Child ; Choristoma ; pathology ; surgery ; Ear, Middle ; pathology ; surgery ; Female ; Hamartoma ; pathology ; surgery ; Humans ; Pharyngeal Neoplasms ; pathology ; surgery ; Salivary Glands ; pathology ; surgery

Choristoma ; pathology ; surgery ; Dermoid Cyst ; diagnosis ; Diagnosis, Differential ; Dissection ; methods ; Eyelids ; pathology ; Hemangioma ; diagnosis ; Humans ; Infant, Newborn ; Male ; Neuroglia ; pathology ; Orbit ; diagnostic imaging ; Orbital Neoplasms ; diagnosis ; Treatment Outcome ; Ultrasonography ; methods

OBJECTIVECongenital esophageal stenosis owing to tracheobronchial remnants (TBR) is a rare condition. This study was conducted to understand the clinical features of TBR.

METHODThe data of the four cases with TBR admitted to our hospital and 76 patients identified from the literature were reviewed. The clinical manifestation, X-ray, endoscopy, biopsy and treatment were studied retrospectively.

RESULTOf the total of 80 cases, 45 were male, 33 were female, and for 2 cases the gender was unknown. Symptoms of dysphagia and regurgitation developed at the age of 1-day to 12-month. Definitive treatment was carried out at the age of 1-month to 16-year. Twenty-seven patients had associated anomalies with esophageal atresia being the most prevalent. X-ray esophagography showed segmental stenosis at the distal third of the esophagus in all patients except three. An abrupt narrow segment at the lower esophagus with marked proximal dilatation was found in 32 cases. Esophagography of 12 cases showed distal esophageal stenosis with tapered narrowing. Esophagography of 20 cases showed flask-shaped shadow of distal esophageal stenosis and one patient showed linear projection of barium at the level of stenosis. Endoscopy found almost complete obstruction of the lower esophageal lumen without signs of the esophagitis or reflux. Esophagoscopic dilatation of the stenosis was attempted in 24 cases, but was ineffective, and 3 patients suffered esophageal perforation. Seventy-nine patients underwent resection of the stenotic segment. Histologic examination of the resected specimen showed cartilage, mucus glands, resembling bronchal tissue. Post-operative complication included anastomotic stenosis, anastomotic leakage, hiatal hernia, and gastroesophageal reflux.

CONCLUSIONTBR should be suspected in patients who present with a typical history of dysphagia after ingestion of solid food. Esophagography and esophagoscopy are the essential means for diagnosis. TBR should be different from achalasia and should be diagnosed by biopsy. Operation is the only choice of treatment.

Anastomosis, Surgical ; Barium Sulfate ; Biopsy ; Child, Preschool ; Choristoma ; complications ; Dilatation ; Esophageal Atresia ; complications ; Esophageal Perforation ; etiology ; Esophageal Stenosis ; congenital ; diagnosis ; pathology ; surgery ; Esophagoscopy ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Postoperative Complications ; epidemiology ; Retrospective Studies ; Tracheoesophageal Fistula ; etiology

Adult ; CD5 Antigens ; metabolism ; Carcinoma, Papillary ; metabolism ; pathology ; Carcinoma, Squamous Cell ; metabolism ; pathology ; surgery ; Choristoma ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Hamartoma ; metabolism ; pathology ; Humans ; Proto-Oncogene Proteins c-kit ; metabolism ; Thymoma ; metabolism ; pathology ; Thymus Gland ; pathology ; Thymus Neoplasms ; metabolism ; pathology ; Thyroid Neoplasms ; metabolism ; pathology ; surgery ; Thyroidectomy ; methods

Cutaneous lung tissue heterotopia is a very rare disorder where mature lung tissues develop in the skin. This is only the second known report of cutaneous lung tissue heterotopia, with the first by Singer et al. in 1998. A newborn infant had a hemangioma-like, freely movable mass connected to the anterior aspect of the sternal manubrium. Pathologic findings showed mature lung tissues with bronchi, bronchioles, and alveoli through the dermis and subcutis, and it was diagnosed as cutaneous lung tissue heterotopia. Cutaneous lung tissue heterotopia is hypervascular, so grossly it looks like a hemangioma. It can be differentiated from pulmonary sequestration, teratoma, bronchogenic cyst, and branchial cleft cyst by histology and the location of the mass. We describe the clinical, radiologic, and pathologic findings of a cutaneous lung tissue heterotopia, the first reported in Korea.
Branchioma/pathology/surgery ; Bronchogenic Cyst/pathology/surgery ; Choristoma/*pathology/surgery ; Humans ; Infant, Newborn ; *Lung ; Magnetic Resonance Imaging ; Male ; Republic of Korea ; Skin Diseases/*pathology/surgery ; Skin Neoplasms/pathology ; Tomography, X-Ray Computed

