Metastatic Prostate Cancer to the Optic Canal: A Large-Cohort Analysis.

Prostate cancer (PCa) can present with metastases in rare cases, including those to the optic canal. Currently, no guidelines exist for managing PCa metastases in this patient population. This article aims to examine optic canal metastases through a large-cohort analysis. It involves a systematic review of the literature through a pooled analysis of published cases of PCa with optic canal or orbital metastasis, including one case previously reported from our institution. A subgroup analysis was employed to compare cases with optic metastases as their initial PCa presentation, hormone-naive (HN), versus those with optic metastases after PCa diagnosis, hormone-refractory (HR).  A total of 45 patients with ocular metastasis from PCa were included in this study. The mean age at diagnosis overall was 66.8 years (range: 45-85 years). Moreover, 16 cases (50%) had deceased, with a median time-to-death of 22 (range: 2-84) months. Regarding subgroup analysis, the mean age at diagnosis was 69.5 years (53-85 years) in the HN group and 64.5 years (45-83 years) in the HR group. With regard to reported survival, 10 (62.5%) cases belonged to the HN subset with a median follow-up duration of 12 (range: 1.5-36) months. In terms of reported mortality, 10 (62.5%) were from the HR subset with a median time-to-death of 32.5 (range 0.5-84) months. Our study constitutes the largest and most comprehensive examination of patients with optic canal metastases due to PCa so far. While optic canal metastases are a rare manifestation of PCa, they are linked with a poor prognosis. We also observed significant differences between HR and HN cohorts, which may indicate a difference in clinical presentations.

An Unusual Presentation of Cogan's Syndrome With a Renal Tumor: A Report of a Rare Case.

Cogan's syndrome (CS) is a rare disorder of an unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms, primarily affecting young white adults, without a hereditary pattern. The exclusion of other diseases makes diagnosis difficult, and it is likely underreported in the literature. A 74-year-old previously healthy African American male presented with ear and jaw pain, later accompanied by vestibular symptoms, fever of unknown origin, aortitis, and a third-degree heart block. The workup revealed incidental renal cell carcinoma and interstitial keratitis. This case highlights the challenge of diagnosing an atypical presentation of CS with late-onset interstitial keratitis by excluding other complex syndromes.

Treatment-Refractory, Castration-Resistant Prostate Cancer With Liver Metastasis: A Promising Modality of Therapy.

Although significant advances in the treatment of prostate cancer (PC) have recently been made, the treatment of metastatic liver disease remains challenging. Recent advances have led to multiple novel therapies and multi-treatment approaches combining systemic and locoregional modalities, such as thermal ablation, representing a promising strategy that has received attention in recent years. Nevertheless, no standard locoregional treatment regimens exist for the management of liver metastases of PC. In addition, regional therapy alone is unlikely to provide durable cancer control. Here, we report for the first time a successful treatment of hepatic metastases of PC using stereotactic image-guided percutaneous microwave ablation and the poly (ADP-ribose) polymerase-1 inhibitor, olaparib.

An Unusual Case of Concurrent Herpes Simplex Virus Type 1 and Cytomegalovirus Encephalitis Complicated by Primary Central Nervous System Lymphoma as an Initial Presentation of Acquired Immunodeficiency Syndrome.

Primary central nervous system lymphoma (PCNSL) is an uncommon brain tumor with a puzzling diagnosis. It has an incidence of seven cases per 100,000,000 people in the United States, which is further lower in immunocompromised patients. Cytomegalovirus (CMV) is a common cause of various malignancies, notably Burkitt's lymphoma, nasopharyngeal carcinoma, Hodgkin's lymphomas, and non-Hodgkin's lymphomas (NHL) including PCNSL. Cases with PCNSL can vary in presentation with either focal or non-focal manifestations progressively worsening over a period that could last up to a few months. In this report, we discuss the case of a 39-year-old woman with a past medical history of bipolar disorder who presented with PCNSL as the initial presentation of acquired immunodeficiency syndrome (AIDS). This case report emphasizes the importance of a multidisciplinary team (MDT) approach for the interpretation as well as for correlating the laboratory and imaging results with clinical findings given the challenging diagnosis, to choose an appropriate management approach that is tailored to the patient's presentation.

