Patient-nephrologist prognostic awareness and discordance in end stage renal disease on renal replacement therapy.

PURPOSE: The 1-year mortality rate of patients with end-stage renal disease (ESRD) on renal replacement therapy (RRT) is 20-25% comparable to many cancers. Studies have shown that cancer patients commonly overestimate their likelihood of survival relative to their physicians. It is unclear if this translates into other terminal illnesses. METHODS: Adult and elderly patients with ESRD on RRT without cognitive defect were interviewed to evaluate their prognostic estimates at 1 and 5 years. Past medical history and demographic data was abstracted from their medical charts. Each patient's proper nephrologist was then interviewed regarding his proper prognostic estimate for this patient. Both the patient and the nephrologist's estimates were compared and a difference of greater than 20% was defined as the threshold for prognostic concordance. RESULTS: 77% of patients were found to be in prognostic discordance with their nephrologists. This group was older, had more comorbidities, a lower albumin level and a poorer prognosis. The majority of patients were in disagreement with their nephrologists regarding whether a discussion about prognosis had taken place. The choice of end of life care for 55% of patients was focused on relieving pain and discomfort. CONCLUSION: Communication of prognosis and discussions related to life expectancy and end of life care are lacking in the routine care of ESRD patients. ESRD patients therefore tend to overestimate their prognosis which might lead to overutilization of invasive procedures with increased acute healthcare costs as well as a delay in instigation of palliative or hospice measures.

Survival benefit of neoadjuvant versus adjuvant radiotherapy in lymph node positive esophageal cancer: a population based analysis.

BACKGROUND: The impact of radiotherapy on the survival of patients with locally advanced esophageal cancer (EC) is presently insufficiently explored. Thus, using data from the Surveillance, Epidemiology, and End Results (SEER) Registry, this study aimed to compare the survival rates of patients with lymph node (LN) positive EC who received curative resection and were treated by neoadjuvant and adjuvant radiotherapy (RT), respectively. METHODS: Retrospectively collected data from the SEER database using all 18 SEER registries on patients that underwent esophagectomy for EC was evaluated. All patients with LN positive pathology who received either neoadjuvant or adjuvant RT and curative intent esophagectomy from 2004 to 2007 were included. A comparison of 5-year relative survival outcome among groups categorized by sex, race, age, histology, and tumor size was performed. RESULTS: A total of 933 patients were evaluated; 636 (69%) and 297 (31%) received RT in neoadjuvant and adjuvant setting respectively. Their overall 5-year relative survival rates were 32.8% (95% CI: 28.7-36.9) and 26.5% (95% CI: 21-32.3) (P=0.058). Patients in the neoadjuvant RT group who underwent curative resection for squamous cell carcinoma (SCC) of EC had an improved 5-year relative survival rate of 43.4% (95% CI: 32.5-53.8) compared to 26.5% (95% CI: 15.4-38.9) measured for the adjuvant RT group (P=0.03). The results further revealed a significant increase in the 5-year relative survival rates for stage T3 and Tx when RT was given in neoadjuvant setting compared to adjuvant RT group (T3 28.5% vs. 20.2%, P=0.011; Tx 46.3% vs. 8.9%, P=0.021). When the patients were grouped according to race, sex or age, or based on the timing of radiation relative to surgery, in the other histological or T stage groups, there were no statistically significant differences in the 5-year survival rates. CONCLUSIONS: Compared to adjuvant radiotherapy, neoadjuvant radiotherapy results in a better 5-year relative survival in patients with squamous cell neoplasms and/or T3, Tx stage disease.

Facial Paralysis and Hearing Loss: A Rare Manifestation of Prostate Cancer Metastases.

