ABSTRACT
Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytosis that usually presents with lymphadenopathy. Although isolated involvement of the CNS was considered to be uncommon, numerous cases have been reported in recent years. For RDD of the CNS, the treatment consists, in general, of surgery. In cases of partial resection or relapse, chemotherapy regimens, corticosteroids, and/or radiotherapy have yielded negative results. The authors describe the case of a 57-year-old man with a history of chronic Q fever who presented with aphasia and partial seizure. Computed tomography of the brain revealed a left frontotemporal lesion that was suggestive of a meningioma. The lesion was partially resected and histopathological evaluation revealed the presence of RDD. Nineteen months later, a Jacksonian seizure prompted MRI evaluation, which disclosed a local recurrence of the tumor. Computed tomography and FDG-PET demonstrated that the RDD involved no other site, but the presence of ileitis, noted on ileoscopy, led to the diagnosis of Crohn disease. Treatment with the purine analog azathioprine was initiated, leading to an objective and sustained response in both the RDD tumor and ileitis over 35 months of follow-up. This case report highlights the potential use of a purine analog in cases of relapsing RDD of the CNS and a possible common defect of macrophage regulation in RDD, Crohn disease, and Q fever.
Subject(s)
Azathioprine/therapeutic use , Central Nervous System Diseases/diagnosis , Central Nervous System Diseases/drug therapy , Histiocytosis, Sinus/diagnosis , Histiocytosis, Sinus/drug therapy , Immunosuppressive Agents/therapeutic use , Central Nervous System Diseases/epidemiology , Comorbidity , Crohn Disease/complications , Crohn Disease/diagnosis , Follow-Up Studies , Histiocytosis, Sinus/epidemiology , Humans , Ileitis/drug therapy , Ileitis/etiology , Male , Middle Aged , Positron-Emission Tomography , Recurrence , Remission Induction , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Peribiliary cysts are common in patients with chronic liver disease. Ambiguous imaging features and association with cirrhosis-induced hyperbilirubinemia may lead to misdiagnose an obstructive jaundice. Magnetic resonance cholangiopancreatography (MRCP) may be a useful sequence by showing small cystic structures with a specific periportal distribution on both sides of the portal veins, which do not communicate with the biliary ducts. These abnormalities may be recognized in order to avoid unnecessary endoscopic retrograde cholangiography.
Subject(s)
Bile Duct Diseases/diagnosis , Bile Ducts, Intrahepatic , Bile Duct Diseases/complications , Diagnostic Errors , Dilatation, Pathologic/complications , Dilatation, Pathologic/diagnosis , Humans , Liver Cirrhosis/complications , Male , Middle AgedABSTRACT
Abdominal wall endometriosis is unusual and mostly occurs in scars following Cesarean section. Although malignant transformation is rare, it must be recognized in order to benefit from radical resection. We report a very rare case of mixed endometrioid and serous carcinoma developing in a Cesarean section endometriosis scar and the way we managed it using surgery and chemotherapy. 18-FDG PET-CT imaging was performed to correctly stage the disease.
Subject(s)
Abdominal Neoplasms/diagnosis , Abdominal Wall/pathology , Cystadenocarcinoma, Serous/diagnosis , Endometrial Neoplasms/diagnosis , Endometriosis/diagnosis , Magnetic Resonance Imaging , Abdominal Neoplasms/pathology , Abdominal Neoplasms/surgery , Cesarean Section , Cicatrix/pathology , Cicatrix/surgery , Cystadenocarcinoma, Serous/pathology , Cystadenocarcinoma, Serous/surgery , Diagnosis, Differential , Endometrial Neoplasms/pathology , Endometrial Neoplasms/surgery , Endometriosis/pathology , Endometriosis/surgery , Female , Humans , Laparoscopy , Middle Aged , Surgical MeshABSTRACT
Hepatic necrosis after ethanol ablation for hepatocellular carcinoma (HCC), although rare, is well known and described, particularly in patients with chronic liver disease. The present report describes a rare case of massive hepatic necrosis with partial gastric, splenic, and pancreatic infarctions after local treatment of liver HCC with ethanol ablation and discusses the reasons for this complication. With the increasing use of percutaneous techniques to treat liver tumors, it is imperative for the interventional radiologist to be aware of the potential vascular complications of these techniques. An appreciation of vascular anatomy via multidetector computed tomography (CT) and/or magnetic resonance (MR) angiography is important when planning image-guided interventions.
Subject(s)
Carcinoma, Hepatocellular/therapy , Chemical and Drug Induced Liver Injury/etiology , Ethanol/adverse effects , Infarction/chemically induced , Liver Neoplasms/therapy , Pancreas/blood supply , Spleen/blood supply , Stomach/blood supply , Aged , Antineoplastic Agents/administration & dosage , Antineoplastic Agents/adverse effects , Carcinoma, Hepatocellular/blood supply , Carcinoma, Hepatocellular/diagnostic imaging , Chemical and Drug Induced Liver Injury/diagnostic imaging , Chemical and Drug Induced Liver Injury/therapy , Ethanol/administration & dosage , Humans , Infarction/diagnostic imaging , Infarction/therapy , Injections, Intralesional , Liver Neoplasms/blood supply , Liver Neoplasms/diagnostic imaging , Male , Necrosis , Severity of Illness Index , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Epithelioid hemangioendothelioma of the liver is a very rare vascular tumour in children with intermediate malignant potential. We present a case in which the typical imaging appearances of coalescent peripheral hepatic masses with capsular retraction contributed to the diagnosis. A positron emission tomography-CT (PET-CT) procedure was performed in staging the disease with a strong suspicion of coeliac nodal involvement confirmed after laparotomy and histological analysis. Our case is unique because of the rarity of the disease, the young age of the child, and proven nodal metastases at initial diagnosis. The use of PET-CT allows better staging at initial diagnosis and thus better management with improved follow-up in these patients.
