An Unusual Case of Disseminated Erdheim-Chester Disease.

INTRODUCTION: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic multisystem disorder, deriving from mononuclear phagocytic cells. It is notoriously challenging to diagnose. Here we present a case of a patient with multisystem ECD. CASE PRESENTATION: A 76-year-old female with a history of Hashimoto's thyroiditis who presented with persistent leukocytosis was found to have bilateral renal enlargement with a perinephric mass, a recurrent pericardial effusion, and bilateral pleural effusions. Following biopsies of several sites of involvement, a diagnosis of ECD was made. DISCUSSION: The existing literature on ECD is sparse, and no diagnostic criteria have been put forward due to widely differing presentations, although the most common is skeletal. Definitive diagnosis requires a tissue sample. CONCLUSIONS: In presenting our clinical reasoning and approach, we hope to contribute to the existing body of literature on ECD, with the aim of ultimately having sufficient data to compile a diagnostic framework for other clinicians who encounter ECD.

Significantly Elevated Alkaline Phosphatase Caused by Congestive Hepatopathy in the Setting of Heart Failure with Preserved Ejection Fraction.

INTRODUCTION: We report a rare case of significantly elevated alkaline phosphatase (ALP) caused by congestive hepatopathy in the setting of heart failure with preserved ejection fraction (HFpEF). CASE PRESENTATION: A 44-year-old woman with multiple hospitalizations for acute decompensated HFpEF and abdominal pain had an ALP elevation to almost 8 times the upper limit of normal. A negative inflammatory, infectious, and autoimmune workup led to liver biopsy and diagnosis of congestive hepatopathy. DISCUSSION: The existing literature includes extensive research on the impact of liver function enzymes in heart failure with reduced ejection fraction (HFrEF); however, research on their impact on HFpEF is limited. ALP has been found to be normal or mildly elevated, with very few cases of significantly elevated ALP levels reported in HFrEF patients only. CONCLUSION: Complex cardiohepatic interactions often result in the coexistence of heart failure and liver disease. Unexplained chronic cholestasis in the setting of congestive heart failure should raise the suspicion for congestive hepatopathy.

Metastatic Rectal Small Cell Carcinoma: A Case Report.

We report a case of metastatic small cell carcinoma presenting as a rectal mass in an 80-year-old male with a history of change in bowel movement and rectal pain for six months. A computed tomography (CT) scan of the abdomen and pelvis was done, which showed a large rectal mass with many liver metastases. He had a diagnostic colonoscopy, which showed a large obstructing rectal mass, and the biopsy result came back as small cell carcinoma. He underwent palliative diverting colostomy without complications. Initially, there was a plan to treat the patient with systemic chemotherapy with etoposide and carboplatin, but given the acute kidney injury, there was a delay in treatment. After the first cycle of chemotherapy, the patient had severe nausea and vomiting. After a discussion with the patient and his family, he decided on hospice care and passed away in a few weeks.

Incidence and Risk of Thyroid Dysfunction in Advanced or Metastatic Non-small Cell Lung Cancer Patients Treated with Pembrolizumab: A Meta-analysis.

Thyroid dysfunction is one of the major side effects associated with Pembrolizumab in the treatment of advanced or metastatic non-small cell lung cancer (NSCLC). We performed a systematic review and meta-analysis of randomized clinical trials to determine its overall incidence. A literature search was conducted using the electronic database engines PubMed and Google Scholar from inception to March 2019. Eligible studies were prospective randomized clinical trials with advanced or metastatic NSCLC. The pooled incidence, risk ratio (RR), and 95% confidence interval (CI) of thyroid dysfunction were calculated using the random-effect model. Given the possibility of a between-study variance, we used the random-effect model rather than the fixed-effect model. A total of four studies, including 1603 patients, were selected for analysis. Among patients receiving Pembrolizumab, the overall incidence of all-grade thyroid dysfunction was 19.8% (95% CI: 16.6-23.3%). Pembrolizumab was associated with a significantly increased risk of thyroid dysfunction of all grades, with a relative risk of 3.9 (95% CI: 2.08-7.42%, p= 0.084) in comparison with the controls. Therefore, there is a significant increase in developing thyroid dysfunction in advanced or metastatic NSCLC patients treated with Pembrolizumab.

A Rare Case of Necrotizing Sarcoid Granulomatosis Involving Liver.

Sarcoidosis is a multi-system inflammatory disease, characterized by formation of non-caseating epithelioid granulomas. It usually involves lungs, lymph nodes, skin, joints, eyes and uncommonly liver. Necrotizing sarcoid granuloma is a rare entity. We are presenting a case of necrotizing sarcoid granuloma of liver which is extremely rare.

Systemic Air Embolism Following Computed Tomography-Guided Lung Biopsy.

