Agenesis of dorsal pancreas (ADP) is an extremely rare congenital anomaly defined as an absence of the dorsal ductal system resulting from failure in the embryologic development of the pancreatic dorsal bud. Most of ADP patients are asymptomatic but some of them suffer recurrent pancreatitis and diabetes. Few number of pancreatic adenocarcinoma in association with ADP has been published previously in other countries. There was no such case reported in Korea. We report a case diagnosed as pancreatic adenocarcinoma with ADP.
Adenocarcinoma* ; Adenosine Diphosphate ; Adult* ; Humans ; Korea ; Pancreas* ; Pancreatic Ducts* ; Pancreatitis

Lymphocytic interstitial pneumonia (LIP) is a rare benign lymphoproliferative disorder characterized by diffuse infiltration of the pulmonary parenchymal interstitium by polyclonal lymphocytes and plasma cells. LIP has been associated with a variety of clinical conditions; such as connective tissue disorders and other immune system abnormalities. Treatment usually involves administration of corticosteroids and other immunosuppressants. We report on a 38-year-old female patient who complained of shortness of breath, dry mouth, and dry eyes for more than 1 month, and was positive for Raynaud's phenomenon. Based on surgical biopsy, she was diagnosed as having LIP accompanied by Sjögren's syndrome. The patient was treated with high-dose steroids followed by maintenance therapy for approximately 2 years, and her condition improved.
Adrenal Cortex Hormones ; Adult ; Biopsy ; Connective Tissue ; Dyspnea ; Female ; Humans ; Immune System ; Immunosuppressive Agents ; Lip ; Lung Diseases, Interstitial* ; Lymphocytes ; Lymphoproliferative Disorders ; Mouth ; Plasma Cells ; Steroids

IgG4-related disease is an immune-mediated fibro-inflammatory disease, characterized by lymphoplasmacytic infiltration composed of IgG4-positive plasma cells of various organs with elevated circulating levels of IgG4. This disease is now reported with increasing frequency and usually affects middle-aged men. Massive pleural effusion in children is an uncommon feature in IgG4-related disease. Here, we report a case of a 16-year-old male patient with extensive IgG4-related disease presenting with massive pleural effusion, mediastinal mass, and mesenteric lymphadenopathy.
Adolescent* ; Child ; Humans ; Immunoglobulin G ; Lymphatic Diseases* ; Male* ; Mediastinum ; Plasma Cells ; Pleural Effusion*