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*

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

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

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

Necrobiotic xanthogranulomatous reaction is a multiorgan, non-Langerhans cell histiocytosis with an unknown etiology. Occurrence in the salivary gland is extremely rare. We recently identified a case of necrobiotic xanthogranulomatous sialadenitis in a 73-year-old Korean woman who presented with a painless palpable lesion in the chin. There was no accompanying cutaneous lesion. Partial resection and subsequent wide excision with neck dissection were performed. Pathological examination showed a severe inflammatory lesion that included foamy macrophages centrally admixed with neutrophils, eosinophils, lymphocytes, plasma cells, and scattered giant cells, as well as necrobiosis. During the 12-month postoperative period, no grossly remarkable change in size was noted. Necrobiotic xanthogranulomatous inflammation may be preceded by or combined with hematologic malignancy. Although rare, clinicians and radiologists should be aware that an adhesive necrobiotic xanthogranuloma in the salivary gland may present with a mass-like lesion. Further evaluation for hematologic disease and close follow-up are needed when a pathologic diagnosis is made.
Adhesives ; Aged ; Chin ; Diagnosis ; Eosinophils ; Female ; Follow-Up Studies ; Giant Cells ; Hematologic Diseases ; Hematologic Neoplasms ; Histiocytosis ; Humans ; Inflammation ; Lymphocytes ; Macrophages ; Neck Dissection ; Necrobiotic Disorders ; Necrobiotic Xanthogranuloma ; Neutrophils ; Plasma Cells ; Postoperative Period ; Salivary Glands ; Sialadenitis ; Skin ; Submandibular Gland

BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomal recessive liver diseases that present as neonatal cholestasis. Little is known of this disease in Korea. METHODS: The records of five patients histologically diagnosed with PFIC, one with PFIC1 and four with PFIC2, by liver biopsy or transplant were reviewed, and ATP8B1 and ABCB11 mutation status was analyzed by direct DNA sequencing. Clinicopathological characteristics were correlated with genetic mutations. RESULTS: The first symptom in all patients was jaundice. Histologically, lobular cholestasis with bile plugs was the main finding in all patients, whereas diffuse or periportal cholestasis was identified only in patients with PFIC2. Giant cells and ballooning of hepatocytes were observed in three and three patients with PFIC2, respectively, but not in the patient with PFIC1. Immunostaining showed total loss of bile salt export pump in two patients with PFIC2 and focal loss in two. Lobular and portal based fibrosis were more advanced in PFIC2 than in PFIC1. ATP8B1 and ABCB11 mutations were identified in one PFIC1 and two PFIC2 patients, respectively. One PFIC1 and three PFIC2 patients underwent liver transplantation (LT). At age 7 months, one PFIC2 patient was diagnosed with concurrent hepatocellular carcinoma and infantile hemangioma in an explanted liver. The patient with PFIC1 developed steatohepatitis after LT. One patient showed recurrence of PFIC2 after 10 years and underwent LT. CONCLUSIONS: PFIC is not rare in patients with neonatal cholestasis of unknown origin. Proper clinicopathologic correlation and genetic testing can enable early detection and management.
Bile ; Biopsy ; Carcinoma, Hepatocellular ; Cholestasis ; Cholestasis, Intrahepatic ; Fatty Liver ; Fibrosis ; Genetic Testing ; Giant Cells ; Hemangioma ; Hepatocytes ; Humans ; Jaundice ; Korea ; Liver ; Liver Diseases ; Liver Transplantation ; Recurrence ; Sequence Analysis, DNA

Giant cell reparative granuloma (GCRG) is a type of non-neoplastic lesion that can be rarely found in clinical practices. Due to the lack of specificity in symptoms, signs and auxiliary examinations, it is likely to be misdiagnosed, and thereby affecting the treatment and prognosis. In July 2018, a GCRG patient who was described with "4 years of hearing loss in the left ear, accompanied by 2 months of preauricular swelling" as the first symptom was admitted in our hospital. Both the HRCT and MRI scans for the temporal bone suggested the presence of tumor at the left lateral skull base, but the nature still needed further examination. Intraoperatively, the tumor was completely removed and repaired locally. Pathological examination confirmed the symptoms as GCRG. Immunohistochemistry showed the expression of CD68 and CD163 in the tumor cells. Postoperatively, the patient recovered well without complications, and had the stitches removed before being discharged on schedule.
Bone Neoplasms ; Giant Cell Tumors ; Giant Cells ; Granuloma, Giant Cell ; Humans ; Temporal Bone