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Gastrointestinal clear cell sarcoma (GICCS)/malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare form of cancer with aggressive clinical behavior. It has distinct pathological, immunohistochemical, ultrastructural, and molecular features. Herein, we present the case of a 20-year-old woman with no notable medical history who presented to the outpatient department with complaints of abdominal pain and vomiting. Symptoms had been evolving for 3 months. The physical examination revealed slight abdominal tenderness and melena. Biological investigations revealed iron-deficiency anemia. The upper and lower endoscopies showed no abnormalities. Magnetic resonance enterography revealed small bowel wall thickening of 15 mm × 2 mm. Exploratory laparotomy revealed an ileal mass with mesenteric lymphadenopathy. A wide resection of the mass was then performed. The final pathological report confirmed the diagnosis of small bowel GICCS/GNET. After 11 months of follow-up, the patient presented with mesenteric lymph node metastases.
Assuntos
Neoplasias Duodenais , Neoplasias Gastrointestinais , Tumores Neuroectodérmicos , Sarcoma de Células Claras , Feminino , Humanos , Adulto Jovem , Adulto , Sarcoma de Células Claras/diagnóstico , Sarcoma de Células Claras/patologia , Sarcoma de Células Claras/cirurgia , Intestino Delgado/patologia , Neoplasias Gastrointestinais/patologia , Tumores Neuroectodérmicos/diagnóstico , Tumores Neuroectodérmicos/patologia , Tumores Neuroectodérmicos/cirurgiaRESUMO
Primary mediastinal large B-cell lymphoma (PMLBCL) is an aggressive tumor originating from thymic B-cells. Clinically, it presents with general signs such as cough, chest pain and dyspnea. Although these symptoms are not specific, they are severe enough to reveal the disease. We report an autopsy case of a 25-year-old man, with a recent past history of cough and dyspnea, for which he consulted twice the emergency department and no diagnosis was made. He presented to the Emergency Unit, with a sudden onset of a dyspnea followed by a loss of consciousness. He was shortly declared dead after, a medico-legal autopsy was requested. On external examination, no traumatic lesions on the body were found, an important cyanosis of the face and ears, was, however, found. On autopsy, a mediastinal mass was found, measuring 19 cm × 25 cm and weighing 600 g, extending to the infra-hyoid region and to the thoracic cage and infiltrating the pericardium. Trachea had a necrotic mucosa with a partially obstructive lymph node mass. The diagnosis of a primary mediastinal large B-cell lymphoma was suspected based on pathological and immunohistochemical findings. The cause of death was finally attributed to respiratory failure due to this tumor.
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Linfoma Difuso de Grandes Células B , Neoplasias do Mediastino , Masculino , Humanos , Adulto , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/diagnóstico , Neoplasias do Mediastino/complicações , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/patologia , Tosse/complicações , Morte Súbita/etiologia , Dispneia/etiologiaRESUMO
Takayasu arteritis is a rare pathology that usually has general and atypical signs that make its diagnosis difficult. These characteristics can delay diagnosis, thus leading to complications and death. We, herein, report an autopsy case of a 25-year-old female patient with a history of multiple consultations for dyspnea. During these consultations, no diagnosis was made. She was found unconscious near her home and shortly after, she was declared dead. Forensic autopsy revealed superficial traumatic lesions. Internal examination revealed complete situs inversus. Multiple bilateral pleural adhesions and bilateral moderate effusion were found. The heart was heavy with thickening of the aortic wall (1.1cm), carotid arteries, and pulmonary trunk, associated with a large aortic valve and evidence of leakage. Histological examination of the aorta and its major branches showed features of panarteritis with segmental involvement. The vascular wall was thick with lymphoplasmacytic infiltrate and giant cells involving mainly the medio-adventitial junction. Disruption of the elastic lamina and reactive fibrosis in the intima were also noted. Diagnosis of large vessel vasculitis and particularly Takayasu arteritis was made. Death was therefore attributed to heart failure due to aortic insufficiency as a complication of Takayasu arteritis.