Dural prostate metastases (DPM) are a rare manifestation of metastatic prostate cancer seen in approximately one to six percent of cases. Presenting symptoms may include signs of elevated intracranial pressure, headache, altered mental status, or cranial nerve palsies. Hearing loss, sensory changes, dysarthria, and dysphagia are rare symptoms in DPM that were present in our patient. We present a case of a 58-year-old male with a known diagnosis of adenocarcinoma of the prostate presenting with symptoms of acute exacerbation of chronic obstructive pulmonary disease (COPD), sub-acute right-sided hearing loss, and right-sided facial paralysis. Over the course of hospitalization, his neurological symptoms worsened and he developed dysarthria, dysphagia, facial numbness, and worsening back pain. He also appeared more withdrawn and lethargic. The symptoms prompted a neurological evaluation and a magnetic resonance imaging (MRI) revealed multiple areas of bone marrow signal abnormality compatible with osseous metastatic disease. There was extensive smooth dural thickening as well as focal nodular thickening, both consistent with dural metastases. The patient was treated with corticosteroids and external beam radiation therapy (EBRT) with improvement in his back pain and facial paralysis. He died two weeks after completing EBRT. Although rare, DPM should be suspected in males over 50 years of age presenting with neurological symptoms. An MRI with gadolinium is most helpful in delineating the presence and extent of dural and calvarial involvement. Corticosteroids and EBRT have been shown to improve neurological function in up to 67% of patients. However, median survival post-radiation remains approximately three months.

Soft Tissue Myoepithelial Carcinoma of the Neck with Spinal Invasion.

Soft tissue myoepithelial neoplasms are a rare yet diverse group of tumors, ranging from benign to malignant lesions. Their presentation in the head and neck region is uncommon and represents a challenging diagnosis. Early identification of myoepithelial carcinoma is crucial given its more aggressive course compared to its benign counterpart, although the histopathological distinction between the two can be difficult. EWSR1 gene rearrangement is found in half the cases and has a speculative role in pathogenesis. Complete excision remains the treatment of choice. The roles of chemotherapy and radiation are unclear. We report the hospital course of a 33-year-old female who presented to our institution with a posterior neck mass with spinal invasion, diagnosed as myoepithelial cancer. A literature review of these rare tumors is discussed here.

Decitabine induced transient cardiomyopathy: a case report.

CASE REPORT: A 75-yr-old gentleman, with a past medical history of diabetes mellitus and Acute Myeloid Leukemia presented to our emergency department with a chief complaint of exertional dyspnea and chest pain. A week prior to this visit, he had recieved a cycle of decitabine chemotherapy at 20 mg/metered square for ten days. This was his second cycle of decitabine. His out patient medications included megesterol, omeprazole, morphine sulfate and insulin glargine. The patient was admitted to the Coronary Care Unit for Acute Coronary Syndrome. His cardiac enzymes were elevated (peak troponin 30 ng/mL, CKMB 67.4 ng/mL). His 12 lead EKG revealed sinus tachycardia with a ventricular rate of 113, but without acute ST-T wave changes. The BNP was 259 pg/mL. A 2D echo revealed moderate diffuse hypokinesis with an EF of 35%. He subsequently underwent a left heart catheterization, which showed non-obstructive CAD. In our patient, the elevated troponins (peak troponin 30 ng/mL) and BNP were seen concomitant with the onset of cardiogenic shock. Two months ago, his 2 D echocardiogram revealed an ejection fraction of about 55%-65% with slightly increased left ventricular (LV) wall thickness. DISCUSSION: The most common adverse effects of decitabine include cytopenia, nausea, pain and erythema/nodules at the injection site. To date, there has been only one reported case of a hypomethylating agent inducing acute myocarditis. We a present a case of reversible, non-ischemic cardiomyopathy secondary to decitabine chemotherapy, which resolved after the drug was discontinued. Trials involving decitabine for the treatment of MDS reported no myocarditis. In our case, the diagnosis of transient cardiomyopathy was highly probable since the patient's troponins and echocardiogram returned to baseline after discontinuation of treatment. Also, the patient never had any further chest pain at his 6 month follow up. In this case, we believe that the elevated Troponin I levels, along with a cardiac catheterization revealing patent coronary vessels, favor our hypothesis that our patient suffered from acute myocarditis as a result of direct toxicity from decitabine chemotherapy. We doubt that there was an underlying infectious etiology, since the patient had three negative blood cultures, two negative urine cultures and a negative viral serology. Our case demonstrates that chest pains in a patient treated with hypomethylating agents should be further explored in order to rule out acute myocarditis.

Development of myelodysplastic syndrome and acute myeloid leukemia 15 years after hydroxyurea use in a patient with sickle cell anemia.

