Metastatic Malignant Disease Research Articles

Recent advances in management of phaeochromocytoma

Pheochromocytoma (PCC) and paraganglioma (PGL) are the neuroendocrine tumours that arise in adrenal medulla or extra adrenal sympathetic and parasympathetic paraganglia. Paraganglia are small organs that mainly consist of neuroendocrine cells derived from the embryonic neural crest that have the ability to synthesize and secrete catecholamines. Sympathetic PGLs are usually located in the chest, abdomen, or pelvis while parasympathetic PGLs are distributed along parasympathetic nerves in the head, neck, and upper mediastinum, also referred to as head and neck PGL (HNP). PCC/PGLs can be either functional (sympathetic) or non-functional (parasympathetic) based on catecholamine synthesis and secretion. In children diagnosed with hypertension, up to 1.7% have a catecholamine-secreting neoplasm. The majority of these tumours in childhood are PCC/PGLs. Incidence rates of PCC/PGLs are estimated at 0.3 cases per million per year or less. Approximately 10 –20% of these cases are diagnosed during childhood. Approximately half of the apparently sporadic PCC/PGLs that present in patients younger than 18 years are due to an identifiable germline mutation and this increases up to 70% in children less than 10 years of age. Hereditary tumor syndromes associated with PCC include Von Hippel-Lindau (VHL) disease, multiple endocrine neoplasia 2 (MEN-2), the familial PGL syndromes and rarely, neurofibromatosis (NF) type 1. Recently, many other genes are found to be involved in pathogenesis of PCC/PGLs. Hereditary PCC/PGLs are often multifocal and, in the case of PCC, frequently bilateral. Children usually present becauseof symptomatic catecholamine hypersecretion or, less often, due to tumor mass effects (e.g. pain), or as an incidental radiographic finding, or because of family screening. At present, the diagnostic test of choice is the measurement of fractionated plasma and/or urine metanephrines (metanephrines and normetanephrines). A four-fold increase above the reference range is associated with an almost 100% probability of the presence of a catecholamine-secreting tumor. 123I-metaiodobenzylguanidine(MIBG) is a highly specific test that can confirm the catecholamine-secreting nature of a tumor, localize tumors not seen with cross-sectional imaging, and identify other sites of disease as well as metastasis. Because MIBG testingis not 100% sensitive, particularly in some genetic mutations, recently other functional scans have been studied in the evaluation of PCC/PGL: 18F fluorodihydroxyphenylalanine (DOPA) positron emission tomography (PET), 18F fluorodopamine (FDA) PET,18F fluorodeoxyglucose (FDG) PET. Surgical resection is the mainstay in the treatment of PCC/PGLs. It should be done after adequate alfa and beta adrenergic blockade to minimize the hemodynamic fluctuation during surgery. Laparoscopic adrenalectomy is the preferred procedure for most PCC. Laparotomy should be considered in patients with large PCC and/or a concern for underlying malignancy based upon the clinical presentation or radiographic appearance of the tumor. In the setting of bilateral PCC, cortical-sparing procedures should be considered for the adrenal with the least tumor bulk. The cortical-sparing approach is particularly attractive in young children and children at risk for noncompliance with lifelong glucocorticoid and mineralocorticoid replacement. The surgical approach for removal of a PGL depends upon the location of the tumor but in selected cases can also be performed laparoscopically. Patients with unresectable malignant tumors or distant metastatic disease can usually be treated symptomatically with adrenergic blockade. Radiation therapy or radiofrequency ablation can help with symptomatic metastatic disease, and IV bisphosphonates can be considered bony metastases, particularly in patients with bone pain or lesions that increase the risk of pathological fracture. Systemic treatment modalities are only palliative in nature and include 123I-MIBG therapy, peptide receptor radiotherapy (PRRT) and chemotherapy. PCC/PGLs can have unpredictable behavior and metastasize late in the clinical course. In addition, children in particular are at risk for the development of metachronous tumors. This makes long-term follow-up mandatory in children with PCC/PGL.

