Progressive dyspnea and a right atrial mass in an 80-year-old man.

Hepatocellular carcinoma (HCC) is among the five most frequent causes of cancer death worldwide, according to the WHO. The disease is related to alcohol abuse, viral infections, and other causes of cirrhosis, and unfortunately, in some developed countries, the incidence shows an increasing trend. Although the diagnosis of the HCC often relies upon the context of a chronic hepatopathy, some cases may present a silent course, and the initial symptoms ensue when the disease is in an advanced stage with no chance for any therapeutic attempt. The clinical picture of the HCC is varied, and unexpected forms may surprise the clinician. One of the unusual presentations of the HCC is shock by the blockage of the venous return to the right atrium by the inferior vena cava infiltration. Herein we present a case of an old patient who sought medical care complaining of dyspnea. The clinical workup disclosed a right thorax pleural effusion and imaging exams depicted a mass in the right hepatic lobe, invasion of the inferior vena cava (IVC) and the right atrium (RA). During the attempts of clinical investigation, the patient passed away. The autopsy disclosed an HCC involving the right hepatic lobe, with the invasion of the IVC and the RA. The authors highlight the importance of recognizing the bizarre presentation of not so rare diseases.

Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum.

Primary splenic angiosarcoma (PSA) is a rare neoplasm of vascular origin associated with aggressive behavior and poor prognosis. The clinical presentation is usually non-specific and is mostly characterized by a wasting disease with anemia and splenomegaly, mimicking a wide range of entities. The authors present the case of an 80-year-old woman with cardiovascular comorbidities with a 6-month history of weight loss, fatigue, weakness, pallor, and abdominal pain. The physical examination showed massive splenomegaly and pallor. After a thorough evaluation that ruled out lymphoproliferative diseases, the working diagnosis was a myelodysplastic disorder. A few days after discharge, she returned to the emergency room with severe abdominal pain, worsening fatigue, and a remarkable pallor. Point-of-care ultrasound showed free intraperitoneal fluid. Spleen rupture was confirmed by abdominal computed tomography (CT) scan, and an emergency laparotomy with splenectomy was performed. The postoperative period was uneventful, and the patient recovered in a few days. The histopathology confirmed the diagnosis of PSA and the patient was referred to an oncological center. Two months later staging CT demonstrated liver and peritoneal metastases, and despite the chemotherapy she died 6 months after the diagnosis.

Traumatic injury to the internal carotid artery by the hyoid bone: a rare cause of ischemic stroke.

Central nervous system (CNS) ischemic events, besides being a common and devastating disease, are accompanied by severe disability and other morbidities. The cause of such events is not always that simple to diagnose, and among the young, a broad spectrum of possibilities should be considered. We present the case of a young man who presented two episodes of CNS ischemia with a 1 year gap between them, which occurred in the same situation while he was walking and carrying a heavy backpack. The second event first presented as a transient ischemic attack followed by a stroke the day after. The diagnostic work-up showed an indentation of the greater cornu of the hyoid bone over the internal carotid artery, which injured the media and intimal layers. At the arterial injury site, a micro thrombus was found, which explained the source of the embolic event to the CNS. The patient was operated on, and the procedure included the resection of the posterior horn of the hyoid bone, the resection of the injured segment of the internal carotid artery followed by carotid-carotid bypass with the great saphenous vein. The postoperative period and the recovery were uneventful as was the 5-month follow-up. We call attention to this unusual cause of stroke and present other cases reported in the literature.

Bone marrow necrosis and fat embolism syndrome: a dreadful complication of hemoglobin sickle cell disease.

Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management. Although there is a high burden of the disease, in many countries, the new-born sickle cell screening test is being performed and is rendering an early diagnosis; however, it is still difficult for sickle cell patients to find proper treatment and adequate follow-up. Moreover, in many countries, patients are neither aware of their diagnosis nor the care they should receive to prevent complications; also, they do not receive adequate genetic counseling. Hemoglobin SC (HbSC) disease is the most frequent double sickle cell heterozygosis found in Brazil. The clinical course tends to be more benign with fewer hospitalizations compared with double homozygotic SS patients. However, HbSC patients may present severe complications with a fatal outcome. We report the case of a 36-year-old man who presented to the emergency care facility with symptoms consistent with the diagnosis of sickling crisis. The outcome was unfavorable and death occurred just hours after admission. The autopsy revealed a generalized vaso-occlusive crisis by sickled red cells, bone marrow necrosis, and fat embolism syndrome.

Fatal hemoperitoneum due to segmental arterial mediolysis.

