Review of real-world experience with lurbinectedin in relapsed/refractory small cell lung cancer.

Lurbinectedin, a novel antineoplastic agent, was granted the orphan drug designation by the United States Food and Drug Administration (US FDA) and approved for use in relapsed/refractory small cell lung cancer in June 2020. The approval was granted after its efficacy was demonstrated in a multicenter open-label, multi-cohort study enrolling 105 participants. Since then, real-world studies have examined the efficacy and safety profiles of lurbinectedin in clinical practice. By examining these outcomes, this review aims to provide clinicians with the tools necessary to make informed clinical decisions.

Miller-Fisher Syndrome Unveiled in the Presence of Cholangiocarcinoma.

Miller-Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome, characterized by ataxia, areflexia, ophthalmoplegia, and possible facial, swallowing and limb weakness alongside respiratory failure. Variations within MFS may include respiratory and limb weakness and Bickerstaff brainstem encephalitis (BBE), marked by altered consciousness, ataxia, ophthalmoparesis, and paradoxical hyperreflexia. MFS can emerge in both children and adults, often following bacterial or viral illness. While autoimmune-driven nerve damage occurs, most MFS patients recover within six months without specific treatment, with a low risk of lasting neurological deficits or relapses. Rarely fatal, MFS's co-occurrence with cholangiocarcinoma (CCA) presents unique management challenges. CCA, primarily affecting bile ducts, has a bleak prognosis; surgical resection offers limited cure potential due to late-stage detection and high recurrence rates. Advances in CCA's molecular understanding have led to novel diagnostic and therapeutic approaches, requiring a comprehensive interdisciplinary care approach for optimal MFS and CCA management outcomes. Herein, we present a 50-year-old male with a complex medical history who was admitted to the hospital due to abdominal discomfort, nausea, vomiting, and ascites. Imaging revealed pneumonia and secondary bacterial peritonitis. Later, he developed neurological symptoms, including weakness, gait abnormalities, and brainstem symptoms, leading to the diagnosis of MFS. Despite treatment efforts, his condition deteriorated, leading to acute liver failure and unexplained anasarca. N-acetyl cysteine was initiated for liver issues. Neurologically, he showed quadriparesis and areflexia. Intravenous immunoglobulin (IVIG) treatment improved his neurological symptoms but worsened gastrointestinal issues, including ileus and elevated CA19-9 levels, suggesting a potential carcinoma. A liver biopsy was performed. After IVIG treatment, he experienced widespread discomfort, emotional unresponsiveness, swallowing difficulties, and aspiration risk, ultimately leading to his demise.

Recurrent Left Renal Fossa Clear Cell Renal Cell Carcinoma With Pancreatic Tail Invasion Presenting as Intestinal Obstruction.

Distant renal cell carcinoma (RCC) metastatic disease is mostly seen in the lungs, bones, and lymph nodes. The incidence of local recurrences within the ipsilateral retroperitoneum (RFR) is very low. We report a case of a 79-year-old male with recurrent left renal fossa RCC with pancreatic tail invasion who presented with large bowel obstruction. To the best of our knowledge, no cases have been reported of recurrent left renal fossa RCC initially presenting as extrinsic large bowel obstruction.

A Diagnostic Dilemma: A Case of Neurosarcoidosis Without Systemic Sarcoidosis.

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology characterized by non-caseating granulomas in involved organs. Approximately 10% of patients with sarcoidosis exhibit central nervous system involvement. However, the occurrence of isolated neurosarcoidosis without concurrent systemic signs is very rare, affecting less than 1% of patients. We report a case of isolated neurosarcoidosis in a previously healthy patient who initially presented with a single episode of seizure and loss of consciousness. Brain MRI showed T2/fluid-attenuated inversion recovery (FLAIR) hyperintense extra-axial soft tissue mass over the left cerebral convexity measuring approximately 14 mm in maximum depth. Excisional biopsy of the brain mass showed chronic non-caseating granulomatous inflammation with epitheloid cells that was consistent with sarcoidosis. Treatment with high dose-steroids led to significant clinical improvement. At a two-year follow-up, there were no signs of systemic disease or recurrence of the meningeal mass. This case emphasizes the rarity of such presentation, diagnostic difficulties, and the importance of high suspicion and timely management to prevent debilitating neurologic complications.

The Role of Dual Antiplatelet Therapy (DAPT) vs Surgery in a Case of Moyamoya Disease: A Case Report and Review of the Literature.

Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by non-atherosclerotic and non-inflammatory progressive narrowing of the intracranial part of the carotid artery and its proximal branches. The disease process is commonly associated with the development of weak, dilated collateral blood vessels at the base of the brain. This gives it a classic smoky appearance on cerebral angiograms and hence the name "Moyamoya" which means "puff of smoke" in Japanese. When a patient has similar vasculopathy in the setting of another disease then it is known as Moyamoya syndrome (MMS). The associated diseases are sickle cell anemia, neurofibromatosis, long-standing diabetes, uncontrolled hypertension, or chemotherapy. Despite being known as a disease of the East Asian population, the disease is no longer exclusive to Asians, as evidenced by the rising incidence among non-Asian groups such as Caucasians, Hispanics, and African Americans. Patients can remain asymptomatic or present with ischemic or hemorrhagic stroke, headache, seizures, or recurrent transient ischemic attacks. Conventional cerebral angiography is considered the gold standard for diagnosing MMD. Treatment may be supportive, medical, or surgical. We present the case of a 42-year-old African American woman with several comorbidities who presented with sudden onset of ischemic stroke and upon further workup was found to have MMD. Equally important is to identify the most effective therapeutic approaches based on individual patients to achieve better clinical outcomes. Our case report highlights the importance of surgery in symptomatic MMD with a lack of supporting evidence indicating the benefits of dual antiplatelet therapy (DAPT).

Demographics and outcomes of hemoglobin genotype in hospitalized patients with COVID-19 and sickle cell disease in the United States.

INTRODUCTION: Coronavirus disease 2019 (COVID-19) is associated with poor outcomes in sickle cell disease (SCD) patients. However, there is a paucity of data comparing hemoglobin (Hb) genotypes in SCD and infection outcomes. METHODS: The National Inpatient Sample was used to identify the record of hospitalizations with COVID-19 and SCD in 2020 using the International Classification of Disease, Tenth Revision codes. Study outcomes (invasive mechanical ventilation, extracorporeal membrane oxygenation, shock, vasopressor use, measures of resource utilization, and in-hospital mortality) were compared between hemoglobin SS, SC, and S-beta thalassemia (Sß). RESULTS: Of the 102 975 COVID-19 hospitalizations with SCD, 87.26% had HbSS, 7.16% had HbSC, and 5.58% had HbSß. Younger patients were more likely to have HbSS, while older patients were likely to have HbSC and HbSß. HbSS was more frequent with Blacks, while HbSß was more prevalent with Whites and Hispanics. Though measures of resource utilization were higher in HbSS, there was no significant difference in in-hospital outcomes between the three genotypes. CONCLUSION: There is no difference in COVID-19 outcomes among Hb genotypes in SCD. Further studies are needed to explore the reasons for this observation.

An Unusual Source of Cerebral Embolism Caused by Lambl's Excrescences.

Lambl's excrescences (LEs) are delicate filiform strands formed by connective tissue located along the valve closure lines within the cardiovascular system. Most cases of these excrescences manifest without discernible symptoms, and the exact etiological factors contributing to their formation remain unknown. These excrescences may embolize to the brain, causing strokes. It is essential that all other possible causes of stroke be eliminated prior to identifying Lambl's excrescences as the cause of the stroke. Herein, we present a case of a patient who suffered a stroke, and all conventional testing for common causes of embolic strokes was ruled out. In pursuit of a comprehensive evaluation encompassing the classification of the stroke as cryptogenic, a transesophageal echocardiogram (TEE) was performed, which effectively disclosed the existence of LEs situated on the aortic valve leaflets. The patient was treated with anticoagulation and discharged with close follow-up monitoring. To culminate, the inclusion of this case within our study augments the currently scarce pool of instances that exhibit such characteristic cardioembolic phenomena, thereby accentuating the necessity for additional prospective investigations to substantiate the existence of a causative association linking LEs and cardioembolic strokes.

COVID-19-Associated Disseminated Intravascular Coagulopathy Presenting As Inferior ST-Segment Elevation Myocardial Infarction.