18F-FDG PET-CT in the Management of Patients Receiving Definitive Radiotherapy for Malignancies of the Head and Neck

The study investigated the utility and timing of 18F-FDG PET-CT to evaluate for residual/recurrent or metastatic HNC in patients treated with definitive intensity modulated radiation therapy (IMRT) with or without chemotherapy, planned with 18F-FDG PET-CT. The incidence and timing of locoregional recurrence, distant metastatic disease, new primary malignancies, and death were evaluated in 261 patients retrospectively. Findings were classified based on pathology or clinical follow-up and the sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of FDG PET-CT were determined overall as well as at the time of each 18F-FDG PET-CT. The overall accuracy for 18F-FDG PET in the detection of residual/recurrent malignancy or metastatic disease was 96.4%. Of those in whom cancer recurred locally, 57% were identified based on physical examination and other imaging findings and 43% were identified initially on 18F-FDG PET-CT surveillance imaging when no disease was evident clinically. 18F-FDG PET-CT has a high diagnostic capability of detecting residual/recurrent malignancy or malignant metastatic disease in patients with HNC following IMRT ± concurrent chemotherapy, supporting 18F- FDG PET-CT’s use to evaluate patients for recurrent malignancy in the post-IMRT period, even without clinical evidence of disease.

Solitary Fibrous Tumor of the Kidney: A Wide Spectrum of an Uncommon Entity from Benignity to Malignant Metastatic Disease

Solitary fibrous tumour (SFT) is a spindle cell neoplasm that rarely occurs in the kidney. It has been reported mainly in the pleura, but it can also arise from almost every organ. Benign SFT of the kidney has been described in several cases, even with metastatic lesions, but only seven cases of malignant SFT including this case have been documented; and only three cases out of seven had distant metastasis. Clinical features are indistinguishable from other renal neoplasms. Diagnosis is based on inmunohistochemical analysis, showing positive staining for CD34, as a key for diagnosis. We report two different cases, with very different behaviour; showing the wide spectrum of malignancy of this entity; with a comprehensive review of the literature.

The Evaluation and Treatment of Polyostotic Lesions.

The approach to skeletal lesions has been well described1-3. Nevertheless, these lesions remain intimidating to both clinicians and patients. This is particularly true in the setting of polyostotic lesions, which can represent a metastatic process. Although it is imperative to consider this diagnosis, especially in adults, multicentric skeletal lesions can be the manifestation of a plethora of processes, ranging from non-neoplastic and benign conditions to more devastating malignant lesions and metastatic disease (Table I). In order to ensure accurate diagnosis and treatment, orthopaedic surgeons must be familiar with these conditions and the necessary workup. This article presents the approach to polyostotic disease while also reviewing some of the more common conditions presenting as multicentric skeletal lesions. View this table: TABLE I Non-Neoplastic, Benign, and Malignant Conditions That Can Present As Polyostotic Disease* There are many ways in which patients with polyostotic lesions present to orthopaedic surgeons. Some patients already will have had imaging studies performed, whereas others are being seen for the first time. Regardless, a thorough history and physical examination must be performed as it is only with the combination of a history, physical examination, and imaging that a differential diagnosis can be appropriately formulated. Once a lesion is identified, a bone scan is useful for …

Impact of a cancer of unknown primary (CUP) service on end-of-life planning for patients with metastatic cancer.

66 Background: Cancer of Unknown Primary (CUP) is the 4th most common cause of cancer death in England and Wales. [(Office for National Statistics, Mortality Statistics: Deaths registered in England and Wales. http://www.ons.gov.uk/ons/search/index.html?newquery=series+dr (accessed 1/7/14).] Patients with CUP have poor outcomes secondary to delayed, inappropriate investigations and poor treatment response. In line with NICE guidance [(NICE guidelines: CG 104 (July 2010).]. Metastatic malignant disease of unknown primary: diagnosis and management of metastatic malignant disease of unknown primary.) a CUP service was set-up at the Royal Free in June 2012. This analysis assessed its impact on patients diagnosed with metastatic cancer who were not fit for active treatment. Methods: Clinical notes were reviewed for all admissions with an imaging-based diagnosis of metastatic cancer. A retrospective analysis (January-April 2009) was compared to prospective data following the launch of the CUP service (June-December 2012). Results: The notes of 9 patients (2009) and 15 patients (2012) were compared (see table). Age and length of stay (LOS) were analysed using t-test, other data were compared using chi-squared test. All patients were reviewed by Oncology and Palliative Care teams. There was a significant reduction in mean LOS (6.7 days, 18.6 days p<0.004) and the number who died in hospital (7%, 67% p<0.002) with only one patient not dying in his preferred place. Conclusions: Prompt assessment by the CUP service facilitates early input from Oncology and Palliative Care teams resulting in reduced LOS and fewer deaths in hospital in keeping with the agenda of the End of Life Care Strategy [(End of Life Care Strategy: Promoting high quality care for all adults at the end of life (July 2008). DOH (England).]. [Table: see text]

Low socioeconomic status is associated with more aggressive end-of-life care for working-age terminal cancer patients.