We report a 41 year old male with sickle cell disease who developed a myelodysplastic syndrome and acute myeloid leukemia with complex karyotype involving chromosomes 5, 7 and 17 after 15 years of hydroxyurea treatment. He responded poorly to induction chemotherapy with cytarabine/idarubicin followed by high dose cytarabine and succumbed to neutropenic sepsis. Multiple systematic reviews, observational studies and clinical trials were conducted to identify the toxicity profile of hydroxurea. Only six cases of leukemia/myelodysplastic syndrome were identified in patients with sickle cell anemia treated with hydroxyurea. Subsequently, it was concluded that hydroxyurea is not leukemogenic. However, it was noted that most of the published studies had only up to 9 years of follow-up. Our patient was started on hydroxyurea in 1990, before the widespread use of the drug and took hydroxyurea for 15 years. His presentation may reflect an outcome otherwise not yet observed because of the short follow-up of prior studies. We believe that the leukemogenic risk of hydroxyurea should be discussed with the patients and their families. Studies evaluating the adverse effects of hydroxyurea should have longer follow-up before definitive conclusions are drawn.

Clinical usefulness of haptoglobin levels to evaluate hemolysis in recently transfused patients.

Introduction. Haptoglobin binds the globin portion of free hemoglobin. Serum haptoglobin measurement is used as a laboratory marker for the diagnosis of hemolytic anemia. Since stored blood contains free hemoglobin, transfusion may affect haptoglobin levels. Study Objectives. The aim of the study was to evaluate whether serum haptoglobin could be measured to assess hemolysis in recently transfused patients. Patients and Methods. Twenty-one patients, receiving more than one unit of packed red blood cells (PRBCs) for presumed nonhemolytic indications, were enrolled. Serum haptoglobin levels were recorded before, immediately after, and 24 and 48 hours after transfusion. Observations and Results. Analysis of variance with a repeated measures was used to examine the serum haptoglobin levels at different time periods and no significant difference was found (P = .28). Conclusion. The results suggest that serum haptoglobin can be used in the diagnosis of hemolysis in patients receiving multiple units of PRBC.

A case of metastatic mucoepidermoid carcinoma complicated by resistant hypercalcemia.

Tumors of salivary glands are uncommon and comprise of about 2%-4% of all head and neck tumors. About 75%-80% of these tumors are benign and include pleomorphic adenoma, monomorphic adenoma, oncocytoma, and papillary cystadenoma lymphomatosum. Mucoepidermoid carcinoma is the most common malignant tumor of salivary glands, representing 5-10% of all salivary gland tumors. Although known to be metastatic to local lymph nodes, distant metastases are rare (especially, with low and intermediate grade tumors). Histologic grade and the expression of various mucin glycoproteins are useful prognostic indicators. We present a case of mucoepidermoid carcinoma of parotid gland origin with distant metastases which is an uncommon occurrence with intermediate grade tumors. Also, this is the first reported case of humoral hypercalcemia of malignancy secondary to mucoepidermoid carcinoma.

Severe refractory autoimmune hemolytic anemia with both warm and cold autoantibodies that responded completely to a single cycle of rituximab: a case report.

INTRODUCTION: Mixed warm and cold autoimmune hemolytic anemia runs a chronic course with severe intermittent exacerbations. Therapeutic options for the treatment of hemolysis associated with autoimmune hemolytic anemia are limited. There have been only two reported cases of the effective use of rituximab in the treatment of patients with mixed autoimmune hemolytic anemia. We report a case of severe mixed autoimmune hemolytic anemia that did not respond to steroids and responded to four weekly doses of rituximab (one cycle). CASE PRESENTATION: A 62-year-old Caucasian man presented with dyspnea, jaundice and splenomegaly. His blood work revealed severe anemia (hemoglobin, 4.9 g/dL) with biochemical evidence of hemolysis. Exposure to cold led to worsening of the patient's hemolysis and hemoglobinuria. A direct antiglobulin test was positive for immunoglobulin G and complement C3d, and cold agglutinins of immunoglobulin M type were detected. A bone marrow biopsy revealed erythroid hyperplasia. A positron emission tomographic scan showed no sites of pathologic uptake. There was no other evidence of a lymphoid or myeloid disorder. Initial therapy consisted of avoidance of cold, intravenous methylprednisolone and a trial of plasmapheresis. However, there was no clinically significant response, and the patient continued to be transfusion-dependent. He was then started on 375 mg/m2/week intravenous rituximab therapy. After two treatments, his hemoglobin stabilized and the transfusion requirement diminished. Rituximab was continued for a total of four weeks and led to the complete resolution of his hemolytic anemia and associated symptoms. At the patient's last visit, about two years after the initial rituximab treatment, he continued to be in complete remission. CONCLUSION: To the best of our knowledge, this is the first reported case of mixed-type autoimmune hemolytic anemia that did not respond to steroid therapy but responded completely to only one cycle of rituximab. The previous two reports of rituximab use in mixed autoimmune hemolytic anemia described an initial brief response to steroids and the use of rituximab at the time of relapse. In both of these case reports, the response to one cycle of rituximab was short-lived and a second cycle of rituximab was required. Our case report demonstrates that severe hemolysis associated with mixed autoimmune hemolytic anemia can be unresponsive to steroid therapy and that a single cycle of rituximab may lead to prompt and durable complete remission.

