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Erdheim-Chester disease (ECD) is a rare, multisystemic, inflammatory, non-Langerhans cell histiocytic neoplasm. The discovery of recurrent and somatic mutations in the mitogen-activated protein kinase signaling pathway, most commonly BRAFV600E, has led to a reclassification of ECD from an inflammatory disorder to a neoplastic process. It is now included in the revised 2016 World Health Organization classification of hematopoietic tumors and in the Langerhans group in the revised 2016 Histiocytosis Classification of the Histiocyte Society. When symptomatic, ECD most commonly manifests with bone pain and fatigue. Also, neurologic manifestations, central diabetes insipidus, exophthalmos, and periorbital xanthelasma-like lesions are frequently encountered. Pathologic findings may vary depending on the site of biopsy and may display a spectrum of features. Thus, due to the diverse clinical presentation and variable histologic findings, imaging can often show the first sign of the disease. Radiologic findings are, however, interpreted in conjunction with clinical and histologic findings to establish the diagnosis of ECD. From providing classic findings that facilitate diagnosis to helping radiologists determine the extent of disease and predicting a prognosis, the role of radiology in ECD has evolved with the understanding of the disease itself. Insights into the molecular pathogenesis and the development of targeted therapeutic agents along with approval of vemurafenib and cobimetinib have necessitated revision of the guidelines for the management of ECD. The authors discuss various radiologic findings of ECD and differential diagnoses by using an organ system-based approach and briefly describe the revised consensus recommendations for evaluation, diagnosis, and treatment based on the International Medical Symposia on ECD from a radiologist's perspective. ©RSNA, 2024 Supplemental material is available for this article. The full digital presentation is available online.
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Erdheim-Chester Disease , Erdheim-Chester Disease/diagnostic imaging , Humans , Diagnosis, DifferentialABSTRACT
OBJECTIVE: Characterize the CT findings of abdominopelvic Castleman disease, including a new observation involving the perinodal fat. METHODS: Multi-centre search at 5 institutions yielded 76 adults (mean age, 42.1 ± 14.3 years; 38 women/38 men) meeting inclusion criteria of histopathologically proven Castleman disease with nodal involvement at abdominopelvic CT. Retrospective review of the dominant nodal mass was assessed for size, attenuation, and presence of calcification, and for prominence and soft-tissue infiltration of the perinodal fat. Hypervascular nodal enhancement was based on both subjective and objective comparison with aortic blood pool attenuation. RESULTS: Abdominal involvement was unicentric in 48.7% (37/76) and multicentric in 51.3% (39/76), including 31 cases with extra-abdominal involvement. Histopathologic subtypes included hyaline vascular variant (HVV), plasma cell variant (PCV), mixed HVV/PCV, and HHV-8 variant in 39, 25, 3 and 9 cases, respectively. The dominant nodal mass measured 4.4 ± 1.9 cm and 3.2 ± 1.7 cm in mean long- and short-axis, respectively, and appeared hypervascular in 58.6% (41/70 with IV contrast). Internal calcification was seen in 22.4% (17/76). Infiltration of the perinodal fat, with or without hypertrophy, was present in 56.6% (43/76), more frequent with hypervascular vs non-hypervascular nodal masses (80.5% vs 20.7%; P < .001). Among HVV cases, 76.9% were unicentric, 71.1% appeared hypervascular, and 69.2% demonstrated perinodal fat infiltration. CONCLUSION: Hypervascular nodal masses demonstrating prominence and infiltration of perinodal fat at CT can suggest the specific diagnosis of Castleman disease, especially the HVV. ADVANCES IN KNOWLEDGE: Abdominopelvic nodal masses that demonstrate hypervascular enhancement and prominent infiltration of the perinodal fat at CT can suggest the diagnosis of Castleman disease, but nonetheless requires tissue sampling.
Subject(s)
Castleman Disease , Tomography, X-Ray Computed , Humans , Castleman Disease/diagnostic imaging , Castleman Disease/pathology , Female , Adult , Male , Retrospective Studies , Tomography, X-Ray Computed/methods , Middle Aged , Lymph Nodes/diagnostic imaging , Lymph Nodes/pathology , Radiography, Abdominal/methods , Pelvis/diagnostic imaging , AgedABSTRACT
As the use of cross-sectional abdominal and pelvic imaging has increased exponentially in the past several decades, incidental musculoskeletal findings have become commonplace. These are often unrelated to the indication for the examination and are frequently referred to as the "radiologist's blind spot" on these studies. The differential diagnosis for abnormalities of the paraspinal and pelvic musculature is, in many cases, quite different from the anterior abdominal wall muscles. Furthermore, due to their relatively deep location, pathology involving the former muscle groups is more likely to be clinically occult, often presenting only incidentally when the patient undergoes cross-sectional imaging. Effective treatment of diseases of these muscles is dependent on adherence to a diverse set of diagnostic and treatment algorithms. The purpose of this review article is to familiarize the radiologist with the unique pathology of these often-overlooked muscles of the abdomen and pelvis.