Disseminated intravascular coagulopathy (DIC) is infrequently associated with COVID-19 infection. COVID-19 infection can predispose to thrombotic events through inflammation and microvascular injury. DIC is rarely associated with coronary artery disease, especially myocardial infarction (MI). In this case report, we present an uncommon case of a patient with concurrent DIC and MI in the setting of COVID-19 infection. A 73-year-old male patient with no known cardiovascular risk factor presented with syncope. Assessment in the field by emergency medical service (EMS) showed the patient had a third-degree atrioventricular block and a heart rate of 40 beats per minute. He was given atropine and transcutaneously paced. Upon admission, he was found to have an inferior wall ST-elevation myocardial infarction (STEMI) and tested positive for COVID-19. Cardiac catheterization was performed urgently and revealed triple vessel disease. Attempts to revascularize the vessels were unsuccessful. He subsequently developed cardiogenic shock. He was started on multiple pressor support. Laboratory workup was suggestive of DIC, and he later developed multiorgan failure. Continuous renal replacement therapy was initiated but failed due to persistent thrombosis of the dialysis access. Despite all measures, the patient developed cardiac arrest and passed away on the third day of hospitalization. Our understanding of COVID-19 and its complications has grown exponentially since the beginning of the pandemic. The pro-inflammatory state induced by the disease creates a hypercoagulable state that may result in thrombotic complications, including MI. In severe cases, a consumptive coagulopathy may develop, leading to DIC. This unique case report seeks to highlight the importance of staying vigilant about the potential complications of MI and DIC induced by COVID-19 so that prompt diagnosis can be made to reduce morbidity and mortality in these patients.

Outcomes of COVID-19 hospitalizations in patients with sickle cell disease: A nationwide analysis.

INTRODUCTION: There is a paucity of data on the outcomes of coronavirus disease 2019 (COVID-19) in patients with sickle cell disease (SCD) in the United States. We examined the outcomes of patients with COVID-19 and SCD. METHODS: We utilized the National Inpatient Sample (NIS) to identify the data of patients diagnosed with COVID-19 and SCD in 2020 using the International Classification of Disease, Tenth Revision codes. In-hospital outcomes (invasive mechanical ventilation and mortality) were compared between SCD and non-SCD groups. RESULTS: Of the 1 057 550 COVID-19 hospitalizations, 2870 (0.3%) had SCD. The median age of the SCD group was 42 (IQR: 31) vs. 66 (IQR: 23) in the non-SCD group (p < .0001). Patients with SCD were likely to be females (62.02% vs. 37.98%, p < .0001), Blacks (87.81% vs. 12.19%, p < .0001), and in the lowest income quartile (50.62% vs. 11.15%, p < .0001). There was no difference in the outcomes between the two groups. There were increased odds of invasive mechanical ventilation and in-hospital mortality in COVID-19 in Asians, Hispanics, Native Americans, and Blacks (except for in-hospital mortality) compared to Whites. CONCLUSION: In-hospital mortality and invasive mechanical ventilation outcomes in SCD are comparable to that in non-SCD patients hospitalized with COVID-19.

Pituitary Metastases From Differentiated Thyroid Cancers: A Systematic Review.

Background: Pituitary metastasis (PM) from differentiated thyroid cancer (DTC) is extremely rare and may adversely affect outcomes. We aimed to assess the characteristics and outcomes of patients with PM from DTC. Methods: We systematically reviewed the literature on publications on PM and the different DTC histologic types (papillary, follicular, and Hurthle cell cancers). Three databases (PubMed, Embase, and Scopus) were searched for articles published from 1967 to 2022. Survival time was estimated as the period from the first treatment of PM to the time of death or last follow-up. Results: Twenty-five articles comprising 27 cases that met the eligibility criteria were identified using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). The median age of the patients was 60 years (23 - 86). A preponderance of females (66.7%) with PM most commonly reported papillary thyroid cancer (55.6%). This was followed by follicular thyroid cancer (37.0%) and Hurthle cell cancer (7.4%). The most common presentations were headache, nausea, and vomiting, with visual symptoms in 44.4%. Diabetes insipidus was an infrequent finding (7.4%). The median time from diagnosis or first treatment of DTC to the diagnosis of PM was 3 years (0 - 25). The most common endocrine abnormality was hyperprolactinemia (63.2%), while the most frequently deficient hormone was luteinizing hormone (50%). The most common treatment modality for PM was a combination of radiotherapy and surgery with or without radio-iodine. At the end of the follow-up, 30% of the patients died. Only 33.3% of the patients achieved complete resolution of symptoms. The overall median survival time was 12 months (3 - 108). There was a moderate inverse correlation between the age of patients and survival, which was, however, not statistically significant (rs = -0.45, P = 0.103). Conclusion: PM from DTC is extremely rare, and Hurtle cell cancer appears to be the least associated with PM. Diabetes insipidus is a rare initial manifestation of PM from DTC. Complete resolution of symptoms is less likely to be achieved in PM from DTC. Older age may confer an increased survival tendency, probably due to more intracranial space volume in older people compared to the younger population. Larger studies are needed to examine the relationship between age and survival in PM from DTC. Also, more observational data are required to determine the predictors of survival and compare the efficacy of the different treatment modalities in patients with PM from DTC.