An Unusual Case of Neisseria flavescens/subflava Group Tricuspid Valve Endocarditis in a Patient With Previously Treated Methicillin-Resistant Staphylococcus aureus Endocarditis.

Infective endocarditis (IE) is classified as an infection of any cardiac valve or endocardial surface and is often associated with high morbidity and mortality. Certain bacteria such as gram-positive cocci and viridans group streptococci have high predilection for both naïve and damaged cardiac valvular tissues, accounting for the majority of IE cases. In very infrequent instances, gram-negative bacteria (GNB), more specifically non-meningococcal, non-gonococcal GNB, have been shown to cause IE. The following is a case of a young male diagnosed with Neisseria flavescens/subflava tricuspid valve endocarditis after being previously treated for Methicillin-resistant Staphylococcus aureus (MRSA) endocarditis.

A Rare Case of Leukemia Cutis as the First Presentation of a Myelodysplastic Syndrome to Acute Myeloid Leukemia Transformation.

Leukemia cutis (LC) is a rare presentation of leukemia. It is characterized by the infiltration of leukemic cells into the different layers of the skin causing varying skin manifestations. It can occur before the hematological presentation of leukemia or during the disease course and carries a poor prognosis. Here, we report a patient with therapy-related myelodysplastic syndrome (t-MDS) whose transformation to acute leukemia was heralded by the development of LC. Worrisome cutaneous lesions should not be overlooked, and a skin biopsy should be pursued to confirm the diagnosis. A high index of suspicion is the key to early recognition of sometimes nonspecific skin findings of widespread systemic disease.

A Rare Case of Incidental Urachal Mucinous Cystadenocarcinoma.

The urachus is a remnant of the embryonic allantois that connects the urinary bladder to the umbilicus. In most cases, it commonly obliterates before birth. Here we present the case of a 60-year-old man with a past medical history of human immunodeficiency virus (HIV) and anal human papillomavirus (HPV). He was referred for an abdominal mass that had been found incidentally while being admitted for an acute kidney injury; it had been excised, and pathological examination showed urachal mucinous cystadenocarcinoma. Surgical excision is performed for the majority of cases with a higher survival rate when diagnosed early.

Pericardial Effusion and Tamponade Complicating Acute Pericarditis in a Human Metapneumovirus-Infected Adult: A Unique Case Report.

Acute pericarditis (AP), or pericardial sac inflammation, is a self-limited condition in healthy individuals. Viruses, including adenoviruses, enteroviruses, cytomegalovirus, and influenza virus, have been well documented to cause AP. In contrast, human metapneumovirus (hMPV), a relatively newer virus, has been described in a few cases to cause serious cardiac complications. Here we report a patient who developed an imminent cardiac tamponade associated with hMVP respiratory infection.

Chronic Myeloid Leukemia: Atypical Presentation and Diagnostic Pitfall in the Workup of Isolated Thrombocytosis.

Chronic myeloid leukemia (CML) is one of the classic types of myeloproliferative neoplasms. It typically manifests with leukocytosis, but rarely with isolated thrombocytosis. Here we describe a unique case of isolated thrombocytosis as an initial presentation of CML in a 21-year-old woman, where the BCR-ABL1 fusion gene was detected in bone marrow (BM) aspiration and biopsy specimen after a negative peripheral blood (PB) fluorescence in situ hybridization testing. It is crucial to pursue workup for patients with isolated thrombocytosis through testing for the presence of the BCR-ABL fusion gene or the Philadephia chromosome in both PB and the BM in order to distinguish CML from essential thrombocythemia.

A Retrospective Review of Tumor Lysis Syndrome Associated With Colorectal Cancer.

