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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.
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Neoplasias Duodenales , Neoplasias Gastrointestinales , Tumores Neuroectodérmicos , Sarcoma de Células Claras , Femenino , Humanos , Adulto Joven , Adulto , Sarcoma de Células Claras/diagnóstico , Sarcoma de Células Claras/patología , Sarcoma de Células Claras/cirugía , Intestino Delgado/patología , Neoplasias Gastrointestinales/patología , Tumores Neuroectodérmicos/diagnóstico , Tumores Neuroectodérmicos/patología , Tumores Neuroectodérmicos/cirugíaRESUMEN
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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Acute localized exanthematous pustulosis (ALEP) is a rare localized variant of acute generalized exanthematous pustulosis (AGEP). We report a case of ALEP localised on the trunk and induced by a mosquito bite in a breast cancer treated female patient.
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INTRODUCTION: Breast cancer is a major public health problem worldwide. It is the leading cause of cancer deaths in females. In developing countries like Tunisia, the frequency of this cancer is still growing. The aim of this study was to determine the crude and standardized incidence rates, trends and predictions until 2030 of breast cancer incidence rates in a Tunisian governorate. METHODS: This is a descriptive study including all female patients diagnosed with breast cancer in Monastir between 2002 and 2013. The data were collected from the cancer register of the center. Tumors were coded according to the 10th version of international classification of disease (ICD-10). Trends and predictions until 2030 were calculated using Poisson linear regression. RESULTS: A total of 1028 cases of female breast cancer were recorded. The median age of patients was 49 years (IQR: 41-59 years) with a minimum of 16 years and a maximum of 93 years. The age-standardized incidence rate (ASR) was of 39.12 per 100000 inhabitants. It increased significantly between 2002 and 2013 with APC of 8.4% (95% CI: 4.9; 11.9). Prediction until 2030 showed that ASR would reach 108.77 (95% CI: 57.13-209.10) per 100000 inhabitants. CONCLUSION: The incidence and the chronological trends of breast cancer highlighted that this disease is of a serious concern in Tunisia. Strengthening preventive measures is a primary step to restrain its burden.
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Neoplasias de la Mama , Adulto , Neoplasias de la Mama/epidemiología , Femenino , Humanos , Incidencia , Clasificación Internacional de Enfermedades , Persona de Mediana Edad , Sistema de Registros , Túnez/epidemiologíaRESUMEN
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.
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Cardiomiopatías , Cardiopatías , Adulto , Autopsia , Cardiomiopatías/diagnóstico , Muerte Súbita/etiología , Muerte Súbita Cardíaca/etiología , Femenino , Fibrosis , Cardiopatías/patología , Humanos , Cirrosis Hepática/complicaciones , Cirrosis Hepática/patología , Miocardio/patologíaRESUMEN
Since its outbreak in December 2019, a consistent number of case reports have been published describing a complex spectrum of skin manifestations associated with COVID-19. We report a first observation of demodicosis of the scalp after a severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) infection.
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Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC), is a new entity that is restricted to the sinonasal tract and is associated with high-risk HPV. This tumor is suggested to have an indolent behavior with a better prognosis than other carcinomas. We report a unique case of HMSC with a locally aggressive behavior. It is about a 61-year-old men presented with 12 months of unilateral progressive olfactory dysfunction accompanied by exophthalmia of the left eye, declining vision and headaches for 6 months. Computed tomography imaging revealed a voluminous mass occupying the ethmoid, maxillary and frontal sinus with bony destruction of the left ethmoidal blade. Histology showed a tumor composed of variably sized nests, separated by thick mucoid stroma. Tumor cells are plasmacytoid with hyperchromatic nuclei and frequent mitoses. Immunohistochemistry revealed that these cells were positive for cytokeratin AE1/AE3, p16 and negative for CK7, CK20, CD117, p40, p63, S100, synaptophysin and chromogranin.
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Infecciones por Papillomavirus/complicaciones , Neoplasias de los Senos Paranasales/diagnóstico , Progresión de la Enfermedad , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Neoplasias de los Senos Paranasales/patología , Neoplasias de los Senos Paranasales/virología , Tomografía Computarizada por Rayos XRESUMEN
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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Mycetoma, commonly known as Madura foot, is a chronic granulomatous infection caused either by fungi (eumycetoma) known as actinomycete. This disease occurs preferentially in young adults, and it affects the foot in particular. We report a Tunisian case of mycetoma occurring in an old patient, particular by its cystic presentation.
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Cemento-osseous dysplasia is a benign fibro-osseous lesion affecting the alveolar bone. It is classified into three forms: periapical, focal or florid dysplasia. It is often asymptomatic and fortuitously discovered during a routine radiological examination. However, it may become symptomatic after superinfection, after patient's exposure to oral bacterial flora. We here report a case of florid cemento-osseous dysplasia associated with actinomycosis of bone in a 53-year-old Tunisian woman. This superinfection has been rarely reported in the literature; Boolean Searching on PubMed for the keywords "cemento-osseous dysplasia AND actinomyces" displays a single article (Smith et al. 2011). The treatment of actinomycosis infection often requires long-term antibiotic therapy, sometimes associated with surgical debridement, as in the case of this patient who underwent piezosurgery to treat dysplasia and necrotic bone.