Prevalence and outcomes of pulmonary embolism with sickle cell disease: Analysis of the Nationwide Inpatient Sample, 2016-2020.

INTRODUCTION: The outcomes of pulmonary embolism (PE) in sickle cell disease (SCD) are poorly established in the literature. This study examined the prevalence and outcomes of patients with PE and SCD. METHODS: The National Inpatient Sample was used to identify patients' data with a diagnosis of PE and SCD in the United States from 2016 to 2020 using the International Classification of Disease, 10th Revision codes. Logistic regression was used to compare outcomes between those with and without SCD. RESULTS: Of the 405 020 patients with PE, 1504 (0.4%) had SCD, and 403 516 (99.6%) did not have SCD. The prevalence of PE with SCD was stable. Patients in the SCD group were more likely to be female (59.5% vs. 50.6%; p < .0001), Black (91.7% vs. 54.4%; p < .0001), with a lower rate of comorbidities. The SCD group had higher in-hospital mortality (odds ratio [OR] = 1.41, 95% confidence interval [CI]:1.08-1.84; p = .012) but lower catheter-directed thrombolysis (OR = 0.23, 95% CI: 0.08-0.64; p = .005), mechanical thrombectomy (OR = 0.59, 95% CI: 0.41-0.64; p < .0029), and inferior vena cava filter placement (OR = 0.47, 95% CI: 0.33-0.66; p < .001). CONCLUSION: In-hospital mortality remains high in PE with SCD. A proactive approach, including maintaining a high index of suspicion for PE, is needed to reduce in-hospital mortality.

Malignant Pleural Effusion As the Initial Presentation of Renal Cell Carcinoma: A Case Report and Literature Review.

Renal cell carcinoma is the most common renal neoplasm. Its presentation is often very occult, and it may be discovered incidentally. It may present with the classic symptoms of back pain, flank pain, hematuria, or hypertension. Renal cell carcinoma may also present with malignant pleural effusion at diagnosis; however, it is very rare. In this case report and literature review, we describe a 77-year-old male who was diagnosed with renal cell carcinoma after presenting with a malignant pleural effusion - an extremely rare phenomenon. An analysis of the literature yielded 13 case reports, including ours, where the diagnostic presentation of renal cell carcinoma was a malignant pleural effusion. Our patient presented with left-sided chest pain. Imaging was suggestive of pleural effusion. CT and MRI imaging demonstrated masses in the upper and lower poles of the right kidney suggestive of renal cell carcinoma. CT imaging also showed lung nodules that were suggestive of pulmonary metastases. Biopsy and immunostaining of pleural tissue were positive for clear cell renal cell carcinoma. Therapeutic thoracentesis was performed. Despite this, the patient developed recurrent large-volume pleural effusions requiring drainage and placement of a pleural catheter. Our patient's extremely rare presentation of malignant pleural effusion as the diagnostic presentation of renal cell carcinoma along with recurrent, large-volume effusions requiring drainage has only been reported in the form of case reports in the literature.

Recurrence of T-Cell Prolymphocytic Leukemia With a Rare Presentation as Diffuse Generalized Skin Lesion.

T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive neoplasm that originates from mature post-thymic T-cells. Cutaneous manifestations are a common presentation in T-PLL but rarely are a presentation in the recurrent setting. Here, we describe the case of a 75-year-old female with a history of T-PLL-who at the time of initial diagnosis did not exhibit any rash-presenting with diffuse rash, facial swelling, sore throat, and dysphagia 7 months later and was found to have recurrent T-PLL. She had diffuse lymphadenopathy and diffuse skin lesions. Biopsy of the skin lesions also confirmed infiltration with T-PLL cells. After review of the literature, no previously reported cases of recurrent T-PLL presented as diffuse skin lesions. This case demonstrates that recurrent T-PLL may present with diffuse rash, respiratory distress, and anasarca. It is important to stay vigilant in patients with history of T-PLL to recognize signs of recurrent disease to allow prompt diagnosis and treatment.

