Bilateral Orbital Plasmacytomas With Orbital Compartment Syndrome.

Orbital plasmacytomas are uncommon soft-tissue plasma cell neoplasms that are associated with a monoclonal or myeloma protein. There are four types of plasma cell neoplasms: multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS), amyloidosis, and plasmacytoma. Plasmacytomas may be classified as medullary, occurring only within the bone, or extramedullary, occurring in soft tissues. Orbital plasmacytomas are extramedullary manifestations associated with MM and they may present with signs and symptoms such as unilateral proptosis, conjunctival injection, ocular pain, diplopia, and vision changes. The diagnosis of orbital plasmacytomas is based on tissue biopsy and histologic and immunohistochemical confirmation of a homogenous infiltrate of monoclonal plasma cells. In this report, we present a case of a 60-year-old female patient with a prior diagnosis of MM and new-onset bilateral orbital plasmacytomas following an autologous peripheral blood stem cell transplant; her condition improved significantly following treatment with dexamethasone, cisplatin, doxorubicin, cyclophosphamide, and etoposide along with palliative radiation therapy (RT) of 2000 cGy in 10 fractions to the orbits. Unfortunately, three months later, she had progression of extramedullary disease with parotid gland involvement. She had multiple complicated hospitalizations and eventually expired. As patients with orbital plasmacytomas classically have lower remission and survival rates compared to those with extramedullary plasmacytomas involving other locations, they must be considered high-risk patients who require a multidisciplinary approach for early diagnosis and timely treatment in order to prevent disease progression and to alleviate symptoms related to the disease.

Metastatic BRAF V600E-Mutated Adenocarcinoma of the Lung Presenting as Extreme Neutrophilia and Eosinophilia.

Hematologic paraneoplastic syndromes with extreme neutrophilia and eosinophilia are very rarely associated with adenocarcinoma of the lung. We describe a case of a 57-year-old female who presented with neutrophil- and eosinophil-predominant hyperleukocytosis and hypoxic respiratory insufficiency. Bone marrow biopsy confirmed metastatic adenocarcinoma, similar to the biopsy-proven adenocarcinoma of the lung. She was administered one dose of cytotoxic chemotherapy with carboplatin and pemetrexed and started on leukoreductive therapy with hydroxyurea. Molecular testing revealed a BRAF V600E mutation and she was started on dabrafenib and trametinib with significant clinical improvement. This is the first reported case of metastatic BRAF V600E mutated non-small cell lung cancer presenting with extreme neutrophilia and eosinophilia treated with a combination BRAF and mitogen-activated extracellular kinase (MEK) inhibitor.

Seizure prevalence, contributing factors, and prognostic factors in patients with leptomeningeal disease.

BACKGROUND: To determine seizure prevalence and contributing factors in patients with leptomeningeal disease (LMD). METHODS: Authors performed a retrospective review of 79 consecutive patients with a diagnosis of LMD. Associations between categorical variables were assessed using Chi-Square tests or Fisher's Exact tests. Survival was plotted with Kaplan Meier curves. Variables with a log-rank p-value of <0.20 were entered into a Cox Proportional Hazard regression analysis. RESULTS: Seizure prevalence in those with and without brain metastases was 22%. Of those who seized, 65% were admitted for this at least once while only one patient required intubation. Primary malignancy, type or route of chemotherapy administration, form of radiation therapy (craniospinal, focal, or whole brain), surgical treatment, location of LMD, and number of brain metastases did not influence seizure development. Only 13% of patients who never had seizures were on a prophylactic AED (anti-epileptic drug). In patients who had brain metastasis, there was no significant difference in prevalence of seizure before versus after LMD diagnosis suggesting that LMD does not significantly increase the risk of seizure compared to brain metastasis alone. A multivariate analysis revealed that while males trended toward inferior survival, only performance status and treatment with systemic chemotherapy showed a significant association with survival. Median survival time of patients after LMD diagnosis was four months. CONCLUSION: The prevalence of seizure in LMD patients is 22%. There were no statistically significant predisposing factors to seizure development. ECOG and use of systemic chemotherapy were found to be significant prognostic factors.

Prostate cancer leading to monoclonal gammopathy of undetermined significance: A case report.

Prostate cancer is one of the most common cancer types, affecting millions of individuals worldwide. The present study reported two cases of metastatic prostate cancer presenting with newly diagnosed monoclonal gammopathy of undetermined significance (MGUS). To the best of our knowledge, prostate cancer leading to MGUS has not been documented previously. MGUS is generally thought to be benign and has been demonstrated to convert into multiple myeloma (MM), as well as other lymphoproliferative disorders. Due to the high mortality rate associated with MM, further studies are required to confirm and clarify the association between prostate cancer and MGUS. Additionally, patients can be counseled on the requirement for follow up studies following a diagnosis of prostate cancer.

A Rare Case of Pancreatic Tuberculosis.

