Neuro-Behcet disease mimicking brain tumor: A case report.
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ABSTRACT:
SUBMITTER: Tramontini PL
PROVIDER: S-EPMC5473079 | biostudies-other | 2017
REPOSITORIES: biostudies-other
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Project description:The authors report a case of immunoglobulin G4-related disease (IgG4-RD) presenting as a paraspinal, epidural mass. This disease encompasses a host of autoimmune conditions that were previously thought to be separate entities. It is characterized by fibrosis, mediated by the aberrant proliferation and tissue invasion of IgG4-positive plasma cells, which can occur in any organ. As with other autoimmune conditions, it tends to be responsive to steroids and other immunosuppressants. It can rarely present as a tumefactive lesion of the central nervous system, creating the potential for misdiagnosis (given its similar radiological appearance to malignancy) and mistreatment. In 2015, a panel of experts convened to set forth guidelines for the diagnosis and treatment of IgG4-RD. In the case presented here, the patient initially presented with pain and weakness in the left upper extremity. Initial neuroimages revealed a contrast-enhancing mass extending from C-4 to T-1, invading the epidural spinal canal, encasing the exiting nerve roots, infiltrating the paraspinal musculature, and surrounding the left vertebral artery. A PET scan confirmed the mass was hypermetabolic, but results of fine-needle aspiration and CT-guided biopsy were inconclusive. Open biopsy yielded fibrotic tissue that met the pathological criteria for IgG4-RD: lymphoplasmacytic infiltrate, fibrosis in a storiform pattern, and obliterative phlebitis. The patient was treated with 2 doses of 4 mg of dexamethasone (Decadron) and then 50 mg of prednisone per day. Within 2 weeks, the mass was radiologically shown to have drastically decreased in size. The prednisone dose was decreased to 40 mg per day, and 100 mg of azathioprine per day was added. The patient continued to improve and the mass continued to decrease over the next 6 months. Currently, she has been weaned from all steroids and will be maintained on a daily dose of 100 mg of azathioprine.
| S-EPMC5548420 | biostudies-other
Project description:BackgroundAngioleiomyoma is a benign lesion of mesenchymal origin, which always occurs in the uterine system. Pathologically, angioleiomyoma is usually composed of well-differentiated smooth muscle cells with few mitotic features. However, primary intracranial angioleiomyoma represents an exceedingly rare tumor, since the first case reported in 1994.Case descriptionHere, we reported a case of primary intracranial angioleiomyoma, which mimicking meningioma in pre-operative images. The patient was a 42-year-old male, presented with dizziness and unsteady walking for about 6 months, without symptoms of cranial nerve deficit. Head computer tomography scan showed a well-defined lesion adjacent to right brain stem with high intensity. Contrast brain magnetic resonance imaging (MRI) scan exhibited an extra-axial mass with homogeneous enhancement located at the right pontine, presented as meningioma features; however, other tumors including lymphoma should be differentiated as well. The patient underwent sub-temporal craniotomy for the tumor resection. Histological analysis confirmed the diagnosis of angioleiomyoma. Follow-up brain MRI scan (6 months after surgery) showed total resection of the lesion without residual.ConclusionsIn summary, primary intracranial angioleiomyoma is rare. Thus, diagnosis and differential diagnosis are important before surgical resection, which was mimicking meningioma in our case. Pathological analysis could reveal spindle shaped cells with few mitotic features, and confirm the diagnosis of angioleiomyoma. Currently, the optimal therapy for primary intracranial angioleiomyoma is surgical resection, and adjuvant radiation therapy for the residual tumor. However, long-term prognosis of the disease should be monitor in the future.
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Project description:Löffler's endocarditis (cardiac involvement in hypereosinophilic syndrome) is rare yet life-threatening if left untreated. We describe a case of hypereosinophilic syndrome presenting as a cardiac mass with an abnormal electrocardiogram. Diagnostic studies of the cardiac mass strongly suggested a malignant cardiac tumor invading the papillary muscle. Thus, excision of the cardiac mass and endomyocardial resection with mitral valve replacement were successfully performed. Pathology revealed various stages of thrombosis and irreversible myocardial damage caused by eosinophilic infiltration with no malignancy, leading to the correct diagnosis of late-stage Löffler's endocarditis. The subsequent combination of anticoagulation and corticosteroids was effective with a favorable outcome. This case highlights pitfalls in multimodality imaging of cardiac thrombus and the clinical significance of considering Löffler's endocarditis in the diagnostic work-up of a cardiac mass.