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BACKGROUND: Spontaneous rupture of hepatocellular carcinoma (rHCC) is a life-threatening complication that occurs in 3 % to 15 % of patients with hepatocellular carcinoma (HCC). This review aimed to discuss the most recent updates in the epidemiology, pathophysiology, risk factors, diagnosis as well as presentation, management, and prognostic factors of rHCC. METHODS: A comprehensive systematic review was conducted using Medline/PubMed and Web of Science databases with the end of search date being December 1, 2023 regarding rHCC diagnosis, imaging, and management. RESULTS: Achieving adequate hemostasis and stabilization of the patient remains the primary objective in the management of patients with rHCC. In earlier studies, the mortality rate in the acute phase of rHCC was reported to be 25 % to 75 %. However, more recent studies have demonstrated that transcatheter arterial embolization (TAE)/transcatheter arterial chemoembolization (TACE) followed by elective hepatectomy in select patients may offer improved survival benefits and decrease perioperative complications compared with TAE/TACE alone or emergent/1-stage hepatectomy. CONCLUSION: Although the prognosis for rHCC remains the worst among causes of death related to HCC, more recent studies have demonstrated that improved short- and long-term patient outcomes may be achieved through active surveillance efforts for HCC combined with advanced multimodal diagnostic tools and multidisciplinary management strategies.
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Carcinoma, Hepatocellular , Hepatectomy , Liver Neoplasms , Humans , Carcinoma, Hepatocellular/therapy , Carcinoma, Hepatocellular/diagnosis , Liver Neoplasms/therapy , Liver Neoplasms/diagnosis , Rupture, Spontaneous/therapy , Prognosis , Risk Factors , Chemoembolization, Therapeutic , Embolization, Therapeutic/methodsABSTRACT
OBJECTIVES: Fluoroscopy has significantly improved lead placement and decreased surgical time for implantable sacral neuromodulation (SNM). There is a paucity of data regarding radiation and safety of fluoroscopy during SNM procedures. Our study aims to characterize fluoroscopy time and dose used during SNM surgery across multiple institutions and assess for predictors of increased fluoroscopy time and radiation dose. METHODS: Electronic medical records were queried for SNM procedures (Stage 1 and full implant) from 2016 to 2021 at four academic institutions. Demographic, clinical, and intraoperative data were collected, including fluoroscopy time and radiation dose in milligray (mGy). The data were entered into a centralized REDCap database. Univariate and multivariate analysis were performed to assess for predictive factors using STATA/BE 17.0. RESULTS: A total of 664 procedures were performed across four institutions. Of these, 363 (54.6%) procedures had complete fluoroscopy details recorded. Mean surgical time was 58.8 min. Of all procedures, 79.6% were performed by Female Pelvic Medicine and Reconstructive Surgery specialists. There was significant variability in fluoroscopy time and dose based on surgical specialty and institution. Most surgeons (76.4%) were considered "low volume" implanters. In a multivariate analysis, bilateral finder needle testing, surgical indication, surgeon volume, and institution significantly predicted increased fluoroscopy time and radiation dose (p < 0.05). CONCLUSIONS: There is significant variability in fluoroscopy time and radiation dose utilized during SNM procedures, with differences across institutions, surgeons, and subspecialties. Increased radiation exposure can have harmful impacts on the surgical team and patient. These findings demonstrate the need for standardized fluoroscopy use during SNM procedures.
