Extraadrenal pheochromocytomas developed from the paraganglia other than adrenal medulla are uncommon tumors. Such lesions have been reported that as much as 10% of retroperitoneal paragliomas arise outside the adrenal gland. But their incidence and prognosis are changing partly as a result of progression in developmental mechanism, diagnostic tools and concepts including ultrastructure, immunohistochemistry. The ultrastructure of chromaffine neoplasm from adrenal and extraadrenal tissues has been studied with chrarnaffine granule specificity and type of catecholamine content. With rare exceptions, extraadrenal chrornaffine tumor mainly has high concentration of norepinephrine which is suggested as dict relationship to numoruos electrone dense granular bodies in the cell cytoplasrn. High or intermediate level of epinephrine in tissue is associated with cytoplasmic granular bodies of much less density, Recently we experienced the exception, a case of norepinephrine secreting pheochrom-ocytoma in the organ of Zuckerkandl with epinephrine granules in cytoplasm and this discrepancy probably due to the plasticity
Adrenal Glands ; Adrenal Medulla ; Cytoplasm ; Epinephrine ; Immunohistochemistry ; Incidence ; Norepinephrine ; Para-Aortic Bodies ; Pheochromocytoma ; Plastics ; Prognosis ; Sensitivity and Specificity

Churg-Strauss syndrome is one of idiopathic, systemic necrotizing vasculitis characterized by a history of asthma or allergic rhinitis, eosinophilia of peripheral blood and variable symptoms of systemic vasculitis. There have been several recent case reports of Churg-Strauss syndrome in association with leukotriene modifier therapy when oral corticosteroids were withdrawn in asthmatic patients. We report a case of a 31-year-old man with steroid-dependent asthma who developed Churg-Strauss syndrome after 9 months of steroid withdrawal period, while he was receiving leukotriene modifier (Pranlukast).
Adrenal Cortex Hormones* ; Adult ; Asthma ; Churg-Strauss Syndrome* ; Eosinophilia ; Humans ; Rhinitis ; Systemic Vasculitis ; Vasculitis

Congenital cystic adenomatoid malformation(C.C. A.M.) is a rare variant of congenital pulmonary cystic diseases. C.C.A.M. was first distinguished from other pulmonary cystic lesions by Ch`in and Tang in 1949. It is characterized by cessation of bronchiolar maturation with an overgrowth of mesenchymal elements and proliferation of polypoid glandular tissue in the absence of normal alveolar differentiation. In 80% to 95% of cases, C.C.A.M. of the lung is confinded to a single lobe and there is no lobe predilection and right and left predominance. The clinical presentation may be widely variable, ranging from intrauterine fetal death secondary to nonimmune hydrops fetalis to discovery in childhood after recurrent pulmonary infections. Definitive treatment is removal of affected lobe. In rare instances, there is cases of myxosarcoma, embryonal rhabdomyosarcoma. Bronchoalveolar carcinoma arising in C.C.A.M.. So, resection is recommended even if they are asymtomatic. We have experienced a case of congenital cystic adenomatoid malformation (type II) of the lung in 15 year old man with pneumonia. After the improvement of pneumonia, surgical operation was performed. A brief review of the related literature is presented.
Adolescent ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Fetal Death ; Humans ; Hydrops Fetalis ; Lung* ; Myxosarcoma ; Pneumonia ; Rhabdomyosarcoma, Embryonal

BACKGROUND AND PURPOSE: This retrospective cross-sectional study included 18 patients from unrelated families harboring mutations of the transthyretin gene (TTR), and analyzed their characteristics and geographical distribution in South Korea. METHODS: The included patients had a diagnosis of systemic amyloidosis, clinical symptoms, such as amyloid neuropathy or cardiomyopathy, and confirmation of a TTR gene mutation using genetic analysis recorded between April 1995 and November 2014. RESULTS: The mean age at disease onset was 49.6 years, and the mean disease duration from symptom onset to diagnosis was 3.67 years. Fifteen of the 18 patients were classified as mixed phenotype, 2 as the neurological phenotype, and only 1 patient as the cardiac phenotype. The most-common mutation pattern in South Korea was Asp38Ala, which was detected in eight patients. Thirteen patients reported their family hometowns, and five of the eight harboring the Asp38Ala mutation were from the Gyeongsang province in southeast Korea. The other eight patients exhibited a widespread geographical distribution. A particularly noteworthy finding was that the valine at position 30 (Val30Met) mutation, which was previously reported as the most-common TTR mutation worldwide and also the most common in the Japanese population, was not detected in the present South Korean patients. CONCLUSIONS: South Korean patients with hereditary TTR amyloidosis exhibited heterogeneous TTR genotypes and clinical phenotypes. The findings of this study suggest that the distribution of TTR amyloidosis in South Korea is due to de novo mutations and/or related to the other countries in East Asia.
Amyloid Neuropathies ; Amyloidosis* ; Asian Continental Ancestry Group ; Cardiomyopathies ; Cross-Sectional Studies ; Diagnosis ; Far East ; Genotype ; Humans ; Korea ; Phenotype ; Prealbumin* ; Retrospective Studies ; Valine