Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare tumors originated in cells derived from the neural crest. The first ones are located in the adrenal medulla, and the second ones in the sympathetic and parasympathetic nervous system. These kind of tumors may secrete excess catecholamines, including epinephrine, norepinephrine, dopamine and/or their metabolite metanephrine, normetanephrine and 3-methoxytyramine, respectively. Its clinical manifestations depend on the location, the secretory profile and the malignant potential of the tumor. These tumors are frequently benign in their presentation. Some arise in the context of familiar syndromes, accounting for up to one-third of the total of diagnosis. The metastatic form is the most common presentation of the tumors with familiar origin and due to their rarity, their diagnosis and management is often difficult. Over the years, our knowledge and perception of PHEO and PGL has greatly expanded and changed. This review article aims to focus on the genetic, clinical, diagnostic, therapeutic and prognostic approaches, to give the clinician knowledge of the most recent updates regarding these themes.

Adult-onset Still's disease (AOSD) is an uncommon auto-inflammatory disease of unknown etiology, with a classical triad of fever, arthritis, and evanescent rash. Its low prevalence and lack of specific guidelines contribute to frequent delays in diagnosis and treatment. Clinical manifestations vary greatly between mainly systemic or articular symptoms and the clinical pattern between monocyclic, polycyclic, or chronic illness. Treatment options include nonsteroid anti-inflammatory drugs (NSAIDs), systemic corticoids, disease-modifying antirheumatic drugs (DMARDs), and, more recently, biological agents directed at identified immune pathological pathways like anti-interleukin-1 (IL-1) or anti-tumor necrosis factor-alpha (TNFalpha). We report a case of a 40-year-old male with persistent fever, polyarthralgia, sore throat, and rash for two weeks despite antibiotic treatment for suspected bacterial pharyngitis. During hospitalization and after extensive diagnostic workup, an AOSD diagnosis was made according to Yamaguchi's criteria and successfully managed with systemic corticoids.

A 50-year-old female with a past medical history of bone tuberculosis diagnosed nine months ago was admitted in our infirmary for persistent fever with no evident cause. The patient was treated with isoniazid, rifampicin, pyrazinamide, and ethambutol for seven months and for the past two months, she was taking isoniazid and rifampicin. She went to our emergency room (ER) for back pain and fever that she had been experiencing for the last month. She was admitted with suspicion of disseminated tuberculosis that was never confirmed. Physical examination was unremarkable. Blood tests showed an elevation of inflammation parameters. A computed tomography (CT) scan of the chest showed a mild pleural effusion. She remained with fever during the three weeks in the infirmary while undergoing many other studies that were all negative. The back pain would change sides, and three consecutive thoracic radiographies showed a small-sized pleural effusion that was either predominantly right-sided or left-sided. Several differential diagnoses were considered in the process, namely an active infection, neoplasia, or autoimmune disease. The search for circulating lupus anticoagulant was positive. Antinuclear antibodies (ANA) were positive and the anti-histone antibody was strongly positive. At this point, we suspected a drug-induced lupus diagnosis, and isoniazid was discontinued. Following discontinuation of isoniazid, back pain and fever subsided and patient was discharged after one week. This case is a diagnostic challenge because of the rarity and symptom severity of isoniazid-induced lupus. Isoniazid rarely induced this lupus-like syndrome, with an incidence of considerably less than 1%.

Despite a recent decline, tuberculosis (TB) infection is still a frequent diagnosis in Portugal. Adenosine deaminase (ADA) measurement has become an important tool in the timely diagnosis of this infection. However, ADA elevation in bodily fluids is not pathognomonic of TB infection. We present the case of a 70-year-old woman, undergoing treatment for pleural TB, diagnosed based on elevated ADA levels in a pleural effusion. Due to worsening symptoms she was readmitted, and the previous diagnosis was reconsidered. Thoracocentesis was repeated and cytometry analysis of the fluid was performed, showing the presence of diffuse large B cell lymphoma (DLBCL). DLBCL is the most frequently occurring non-Hodgkin lymphoma (NHL). Pleural involvement is rare in the initial stages. ADA elevation >250 U/l should raise suspicion of malignancy, especially in association with markedly elevated LDH levels. The purpose of this case report is to highlight that in the absence of microbiologic or histologic confirmation, a presumptive TB diagnosis should not be lightly made, and alternative diagnoses should be systematically ruled out.

Multiple myeloma (MM) is characterized by a proliferation of malignant plasma cells and a subsequent overabundance of monoclonal paraprotein. This disease commonly presents with hypercalcemia, kidney failure, anemia, and bone lesions. Acute kidney failure (AKF) as an initial presentation of MM has rarely been reported. Herein, we present a case of a 49-year-old female who was admitted to our intensive care unit (ICU) for AKF in June 2017. The patient was admitted to our emergency room (ER) with abdominal pain and biliary vomiting within six days. From the laboratory tests, we highlight a serum creatinine of 19 mg/dl and urea of 377 mg/dl. The physical examination was globally unremarkable. Once clinically stable, she was admitted to our infirmary with a creatinine of 8.00 mg/dl. The patient underwent an extensive study: markers for hepatitis B and C, human immunodeficiency syndrome (HIV), and autoimmune markers were all negative; renal ultrasound, abdominal and pelvic CT had no relevant alteration; and the skeletal survey had no significant change. Peripheral blood smear showed no abnormalities. Serum immunoglobulin analysis revealed an elevated immunoglobulin A (IgA). Serum protein electrophoresis showed a monoclonal spike and urine protein electrophoresis showed an increased amount of protein consistent with Kappa light chains. The Kappa:Lambda chain ratio was increased. In order to understand the etiology of this AKF, we ended up performing a kidney biopsy, which was compatible with a myeloma kidney. The patient was transferred to the Portuguese Oncology Institute in Porto and initiated chemotherapy. Two months after the hospital discharge, creatinine levels were stable around 1.5 g/dL. This case illustrates AKF as the initial and sole presentation of MM. This presentation, even though previously reported, is very uncommon, especially considering that it occurred in a young woman and it was associated with light chain precipitation of IgA. MM is an important differential diagnosis in AKF, particularly when excluded pre and post-renal etiologies. Although being an invasive procedure with inherent possible complications, a kidney biopsy is still a very important procedure that was essential in this case to achieve a final diagnosis and, therefore, the patients' treatment.