The relationship between low socioeconomic status (SES) and aggressiveness of end-of-life (EOL) care in cancer patients of working age (older than 18 years and younger than 65 years) is not clear. We assessed the association between aggressiveness of EOL care and differences in SES among working-age terminal cancer patients from Taiwan between 2009 and 2011. A total of 32,800 cancer deaths were identified from the Taiwan National Health Insurance Research Database. The indicators of aggressive EOL care (chemotherapy, more than one emergency room [ER] visit or hospital admission, more than 14 days of hospitalization, intensive care unit [ICU] admission, and death in an acute care hospital) in the last month of life were examined. The associations between SES and the indicators were explored. Up to 81% of the cancer deaths presented at least one indicator of aggressive EOL care. Those who were aged 35-44 years and male, had low SES, had metastatic malignant disease, lived in urban areas, or were in hospitals with more abundant health care resources were more likely to receive aggressive EOL care. In multilevel logistic regression analyses, high-SES cancer deaths had less chemotherapy (p < .001), fewer ER visits (p < .001), fewer ICU admissions (p < .001), and lower rates of dying in acute hospitals (p < .001) compared with low-SES cancer deaths. Working-age terminal cancer patients in Taiwan received aggressive EOL care. EOL cancer care was even more aggressive in those with low SES. Public health strategies should continue to focus on low-SES patients to provide them with better EOL cancer care.

The biological and clinical importance of epithelial-mesenchymal transition in circulating tumor cells.

Movement of tumor cells from a primary tumor to a nonadjacent or distant site is a contiguous and complex process. Among the multiple natural cellular programs that promote initiation and progression of tumor metastasis, epithelial-mesenchymal transition (EMT) may play a key role in the ultimate generation of a metastatic foci. Acquisition of the EMT phenotype by tumor cells not only increases their migration and invasion potentials, thereby facilitating their ability to infiltrate blood vessels and to produce circulating tumor cells (CTCs), but also promotes survival of CTCs in the bloodstream and their ability to extravasate out of the circulatory system and invade proximal tissues. In organs distal to the primary tumor, the phenotypic switching mechanism of mesenchymal-epithelial transition (MET) enables CTCs to grow and colonize, enhancing the likelihood of establishing metastasis. In addition, CTCs that have undergone EMT attain increased resistance to chemotherapy and targeted therapy. CTCs with the EMT phenotype have become recognized as an active source of metastases, and targeting EMT/MET processes during the individual steps of tumor metastasis represents a promising new approach for alleviating cancer metastasis and recurrence. In this article, we focus on the biological and clinical importance of EMT and/or MET in CTCs during the individual steps of tumor metastasis, summarizing the recent findings of the regulatory roles played by EMT and/or MET in the generation, survival, and recolonization of CTCs and discussing the EMT-targeting strategies developed for tumor diagnosis as well as their potential for management of metastatic malignant diseases.

UNKNOWN PRIMARY INVESTIGATION SERVICE EVALUATION

BackgroundIn July 2010, NICE developed the guidelines for the diagnosis and management of Metastatic Malignant disease of Unknown Primary (MUO). At Chesterfield Royal Hospital (CRH) the Primary Investigation Service is...

Polypharmacy and excessive polypharmacy in octogenarians and older acutely hospitalized patients

The aim of this study was to assess the occurrence of polypharmacy and excessive polypharmacy in very old hospitalized patients based on their comorbidities. The documentation of patients aged 80 years or older admitted to our department in the year 2010 was analyzed. Based on the Charlson index of comorbidity, a multiple logistic regression model with stepwise backward elimination was performed. Patients were stratified by gender and four age-groups, and factors of a change in the number of medications during the hospital stay were assessed. Chronic pulmonary disease [odds ratio (OR): 2.40], diabetes mellitus with (OR: 4.65) or without (OR: 1.65) microvascular complications, congestive heart failure (OR: 2.37), connective tissue disease (OR: 3.02), and peripheral vascular disease (OR: 2.30) were statistically significantly associated with polypharmacy, while some of these diseases were also associated with excessive polypharmacy. The number of medications showed a gradual decrease with age, which was concordant with a decrease in total Charlson index score. "Admission for myocardial infarction" was associated with an increase in pharmaceuticals during hospital stay, whereas a known diagnosis of dementia or metastatic malignant disease was protective against a further increase in medications. Several medical conditions seem to predispose to polypharmacy in very old patients. To attain old age seems to be associated with few comorbidities, which reduces the need for a high number of pharmaceuticals. Physicians should pay attention to the identified predictors in very old patients, as polypharmacy may lead to adverse events and unnecessary hospitalization.

