiogram (EKG), demonstrating biphasic or deeply inverted T waves in leads V2-3 (may extend to involve all precordial and lateral limb leads - the type B Wellens' pattern). A Wellens' EKG pattern is considered highly specific for critical stenosis involving the ostial/proximal left anterior descending artery (LAD). However, there are no reported cases of an association of a Wellens' EKG pattern with myopericarditis. Here, we present such a rare case. Case description: A thirty-one-year-old female with known essential hypertension and psoriatic arthritis presented with a constant, central chest pain radiating to the shoulders and back. The patient's physical examination was unremarkable at presentation other than elevated blood pressure at 170/68 mmHg. An EKG at presentation demonstrated deep symmetric T-wave inversions in anterolateral leads with elevated high-sensitivity troponin, and an elevated erythrocyte sedimentation rate. The patient was referred to the cardiac catheterisation laboratory for concerns of a Wellens' EKG pattern; however, invasive angiography demonstrated only obtuse marginal branch disease - no LAD disease was noted. Cardiac magnetic resonance (CMR) imaging confirmed the diagnosis of myopericarditis and absence of myocardial infarction. The patient was medically managed and discharged home in a stable condition. Conclusion: In literature and established clinical practice, the Wellens' EKG pattern is considered highly concerning for critical ostial/proximal LAD stenosis. However, we now propose that myopericarditis may be considered in a differential diagnosis for this EKG pattern. LEARNING POINTS: Wellens' syndrome is characterised by a history of chest pain with an abnormal electrocardiogram (EKG), demonstrating biphasic or deeply inverted T waves in leads V2-3.A Wellens' EKG pattern is considered highly specific for critical stenosis involving the ostial/proximal left anterior descending artery (LAD).Association of Wellens' pattern EKG has been described in association with various other pathologies; however, its association with acute myopericarditis has not been well described.

Case Report and Literature Review of an Anomalous Course of the Left Main Coronary Artery (LMCA) Arising From the Right Sinus of Valsalva (RSV) Presenting as Takotsubo Cardiomyopathy.

Takotsubo cardiomyopathy (TC) mimics myocardial infarction with symptoms like chest pain, electrocardiogram (EKG) changes, and elevated troponin levels, although it typically features normal coronary arteries upon angiography. While often asymptomatic, coronary artery anomalies (CAAs) can cause intermittent vasospasm and endothelial dysfunction, potentially inducing TC. We report the case of a 74-year-old female with a history of hypertension, hyperlipidemia, and peripheral artery disease, who presented with sudden onset chest pain. Initial EKG and elevated troponin suggested myocardial infarction. However, coronary angiography revealed an anomalous left main coronary artery (LMCA) originating from the right coronary artery (RCA), with no significant stenosis. Subsequent transthoracic echocardiography indicated TC, with the left ventricular ejection fraction improving from 35-40% to 60-65% within days. Cardiac computed tomography angiography (CCTA) revealed that the anomalous LMCA originated from the common trunk at the right sinus of Valsalva (RSV), which further continued as a large, dominant RCA. The LMCA branched into a small to moderate left anterior descending artery (LAD) and a non-dominant left circumflex artery (LCx). The LMCA followed a prepulmonic/anterior course, while the LCx took an interarterial course between the aorta and pulmonary artery. The patient was referred for further surgical evaluation. We conclude that the CAA was an incidental finding and was not related to underlying TC. Although rare, this case suggests a possible correlation between CAAs and a predisposition to stress-induced cardiomyopathy, warranting further investigation.

An Unusual Case of Thalamic Stroke in a Young Adult With Patent Foramen Ovale and Finasteride Use.

Symptomatic cerebral infarcts with cryptogenic ischemic stroke pose diagnostic challenges due to unknown etiology. Notably, up to half of young individuals with cryptogenic stroke exhibit patent foramen ovale (PFO), while finasteride, which is used for male pattern baldness, elevates testosterone levels, potentially increasing the risk of thrombosis. Here, we present a case of thalamic infarction in a 21-year-old male devoid of cerebrovascular risk factors but with PFO and finasteride use. The patient presented with short-term memory issues, otherwise lacking medical history or substance use. Examination revealed neurological deficits, with imaging indicating a left thalamic infarct. Subsequent investigations identified PFO, prompting referral for closure, yielding symptomatic improvement. Furthermore, discontinuation of finasteride was advised due to its thrombotic association. Finasteride's inhibition of 5-alpha reductase 2 increases testosterone conversion to estrogen, potentially promoting thrombosis. Finasteride use can cause thrombotic events, emphasizing its risk. In conclusion, young embolic stroke patients warrant PFO evaluation alongside hypercoagulable workup, with closure benefiting those under the age of 55. Additionally, discontinuing finasteride may mitigate thrombosis risk.