Renal epithelioid angiomyolipoma: a malignant disease.

BACKGROUND: Renal angiomyolipomas are recognized as clonal neoplasms with clonal chromosomal aberrations and a common progenitor cell, the perivascular epithelial cell (PEC). The epithelioid variant is a recently identified entity, characterized by predominance of PEC and a unique morphologic and immunohistochemical profile. There is accumulating evidence suggesting that renal epithelioid angiomyolipoma (REA) is a malignant disease. METHODS: We searched the literature for descriptions of clinical behavior of REA. A Pubmed search was performed using the following key words: angiomyolipoma, epithelioid, perivascular epithelial cell or/and renal tumors. We reviewed a case of fatal REA at our institution. A pathologist reviewed slides to confirm the diagnosis. RESULTS: Upon review of 140 articles, 37 eligible articles were found including 10 articles describing the clinical course of REA. Almost all of the patients described, for whom there was a follow-up available, died of neoplastic progression of the disease, with liver, lung and bone metastases. Four cases were reclassified after retrospective pathology review, and they were fatal. Three of these had been misdiagnosed as renal cell carcinoma (RCC), while 1 was diagnosed as classic angiomyolipoma. CONCLUSION: Unlike commonly benign classic angiomyolipoma, REA behaves aggressively. It is crucial for the clinician to be aware of and identify this epithelioid variant as a malignant disease. It should be carefully differentiated from RCC. Resection alone may not be curative, and adjuvant therapy should be considered. A multimodality treatment approach needs to be explored for this newly recognized malignant variant renal angiomyolipoma.

A phase I/II trial of fixed-dose docetaxel plus irinotecan and escalating doses of estramustine phosphate for second-line or greater treatment of selected advanced solid tumors.

This phase I/II study evaluated the safety of the combination of irinotecan, docetaxel, and estramustine for selected advanced solid tumors and also obtained initial efficacy data. Twenty-two patients were enrolled in the study. The regimen consisted of docetaxel 30 mg/m(2) and irinotecan 60 mg/m(2) both given intravenously on days 1 and 8 every 21 days in combination with escalating doses of estramustine (500 mg/m(2)/day escalated to 750 mg/m(2)/day on days 0, 1, 2, 7, 8, and 9 given every 21 days) during phase I. Dose escalation was continued until the maximum planned dose level of estramustine (750 mg/m(2)/day) was reached. After the appropriate phase II dose of estramustine was found additional patients were enrolled. Twenty-one of the 22 patients were evaluable for toxicity and 17 for tumor response. The recommended phase II dose of estramustine was found to be 750 mg/m(2)/day orally on days 0, 1, 2, 7, 8, and 9 given every 21 days. Hematologic toxicity was fairly mild, with only one episode of grade 3 neutropenia. Diarrhea was the most common nonhematologic toxicity with grade 3 toxicity occurring in five of 21 patients. Only one episode of venous thrombosis was observed. Objective response rate was 15.8%, overall clinical benefit rate was 63%, and median time to progression was 15 weeks. Estramustine in combination with the doublet of docetaxel and irinotecan is a well-tolerated regimen with minimal hematologic toxicity, mild to moderate nonhematologic toxicity, and promising initial antitumor activity in previously treated patients with advanced solid tumors.

