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
;
Anemia, Hemolytic, Autoimmune
;
Biopsy
;
Giant Cells
;
Hepatitis
;
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
;
Biopsy
;
Child
;
Cholestasis
;
Cholestasis, Intrahepatic
;
Female
;
Fibrosis
;
Giant Cells
;
Hepatitis
;
Humans
;
Jaundice
;
Liver
;
Liver Function Tests
;
Pruritus
;
Rifampin
;
Siblings
;
Steatorrhea
9.Surgical Treatment of Recurrent Giant Cell Tumor Occurring at the First Metatarsal
Kap Jung KIM ; Kwang Won LEE ; Jong Shin LEE
The Journal of the Korean Orthopaedic Association 2019;54(2):182-186
Giant cell tumor is a benign but locally aggressive tumor with common recurrence. Most cases occur around the knee joint. Giant cell tumor of the foot is rare and very few cases involving the first metatarsal have been reported. Its characteristics and treatment in adult patients remain unclear. This paper reports a case of recurrent giant cell tumor at the first metatarsal that was excised surgically and subsequently reconstructed with non-vascularized fibula graft.
Adult
;
Fibula
;
Foot
;
Giant Cell Tumors
;
Giant Cells
;
Humans
;
Knee Joint
;
Metatarsal Bones
;
Recurrence
;
Transplants
10.Three Cases of Recurrent Cheilitis Granulomatosa Treated with Variable Combination Therapy
Seung Pil HAM ; Hee Jae PARK ; Cheong Ha WOO ; Mira CHOI ; Hai Jin PARK
Korean Journal of Dermatology 2019;57(4):209-212
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

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