Subject(s)
Electric Stimulation Therapy , Radiation Exposure , Surgeons , Urinary Bladder, Overactive , Humans , Female , Urinary Bladder, Overactive/therapy , Electric Stimulation Therapy/methods , Sacrum , Radiation Exposure/adverse effectsABSTRACT
While a plethora of articles discuss management of deep venous thromboses in extremities, there is a relative scarcity of literature comprehensively describing intra-abdominal venous thromboses, and their management. Intra-abdominal venous thromboses include iliocaval venous obstruction (ICVO), hepatic venous thrombosis (HVT), portal venous thrombosis (PVT), renal vein thrombosis (RVT), splenic vein thrombosis (SVT), and gonadal vein thrombosis (GVT); each of which provides unique microenvironmental challenges to management. Doppler ultrasound is the first line imaging modality for diagnosis, and computed tomography and magnetic resonance imaging can help define the extent of thrombus burden and aid with interventional planning. Systemic anticoagulation remains the common medical treatment for intra-abdominal venous thrombosis, however, catheter directed thrombolysis and thrombectomy show positive outcomes in ICVO, HVT, PVT, and RVT, with transjugular intrahepatic portosystemic shunt (TIPS) creation especially beneficial in HVT and PVT. In this review article, we describe pathophysiology, clinical features, imaging findings, and current management options for intra-abdominal venous thromboses.
Subject(s)
Venous Thrombosis , Humans , Venous Thrombosis/diagnostic imaging , Venous Thrombosis/therapy , Portal Vein/diagnostic imaging , Thrombectomy/methods , Abdomen/diagnostic imaging , Abdomen/blood supply , Anticoagulants/therapeutic use , Thrombolytic Therapy/methods , Portasystemic Shunt, Transjugular Intrahepatic/methodsABSTRACT
An otherwise healthy, 34-year-old man presented to the emergency department with abdominal pain, nausea, and vomiting, which began a day after he ingested a banana-stuffed condom. A contrast-enhanced computed tomography (CT) abdomen and pelvis revealed a dilated small bowel with a transition point, findings consistent with small bowel obstruction. Abdominopelvic ascites was also noted on imaging, which was concerning for bowel distress. He was taken to the operating room for an exploratory laparotomy, which revealed a high-grade obstruction by a foreign body, a banana stuffed in a condom, in the mid-jejunum. An enterotomy was performed to relieve the obstruction. The postoperative course was uneventful, and he was discharged three days later.
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Segmental testicular infarct is a rare clinical entity and can be a diagnostic challenge. Although cases are often idiopathic, underlying etiologies can include testicular torsion, epididymo-orchitis, trauma, vasculitis, and hypercoagulable states. Once suspected, an underlying testicular neoplasm should be excluded. We present a case of a 43-year-old male who developed acute onset left sided scrotal pain. A diagnostic scrotal ultrasound showed a focal, heterogeneous region in left testicle with absent focal Doppler signal, concerning for a segmental testicular infarction. There was no history of trauma, urinary symptoms, sexually transmitted diseases, or constitutional symptoms. Work up for associated underlying etiologies was negative. A computed tomography angiogram scan of the abdomen and pelvis revealed an incidental left testicular artery aneurysm. The patient's consulting multidisciplinary care teams included urology and vascular surgery. Urology deemed surgical intervention inappropriate for the segmental testicular infarct, and vascular surgery elected not to intervene on the testicular artery aneurysm due to risk of completing testicular infarct and damaging blood supply to the testis. The patient was discharged after achieving adequate pain control, and completion of inpatient work up. No underlying malignancy was diagnosed on follow up, and pain symptoms resolved. To the authors' knowledge, no literature exists describing the concurrent incidence of a segmental testicular infarct and an ipsilateral testicular artery aneurysm. In this report, we aim to further describe both diagnoses, and explore the association between the 2 entities.
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A 52-year-old female presented with epigastric pain, yellowing of the sclera, and vomiting for three weeks. Laboratory investigations revealed markedly elevated serum bilirubin and alkaline phosphatase levels, accompanied by a modest rise in transaminases. A clinical diagnosis of obstructive jaundice was established. Ultrasound of the abdomen depicted a mass in the region of the head of the pancreas. Contrast-enhanced computed tomography (CECT) of the abdomen revealed an infiltrative gastric mass spreading across the gastroduodenal junction and involving the ampulla of Vater. Owing to comorbid conditions and widespread lymphadenopathy, a palliative gastrojejunostomy with excision biopsy was performed. Histopathology confirmed an undifferentiated gastric adenocarcinoma.