Tumor lysis syndrome (TLS) is a life-threatening oncologic condition that is most commonly linked with hematologic malignancies and uncommonly seen in solid tumors, including colorectal cancer (CRC). Therefore, a lack of awareness regarding TLS in CRC could lead to significant morbidity and mortality. This study aims to explore the clinical characteristics and outcomes of TLS in patients with CRC. A systematic review of the literature was performed by searching PubMed using the keywords "tumor lysis syndrome" and "colorectal cancer". The English-language case reports and abstracts that the search results yielded were reviewed, and additional articles of interest were identified from reference lists. Information regarding the patients (age at diagnosis, presentation, and comorbidities), the tumors (histology, grade, and stage), radiologic investigations, treatment modalities (surgery, radiation, and systemic therapy), and the outcomes (response, adverse events) were recorded, when available. Descriptive statistics, such as frequency counts, medians, and ranges, were used to characterize the pooled sample. Nine case reports of TLS in CRC were identified in the literature; one additional case was added from our patient database. The median age of these patients was 58.5 years (range: 42-82 years) with 70% of these patients being male. Of note, 100% of these patients had metastatic colon cancer and 80% had metastatic involvement of the liver; 70% of these cases were associated with therapy-induced TLS with the median time-to-event being three days (range: 18 hours-30 days) after receiving chemotherapy. When looking at laboratory parameters, uric acid and lactate dehydrogenase (LDH) were consistently elevated in all the cases, but 50% of the cases had hyperkalemia and 50% had hyperphosphatemia. Treatment of TLS included supportive measures with IV hydration. Five out of 10 patients received urate oxidase and only one underwent hemodialysis. The overall mortality was 60%. TLS can occur with CRCs that demonstrate a high tumor burden. While most cases are associated with therapy, some cases are spontaneous in nature. Keeping in mind the high mortality associated with TLS, physicians should have a high degree of suspicion and should be aware of the fatal complications associated with TLS. Timely implementation of prophylactic and therapeutic measures including IV hydration as well as the use of xanthine oxidase inhibitors such as allopurinol can be life-saving in these cases.

Clinical Features and Outcomes of Patients With Chronic Myeloid Leukemia Presenting With Isolated Thrombocytosis: A Systematic Review and a Case From Our Institution.

Chronic myeloid leukemia (CML) represents a common condition in the spectrum of myeloproliferative disorders (MPD). It classically exhibits leukocytosis, but rarely presents with isolated thrombocytosis. This paper is designed to review the clinicopathologic features, treatment, and outcomes of patients with CML who present with isolated thrombocytosis. We searched PubMed, MEDLINE®, ScienceDirect, and Scopus for English-language articles about case series and case reports for the period 2000-2020 with the terms "chronic myeloid leukemia" and "thrombocytosis" and pooled them with a case from our institution. Cases were also incorporated from the reference list and screened for inclusion. A total of 20 cases were included in the final cohort. The male-to-female ratio was 1:1.86. The mean age of the patients at the time of initial diagnosis was 40.5 years (range: 9-77 years). Out of 17 cases with available data, seven (41%) were asymptomatic and found to have thrombocytosis incidentally upon routine blood work. Five cases (29.4%) either had a history of thrombotic events or presented with severe thrombotic complications, including ischemic cerebrovascular accidents (CVA), myocardial infarction (MI), pulmonary embolism (PE), and/or miscarriages. Four cases (23.5%) had more than one symptom at presentation, including headache, syncope, and bruising. The average platelet count was 1,923 × 109/L (range: 584-8,688 × 109/L), and one case (5%) had anemia. The bone marrow (BM) examination showed normal cellularity and normal myeloid to erythroid (M/E) ratio in seven (50%) and 11 (84.6%) out of the 14 and 13 cases with reported data, respectively. Moreover, megakaryocytes in the BM were small in 10 cases (71.4%), pleomorphic in three cases (21.4%), and dysplastic in one case (7.1%). Accurate differentiation among MPD subtypes and the exclusion of CML is critical in reaching a proper diagnosis to decide on proper therapy and eventually modify outcomes. Prompt evaluation for the precise diagnosis of patients presenting with isolated marked thrombocytosis will help expedite their diagnosis and initiation of a specific tyrosine kinase inhibitor (TKI) therapy, thereby promptly inducing remission, preventing thrombotic complications, and avoiding adverse drug events, which would eventually improve outcomes.