A 61-year-old male with a history of poorly differentiated squamous cell carcinoma of tongue who completed chemo-radiation was found to have bilateral lung nodules on follow-up positron emission tomography (PET) scan. He underwent computed tomography (CT)-guided lung biopsy. Sequential chest scans done during the procedure showed air-fluid level in the left ventricle, suggestive of air embolism. He was hemodynamically stable during the procedure, however at the end of the procedure he developed right-sided face and arm weakness with aphasia. Emergent CT scans including angiography of head and neck were done which did not show any bleed and was also negative for any air in intracranial vasculature. Patient was treated with 100% oxygen. His neurological symptoms resolved in 30 minutes and he was subsequently admitted to intensive care unit (ICU) for further management. Six hours later, repeat CT of chest was done which showed resolution of air embolism.

Severe Hyponatremia Due to Cisplatin-induced Syndrome of Inappropriate Secretion of Antidiuretic Hormone.

We report a rare case of severe hyponatremia due to cisplatin-induced syndrome of inappropriate secretion of antidiuretic hormone (SIADH) in a 74-year-old male with a history of squamous cell carcinoma of the tongue who presented with a five days history of worsening confusion. He was found to have severe hyponatremia. His clinical and laboratory findings were consistent with hyponatremia due to SIADH. He was treated with fluid restriction and salt tablets after which his serum sodium markedly improved. Drugs including diuretics, antidepressants, antipsychotics are well known to cause hyponatremia. Cisplatin is a very common antineoplastic drug for solid tumors. Clinicians need to have prompt recognition of this rare side effect of cisplatin to prevent life-threatening hyponatremia.

Osteoclast Giant Cell Tumor of Pancreas: A Case Report and Literature Review.

We report a rare case of undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UC-OGC) in a 62-year-old female who presented with a three-month history of bilateral flank pain and significant unintentional weight loss. Computed tomography (CT) scan of the abdomen showed pancreatic tail mass, concerning for malignancy. She underwent endoscopic ultrasound (EUS) which also revealed a pancreatic mass invading into the splenic artery. CT chest and positron emission tomography (PET) scan did not reveal any metastases. The patient had a distal pancreatectomy, splenectomy, and left partial adrenalectomy. Histopathology revealed undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (OGCs). The patient was recently started on adjuvant chemotherapy with capecitabine and gemcitabine and the plan is to repeat imaging to assess response. We present this case to increase clinical awareness of this rare clinical entity, and also review controversies in the management and surveillance of UC-OGC.

Efficacy of Pembrolizumab and Nivolumab in Crossing the Blood Brain Barrier.

Metastatic brain tumors are the leading cause of central nervous system malignancies in adults, surpassing primary central nervous system with non-small cell lung cancer, accounting for more than 50% of all cases. The emergence of immunotherapies such as antibodies targeting the immune check points has led to significant advancement in the field of cancer treatment since these approaches have overwhelmingly impacted outcomes in patients with metastatic non-small cell lung cancer. Here we report two cases of metastatic non-small cell lung cancer treated with immunotherapy. While one patient achieved an excellent systemic response but developed new metastatic brain lesions, the other showed remarkable systemic as well as central response. These cases highlight variable central nervous system penetration of programmed death 1 (PD-1) antibodies, and we also review the available literature on blood brain barrier permeability of PD-1 antibodies.

Autoimmune necrotizing myopathy after statin discontinuation.

We report a case of autoimmune necrotizing myopathy related to statin use in a 70-year-old woman who came to the hospital because of progressive lower extremity weakness. Laboratory, electromyography and muscle biopsy results were consistent with autoimmune necrotising myopathy. The patient was treated with intravenous immunoglobulin with improvement in muscle strength.

Severe hypocalcaemia following denosumab in a patient with cancer with vitamin D deficiency.

Denosumab is a monoclonal antibody that has been widely used for the prevention of skeletal-related events in patients with cancer with solid tumours and bone metastases, and acts by reducing the release of calcium from bones into the bloodstream. Severe hypocalcaemia is a rare and dangerous side effect of denosumab. We present a case of a patient with metastatic prostate cancer who developed severe hypocalcaemia after receiving a single dose of denosumab. Further laboratory analysis showed that the patient had a low vitamin D level, which contributed to the development of hypocalcaemia. He required an inpatient admission for repeated doses of intravenous calcium.

Hodgkin's Lymphoma presenting as an obstructing endobronchial mass-A rare presentation.

We report a case of Hodgkin's lymphoma presenting as an endobronchial mass in a 40-year-old man with history of 8 months of non-specific symptoms like cough, fatigue and weight loss. Initially he was treated with broad-spectrum antibiotics for suspicion of pneumonia without recovery. Radiographic work-up showed cavitary consolidation of the upper lobe of the left lung, followed by bronchoscopy which showed obstructing mass of the upper lobe of the left lung mimicking primary lung carcinoma. Immunohistochemical staining of the specimen was suggestive of Hodgkin's lymphoma. The patient responded well to the chemotherapy regimen.