Paraneoplastic cerebellar degeneration (PCD) is an immune-mediated neurological disease characterized by adaptive immune response against onconeural antigens physiologically expressed in the cerebellum. It is characterized by presence of highly specific onconeural autoantibodies such as anti-Yo, anti-Hu, anti-Ri, and anti-Ma2 in the serum and cerebrospinal fluid as diagnostic biomarkers. Antiamphiphysin autoantibodyrelated paraneoplastic encephalitis is a less commonly seen autoimmune neurological disorder usually presenting as stiff person syndrome. We present an unusual case of isolated antiamphiphysin antibody-related PCD presenting as vestibulocerebellar syndrome with associated sensorineural hearing loss and sensory neuropathy. FDG PET helped in topographical localization of brain lesion along with early detection of extragonadal germ cell tumor in the retroperitoneum.
Immunoglobulin G4 (IgG4)–related disease is known to mimic multiple malignancies and always poses a diagnostic challenge. We report a case of a 20-year-old young man, who presented with unexplained recurrent episodes of fever and pain abdomen. 18F-FDG PET/CT revealed intense focal FDG avidity in the prostate. On further workup, he had an elevated serum IgG4 level, and a clinical diagnosis of immunoglobulin G4–related disease was kept. A follow-up FDG PET/CT after glucocorticoid therapy revealed resolution of FDG avidity in the prostate with fall in serum IgG4 levels, hence confirming a diagnosis of atypical immunoglobulin G4–related disease involving isolated prostate gland.
Purpose
This study was conducted to identify the role of hepatobiliary scintigraphy (HBS) in the management of biliary tract injuries.
Methods
We retrospectively studied 54 patients (28 male and 26 female patients) aged 3 to 78 years with evidence of bile leak on HBS performed between January 2015 and October 2017. Following intravenous injection of 99mTc-mebrofenin, dynamic images were acquired for 30 minutes followed by static images until 24 hours. SPECT/CT was performed in patients with suspicion of bile leak on the planar images. Patients were classified as those with free intraperitoneal or localized bile leak. Any abdominal drain output was documented, and its statistical significance was assessed using Mann-Whitney U test. Subsequent management was also documented.
Results
Of the 54 patients, 28 demonstrated free intraperitoneal bile leak and 26 localized leak on HBS. Thirty-four patients (24 with free intraperitoneal leak and 10 with localized leak) had an abdominal drain. Drain output was significantly higher in patients with free intraperitoneal leak compared with patients with localized leak (370 vs 78 mL/d per patient, P < 0.01). All patients with free intraperitoneal bile leak underwent interventional procedures such as endoscopic retrograde cholangiopancreatography and stenting or Roux-en-Y hepaticojejunostomy later on. Patients with localized bile leak were managed conservatively and were free of symptoms at 1-year follow-up.
Conclusions
Our findings indicate that, in addition to detecting biliary leak, HBS may be used to identify patients with localized bile leak (48% of patients in our study) who do not require active intervention.
Wilms’ tumor also called as nephroblastoma is commonly seen extracranial solid tumor involving kidneys in children. Rarely, Wilms’ tumor can arise from ectopic nephrogenic remnants located outside the kidneys. Extrarenal Wilms’ tumor comprises 3% of total Wilms’ tumor with its incidence even less common in adults. We report the staging and restaging fluorodeoxyglucose positron emission tomography-computed tomography findings in a case of locally advanced metastatic extrarenal adult Wilms’ tumor involving the retroperitoneum.
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