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Project description:IntroductionAn Inflammatory myofibroblastic tumor, a neoplasm of intermediate biological potential, of the small bowel mesentery, is a rare tumor, most commonly reported in but not confined to the pediatric age group.Case presentationThis case report underlines a case of a (small bowel) mesentery IMT in an adult female presenting with recurrent symptoms similar to acute appendicitis.DiscussionIt can present with symptoms similar to acute appendicitis necessitating a high index of suspicion for its prompt diagnosis. Treatment primarily includes surgical resection with recent advances in targeted therapy with tyrosine kinase inhibitors showing promising results.ConclusionIMTs can present as clinical as well as histopathological mimickers of a variety of diseases especially in the abdomen. Prompt diagnosis requires both imaging and histopathological examination.
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Project description:Behçet disease (BD) is a form of widespread vasculitis that involves both arteries and veins. Cardiac involvement in BD is exceedingly rare and can present as a form of non-bacterial- thrombotic-endocarditis (NBTE). A 54-year-old man with Behçet disease was admitted to our hospital after presenting with abdominal pain and fever. He had been recently discharged from a hospital in another country with the diagnosis of infective endocarditis of the mitral valve and mycotic embolization to the superior mesenteric artery. At presentation, oral and genital ulcers were present, raising the suspicion of a flare of BD. Transesophageal echocardiography showed a small vegetation on the anterior leaflet of the mitral valve. Blood cultures results were negative. Computed tomography of the abdomen showed extensive inferior vena cava thrombosis. The aneurysm and thrombotic phenomena were interpreted as related to BD: the vegetation on the mitral valve was diagnosed as NBTE of which BD is a recognized cause. With corticosteroid and anticoagulant therapy, the patient's symptoms steadily improved. NBTE is a rare manifestation of BD; differential diagnosis with infective endocarditis can be challenging and should be focused on identifying predisposing conditions. The mainstay of treatment in NBTE is medical therapy with anticoagulation and treatment of the underlying disease. <Learning objective: Cardiac involvement in the setting of Behçet Disease (BD) is rare and several different manifestations are described (intracardiac thrombus, non-infective endocarditis, myocarditis, pericarditis, endomyocardial fibrosis, coronary arteritis). Non-bacterial thrombotic endocarditis (NBTE) is also a rare finding which can happen in the setting of BD. Its prevalence is unknown and evidence is derived only from scarce case reports.The role of BD as a potential cause of NBTE must be acknowledged to avoid potential misdiagnosis.>.
| S-EPMC8758554 | biostudies-literature
Project description:BackgroundImmunoglobulin G4-related disease (IgG4-RD) is an autoimmune disease that can affect any organ or tissue in the body, and is characterized by intensive infiltration of IgG4-positive plasma cells, and elevated serum IgG4 levels. IgG4-RD causes renal impairment of unknown pathogenesis that may progress to kidney failure. However, few case of IgG4-RD mimicking malignant ureter tumor leading to severe hydronephrosis.Case descriptionThis report describes a 38-year-old male patient who was hospitalized for sudden waist pain. Enhanced abdominal computed tomography (CT) revealed a mass involving the right ureter. He presented to the urologist with severe right hydronephrosis. Urinalysis revealed occult blood (3+), and atypical cells were observed in urine cytology, raising the possibility of a ureteral malignancy. After that, the patient underwent diagnostic ureteroscopy instead of direct nephroureterectomy and was found not to have any malignancy. The patient received laparoscopic partial ureteral resection and anastomosis. Histologically, there were observations of IgG4-positive plasma cell infiltration exceeding 10 cells per high-power field, as well as a high ratio of IgG4-positive/IgG-positive cells exceeding 40%. And histopathology revealed ureteral IgG4-related disease, with no evidence of urothelial carcinoma.ConclusionsIgG4-RD has previously been reported in lesions involving the ureters, but misdiagnosis and subsequent radical nephroureterectomy can cause lifelong regret for the patient in having lost one side of the urinary tract. To avoid such misdiagnoses, clinicians should consider IgG4-RD as a potential condition.
| S-EPMC10789895 | biostudies-literature
Project description:IntroductionNeuroendocrine tumors (NETs) are neoplasms that arise from neuroendocrine cells that have properties of both neuronal and endocrine cells. NETs are most common in the small intestine, rectum, and the appendix and frequently termed carcinoid.Presentation of caseA 30-year-old male presented with abdominal pain and tenderness in the RLQ. Computerized tomography revealed findings consistent with acute appendicitis. The patient underwent an uneventful laparoscopic appendectomy for an acutely inflamed appendicitis. Histopathological examination, showed a 0.5 cm well-differentiated neuroendocrine tumor of the distal appendix, with clear margins. The mitotic rate was <2 mitoses/10 high power field. Following guidelines, no further procedures were performed and follow-up one week later was uneventful.DiscussionAppendectomy for the treatment of appendiceal NETs smaller than 1 cm has been recommended as the treatment of these neoplasms by the guidelines set by The North American Neuroendocrine Society (NANETS). NANETS recommends right hemicolectomy for tumors originating at the base of the appendix, for tumors >2 cm in size, if there is evidence of lymphovascular or meso-appendiceal invasion, with mesenteric lymph node metastases, or for intermediate or high-grade tumors.ConclusionWe present the case of a 30-year old male that presented with an appendiceal, well-differentiated NET that manifested as appendicitis and laparoscopic appendectomy was performed. The appendix was resected with clear margins. Given appropriate markers appendectomy can be curative.