Komplikationsmanagement nach Resektion und Defektrekonstruktion hüftgelenknaher Tumoren

Tumorous destruction of the periacetabular region and the proximal femur are a consequence of either primary malignant bone tumor manifestation or metastatic disease, which is observed much more frequently and occurs typically in these skeletal segments. Pathological fractures of the proximal femur and periacetabular regions of the pelvis have a high incidence and ultimately lead to severe pain and immobilization. Advanced resection techniques and different types of defect reconstruction, allowing for oncologically sufficient resection of extensive tumors have contributed to a marked increase in the limb salvage rate. However, these procedures are associated with an increasing rate of several, sometimes severe intraoperative and postoperative complications. Compared to elective total hip arthroplasty, the rate of postoperative deep infections, dislocations, the incidence of pathological and periprosthetic fractures and the prevalence of deep vein thrombosis are increased with high rates of postoperative mortality and local tumor recurrence, being the most serious complications. Pelvic involvement and subsequent periacetabular resection have the highest complication rate when compared to proximal femur resection with endoprosthetic treatment. In order to minimize the risk of these intraoperative and postoperative complications wide resection and advanced reconstruction as well as complicated palliative stabilization due to malignant bone tumor growth around the hip joint should be performed in musculoskeletal tumor centers with profound expertise in osteosynthetic and endoprosthetic reconstruction of the pelvis and the proximal femur. Only in specialized centers an effective, multidisciplinary emergency management of these complications and, more importantly, reliable prevention of complications can be ensured.

Uncertainty and anxiety in the cancer of unknown primary patient journey: a multiperspective qualitative study

BackgroundPatients with cancer of unknown primary (CUP) have metastatic malignant disease without an identifiable primary site; it is the fourth most common cause of cancer death.ObjectivesTo explore patients’ informal and...

Hepatocellular carcinoma metastasis to the maxilla: a rare case

The manifestation of metastatic malignant disease in the oral cavity can present in a number of ways and may mimic benign conditions. This case reports a rare presentation from a metastasis of a hepatocellular carcinoma that had invaded the attached gingiva of the maxilla and which on initial inspection could have been misdiagnosed as a pyogenic granuloma. With the advent of new classes of drugs to manage this disease it could be expected that this presentation may become more common.

Bone Scintigraphic Findings of Hypophosphatemic Osteomalacia Associated With Adefovir Therapy

Occurrence of hypophosphatemic osteomalacia (HO) induced by administration of low-dose adefovir therapy is rare, and bone scintigraphy of the disease has rarely been reported. We report on bone scintigraphic findings in patients with HO. Multiple uptakes in ribs, spines, and lower extremities, suggesting multiple fractures, were observed on bone scintigraphy. Patients were diagnosed as HO associated with adefovir therapy. Owing to the same predilection sites, multiple uptakes on the axial skeleton can cause confusion with malignant metastatic disease; however, involvement of lower extremity skeletons can provide a scintigraphic clue for use in differentiation of HO from malignant osseous metastasis.

Intravenous Alteplase for acute ischemic stroke in patients with current malignant neoplasm

BackgroundThe current European license for Alteplase in acute ischemic stroke excludes the patients who have neoplasm with increased bleeding, but this criterion is not clearly defined.The aim of the present study is to assess whether intravenous (IV) thrombolysis is safe and effective in patients with current non-malignant neoplasm in absence of metastatic disease or other additional risk factors of bleeding. MethodsWe reviewed the clinical characteristics of 11 thrombolysed patients with current malignant neoplasm. We decided to treat only patients who did not have one or more of the following additional risk factors of bleeding: metastatic malignant disease; recent bleeding; documented hemorrhagic diathesis, also including baseline normal platelet count, activated partial thromboplastin time and prothrombin time value; and anticoagulant treatment on admission. ResultsCancer was diagnosed before IV thrombolysis in 9 patients, while non-metastatic malignant disease was incidentally detected after IV thrombolysis in 2 patients. None of the patients showed severe hemorrhagic complications. At 7days, all patients that showed a reduction of 4 points or more in the National Institutes of Health Stroke Scale from baseline were asymptomatic. At 3months, functional outcome was favorable in 7 (73%) patients. ConclusionsOur experience suggests that IV thrombolysis does not appear to increase the risk of hemorrhagic complications in current non-metastatic cancer patients in absence of additional risk factors of bleeding. In addition, these patients showed clinical improvement after IV thrombolysis, although replication of our findings in a randomized controlled trial is required to confirm our results.