Assuntos
Situs Inversus , Arterite de Takayasu , Humanos , Feminino , Adulto , Arterite de Takayasu/complicações , Arterite de Takayasu/diagnóstico , Arterite de Takayasu/patologia , Autopsia , Morte Súbita/etiologia , Aorta/patologia , Situs Inversus/complicações , Situs Inversus/patologiaRESUMO
Erythema nodosum (EN), the most common form of panniculitis, is a reactive inflammation of the subcutaneous fat clinically presented with a sudden onset of painful, erythematous, nodular, subcutaneous lesions, typically localized to the pretibial area. EN is commonly caused by numerous infections (especially beta-haemolytic streptococcal infections), autoimmune diseases (sarcoidosis), inflammatory bowel conditions and drugs. EN induced by Covid-19 vaccines is rarely reported. We describe an original clinical observation of a 75-year-old woman who presented with EN after receiving the second dose of BNT162b2, an mRNA vaccine.
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Doenças Autoimunes , COVID-19 , Eritema Nodoso , Idoso , Feminino , Humanos , Doenças Autoimunes/complicações , Vacina BNT162 , COVID-19/prevenção & controle , COVID-19/complicações , Vacinas contra COVID-19/efeitos adversos , Eritema Nodoso/induzido quimicamente , Eritema Nodoso/diagnósticoRESUMO
Introduction and importance: Primary Retroperitoneal mucinous cystadenoma (PRMC) is an extremely rare benign tumor, predominantly occurring in women, with unclear pathogenesis. Case presentation: A 31-year-old woman, with no medical or surgical history, presented with left flank pain. Clinical discussion: An abdominal computed tomography (CT) scan revealed an 11cm retroperitoneal cyst. Due to its large size, percutaneous CT-guided drainage followed by a laparotomy surgical resection, were performed. Post-operative course was uneventful. Histological and immunohistochemical findings were consistent with PRMC. The patient was disease-free after a 6-month follow-up. Conclusion: Mucinous cystadenoma is a very odd finding in the retroperitoneum. Multiple differential diagnoses are to be considered beforehand, as most of cystic lesions in this anatomical region are malignant and require a different surgical approach. Radical resection, by laparotomy or laparoscopy, is the treatment of choice.
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Cirrhosis cardiomyopathy is defined by cardiac dysfunction in cirrhotic patients. It is characterized by the reduced contractile response to stress and/or impaired diastolic relaxation associated with electrophysiological disturbances with unknown cardiac disease. Here we report a case of sudden death in a 44-year-old woman, with no personal and family medical history and in apparently good health before death. The death was occurred when performing agricultural activities. The autopsy revealed an elevated weight of the heart with heterogeneous myocardium. The liver was heavy and had a variegated appearance. The histologic examination showed fibrosis and partially disturbance of the texture of the left ventricular myocardial tissue with storiform patterns and circumscribed hypertrophic cardiomyocytes. The microscopic examination of the liver showed cirrhosis with no specific features of etiology. The death was attributed to arrythmia due to cirrhotic cardiomyopathy. Our case highlighted the importance of pathological examination to con-sider the diagnosis of cirrhotic cardiomyopathy in case of sudden death for patient with known or unknown cirrhosis.
Assuntos
Cardiomiopatias , Cardiopatias , Adulto , Autopsia , Cardiomiopatias/diagnóstico , Morte Súbita/etiologia , Morte Súbita Cardíaca/etiologia , Feminino , Fibrose , Cardiopatias/patologia , Humanos , Cirrose Hepática/complicações , Cirrose Hepática/patologia , Miocárdio/patologiaRESUMO
Psoas abscess is a rare pathology that usually presents with non-specific signs and rare clinical features. These characteristics can delay the diagnosis leading to complications and death. We report a forensic autopsy case of a 65-year-old male, alcoholic, smoker, with a history of hypertension, and urinary infection, who presented to the emergency room for anorexia and consciousness disorder. On physical examination, the patient was febrile and confused. Laboratory exams revealed leukocytosis and elevated C-reactive protein (CRP). Two days later, he died despite extensive resuscitation. Forensic autopsy revealed a large amount of green pus in the left psoas muscle extending to the muscles of the thigh of the same side with multiple cavities. The pus extended to the left kidney with destructive parenchyma and coralliform lithiasis. Histological examination showed destroyed renal tissue by lesions of chronic and acute pyelonephritis with dilatation of the pyelocaliceal cavities. Bacteriological analysis of the pus showed the presence of Escherichia coli. The psoas abscess was secondary to pyonephrosis favored by the immunodeficiency. Thus, death was attributed to a septic shock secondary to a psoas abscess complicating pyonephrosis.