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Project description:Introduction and importanceTextiloma is a retained surgical item such as a sponge or gauze that is unintentionally left in the surgical field after the wound closure. Here, we present the first reported surgical gauze that penetrated the intestine, made a duodenal-ceca fistula and then stuck far away in the ileum. Mechanical obstruction didn't appear clinically or even in radiological investigations because of the fistula which provided the intestinal continuity.Case presentationWe report a 34-year-old man with a previous abdominal interventions complained of cramping, frequent vomiting and presence of undigested food in stool. The frequency of the bowel movement increased recently. Endoscopies, radiological investigations and pathological findings figure out a duodenal-ceca fistula with nonspecific inflammatory tissues in the intestinal biopsy. When we performed the abdomen surgery, retained gauze in the ileum was taken out and the duodenal-ceca fistula was fixed.Clinical discussionGauze or sponge that is forgotten in the surgical field called gossypiboma, textiloma, gauzoma or cottonoid. It could present with various complaints; as an acute or chronic problem, clear or ambiguous symptoms. It could reside in a space; extend across a gap, migrate through a tissue, or even make a fistula between lumina like in our case.ConclusionTextiloma could change pre-operative diagnosis, intra-operative techniques, postoperative follow-up plan and prognosis. This is the first report proves its ability to mimic inflammatory diseases that penetrate two different lumina and perform fistula. So it should be written in the list of any differential diagnosis when the patient has a previous procedure or surgery.
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Project description:RATIONALE:Behcet disease is an autoimmune vasculitis, involving multiple systems and organs. It is often chronic and recurrent, and involves all levels of arteries and veins. PATIENT CONCERNS:A 40-year-old Chinese male with sudden vision loss in his right eye for 3 days was reported. DIAGNOSES:The patient was diagnosed as bilateral uveitis (obstructive retinal vasculitis in the right eye) and Behcet disease. INTERVENTIONS:The patient was given topical corticosteroids and compound tropicamide eye drops in our department. In addition, he was also hospitalized in the Rheumatology Department, and received systemic infusion of methylprednisolone and cyclophosphamide. OUTCOMES:Best corrected visual acuity vision acuity of the left eye was 20/20 and that of the right eye was no light perception after 2 weeks. During the telephonic follow-up after 1 year, the patient reported 2 recurrent episodes of uveitis in the left eye, which improved after treatment at the local hospital. LESSONS:It cautions ophthalmologists of the high possibility of vasculitis in young and middle-aged patients with retinal vascular occlusion. It also reminds the ophthalmologists investigate systemic diseases, and obtain detailed medical history, not just depending on specific laboratory findings. This case further suggests that Behcet disease has diverse manifestations, complex diagnosis, and severe lesions, and the recurrence rate must be controlled to minimize irreversible consequences such as sudden vision loss during recurrence.
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Project description:Neuro-Behçet's disease (NBD) is subcategorized into parenchymal-NBD (P-NBD) and non-parenchymal-NBD types. Recently, P-NBD has been further subdivided into acute P-NBD (A-P-NBD) and chronic progressive P-NBD (CP-P-NBD). Although an increasing number of studies have reported the various clinical features of A-P-NBD and CP-P-NBD over the last two decades, there was a considerable inconsistency. Two investigators systematically searched four electrical databases to detect studies that provided sufficient data to assess the specific characteristics of A-P-NBD and CP-P-NBD. All meta-analysis was carried out by employing the random-model generic inverse variance method. We included 11 reports consisted of 184 A-P-NBD patients and 114 CP-P-NBD patients. While fever (42% for A-P-NBD, 5% for CP-P-NBD, p?<?0.001, I2?=?93%) was more frequently observed in A-P-NBD cases; sphincter disturbances (9%, 34%, P?=?0.005, I2?=?87%), ataxia (16%, 57%, P?<?0.001, I2?=?92%), dementia (7%, 61%, P?<?0.001, I2?=?97%), confusion (5%, 18%, P?=?0.04, I2?=?76%), brain stem atrophy on MRI (4%, 75%, P?<?0.001, I2?=?98%), and abnormal MRI findings in cerebellum (7%, 54%, P?=?0.02, I2?=?81%) were more common in CP-P-NBD. Cerebrospinal fluid cell count (94/mm3, 11/mm3, P?=?0.009, I2?=?85%) was higher in A-P-NBD cases. We demonstrated that A-P-NBD and CP-P-NBD had clearly different clinical features and believe that these data will help future studies investigating P-NBD.
| S-EPMC5579041 | biostudies-literature