Spontaneous hemoperitoneum due to vascular injury is a life-threatening condition mostly associated with aortic or splanchnic arterial disease, which stems from atherosclerotic, inflammatory, or infectious origin. However, in 1976, Slavin and Gonzales described a nonatherosclerotic arterial disease that may render aneurysmal formation predominantly in the splanchnic arterial bed. The clinical presentation is diverse, but abdominal pain and shock prevail. We report the case of a middle-aged man who presented a hemoperitoneum due to a middle colic artery aneurysm rupture and died after undergoing a surgical treatment attempt. The preoperative imaging study revealed the presence of a huge hematoma in the epiplon retrocavity, and abdominal free liquid as well as extensive arterial disease with multiple aneurysms. The autopsy findings included hemoperitoneum, hematoma in the upper left abdominal quadrant, the surgical ligature of the middle colic artery, and histologic features consistent with segmental arterial mediolysis. The authors call attention to this rare entity and highlight the autopsy as a fundamental examination to accurately reach this diagnosis.

Fatal pancreatic pseudocyst co-infected by Raoultella planticola: an emerging pathogen.

Raoultella planticola is an aerobic Gram-negative bacterium belonging to the Enterobacteriaceae family. Initially identified in the 1980s, its pathogenic potential was further recognized when the first case of bacteremia was reported. Since then, only a few infections caused by this pathogen have been described. Although considered an opportunistic agent, fatal outcomes are associated with the infection by this pathogen, since it is more prevalent among the patients with immunodeficiency. The authors report the case of a middle-aged man diagnosed with end-stage renal disease and alcoholic pancreatitis, who was admitted to the emergency department with septic shock. Physical examination disclosed peritoneal irritation and a laparotomy was undertaken. Purulent peritonitis was found as well as a retroperitoneal abscess, which was drained. The postoperative period was troublesome, and the patient died. The autopsy showed a ruptured, infected pancreatic cyst and purulent peritonitis, among other findings. The culture of the peritoneal fluid and two blood sample sets were positive for R. planticola. The authors call attention to the importance of this emerging pathogen associated with severe gastrointestinal infections.

Gonococcal endocarditis: an ever-present threat.

The incidence of severe complications of the Neisseria gonorrhoeae infection has presented variations over recent decades since the advent of penicillin. Gonococcal endocarditis (GE) still remains an ever-present threat afflicting the society's poor and sexually active young population. This entity frequently requires surgical intervention and usually exhibits a poor outcome. The interval between the onset of symptoms and the diagnosis does not usually exceed 4 weeks. One of the characteristics of GE is a proclivity for aortic valve involvement with large vegetation and valve ring abscess formation. The authors report the case of a young man with a 2-week history of fever, malaise, weakness, and progressive heart failure symptoms, who had no previous history of genital complaints or cardiopathy. The physical examination was consistent with acute aortic insufficiency, which was most probably of an infectious origin. The echocardiogram showed thickened aortic cusps and valve insufficiency. After hospital admission, the patient's clinical status worsened rapidly and he died on the second day. The autopsy findings disclosed aortic valve destruction with vegetation and a ring abscess besides signs of septic shock, such as diffuse alveolar damage, acute tubular necrosis, and zone 3 hepatocellular necrosis. The blood culture isolated N. gonorrhoeae resistant to penicillin and ciprofloxacin. The authors call attention to the pathogen of this particular infectious endocarditis, and the need for early diagnosis and evaluation by a cardiac surgery team.

Indeterminate cell histiocytosis successfully treated with phototherapy.

First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome.

Lemierre's syndrome due to intratumoral abscess of the uvula.

Lemierre's syndrome (LS), described in detail in 1936, used to be a life-threatening entity until the advent of antibiotics. Tonsillitis or pharyngitis are the main primary infections and oropharyngeal anaerobic flora is the predominant etiology. However, other primary site infections, as well as other microbiological agents have been reported since the first description. Inflammatory symptoms in the neck and marked findings on physical examination predominate the majority of cases. Nonetheless, the authors report the case of a 54-year-old man with a history of dysphagia followed by cough, purulent expectoration, and fever. The bad condition of his dentition was noteworthy. During the diagnostic work-up, an ulcerated lesion in the uvula and a middle lobe pneumonia were disclosed. Streptococcus viridans was isolated from blood culture. On the fifth day of hospitalization, the patient died after a copious episode of hemoptysis. The autopsy findings depicted an abscess within a squamous cell carcinoma of the uvula, pharyngitis with carotid sheath spreading accompanied by pylephlebitis and thrombosis of the internal jugular vein up to the innominate vein, surrounded by an abscess in the mediastinum. Alveolar hemorrhage and pneumonia were also present. We conclude that the ulcerated carcinoma of the uvula housed an abscess, facilitated by the poor oral hygiene, which triggered LS and the descending mediastinitis. Pulmonary involvement was due to the septic embolism from the internal jugular vein. We would like to highlight the uvula abscess as the primary site of infection in this case of LS with S. viridans as the causative agent.