Tuberculosis remains a major public health concern, although it has reportedly shown a decline in prevalence both in the United States and abroad in recent years. The focus on diagnosing and treating tuberculosis is on pulmonary tuberculosis. Meanwhile, pancreatic tuberculosis remains exceedingly rare, accounting for less than 5% (Gastroenterol Rep (Oxf), 2014;2:154-157) of all tuberculosis cases in the developing world. A literature review shows that the head is primarily affected in pancreatic tuberculosis (Gastroenterol Rep (Oxf), 2014;2:154-157).

Isolated Streptococcus intermedius pulmonary nodules.

Streptococcus intermedius, a member of the Streptococcal anginosus group of alpha-hemolytic streptococci, remains a notable infectious agent causing necrotizing pneumonia and abscesses. We report a case of a young male with pulmonary nodules found to have Streptococcus intermedius and no other underlying hepatic or dental history who was treated with ceftriaxone and avoided surgical intervention. A review of the literature reveals cases that are typically treated with a combined surgical and medical approach. These same cases often involve middle aged to elderly individuals who have a past history of tobacco use, alcoholic liver cirrhosis and/or poor dentition. This case stands out given the patient's atypical presentation.

A rare cause of acute abdomen: spontaneous dissection of the superior mesenteric artery.

Spontaneous superior mesenteric artery (SMA) dissection is a rare, but potentially fatal disease. Prompt diagnosis and treatment of SMA dissections result in a lower prevalence of intestinal infarction and mortality. In the current era, imaging techniques can promptly diagnose SMA dissection; however, no definitive guidelines have been established to treat this condition.

Clinical use of blinatumomab for B-cell acute lymphoblastic leukemia in adults.

Adults with relapsed or refractory B-cell acute lymphoblastic leukemia have a dismal prognosis with a short median overall survival that can be measured in months. Because most patients will have chemotherapy-resistant disease, allogeneic hematopoietic stem cell transplantation remains the only potentially curative treatment. Despite advances in current management, patients continue to have poor outcomes and lack of durable responses. Thus, new therapies with alternative modes of actions are currently being investigated. Blinatumomab is a novel bispecific T-cell engager that simultaneously binds CD3-positive cytotoxic T-cells and CD19-positive B-cells, resulting in selective lysis of tumor cells. It has shown promising results in patients with relapsed or refractory acute lymphoblastic leukemia or those achieving hematologic response with persistent minimum residual disease. Future clinical trials will answer questions regarding its optimal place in the treatment paradigm. Dose-limiting toxicities include immunological toxicities and cytokine release syndrome. However, most patients tolerate the therapy relatively well. This review will focus on the pharmacology, clinical efficacy, and safety of blinatumomab in the treatment of adult B-cell acute lymphoblastic leukemia while highlighting its unique drug warnings and toxicity management.

Pericardial Metastasis Induced Tamponade from Urothelial Carcinoma: A Rare Entity.

Urothelial carcinoma in a few cases may result in cardiac metastasis. A rare presentation of this condition is its diagnosis as a result of cardiac tamponade. Tamponade is an unusual entity as a result of urothelial carcinoma and has only been reported in four cases. There have also been only a total of fifteen cases of cardiac metastasis from this form of malignancy. It is through this discussion that we emphasize the importance of early detection and monitoring of cardiac symptoms with the implementation of echocardiogram imaging. Although not feasible in all patients it may be considered in those presenting with cardiac and pulmonary symptoms. In this case we discuss the presentation of a 71-year-old gentleman with a history of urothelial carcinoma after cystectomy and while on chemotherapy presented with new onset atrial fibrillation and later was diagnosed with cardiac tamponade as a result of malignant metastasis.

Bladder cancer in a young patient: Undiscovered risk factors.

Bladder cancer is one of the most common forms of malignancies involving the urinary system and multiple risk factors have been associated with its etiology. The most common of which include cigarette smoking and various occupational or chemical exposures. It is usually diagnosed in older individuals with an average age of 70. In rare cases it is observed in children as well as young adults where it usually presents as a low-grade, non-invasive disease. In the present case report a 27-year-old male patient is discussed: The patient presented with no significant risk factors and was treated for mucinous adenocarcinoma of the bladder while further investigations were performed to identify other associated factors related to this form of malignancy. Debate in the literature exists in regards to the characteristics of bladder neoplasms in younger patients compared with older patients, however there is a lack of research into the etiology or prognosis in young patients. The present case study illustrates the case of a young adult with no clear risk factors who was diagnosed with a rare case of mucinous adenocarcinoma of the bladder.

Muir-Torre Syndrome Presenting as Sebaceous Adenocarcinoma and Invasive MSH6-Positive Colorectal Adenocarcinoma.