Improving access to breast health services with an interdisciplinary model of care.

This article reports a hospital's experience confronting a community crisis, stemming from local and national breast health access issues, and evaluates the subsequent effectiveness of the initiative to improve breast care service. An interdisciplinary Breast Care Facility was developed adjacent to a Community Hospital. Patients receiving breast cancer screening during the year prior to the Breast Center opening (2002) were compared with patients in subsequent years (2003-2005). Program effectiveness was evaluated by examining screening mammography volume, wait times and cancer detection rates. Screening volume increased by 29.6%. Wait times declined from 30 weeks to 3.5 weeks. Initially, patients with a suspicious screening mammography had a 2-3 week delay for diagnostic mammography and the subsequent evaluation took another 3-4 weeks. Both times improved to an average of 2-5 days. Screening cancer detection rates increased from 3.2 per 1,000, to 6.3 per 1,000. In addition, the number of cancers identified by screening increased from 40% to 58%, p = 0.002. Patient satisfaction measured by survey was over 95%, in areas of courtesy, counseling, and overall care. Our study demonstrates that a comprehensive breast center model can increase access to breast care services, improve patient satisfaction and address focal areas of shortage. Furthermore, in the years after the opening of the breast center the cancer detection rate during screening increased, an important observation that needs to be investigated with future studies.

Need for parenteral iron therapy after bariatric surgery.

BACKGROUND: Malabsorptive bariatric procedures such as Roux-en-Y gastric bypass and biliopancreatic diversion/duodenal switch expose the patient to malnutrition and commonly cause iron deficiency. The optimal replacement and monitoring method remain undetermined. To identify high-risk patients who might need intravenous iron supplementation after bariatric surgery, we evaluated bariatric surgery patients who received parenteral iron at a university hospital-affiliated hematology center. METHODS: We performed a retrospective analysis and reviewed the records of 165 patients who had received parenteral iron from May 2004 to June 2007. Of the 165 patients, 42 bariatric surgery patients were identified. The type of bariatric procedure and menstrual status of the patients were compared. RESULTS: The average patient age was 40 years. Of the 42 patients, 2 were men and 40 were women. Of the 40 women, 32 were premenopausal and 8 were postmenopausal. The patients in the biliopancreatic diversion/duodenal switch group had a significantly lower hemoglobin at presentation (P = .02), relatively lower ferritin levels, and required more additional parenteral iron treatment after the initial resolution of anemia (P = .001). The premenopausal women required earlier parenteral replacement (P = .008) and were at a greater risk of anemia-related hospitalization (P = .00033). CONCLUSION: The available published studies lack any data regarding parenteral iron replacement needs after bariatric surgery. Our results have identified the need for long-term parenteral iron replacement therapy after malabsorptive bariatric procedures, especially in premenopausal women. Patients who do not respond to oral iron therapy should be referred early for parenteral iron replacement therapy to prevent anemia-related complications and to maintain patients' quality of life. Iron monitoring should continue indefinitely even after the initial repletion of iron stores and the resolution of anemia.

Breast cancer screening in women with chronic kidney disease: the unrecognized effects of metastatic soft-tissue calcification.

Patients with chronic kidney disease (CKD) or end-stage renal disease (ESRD) are known to develop metastatic soft-tissue calcification, secondary to hyperparathyroidism, in tissues including the breast. Such calcifications in women could pose a problem for interpretation of mammograms, since they are thought to mimic malignant lesions and interfere with differentiation of benign from malignant disease. Investigation of this issue is important to provide high-quality, accurate breast care to women with CKD or ESRD, but little evidence is so far available. In a systematic review of the literature on the types and patterns of breast calcifications, we found only three studies that examined metastatic soft-tissue calcifications of the breast. The studies did, however, confirm that women with CKD or ESRD have a higher frequency of breast calcification than women with normal kidney function. The two older studies reported that these breast calcifications are not associated with malignancy, but the later study reported a raised rate of suspicious breast calcification among women with ESRD receiving hemodialysis, leading to an increased biopsy referral rate. In this Review we discuss the strengths and limitations of the available data and whether mammography is recommended in women with CKD or ESRD.