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Actinomicosis/diagnóstico , Displasia Fibrosa Ósea/diagnóstico , Osteomielitis/diagnóstico , Piezocirugía/métodos , Actinomyces/aislamiento & purificación , Actinomicosis/microbiología , Actinomicosis/cirugía , Femenino , Displasia Fibrosa Ósea/microbiología , Displasia Fibrosa Ósea/cirugía , Humanos , Persona de Mediana Edad , Osteomielitis/microbiología , Osteomielitis/cirugía , Radiografía , Sobreinfección/diagnóstico , Sobreinfección/microbiologíaAsunto(s)
Equinococosis , Quiste Paraovárico , Equinococosis/diagnóstico por imagen , Femenino , Humanos , Hallazgos Incidentales , ÚteroRESUMEN
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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Colorantes/efectos adversos , Dermatitis por Contacto/etiología , Dermatitis por Contacto/patología , Dermatitis Profesional/etiología , Dermatitis Profesional/patología , Dermatitis por Contacto/complicaciones , Femenino , Humanos , Persona de Mediana Edad , Industria Textil , Vitíligo/complicacionesRESUMEN
Fetus in Fetu (FIF) is an extremely rare congenital anomaly defined as a mass containing a vertebral axis often associated with other organs or limbs around this axis. We report the case of a female fetus aged 4 months presenting with retroperitoneal mass measuring 7x6x4cm, suggesting teratoma on computerized tomography (CT) scan. The mass was resected. Macroscopic examination showed fetiform mass covered by skin tissue and extending into upper and lower limb buds (A). Sectional views showed that it was centered by several osteocartilaginous fragments arranged in a linear fashion reminding the vertebral axis (B). Histological examination objectified the presence of glial tissue around a cerebral ventricle as well as skin, muscle and bone tissue. The diagnosis of FIF was retained. FIF is mainly reported at the level of the retroperitoneum followed by the sacro-caudal, intra-abdominal, cranial, buccal, mediastinal, pulmonary, renal and scrotal area. Diagnosis is made during the antenatal period in 15% of cases. The etiopathogenesis of FIF includes both the theory of monochorionic diamniotic monozygotic pregnancy in which an aberrant asymmetric twin becomes internalized in the other twin and the theory of a defective embryo implantation in the mesenchyme of its twin instead of the uterine wall. The differential diagnosis includes teratoma, meconium pseudocyst and ectopic pregnancy.
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A 2-year-old boy with prenatal diagnosis of a malformation uropathy was referred to paediatric surgery department. On systemic examination, there were no palpable masses. An ultrasonography of abdomen with color Doppler, a renal artery angiographic and scintigraphy revealed a preostial aneurysm at the left renal artery. The patient had a left nephrectomy. Grossly, the specimen measured 75 mm × mm 50 × 20 mm with renal artery aneurysm measuring 30 mm × 35 mm. On cut section, the renal parenchyma contained a whitish tumor that measured 35 mm × 10 mm. Histopathologically, this tumor was diagnosed as metanephric stromal tumor.
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Neoplasias Renales/diagnóstico , Riñón/diagnóstico por imagen , Nefrectomía/métodos , Biopsia , Preescolar , Diagnóstico Diferencial , Humanos , Riñón/cirugía , Neoplasias Renales/cirugía , Masculino , Enfermedades Raras , Células del Estroma/patología , Ultrasonografía DopplerRESUMEN
INTRODUCTION: Gastroblastoma is a rare gastric biphasic tumor with both epithelial and mesenchymal components. To the best of our knowledge only eight cases have been reported in the English literature till date. PRESENTATION OF CASE: We report a case of a 29-year-old female, hospitalized for epigastric pain with poor general condition. An upper gastrointestinal endoscopy showed a polypoid mass in the stomach near the gastric cardia suspicious of gastrointestinal stromal tumor. The patient underwent atypical proximal gastrectomy with splenectomy. Detailed histopathological examination of the resected specimen revealed the diagnosis of gastroblastoma. After six months, the patient developed loco-regional recurrence for which surgical debulking was performed. DISCUSSION: Gastroblastoma is predominantly seen in young adults with non-specific complaints. They appear as submucosal lesion in the stomach mimicking gastrointestinal stromal tumor. Preoperative diagnosis is often difficult. Surgical resection remains the mainstay of treatment. On histology, they consist of mesenchymal component which stain positively for vimentin and CD10 and epithelial component which is positive for cytokeratin on immunohistochemistry. CONCLUSION: Gastroblastoma is a malignant tumor with risk of local recurrence after curative resection.