Doege-Potter Syndrome and Pierre-Marie-Bamberger Syndrome in a Patient With Pleural Solitary Fibrous Tumor: A Rare Case With Literature Review.

Solitary fibrous tumors (SFT) represent a unique subset of mostly benign heterogeneous tumors with mesenchymal cell origins. These tumors have been reported in the past as being mostly indolent, with a slowly evolving clinical course and low potential for malignancy. Although found systemically, the incidence of SFT arising intrathoracically, from the pleura of the lung, is relatively poorly documented in the medical literature. SFT is a rare phenomenon, but in even rarer circumstances, these tumors are associated with distinctive paraneoplastic syndromes, such as Pierre-Marie-Bamberger syndrome (PMBS) and Doege-Potter syndrome (DPS). PMBS presents as digital clubbing and hypertrophic pulmonary osteoarthropathy. DPS has been characterized as a non-islet cell tumor hypoglycemia due to the ectopic secretion of insulin-like growth factor 2 (IGF-2), a pattern seen in fewer than 5% of cases of SFT. Treatment is typically through surgical resection. In our research of the medical literature, we found only very few cases in which the association with SFT and both paraneoplastic syndromes were described. Here, we report an uncommon case of a 68-year-old male patient found to have an incidental right hemithoracic tumor with digital clubbing and intermittent severe episodes of fasting hypoglycemia after initially presenting with a syncopal episode.

Tumor Lysis Syndrome in Solid Tumors: A Comprehensive Literature Review, New Insights, and Novel Strategies to Improve Outcomes.

Tumor lysis syndrome (TLS) is a life-threatening oncological condition that is typically characterized by metabolic derangements that are often labeled as an acute kidney injury. The recent advancement in cancer treatment has led to the mounting rate of TLS in solid tumors that were previously rarely linked to this complication. Given that its prognosis is dismal, it is essential to increase recognition of this condition by describing more sensitive markers. Currently, the management of TLS is mainly supportive due to the lack of specific therapy targeting its specific pathology. This review aims to summarize the most recent literature on the underlying mechanism of TLS and the potential implications for novel TLS therapy.

Misdiagnosis of Small Cell Prostate Cancer: Lessons Learned.

Small cell carcinoma (SCC) of the prostate is rare in the spectrum of prostate tumors. Clinically, prostate SCC is more aggressive and has a poorer prognosis when compared to prostate acinar adenocarcinoma. Delay in diagnosis and misdiagnosis further worsens the outcome of this disease. Here we present a 68-year-old man whose prostate SCC was initially misdiagnosed with benign prostate hypertrophy (BPH) by his primary care physician (PCP) and urologist and small cell lung cancer by his first oncologist and pathologist. This case underscores the diagnostic conundrum of prostate SCC and the importance of exercising caution against the possibility of uncommon prostate cancer misdiagnosis in clinical practice.

Not Just Another Headache: Cerebral Venous Sinus Thrombosis in a Patient With Isolated Antithrombin III Deficiency.

Cerebral venous sinus thrombosis (CVST) is a rare condition. Symptoms and signs arise from a combination of thrombosis of cerebral veins and increased intracranial pressure. The most common presenting symptom is a non-descriptive headache, but presentation varies with underlying etiology. CVST requires a high index of suspicion to diagnose, particularly in those without apparent risk factors. Evaluation and diagnosis should include a combination of a thorough history, ophthalmoscopic examination, laboratory studies, and imaging. Management is multidimensional and aims to reverse the underlying causes, and prompt treatment with anticoagulation by heparin to decrease thrombotic burden, risk of permanent neurological deficits, and death. In the present study, we report the case of a 61-year-old man referred to the emergency department by an ophthalmologist for bilateral papilledema and eventually diagnosed with CVST secondary to isolated antithrombin III deficiency. Although CVST is uncommon, this case is worthwhile to report as the presentation is unique, and it requires a high index of clinical awareness for timely diagnosis and early therapeutic intervention.