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Pancreatic fluid collections (PFC) are notorious for their extension beyond the normal confines of the pancreatic bed. This distribution is explained by dissection along the fascial planes in retroperitoneum due to the digestive enzymes within the PFC. In genitourinary track, PFCs have been described to involve the kidneys and the ureters. We report a case of severe acute necrotizing pancreatitis in a 28-year-old male, chronic alcoholic, who on readmission developed features of cystitis. The urine was turbid but did not show significant bacteriuria. Close location of the PFC near the urinary bladder (UB) prompted evaluation of urinary lipase and amylase. Elevated urinary enzyme levels suggested a Pancreatico-vesical fistula, conclusive demonstration of which was established by CT cystography. Percutaneous drainage of the necrosum and stenting of pancreatic duct led to spontaneous healing of the pancreatico-vesical fistula. Our case reiterates the remarkable property of pancreatic enzymes to dissect the fascial planes which is demonstrated by decompression of PFC via UB causing spontaneous Pancreatico-vesical fistula. Further, presence of main pancreatic duct fistulization should prompt endoscopic-guided stenting to obliterate the communication with the fistula and accelerate healing.
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Patients in the intensive care units suffer from a myriad of cardiopulmonary processes for which portable chest radiography is the most utilized imaging modality. A pragmatic approach toward evaluating the nearly similar radiographic findings seen in most of the pathologies with comparative review and strong clinical acumen can help the radiologists and clinicians achieve a rapid and precise diagnosis.
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Cryptococcosis is a life-threatening mycosis typically seen in immunocompromised patients. Pulmonary cryptococcosis generally presents as multiple or solitary nodular opacities. Cryptococcal infection presenting as a destructing cavernoma (cryptococcoma) without diffuse infiltration of the lung is an extremely rare presentation, even in immunocompromised patients. This report presents a healthy, HIV negative, immunocompetent patient who presented with a large solitary lung mass provisionally diagnosed as a lung malignancy on radiological imaging that proved to be a large cryptococcoma after biopsy. The patient was treated with liposomal Amphotericin B and fluconazole, and the lesion showed regression on serial imaging. This case report thus highlights an unconventional presentation of pulmonary cryptococcosis in an immunocompetent individual.
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Genital tuberculosis (TB) is a rare, comprising <0.5% cases of extrapulmonary TB. Among cases of genitourinary TB, glandular TB is even rarer. Its a diagnosis of exclusion. Most patients present later in the course of disease due to the associated stigma in view of site of involvement and the hesitancy on the patients's part. We saw two such cases of nonhealing genital ulcers in immunocompetent males.
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BACKGROUND: The classical theory of Müllerian origin of upper vagina fails to explain complex urogenital malformations like OHVIRA syndrome; the Acien's hypothesis, however, unravels the hidden embryopathogenesis. As per Acien, Wolffian (mesonephric) ducts instead of Müllerian ducts and sinovaginal bulbs, give rise to the vagina. The new hypothesis, however, retains the concept of origin of the ureters (with ureters inducing renal development) by the former and the uterus by Müllerian ducts. Thus, a failure of development of mesonephros/mesonephric duct gives rise to absent ureters and hence absent homolateral kidney; blind ending (obstructed) ipsilateral hemivagina and cessation of support to paramesonephric ducts which leads to unfused uterus (uterus didelphys). Hence, the new hypothesis explains all components of OHVIRA syndrome. On a parallel track, unilateral anomalous development of the mesonephros in males causes atresia of the homolateral ejaculatory duct that results in obstruction of the proximally placed seminal vesicle. Besides, there is absence of the ipsilateral kidney (Zinner syndrome). CASE REPORT: In this manuscript, we describe four cases of OHVIRA syndrome. Case 1 was a 34-year-old nulligravida, married since fourteen years, who presented with a 5-month history of pelvic inflammatory disease and dyspareunia. Regular menses in the patient and azoospermia in her husband delayed the diagnosis. Case 2 was a 14-year-old girl who presented with dysmenorrhea and lower abdominal pain since a few months. Case 3 was a 27-year-old female who presented with infertility and dysmenorrhea. Case 4 was a 15-year-old female who presented with a one-year history of dysmenorrhea and cyclic pelvic pain. In all cases, one of the uterine horns revealed collection due to a hemivaginal septum and an absent ipsilateral kidney; thus, establishing the diagnosis of OHVIRA syndrome. The case 4 additionally revealed homolateral vaginal agenesis. CONCLUSIONS: On the basis of our 4 cases, we support the Acien's hypothesis of Wolffian origin of vagina which explains the development of OHVIRA syndrome. Besides, we emphasize the need to suspect this syndrome in a female with a pelvic mass and absence of homolateral kidney. Finally, we believe that OHVIRA due to its Wolffian origin is a female equivalent of Zinner syndrome in males. Therefore, we propose OSVIRA (Obstructed Seminal Vesicle and Ipsilateral Renal Agenesis) as an acronym for Zinner syndrome analogous to OHVIRA.