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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
Noonan syndrome is a genetic disorder frequently caused by PTPN11 mutations. Patients with Noonan syndrome are characterized by facial dysmorphism, short stature and congenital heart defects and they have a reported predisposition to malignancies such as leukemia, and solid and central nervous system tumors. Here, we report a case of a 14-year-old boy with Noonan syndrome treated for T-cell acute lymphoblastic leukemia who presented with 2 concomitant abnormalities: cerebral abscess and high grade glioblastoma. This exceptional association exhibits to a poorer prognosis and may sometimes delay the diagnosis and therefore the therapeutic intervention.
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Background and Aims: Successful Helicobacter pylori (Hp) eradication with the traditional 7-day course of proton pump inhibitor triple therapy is declining. Prolonging therapy to 14 days is associated with better eradication rates. Most learned societies recommend concomitant quadruple therapy (QC) as a first-line alternative therapy for this bacterial infection. The aim of this study is to compare the efficacy and safety of triple therapy (TT) and QC for the eradication of Hp infection. Methods: A parallel double-blind randomized controlled trial was conducted. The diagnosis of Hp infection was made by pathological examination of gastric biopsies. Patients were randomly assigned to two treatment groups: either QC (esomeprazole 80 mg, amoxicillin 2000 mg, clarithromycin 1000 mg, and metronidazole 1000 mg daily) or triple therapy (esomeprazole 80 mg, amoxicillin 2000 mg, and clarithromycin 1000 mg daily in divided doses) for 14 days. The efficacy of the treatment is defined by Hp eradication attested by a negative breath test performed 6 weeks after the completion of treatment. Treatment outcomes were compared using the chi-square test, while binary logistic regression identified predictors of treatment failure. Results: Ninety-two patients were included. Forty-two patients belonged to the QC group and 50 to the TT group. No significant difference was noted between the two groups concerning the rate of Hp eradication either by intention to treat (81% vs. 72% respectively, p = 0.31) or per protocol (81.6% vs. 76.1% respectively, p = 0.54). Likewise, there was no difference between the two groups in terms of tolerance to treatment (59.5% for QC vs. 58% for TT, p = 0.88). No factor has been associated with treatment failure. Conclusion: There was no significant difference in the rate of HP eradication between the QC and the 14-day triple therapy. Neither regimen should be used topically because of their low eradication rates.
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Key Clinical Message: There has been a recent spike in reporting of noncalcifying variants of CEOT with the WHO 2022 classification of head and neck tumors. The present case describes a rare histopathological variant of CEOT of which a handful of cases have been reported. Abstract: This work aimed to report a rare case of noncalcifying type of Pindborg Tumor involving the right mandible. The absence of calcifications in the calcifying epithelial odontogenic tumor presents a diagnostic challenge and prognostic implications. A literature review of the 16 reported clinical cases of this scarce variant was performed.
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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.
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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
We report a new case of chronic recurrent annular neutrophilic dermatosis in a woman. Through our observation, we aim to make the clinician aware of this rare entity, in order to consider it among the diagnostic hypotheses of annular dermatosis, with centrifugal, recurrent, and chronic evolution.
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Clear cell meningiomas (CCM) are a very rare histologic subtype of meningioma usually affecting younger patients. The reported data on spinal CCM are extremely rare. Until today, only 89 cases have been reported. Furthermore, CCM without dural attachment is even rarer since only 19 cases have been reported in English literature. In this article, we present the twentieth case of a spinal CCM without dural attachment. Our patient was a 58-year-old female who was presented with pain in her lower back and bilateral sciatica for 6 months. Magnetic resonance imaging showed an intra-dural well-demarcated lesion at L3. Via a posterior approach, total resection was possible due to the lack of dural adhesion of the tumor. Histologic diagnosis was clear cell meningioma.