Infective Endocarditis Complicated by Iatrogenic Intracranial Hemorrhage Secondary to Cefazolin-Induced Coagulopathy.

Infective endocarditis can be acute or subacute. It can be caused by viral, bacterial, fungal, and sometimes nonbacterial etiologies. It is an important cause of mortality and morbidity in children as well as adolescents, despite advances in management. A 59-year-old male with a past medical history of aortic valve (AV) replacement on warfarin presented to the Emergency Department with dull right flank pain and poor dentition on examination. Computerized tomography (CT) scans of the abdomen revealed the presence of splenic and renal infarcts. Warfarin was held after the international normalized ratio (INR) was noted to be elevated at 11. Following the activation of the sepsis bundle in the ER, he received intravenous fluids (30 cc/kg) and was started on vancomycin and ceftriaxone. On further evaluation, the transesophageal echocardiogram revealed mobile densities on the aortic surface concerning vegetation. Antibiotics were transitioned to cefazolin, gentamycin, and rifampin for the management of prosthetic valve endocarditis. The patient's INR improved to 3.5 on the third day of hospitalization, and heparin was initiated to maintain anticoagulation for the prosthetic valve. However, on the eighth day of hospitalization, the patient developed left-sided weakness and slurred speech. The CT head showed acute frontoparietal intracranial hemorrhage (ICH), with an INR noted to be 5. Heparin was reversed with protamine sulfate, and vitamin K was administered, following which the INR improved to 2.3. The patient was transferred to intensive care, but on the second day of the ICU stay, the INR again shot up to 6 with normal LFTS. The patient received vitamin K, but the INR only improved to 5. Subsequently, antibiotics were changed from cefazolin to nafcillin. INR thus fell to 1.6 in two days after changing the antibiotics. The patient was soon transferred to a higher center for aortic valve replacement. While few case reports have described severe coagulopathy induced by cefazolin, it is particularly seen with impaired renal function; however, our patient's renal function was completely normal. Coagulopathy is due to the drug's effect on intestinal flora and its structural methyl-thiadiazole side chain, which has similar effects as epoxide reductase inhibitors and results in INR elevation. Patients on cefazolin need to be closely monitored for INR levels every day, as there is a high likelihood of developing complications like ICH, as noted in this patient. While the monitoring of cefazolin levels is not necessarily indicated, it is necessary to place patients on fall precautions and monitor INR levels every day, as mentioned above.

Pericardial Tamponade and Berger's Disease: An Unusual Association.

Cardiac tamponade is considered a medical emergency because a patient can deteriorate easily and die of cardiac arrest if the fluid is not drained immediately. The most common etiologies are the same as pericarditis as fluid accumulates due to pericardial inflammation, including infection, malignancy, trauma, iatrogenic, autoimmune, post-myocardial infarction, radiation, and renal failure. Although the treatment is pericardiocentesis or pericardial window, finding the etiology responsible for the development of pericardial effusion is important. Here, we describe the case of a 40-year-old female who presented to the emergency department with a chief complaint of severe epigastric pain of a two-day duration that was associated with multiple episodes of nausea, vomiting, dysphagia, and severe shortness of breath (New York Heart Association III). The patient was eventually diagnosed with cardiac tamponade as a cause of her dyspnea, as a two-dimensional cardiac echocardiogram detected a large pericardial effusion (>2 cm) with echocardiographic indications for cardiac tamponade with severe pulmonary hypertension. The patient underwent a therapeutic pericardial window with drainage of 250 mL of pericardial fluid. Ultrasound of the abdomen focusing on the kidneys showed an atrophic and echogenic right kidney with a bidirectional flow in the hepatic veins, suggestive of right heart failure. Subsequently, she underwent a kidney biopsy that showed diffuse mesangial proliferative glomerulonephritis with segmental sclerosing features consistent with IgA nephropathy, associated with tubular atrophy, interstitial fibrosis, interstitial inflammation, and moderate arteriosclerosis. The patient was diagnosed with stage V chronic kidney disease secondary to IgA nephropathy. IgA nephropathy is usually common in Caucasian or Asian males in their teens and late 30s, with hematuria as a usual presentation. This case is unique as cardiac tamponade with renal failure is rarely the presenting symptom of IgA nephropathy.