Humans ; Myocarditis/diagnostic imaging* ; Magnetic Resonance Imaging ; Myocardium ; Giant Cells

Humans ; Myocarditis/diagnostic imaging* ; Magnetic Resonance Imaging ; Myocardium ; Giant Cells

Male ; Humans ; Prostate/pathology* ; Adenocarcinoma/pathology* ; Giant Cells/pathology*

Carcinoma/pathology* ; Giant Cells/pathology* ; Humans ; Osteoclasts/pathology* ; Pancreas/pathology* ; Pancreatic Neoplasms/surgery*

Cartilage and giant cell-related neoplastic lesions originating in the temporomandibular joint region have similar clinical, imaging and pathological manifestations, making the diagnosis of these disorders challenging to varying degrees. Diagnostic findings can influence treatment procedures and a definitive pathological diagnosis is important for the prognosis of these conditions. In this article, we discuss the pathological diagnosis and differentiation of four benign cartilage and giant cell related tumors and tumor-like lesions that occur in the temporomandibular joint, namely synovial chondromatosis, tumoral calcium pyrophosphate deposition disease, pigmented villonodular synovitis and chondroblastoma, taking into account their clinical features and histological manifestations, with a view to providing a basis for clinical management.
Humans ; Temporomandibular Joint/pathology* ; Chondromatosis, Synovial/pathology* ; Synovitis, Pigmented Villonodular/pathology* ; Giant Cells/pathology* ; Cartilage

Bone Neoplasms ; Giant Cell Tumors ; Giant Cells ; Granuloma, Giant Cell ; Humans

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.
Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune ; Biopsy ; Giant Cells ; Hepatitis ; Hepatocytes ; Humans ; Infant ; Inflammation ; Jaundice ; Liver ; Liver Diseases ; Male ; Pallor ; Physical Examination ; Rare Diseases ; Recurrence ; Rituximab ; Splenomegaly

Most of temporal arteritis occurs in the older patient over 50 years old, and the histopathologic finding shows a granulomatous inflammation, so this called giant cell arteritis. However, the young patients also present with a nodular lesion in their temple, and juvenile temporal arteritis (JTA) should be considered as one of the differential diagnosis, although it is very rare. For both diagnosis and treatment of JTA, excisional biopsy is essential. The pathologic finding of the temporal artery shows panarteritis with lymphoeosinophilic infiltrates, but no giant cell or granulomatous lesion. JTA is a localized disease with low level of systemic inflammatory marker, so the symptom is usually relieved by excision of affected lesion. Peripheral blood eosinophilia present in some cases of JTA, but its relation with clinical course and prognosis is not yet been known. Herein, we report the case of a 24-year-old man diagnosed with concurrent JTA and hypereosinophilic syndrome. We also reviewed the literature of JTA focusing on the impact of combined peripheral eosinophilia on the course of the disease. Combined peripheral eosinophilia may increase the risk of recurrence of JTA after local treatment such as excision only.
Biopsy ; Diagnosis ; Diagnosis, Differential ; Eosinophilia ; Giant Cell Arteritis ; Giant Cells ; Humans ; Hypereosinophilic Syndrome ; Inflammation ; Prognosis ; Recurrence ; Temporal Arteries ; Young Adult

Benign recurrent intrahepatic cholestasis (BRIC), a rare cause of cholestasis, is characterized by recurrent episodes of cholestasis without permanent liver damage. BRIC type 2 (BRIC2) is an autosomal recessive disorder caused by ABCB11 mutations. A 6-year-old girl had recurrent episodes of jaundice. At two months of age, jaundice and hepatosplenomegaly developed. Liver function tests showed cholestatic hepatitis. A liver biopsy revealed diffuse giant cell transformation, bile duct paucity, intracytoplasmic cholestasis, and periportal fibrosis. An ABCB11 gene study revealed novel compound heterozygous mutations, including c.2075+3A>G in IVS17 and p.R1221K. Liver function test results were normal at 12 months of age. At six years of age, steatorrhea, jaundice, and pruritus developed. Liver function tests improved following administration of phenylbutyrate and rifampicin. Her younger brother developed jaundice at two months of age and his genetic tests revealed the same mutations as his sister. This is the first report of BRIC2 confirmed by ABCB11 mutations in Korean siblings.
Bile Ducts ; Biopsy ; Child ; Cholestasis ; Cholestasis, Intrahepatic ; Female ; Fibrosis ; Giant Cells ; Hepatitis ; Humans ; Jaundice ; Liver ; Liver Function Tests ; Pruritus ; Rifampin ; Siblings ; Steatorrhea

Cheilitis granulomatosa (CG) is a subset of orofacial granulomatosis (OFG) and considered to be a monosymptomatic form of Melkersson-Rosenthal syndrome (MRS), which is characterized by the triad of chronic lip swelling, facial paralysis, and fissured tongue. The labial swelling is thought to be associated with an orofacial swelling, which affects the chin, cheeks, and oral mucosa. Histologically, it is distinguished by noncaseating granulomas consisting of lymphohistiocytes and giant cells. Although systemic steroids with or without intralesional triamcinolone injections are the mainstay of treatment, and various agents have been proposed for this rare disease, no successful treatment modality has been reported in the literature yet. Herein, we present our experience with three different CG cases, which showed a varied level of positive response to the combination therapy.
Cheek ; Cheilitis ; Chin ; Facial Paralysis ; Giant Cells ; Granuloma ; Granulomatosis, Orofacial ; Lip ; Melkersson-Rosenthal Syndrome ; Mouth Mucosa ; Rare Diseases ; Steroids ; Tongue, Fissured ; Triamcinolone