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Infecciones Oportunistas Relacionadas con el SIDA/complicaciones , Dermatitis/diagnóstico , Granuloma/diagnóstico , Neoplasias Cutáneas/diagnóstico , Infecciones Oportunistas Relacionadas con el SIDA/patología , Dermatitis/patología , Diagnóstico Diferencial , Granuloma/patología , Humanos , Neoplasias Cutáneas/patologíaRESUMEN
A boy weighing 4500 g was born at 41 weeks' gestation by Cesarean section due to fetal distress. The pregnancy was complicated by gestational diabetes. He had an Apgar score of 5 and 6 after 5 and 10 minutes, respectively. At birth, the newborn manifested respiratory distress, which needed assisted ventilation for 48 hours. He developed a convulsive attack, diagnosed as a grade 2 hypoxic-ischemic encephalopathy, which was controlled by phenobarbital. Four days later, dermatologic examination revealed subcutaneous and firm nodules, ranging from 1 to 4 cm in diameter, on the cheeks, neck, arms, legs, and back (Figure 1). Some nodules became fluctuant as abscesses. Fine-needle aspiration cytology performed on a nodule revealed a dirty background with necrotic fat-containing characteristic, radially- oriented, refractile, needle-shaped crystals (Figure 2), which was diagnosed as subcutaneous fat necrosis. The diagnosis of subcutaneous fat necrosis of the newborn was made. Laboratory studies revealed hypocalcemia at 1.65 mmol/L, hypomagnesemia at 0.48 mmol/L, and hypokalemia at 3 mmol/L. The infant received calcium, magnesium, vitamin D, and potassium supplementation. On day 18, the serum calcium increased to 3.3 mmol/L. It was associated with hypertriglyceridemia at 2.6 mmol/L. Bilateral nephrocalcinosis was detected on renal ultrasound. So, hyperhydration, diuretics, and withdrawal of vitamin D were indicated. The patient was given betamethasone 0.125 mg/kg/day for 3 weeks. After a two months course, there had been complete healing of the fat necrosis (Figure 3), normalization of the calcium and triglyceride levels, and a normal growth pattern.
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Necrosis Grasa , Cesárea , Necrosis Grasa/diagnóstico , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Necrosis , Embarazo , Grasa Subcutánea , UltrasonografíaRESUMEN
Metastatic parapharyngeal lymph nodes (LNs) from papillary thyroid carcinomas (PTC) are uncommon and can easily remain undetected. We describe a case that involves a 62-year-old woman treated for a PTC, who presented a rise in serum thyroglobulin (TG) levels. A computed tomography scan was performed, and revealed metastatic nodes in the left parapharyngeal space (PPS). A surgical resection of the nodes was performed with external cervical approach. A histological exam confirmed the diagnosis of a metastatic LN of a PTC. The aim of this report is to emphasize on the possibility of parapharyngeal metastatic nodes in PTC and to describe the diagnosis methods, treatment options, and impact on the prognosis.
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BACKGROUND: Secondary solid cancers are severe complications in patients who have undergone allogeneic hematopoietic stem cell transplantation (AHSCT) for malignant and nonmalignant lymphohematopoietic diseases. OBJECTIVE: The aim of this work was to report a case of two lip carcinomas following AHSCT and to warn doctors about the importance of regular check-ups of patients who have received HSCT. Observation. A 57-year-old man was referred by the dermatology department for the management of exophytic budding lesions on the lower lip evolving since 5 months. The patient was in complete remission following allogeneic bone marrow transplantation for acute myeloid leukemia since five years. Clinical and histological findings confirmed the diagnosis of a squamous cell carcinoma of the two lesions. CONCLUSION: It is of paramount importance to seek an oral squamous cell carcinoma in the presence of persistent lesions in HSCT recipients.
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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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Amniotic fluid embolism (AFE) is an unforeseeable, life-threatening complication of pregnancy and child birth. Although rare in an absolute sense, most contemporary series of maternal deaths from developed countries report AFE as a leading cause of mortality in the pregnant population. It has a heterogeneous presentation. This clinical heterogeneity makes the diagnosis of AFE difficult based on a beam of clinical and para-clinical arguments. Rapid diagnosis and immediate interdisciplinary treatment are essential for a good outcome. The present is a case of AFE with a disseminated intravascular coagulation (DIC) and a cardiorespiratory collapse following a vaginal delivery.