Peritoneal Carcinomatosis in an Adult With Sjögren's Syndrome: A Diagnostic Dilemma Revealing a Rare Association With Primary Gastric Adenocarcinoma.

Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects middle-aged women. It is rarely associated with solid neoplasms. We report a 60-year-old woman with a past medical history relevant for SS who was diagnosed with advanced-stage gastric adenocarcinoma upon evaluating for peritoneal carcinomatosis and succumbed two months after her original diagnosis. This case highlights the significance of considering gastrointestinal (GI) malignancy as an essential differential, particularly when evaluating patients with SS who fail conservative treatment for their GI symptoms.

Cerebral Venous Sinus Thrombosis in Adults with Prothrombotic Conditions: A Systematic Review and a Case from Our Institution.

Cerebral venous sinus thrombosis (CVST) is a rare condition characterized by elevated intracranial pressure due to impaired cerebral venous drainage, potentially leading to life-threatening consequences. We searched the PubMed electronic database for 'cerebral venous sinus thrombosis' and 'prothrombotic' cases reported in adults (19+ years) and conducted a systematic review for the published literature in the English language pooled with a case from our institution. Data were analyzed regarding patient demographics, risk factors, clinical features, treatment modalities, and outcomes when available. Thirty cases of CVST were identified (29 case reports, of whom two were described in a case series, and the one case from our institution). The patients' mean age was 39 years (range: 19 - 65). The male: female ratio was 1.14:1. The majority (73.3%) had at least one preexisting risk factor, with prescription drug use being the most common risk factor (33.3%) shared among all patients. Most patients (83.3%) presented with at least two symptoms. The most common presenting symptoms were headache (70%), gastrointestinal disturbance (50%), and seizures (40%). Focal deficits (36.7%), vision disturbances (30%), and altered consciousness (20%) were the remaining presenting complaints. Twelve cases (40%) commented on papilledema, with 10 (83.3%) having papilledema present. Anticoagulation abnormalities were examined in 26 cases (86.7%), out of which four cases (15.4%) had isolated protein S (PS) deficiency, three cases (11.5%) had isolated antithrombin III (ATIII) deficiency, and one case (3.8%) had isolated protein C (PC) deficiency. The most common initial imaging modality (22 cases, 73.3%), and most commonly used overall (23 cases, 76.7%), was computed tomography (CT). Magnetic resonance imaging (MRI) was the second most common imaging modality for initial use (five cases, 16.7%), diagnosis or confirmation of CVST (eight cases, 26.7%), and overall (21 cases, 70%). Heparin treatment was involved in the treatment of 18 cases (60%), and warfarin treatment was used in 10 cases (33.3%). Heparin-warfarin combination treatment was utilized in eight cases (26.7%). Most patients survived (28 cases, 93.3%), while the two remaining patients died secondary to brain death from the CVST (6.7%). The findings from this study highlight the clinical characteristics of CVST. Therefore, this study aims to increase awareness of this rare entity. Physicians should maintain a high index of suspicion in order to diagnose patients presenting in the proper clinical context, given this case shares various forms of presentations with other common clinical conditions but requires long-term anticoagulation.

Molecular Profiling of Benign Metastasizing Leiomyoma of the Uterus Revealing Unique Novel Therapeutic Targets.