A Case of Pheochromocytoma Associated with Diabetic Ketoacidosis and Infective Endocarditis

Pheochromocytoma is a rare neuroendocrine tumor that is usually derived from adrenal medulla or chromaffin cells along with sympathetic ganglia. In Western countries, the prevalence of pheochromocytoma is estimated to be between 1:6,500 and 1:2,500, compared with an incidence in the United States of 500 to 1,100 cases per year. Despite this low incidence, pheochromocytoma should always be considered for differential diagnoses because previous studies have shown that this condition can be cured in approximately 90% of cases. However, an untreated tumor is likely to be fatal due to catecholamine-induced malignant hypertension, heart failure, myocardial infarction, stroke, ventricular arrhythmias or metastatic disease. Symptoms that result primarily from excess circulating catecholamines and hypertension include severe headaches, generalized inappropriate sweating and palpitations (with tachycardia or occasionally bradycardia). Pheochromocytoma, however, has highly variable and heterogeneous clinical manifestations, including fever, general weakness and dyspepsia, and can be observed in patients who are suffering from infectious diseases. Several of such case reports have been presented, but most of these included infectious patients with high blood pressure and severe fluctuations. In this study, we presented the case of a 53-year-old male who showed normal blood pressure, but had a sustained fever. He was diagnosed with diabetic ketoacidosis, infective endocarditis and asymptomatic adrenal incidentaloma. Despite treatment with antibiotics and valve replacement, the fever persisted. After the patient underwent evaluation for the fever, adrenal incidentaloma was identified as pheochromocytoma. After removal of the abdominal mass, his fever improved. (J Korean Diabetes 2013;14:156-161)

Transient Telangiectatic Purpura after Axillary Dissection: A Postoperative Phenomenon

© 2013 The Authors. doi: 10.2340/00015555-1547 Journal Compilation © 2013 Acta Dermato-Venereologica. ISSN 0001-5555 Telangiectasias are small dilated vessels observed after inflammatory diseases, radiotherapy and in vascular malformations, such as Sturge-Weber neurovascular syndrome (1). Purpura is a discoloration of the skin due to extravasation of red blood cells. Extravasated blood is broken down to various metabolites derived from haem within 3 weeks. Purpura can originate from platelet or coagulation disorders, elevated intravascular pressure, vasculitis or toxic reactions to drugs. Purpura lesions do not blanch on applying pressure (diascopy) (2). Treatment of metastatic malignant disease often involves regional lymph node dissection. Axillary lymph node clearance involves removal of all lymph nodes in levels I–III. The intercostobrachial sensory nerves in the area are removed, but the motor nerves (long thoracic and thoracodorsal nn.) are preserved (3, 4). Significant morbidity can be related to axillary dissection including seroma, wound infection, haematoma, neuro praxia, lymphoedema and shoulder dysfunction (3, 5). We present here 2 cases of transient purpura discoloration of the skin after axillary dissection in 2 patients with different malignancies.

Abstract 4400: Novel combination of carbonic anhydrase inhibitor with a phytochemical for treatment of human bronchial carcinoids