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Coagulación Intravascular Diseminada/diagnóstico , Embolia de Líquido Amniótico/diagnóstico , Adulto , Parto Obstétrico , Coagulación Intravascular Diseminada/terapia , Embolia de Líquido Amniótico/terapia , Femenino , Humanos , EmbarazoRESUMEN
INTRODUCTION AND IMPORTANCE: The ossifying fibromyxoid tumor of soft tissue is a rare tumor of intermediate differentiation and uncertain lineage that occurs in adults mostly in the extremities and the trunk. PRESENTATION OF CASE: we present a case of 57 year-old man presenting with a right scapular mass. It was a subcutaneous and painless mass that was largely excised. The diagnosis of ossifying fibromyxoid tumor of the right shoulder was made. The follow up of 1 year was without recurrence and metastasis. CLINICAL DISCUSSION: The ossifying fibromyxoid tumor of soft tissue is exceptional, microscopic diagnosis and management is challenging, considering the scarcity of the tumor. CONCLUSION: More cases and retrospective studies are needed to understand the pathogenesis and to determine optimal treatment regimens.
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The purpose of this study was to describe the clinical, radiological, histological and therapeutic features of breast cancer diagnosed during pregnancy. We performed a review of all medical records of patients with breast cancer diagnosed during pregnancy in the Maternity and Neonatal Center, Monastir-Tunisia, over the period 2004-2019. We collected data on 15 cases. The average age of patients was 34 years; most pregnancy-associated breast cancers (PABCs) had been diagnosed during the postpartum period. Invasive ductal carcinoma was the major histological type (93% of cases), a rare case of secretory breast carcinoma had been observed. The main clinical stages were T2 and T4 breast cancer. Hormone receptor (HR)-negative breast cancers had been reported in 40% of cases, HER2-positive breast cancers in 26.6% of cases. Treatment included surgery, radiotherapy, chemotherapy and palliative chemotherapy. The median overall survival was 32.2 months. Pregnancy-associated breast cancer is a rare entity. Patients' prognosis is generally poor due to the young age at onset and a usually delayed diagnosis. Patients should participate in therapeutic decision making, which is difficult and multidisciplinary. Targeted therapy is the great hope for new therapies.
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Neoplasias de la Mama/patología , Carcinoma Ductal de Mama/patología , Complicaciones Neoplásicas del Embarazo/patología , Adulto , Factores de Edad , Neoplasias de la Mama/diagnóstico , Neoplasias de la Mama/terapia , Carcinoma/diagnóstico , Carcinoma/patología , Carcinoma/terapia , Carcinoma Ductal de Mama/diagnóstico , Carcinoma Ductal de Mama/terapia , Femenino , Humanos , Estadificación de Neoplasias , Periodo Posparto , Embarazo , Complicaciones Neoplásicas del Embarazo/diagnóstico , Complicaciones Neoplásicas del Embarazo/terapia , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia , TúnezRESUMEN
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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Pustulosis Exantematosa Generalizada Aguda/diagnóstico , Antifúngicos/efectos adversos , Arthrodermataceae/aislamiento & purificación , Tiña del Cuero Cabelludo/diagnóstico , Pustulosis Exantematosa Generalizada Aguda/tratamiento farmacológico , Corticoesteroides/farmacología , Corticoesteroides/uso terapéutico , Antifúngicos/uso terapéutico , Preescolar , Griseofulvina/efectos adversos , Griseofulvina/uso terapéutico , Humanos , Tiña del Cuero Cabelludo/tratamiento farmacológicoRESUMEN
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor usually seen within the first and second decade. They are extremely rare in adults, constituting less than 1% of adult lung tumors. It's usually benign, but it had a tendency for local recurrence. We report a case of asymptomatic inflammatory myofibroblastic tumor of lung in a 46-year-old non-smoker woman.