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

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 rare benign bone disease of unknown causes. Trauma is a suspected cause or contributor to the onset of GCRG, of which only a few cases have been reported worldwide. We report a case of temporal bone GCRG in a 4-month-old male newborn, who was born at full term via spontaneous vaginal delivery with recent presentation of right facial palsy. He had a right temporo-occipital craniectomy due to GCRG 1 month earlier. There had been no history of trauma. During the workup of the facial palsy complaint, we identified a lobulated mass in the right temporal bone by computed tomography and magnetic resonance imaging. The mass was mainly in the petrous portion of the temporal bone with extracranial extension through the occipital bone. This was consistent with a GCRG recurrence. The mass was removed via the transcrusal approach, and the final histopathology report confirmed it as recurrent GCRG.
Bone Diseases ; Bone Neoplasms ; Facial Paralysis ; Giant Cells ; Granuloma ; Humans ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Male ; Occipital Bone ; Recurrence ; Temporal Bone