Malignant Pleural Effusion Due to Uterine Serous Carcinoma: An Unusual Presentation.

Endometrial cancer is the most common cancer of the female genital tract. It can rarely metastasize to the pleura and present as a malignant pleural effusion. Here we present the case of a 61-year-old female with two primary malignancies, breast and endometrium, who presented to us with shortness of breath. Imaging was suggestive of a malignant pleural effusion. Diagnostic and therapeutic thoracentesis were performed that were initially suggestive of a breast source. However, final pleural fluid studies showed endometrial serous carcinoma as the source of the effusion. The patient received pembrolizumab and lenvatinib treatment and continues to be followed up in our clinic.

Breast Cancer Complicated by Cardiac Tamponade in a Patient With Neurofibromatosis Type 1.

Pericardial effusion may occur as a result of malignant pericarditis, which may in turn result in cardiac tamponade. This paper reports on a rare case of cardiac tamponade that occurred in an African American patient with breast cancer and neurofibromatosis. Herein, we present a case of a 38-year-old woman with neurofibromatosis type 1 (NF1) and breast cancer. She presented with sudden shortness of breath and hypotension. Computed tomography of the chest and an echocardiogram confirmed the presence of cardiac tamponade. Symptomatic relief was obtained following an emergency pericardiocentesis. The patient experienced a recurrence of symptomatic pleuro-pericardial effusion, requiring repeat therapeutic pericardiocentesis and thoracocentesis. To eliminate accumulating fluid, an indwelling drain was placed. The clinical condition of the patient, however, continued to deteriorate and she expired a few days after admission. When patients with breast cancer present with dyspnea, clinicians should maintain a high index of suspicion of cardiac tamponade; urgent imaging should be performed to exclude tamponade. Further research is needed to identify the factors that predict cardiac tamponade in breast cancer patients as well as the optimal treatment for the condition. It is also necessary to examine the relationship between a history of neurofibromatosis and cardiac tamponade.

Cardiac Tamponade in Patients With Breast Cancer: A Systematic Review.

Cardiac tamponade is a rare presentation in breast cancer and may be associated with poor prognosis. In this article, we reviewed the characteristics and survival outcomes of patients with breast cancer who developed cardiac tamponade. Three databases (PubMed, EMBASE and SCOPUS) were searched for relevant articles published from 1978 to 2022 and 16 articles were identified comprising 64 cases. The median age of the cases was 52 years. Cardiac tamponade was diagnosed with echocardiogram or computerized tomography of the chest or both in 91.9%, 1.6% and 6.5% of the cases, respectively. Cytology of the pericardial fluid was done in 90.5% of the cases while biopsy in addition to cytology was done in 9.5% of cases. Tamponade was proven to be malignant in 97.4% of the cases. The initial treatment for tamponade was pericardiocentesis. Adjunct therapies ranged from the insertion of a pericardial window, pericardiectomy, radiotherapy and chemotherapy. The median time from the first treatment of breast cancer to the onset of tamponade was 24 months while the median survival following diagnosis of tamponade was 13 months. There was no significant correlation (spearman rank-sum correlation coefficient= 0.35, p = 0.165) between time to tamponade (interval time from the first diagnosis of breast cancer and the onset of cardiac tamponade) and survival. Cardiac tamponade may adversely affect survival in patients with breast cancer. Early diagnosis with echocardiogram and cytology may guide management and expectations. Further observational studies are needed to determine the predictors of cardiac tamponade and optimal treatment in patients with breast cancer.

A Rare Case of Metastatic Gastric Signet Ring Cell Adenocarcinoma in a 23-Year-Old Female Presenting as Malignant Pleural Effusion.

Signet ring cell carcinoma (SRCC) is a poorly differentiated mucin-producing adenocarcinoma with greater than 50% signet ring cells. It commonly arises from the gastrointestinal (GI) tract and rarely from extraintestinal organs. This is a rare case of a young African American female who presented with metastatic spread of signet ring cell gastric cancer (pleural and lymph nodal involvement) as the initial presentation of SRCC. Knowledge of the various clinical manifestations of SRCC can help with its early diagnosis, and there is a high need for detailed physical examination, early referral, and prompt treatment in patients with SRCC.