Assuntos
Abscesso do Psoas , Pionefrose , Choque Séptico , Masculino , Humanos , Idoso , Abscesso do Psoas/complicações , Abscesso do Psoas/diagnóstico , Pionefrose/complicações , Pionefrose/patologia , Proteína C-Reativa , Músculos Psoas/patologia , Choque Séptico/etiologiaRESUMO
The histological features of cystic partially differentiated nephroblastoma (CPDN) require a thorough sampling of the surgical specimen and the application of immunohistochemical techniques to confirm the diagnosis.
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INTRODUCTION AND IMPORTANCE: Histiocytic sarcoma (HS) is a rare malignant neoplasm showing morphologic and immunohistochemical features of histiocytes. It is characterized typically by extranodal presentation and a poor clinical course, particularly in cases with disseminated disease. CASE PRESENTATION: This report documents a case of bifocal and aggressive HS in small bowel and lung revealed by acute peritonitis in a 63-year-old man. CLINICAL DISCUSSION: Despite its rarity, we believe that the correct diagnosis of HS is crucial for clinical treatment and prognostic prediction. CONCLUSION: The collection of additional cases of HS are important to obtain further progress in prognosis and guide treatment decisions.
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Isolated pancreatic tuberculosis (PT) is an extremely rare disease, with non-specific clinical characteristics, making the diagnosis often challenging with pancreatic cancers. Here we report a case of a 36-year-old female, who was admitted to our hospital after suffering from a 3-month history of epigastric abdominal pain, night sweats and weight loss. The physical examination was normal. The radiological findings revealed the presence of a pancreatic mass and multiple abdominal lymphadenopathy, suggestive of malignancy. The initial differential diagnosis suspected was pancreatic tuberculosis. Tuberculosis skin test was performed and was highly positive (>22 mm). Computed tomography (CT)-guided biopsy of peripancreatic lymph node was carried out and the histopathological exam confirmed the diagnosis of PT. Therefore, anti-tuberculous therapy was initiated, leading to clinical and radiological improvement. The diagnosis of PT is rare and can sometimes be misleading. It should be considered when a pancreatic mass is observed, especially in endemic countries, to ovoid unnecessary interventions.
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INTRODUCTION: Peutz-Jeghers syndrome (PJS) is a rare hereditary disease characterized by hyperpigmentation on the lips and oral cavity and gastrointestinal hamartomatous polyps. The most common complications in PJS patients are bleeding, bowel obstruction and intussusception. PRESENTATION OF CASE: We hereby report a case of a 33-year-old female, without a family history of the disease, who presented to the emergency room with acute abdominal pain, bloating and not passing gas. On abdominal examination, upper abdominal and periumbilical tenderness was found. Computed tomography (CT) of the abdomen demonstrated suspected ascending colon intussusception. The patient underwent a mid-line laparotomy that showed an ileocolic intussusception. Reduction of this intussusception was successfully done with resection of the affected segment that showed presence of two pedunculated polyps. The specimen was sent thereafter to our department for histopathological evaluation, which confirmed the diagnosis of hamartomatous Peutz-Jeghers polyps with no malignancy. Afterwards, the patient was carefully reexamined and the physical examination revealed multiple pigmented spots on the face and lips. Thus, the diagnosis of Peutz-Jeghers syndrome was made. DISCUSSION: PJS is a rare autosomal dominant disorder that often remain undiagnosed for many years. Acute complications such as intestinal obstruction secondary to intussusception is one of infrequent revealing symptoms. CONCLUSION: Early identification, in patients with PJS and family members, as well as close cancer surveillance can improve certainly prognosis in these individuals.