Extra-uterine manifestations of benign uterine leiomyoma (fibroids) are rare. Benign metastasizing leiomyoma (BML) comprises an uncommon variant characterized by metastatic lung nodules. The pathologic characteristics for BML are well known in the literature; however, the underlying biology and molecular mechanisms remain poorly understood. We present a case of a 43-year-old woman who presented to the hospital complaining of dyspnea and lower extremity edema. Medical history includes a previous hysterectomy for leiomyomata two years prior. A reduced ejection fraction and right atrium globular filling defect are seen on transthoracic echo (TTE). CT scans of the chest, abdomen, and pelvis reported pelvic mass with an extensive inferior vena cava (IVC) thrombus extending into the right atrium, which was subsequently completely resected. Subsequent histopathology for the thrombus reported intravascular leiomyomatosis (IVL) and pelvic mass reported benign leiomyoma. Two years later, the symptoms recurred, and a chest CT revealed new pulmonary nodules. Subsequent pathology from a biopsy of these nodules was consistent with BML with ER+/PR+ on immunohistochemical staining. Genetic testing showed amplification of JUN, cyclin-dependent kinase 4 (CDK4), and MCL1, and loss of SUFU, AT-rich interaction domain 1A (AR1D1A), RB transcriptional corepressor 1 (RB1), and hepatocyte nuclear factor 1-alpha (HNF1A). The patient deemed to be a poor surgical candidate, and, therefore, she was started on hormonal treatment with leuprolide and letrozole. The disease remained stable upon follow-up at 48 months. Here, we report novel genomic profiling findings for the first time in a patient with a newly diagnosed BML. These findings may suggest molecular evidence that IVL may not be as benign as previously thought.  Our study further highlights the value of genetic profiling in the understanding of this tumor's behavior and identification of new patient-specific therapeutic targets.

The Potential Clinical Benefit of Tocilizumab Therapy for Patients with HHV-8-infected AIDS-related Multicentric Castleman Disease: A Case Report and Literature Review.

Castleman disease (CD), also known as angiofollicular hyperplasia, is a rare disorder characterized by nonmalignant mediastinal lymph node enlargement provoked by excess interleukin-6 (IL-6) secretion. It could be unicentric or multicentric (MCD). Here, we describe a 27-year-old man with a prior history of AIDS, Kaposi sarcoma (KS), and latent syphilis who presented to the ED for persistent fatigue, fever, chills, night sweats, and productive cough. Infectious workup was negative, and the patient continued to have a high fever despite empiric antibiotic therapy. Bone marrow biopsy was performed and was negative for malignancy. The patient eventually underwent a left clavicular lymph node biopsy, which showed a plasma cell variant CD with positive immunostaining for human herpesvirus 8 (HHV-8), and high HHV-8 viral load. We started the patient on rituximab and liposomal doxorubicin, but unfortunately, the patient had a severe anaphylactic reaction to the rituximab, so we could not proceed with this treatment. We, therefore, started tocilizumab treatment, which improved the patient's general condition, and he was eventually discharged from our hospital. Upon follow-up 11-months later, a repeat CT scan of the chest and abdomen showed a near-complete treatment response with decreased lymphadenopathy throughout and hepatosplenomegaly. IL-6 overproduction in patients with CD is linked to the production of inflammatory cytokines and has a role in tumor angiogenesis, which makes it potential for IL-6 targeted therapy. The diagnosis of CD, especially MCD, requires a high index of suspicion, and a lymph node biopsy is essential in the diagnosis. Tocilizumab, an IL-6 receptor antibody, could potentially be considered as a practical therapeutic approach in managing HHV-8 positive MCD patients who do not tolerate or respond to initial rituximab therapy.

A Novel Combination of Metachronous Primary Malignancies of the Thyroid and Breast in a Patient with Neurofibromatosis Type 1.

Neurofibromatosis 1 (NF1) is a genetic condition of variable presentations. It has been shown to increase the risk of multiple cancers. Therefore, NF1 has been identified as a tumor-provoking condition. We present a case of a 39-year-old woman with NF1 who was diagnosed initially with papillary thyroid carcinoma (PTC) and subsequently presented with a painful breast lump. Core biopsy revealed an invasive ductal carcinoma (IDC) for which selective estrogen receptor modulator (SERM) therapy was initiated. A lumpectomy was performed soon after, which confirmed IDC. Following surgery, the patient received a combination of anthracycline and cyclophosphamide (AC), which was later followed by a taxol-based chemotherapy regimen. This study aims to throw light on the rare phenomenon of metachronous malignancy: the occurrence of successive primary cancers in the same patient. We believe that raising awareness regarding the different neoplasms associated with NF1 is important to promote appropriate preemptive screening for early detection of a second primary neoplasm, which can help lower the morbidity and mortality associated with this condition through expedited intervention.