Atypical behavioral and psychiatric symptoms: Neurosyphilis should always be considered.

Syphilis still remains a major health concern worldwide because of the possibility of serious medical and psychological consequences, long-term disability, and death. Neurosyphilis (NS) may occur at any stage of infection. Its clinical presentation has been changing over recent years including- psychiatric and neurocognitive symptoms. Several recent studies have described cases with these symptoms as the principal signs of NS. We present the case of neurosyphilis with a psychiatric presentation characterized by mood disturbance and auditory and visual hallucinations.

Budd-Chiari Syndrome: an unnoticed diagnosis.

Budd-Chiari syndrome (BCS) encompasses a group of disorders caused by the obstruction to the hepatic venous outflow at the level of the small or large hepatic veins, the inferior vena cava, or any combination thereof. Clinical manifestation of the subacute form is characterized by supramesocolic abdominal discomfort, abdominal distension, fever, and lower limbs edema. Imaging work-up with hepatic Doppler ultrasound and abdominal computed tomography (CT) enables the diagnosis in the majority of cases. Treatment comprises long-term anticoagulation associated with measures that attempt to re-establish the flow in the thrombosed vessel (thrombolysis or angioplasty) or through the venous blood flow bypasses (transjugular intrahepatic portosystemic shunt or surgical bypass); however, the outcome is often dismal. The authors report the case of a 37-year-old woman presenting a 2-month history of dyspeptic complaints and abdominal distention. Fever was present at the beginning of symptoms. The laboratory work-up disclosed mild hepatic dysfunction, and the ultrasound showed evidence of chronic liver disease. Despite a thorough etiologic investigation, diagnosis was missed and, therefore, management could not be directed towards the physiopathogenetic process. The outcome was characterized by portal hypertension and esophageal varices bleeding. The patient died and the autopsy findings were characteristic of BCS, although an abdominal CT, close to death, had showed signs consistent with this diagnosis. The authors highlight the importance of knowledge of this entity, the diagnostic methods, and the multidisciplinary approach. BCS should be considered whenever investigating etiology for chronic or acute hepatopathy.

Immune thrombocytopenia and autoimmune thyroid disease: a controversial overlap.

Immune thrombocytopenia (ITP) is an entity characterized by a platelet count of less than 100 × 10(9)/L in the absence of other causes of thrombocytopenia, such as viral infections, rheumatic diseases, or drugs. Grave's disease is also an autoimmune condition in which thrombocytopenia is often observed. Moreover, in the literature, many reports show a marked interference of the thyroid dysfunction (mainly hyperthyroidism) in the control of thrombocytopenia. Although this issue still remains debatable, the authors report the case of a young woman with a previous diagnosis of ITP with a brilliant initial response to corticotherapy. Some years after this diagnosis, the patient presented thyrotoxicosis due to Grave's disease and the thrombocytopenia relapsed, but this time there was no response to the glucocorticoids. Only after the radioiodine I-131 thyroid ablation the control of thrombocytopenia was achieved. The authors call attention to this overlap and for testing thyroid function in every patient with an unexpected negative response to corticotherapy.

Septic pelvic thrombophlebitis of unknown origin: an ever threatening entity.

Septic pelvic thrombophlebitis (SPT) is a rare and severe entity, which may occur after abortion, delivery, gynecological diseases, or surgeries. Diagnosis is challenging when no risk factor is clearly present, since clinically, symptoms are non-specific. Nowadays, with the aid of imaging methods, diagnosis has become more achievable, but the treatment still bears some uncertainties. The authors present a fatal case of SPT in a young woman who sought medical care already presenting signs of septic shock, referring fever and non-characteristic abdominal pain. The patient evolved rapidly to multiple organ failure and respiratory distress, which was also due to septic pulmonary embolism. The autopsy confirmed the computed tomographic findings of a right ovarian vein septic thrombophlebitis and multiple septic pulmonary embolization foci. The patient did not present any of the recognized risk factors; neither did she present signs of pelvic inflammatory disease on admission or at autopsy. However, an intrauterine device was present. The authors call attention to this entity in the differential diagnosis of a woman with fever and abdominal pain, as well as for an empiric broad-spectrum antibiotic regimen in these cases.