Muir-Torre syndrome (MTS) is a rare genodermatosis, diagnosed by the presence of sebaceous neoplasms along with an internal malignancy, most commonly colorectal carcinomas. MTS is most commonly caused by microsatellite instabilities of the hMLH1 and hMSH2 mismatch repair genes, and is rarely caused by mutations of the hMSH6 gene. We describe the case of a 56-year-old male who presented with an enlarging mass on his back as well as hematochezia. The back mass was excised, and pathology confirmed microsatellite instability in MSH2 and MSH6. Abdominal CT and colonoscopy confirmed the presence of synchronous masses in the cecum, ascending colon, and the transverse colon. He refused any further workup or treatment, only to return 8 months later complaining of hematochezia and discomfort due to an enlarging mass protruding from the rectum. After consenting to surgical intervention, he agreed to outpatient chemotherapy treatment. The presence of sebaceous neoplasms should raise suspicion for the possibility of an associated internal malignancy.

A rare case of orthostatic headache due to spontaneous intracranial hypotension.

Headache is one of the most common clinical entities, and has a long list of differential diagnoses; however, one of the more uncommon causes of postural headache is spontaneous intracranial hypotension. It is important that clinicians be aware of this condition, as it is often overlooked, leading to invasive and unnecessary diagnostic testing. A good history and physical exam, paired with an MRI of the brain is sufficient to make the diagnosis of SIH, relieve the symptomology of the patient in a quick and efficient manner, and avoid costly invasive procedures.

Severe Hypothyroidism-Induced Volvulus.

Thyroid disorders have been found to be associated with multiple organ systems and thus have a broad spectrum of presenting symptoms and clinical conditions. Certain aspects of the gastrointestinal (GI) system have yet to be fully understood and documented. Hypothyroidism and even hyperthyroidism have been identified in patients with motility symptoms involving the GI tract. These symptoms can vary and can be a complication of undertreated or undiagnosed condition involving the thyroid. Unfortunately, the mechanism in which these hormones can impact intestinal motility remains poorly understood and not well documented. In this case report, we discuss the presentation of a 71-year-old female with poorly managed hypothyroidism presenting with significant abdominal distention and pain secondary to underlying volvulus formation. By better understanding the complications induced by hypothyroidism, physicians may be able to prevent further life-threatening outcomes with early management and intervention.

A New Variant of Combined Pulmonary Fibrosis and Emphysema From Second-Hand Smoke: A Case Report and Review of Literature.

The findings of combined pulmonary fibrosis along with emphysema have been increasingly recognized in the medical literature. Patients presenting with such findings are usually found to be heavy smokers or former smokers. Their presentations begin with severe respiratory distress that gets progressively worse. They are found to have low diffusion capacity (DLCO) although spirometry will show preserved lung volumes. No prior research has presented a documented case of such a fatal condition in a young person with no prior history of smoking. In this case report, we discuss the presentation, diagnosis, and management of a young 29-year-old non-smoker with increasing shortness of breath with a complicated hospital course discovered to have an abnormal variant or presentation of "combined pulmonary fibrosis and emphysema" (CPFE). As most published studies have attributed these findings as a secondary response to a history of smoking, other etiologies and risk factors have yet to be properly analyzed resulting in prolonged hospital course and often missed diagnoses.

A Rare Silent Killer: Right Atrial Metastasis of Thyroid Hürthle Cell Carcinoma.

Hürthle cell carcinoma (HCC) is a variant of a follicular carcinoma with a tendency to higher frequency of metastases and a lower survival rate. However, intracavitary cardiac metastases from thyroid HCC are extremely rare. We describe the case of a 57-year-old female with thyroid HCC, 5 years after total thyroidectomy, who presented with dyspnea associated with hypoxia and hypotension. The computed tomography angiogram showed extensive pulmonary embolism and a 6-cm right atrial mass while the lower-extremity deep vein thrombosis studies were negative. This patient received a cardiac thrombectomy using cardiopulmonary bypass support. However, intraoperatively, we found out that the mass was from the mediastinum, directly extending into the heart and clearly unresectable since it effaced at least 1/3 of the right atrial wall. The core biopsy of the mass confirmed that it was metastatic poorly differentiated HCC of thyroidal origin. The patient eventually died of respiratory failure due to a massive pulmonary embolism. For cancer patients with unexplained dyspnea, cardiac metastases should be considered regardless of anticoagulation prophylaxis, especially when there is no deep vein thrombosis in the lower limbs. Early recognition of intracavitary cardiac metastases may help in providing prompt treatment and improving the prognosis.

Myopericarditis: A Diagnosis of Uncertainty.

Myocarditis can present in many different forms and can be overlooked by more life-threatening conditions. At times it may mimic conditions such as acute myocardial infarction and although it may have features highly suggestive of myocarditis, other etiologies need to be excluded. Thus, due to its clinical presentation, lab findings, and electrocardiogram analysis, it often can be confused with other conditions, making it a diagnostic dilemma of uncertainty. Myopericarditis is normally caused by viral infections, most common of which is coxsackievirus. Here we report a case of a 52-year-old gentleman who presented with a clinical picture of acute myocardial ischemia versus dissection, which overlooked a rather less threatening etiology of myopericarditis.