5.Diagnosis and differentiation diagnosis of primary temporomandibular joint cartilage and giant cell related tumor lesions.
Shi Lei NI ; Zhi Min LI ; Hong Chen SUN
Chinese Journal of Stomatology 2022;57(11):1097-1101
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
7.Successful Treatment of a Korean Infant with Giant Cell Hepatitis with Autoimmune Hemolytic Anemia Using Rituximab
Young Ho KIM ; Ju Whi KIM ; Eun Joo LEE ; Gyeong Hoon KANG ; Hyoung Jin KANG ; Jin Soo MOON ; Jae Sung KO
Pediatric Gastroenterology, Hepatology & Nutrition 2020;23(2):180-187
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
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Anemia, Hemolytic, Autoimmune
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Biopsy
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Giant Cells
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Hepatitis
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Hepatocytes
;
Humans
;
Infant
;
Inflammation
;
Jaundice
;
Liver
;
Liver Diseases
;
Male
;
Pallor
;
Physical Examination
;
Rare Diseases
;
Recurrence
;
Rituximab
;
Splenomegaly
8.Benign Recurrent Intrahepatic Cholestasis Type 2 in Siblings with Novel ABCB11 Mutations
Min Ji SOHN ; Min Hyung WOO ; Moon Woo SEONG ; Sung Sup PARK ; Gyeong Hoon KANG ; Jin Soo MOON ; Jae Sung KO
Pediatric Gastroenterology, Hepatology & Nutrition 2019;22(2):201-206
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
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Biopsy
;
Child
;
Cholestasis
;
Cholestasis, Intrahepatic
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Female
;
Fibrosis
;
Giant Cells
;
Hepatitis
;
Humans
;
Jaundice
;
Liver
;
Liver Function Tests
;
Pruritus
;
Rifampin
;
Siblings
;
Steatorrhea
9.Annular Elastolytic Giant Cell Granuloma: Chronic Heat Exposure, an Underestimated Factor
Ga Ram AHN ; Guk Jin JEONG ; Hye Sung HAN ; Hyun Jung KWON ; Kapsok LI ; Seong Jun SEO
Annals of Dermatology 2019;31(Suppl):S64-S65
No abstract available.
Giant Cells
;
Granuloma, Giant Cell
;
Hot Temperature
10.Evaluation of Local Recurrence in Giant-Cell Tumor of Bone Treated by Neoadjuvant Denosumab
Pramod Shekarappa CHINDER ; Suraj HINDISKERE ; Srinath DODDARANGAPPA ; Utkarsh PAL
Clinics in Orthopedic Surgery 2019;11(3):352-360
BACKGROUND: Giant-cell tumor of bone (GCTB) is a locally aggressive primary benign tumor presenting as an expansile osteolytic lesion affecting the epiphysis of long bones. Denosumab halts the osteolysis by giant cells thereby downstaging the tumor, helping in performing less morbid procedures to remove the tumor. Our aim was to report the incidence of local recurrence (LR) in patients operated following neoadjuvant denosumab, to investigate factors associated with LR following extended curettage for GCTB, and to compare the postoperative functional and oncological outcome of patients operated with and without neoadjuvant denosumab. METHODS: A total of 123 patients with a mean age of 29.6 years undergoing extended curettage for GCTB were retrospectively divided into group 1 receiving neoadjuvant denosumab and group 2 operated without denosumab. The mean follow-up period was 35 months. The perioperative characteristics and outcome were compared between the two groups and the factors for LR of GCTB were analyzed. RESULTS: The incidence of LR among patients operated after neoadjuvant denosumab therapy was 42.8% and was significantly high compared to that in patients without denosumab (p < 0.001). On multivariate logistic regression analysis, use of denosumab as a neoadjuvant was the only factor independently associated with LR following surgery (p = 0.002). Patients treated with denosumab had a lower LR-free survival rate (log-rank, p = 0.018). CONCLUSIONS: Denosumab was independently associated with increased LR following surgery for GCTB. Denosumab has to be used cautiously in patients in whom the burden of downstaging the disease outweighs the possible chance of LR.
Curettage
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Denosumab
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Epiphyses
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Follow-Up Studies
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Giant Cell Tumors
;
Giant Cells
;
Humans
;
Incidence
;
Logistic Models
;
Osteolysis
;
Recurrence
;
Retrospective Studies
;
Survival Rate

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