Abstract Bronchial carcinoids are pulmonary neuroendocrine cell derived tumors comprising typical (TC) and atypical (AT) malignant phenotypes. The 5-year survival rate in metastatic disease, despite multiple current therapies, is 14-25%. Carcinoids are found in the gastrointestinal tract are more frequent (61%) and also more aggressive; however, 31% of carcinoids are located in bronchopumonary system, which can metastize beyond the longs. Therefore, new strategies are needed for effective treatment of carcinoid malignant progression and metastatic disease. The progenitor neuroendocrine cells are O2/CO2 chemosensory. Hypoxia and hypercapnia stimulate secretion of the neuroendocrine associated bioactive amine, serotonin, which can also serve as an autocrine growth factor. CO2 sensing and metabolism is associated with physiological activities of different carbonic anhydrases (CAs) which function in tumor cell pH homeostasis and therein regulation of growth, survival, and metastasis. CAs are abundantly expressed in lung carcinoids. We postulated that acetazolamide (AZ), a pan CA inhibitor, and the anti-tumor phytochemical sulforaphane (SFN), which can inhibit expression of serotonin receptors could function cooperatively and synergistically to inhibit growth of pulmonary carcinoids. A dose dependent effect of AZ (0-80 µM, 48h) and SFN (0-80µM, 48h) on carcinoid cell lines H727 (TC), H835 (intermediate phenotype) and H720 (AT) was assessed in vitro. Both compounds reduced cell viability (via Alamar Blue) and mitochondrial integrity (via JC-1 mitochondrial staining) dose-dependently in all cell lines. IC50 values for cell viability were 9.29 µM (H727), 16.67 µM (H835) and 30.85 µM (H720) for AZ and 51.93 µM (H727), 5.31 µM (H835) and 10.82 µM (H720) for SFN. The mitochondrial integrity JC1 IC50 values were 50.16 µM (H727), 15.52 µM (H835), 11.93 µM (H720) for AZ and 9.29 µM (H727), 16.67 µM (H835) and 30.85 µM (H720) for SFN treatment. Treatment of H727 subcutaneous xenografts in NOD/SCID mice for 2 weeks demonstrated modest growth inhibition with AZ (7%, 20mg/kg) and SFN (23%, 40mg/kg) alone. However, a highly significant reduction (57%; p=0.02) was shown with the combination treatment. Furthermore, the combination did not show any signs of morbidity in treated mice. Since these doses are at the low end and well within clinical range and bioavailability, our results suggest a potential new therapeutic strategy for the treatment of pulmonary carcinoids. The molecular mechanism underlying this synergistic anti-tumor effect, currently under investigation, suggests a novel targeting of tumor cell homeostasis. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 103rd Annual Meeting of the American Association for Cancer Research; 2012 Mar 31-Apr 4; Chicago, IL. Philadelphia (PA): AACR; Cancer Res 2012;72(8 Suppl):Abstract nr 4400. doi:1538-7445.AM2012-4400

Smoking is a major risk factor for anastomotic leak in patients undergoing low anterior resection

To examine modifiable risk factors for anastomotic leak in patients undergoing low anterior resection. In total 233 patients undergoing low anterior resection for benign and malignant disease over a 10-year period at a single surgical unit were identified from a prospective database. The relationships between anastomotic leak and 17 variables were examined, including patient demographics, operative technique, tumour pathology, preoperative physiological function and smoking status. The majority (91%) of operations were carried out for rectal cancers, and 24 procedures (10%) were performed with laparoscopic assistance. The overall anastomotic leak rate was 14% (33/233). Patients with anastomotic leak had higher 30-day mortality (6%vs 1%, P<0.05) and stayed significantly longer in hospital (median 23 vs 10 days, P<0.001). On multivariate analysis, current smokers (OR 3.68, 95% CI 1.38-9.82, P=0.009) and patients with evidence of metastatic malignant disease (OR 3.43, 95% CI 1.29-9.13, P=.013) were at increased risk of anastomotic leak. Smoking and the presence of metastatic disease are major risk factors for the development of anastomotic leak following low anterior resection.

An evaluation of hospice staffs' knowledge of organ and tissue donation

BackgroundThe latest version of the Liverpool Care Pathway directs staff to confirm whether or not the dying patient has previously expressed a wish to donate organs. Staff require basic knowledge...

Bilateral Primary Adrenal Lymphoma Presenting with Adrenal Insufficiency

Lymphoma may occasionally involve the adrenal glands, but primary adrenal lymphoma (PAL) is very rare and only few cases have been reported. We present a case of a 60-year-old, otherwise healthy, woman, with bilateral PAL presenting with adrenal insufficiency. The patient responded initially upon administration of large doses of intravenously hydrocortisone with total remission of symptoms. An abdominal computerized tomography scan demonstrated bilateral adrenal lesions but did not demonstrate any other pathology. Since metastatic malignant disease was suspected a positron-emission-tomography scan was performed only showing significant uptake in the adrenal glands. Endocrine evaluation did not reveal abnormal function of any hormonal system and the patient was scheduled for bilateral adrenalectomy. However the clinical condition deteriorated rapidly and the patient was readmitted to hospital before surgery was performed. A new computerized tomography scan showed rapid progression of disease with further enlargement of the adrenal masses and both pulmonary and hepatic metastasis. Needle biopsy was performed but the patient refused further treatment and died before a diagnosis was obtained. The immuneohistochemical diagnosis was large B-cell lymphoma. This case should remind clinicians that PAL may be a cause of bilateral adrenal incidentaloma especially if the patient presents with adrenal insufficiency.