Adolescent ; Adult ; Child ; Child, Preschool ; Choristoma ; metabolism ; pathology ; surgery ; Encephalocele ; metabolism ; pathology ; surgery ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Infant ; Male ; Meningocele ; metabolism ; pathology ; surgery ; Middle Aged ; Mucin-1 ; metabolism ; Neuroglia ; metabolism ; pathology ; Nose Diseases ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult

PURPOSE: Extensive extirpation of cervico-mediastinal adipose tissue increases the chance of removing ectopic thymic tissues, thus potentially improving the prognosis of myasthenia gravis after thymectomy. We sought to increase efficacy and safety of transsternal maximal thymectomy (TSMT). MATERIALS AND METHODS: Twenty four patients who underwent TSMT from July 2006 to June 2007 were retrospectively reviewed and compared with 73 patients who underwent transsternal extended thymectomy (TSET) from January 2004 to May 2006. Ectopic thymic tissue in additionally excised cervicomediastinal fat tissue was examined histologically. RESULTS: In TSMT group, operation time, amount of cumulative drainage and duration of drainage were significantly higher than TSET group. However, the difference in hemoglobin count, amount of transfusion, duration of intensive care, postoperative hospital stay, and complication rates were not statistically different. There was no operative mortality in either group. Ectopic thymic tissue was found in 50% of patients. All patients had ectopic thymic tissues in the cervical area. Two patients had additional ectopic tissue in the aortopulmonary window, and 1 patient had ectopic tissue at posterior of the left bracheocephalic vein and lateral of the right phrenic nerve. CONCLUSION: TSMT is more effective in the extirpation of ectopic thymic tissues than TSET without significant impairment of safety, especially in the cervical area.
Adipose Tissue/pathology/surgery ; Adult ; Choristoma/pathology/surgery ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis/*surgery ; Neck ; Retrospective Studies ; Thymectomy/*methods ; Thymus Gland ; Young Adult

Brunnera's gland hyperplasia is a benign tumor of the duodenum and it is rarely associated with clinical symptoms. We report on a 64-yr-old man with Brunnera's gland hyperplasia who had undergone a duodenocephalo-pancreatectomy. The reason is that he presented upper gastrointestinal obstructive symptoms and the esophagogastroduodenoscopic finding revealed the lesion to be an infiltrating type mass on the second portion of the duodenum with luminal narrowing. An abdominal computed tomography showed a 2.5 cm-sized mass in the duodenal second portion with a suspicious pancreatic invasion and 7 mm-sized lymph node around the duodenum. Duodenocephalopancreatectomy was successfully performed. Histological examination revealed a Brunnera's gland hyperplasia. The final diagnosis was the coexistence of Brunnera's gland hyperplasia and pancreatic heterotopia with a pancreatic head invasion. The literature on Brunnera's gland hyperplasia is reviewed.
Brunner Glands/*pathology/radiography ; *Choristoma ; Diagnosis, Differential ; Duodenal Neoplasms/*pathology/radiography/surgery ; Duodenum/pathology/radiography ; Endoscopy, Gastrointestinal ; Humans ; Hyperplasia ; Male ; Middle Aged ; Pancreatic Diseases/*pathology/radiography ; Pancreaticoduodenectomy ; Severity of Illness Index ; Tomography, X-Ray Computed

Brain ; Choristoma ; metabolism ; pathology ; surgery ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Infant ; Neurofilament Proteins ; metabolism ; Orbit ; pathology ; surgery ; Orbital Diseases ; metabolism ; pathology ; surgery

OBJECTIVETo study the clinicopathological and immunohistochemical features of ectopic hamartomatous thymoma (EHT), and to discuss its histogenesis.

METHODSThe clinical and pathologic features of two EHT cases of were evaluated. Immunohistochemical study was performed by LSAB method using a panel of antibodies including AE1/AE3, CK5, CK7, CK8, CK20, EMA, vimentin, CD5, CD10, alpha-SMA, calponin, desmin, CD34, S-100 protein, CD57, GFAP, TTF-1 and CD99.

RESULTSBoth cases occurred in males aged 20 years and 40 years respectively. Each patient presented with a solitary mass, one located in the suprasternal fossa and the other in the left supraclavicular region for a period of 6 months and 2 months respectively. Grossly, the masses were well-circumscribed with spherical and ovoid appearance, measuring 5 cm and 3 cm in maximum diameter respectively. On cut section, they were gray-white in color and of soft consistency. Histologically, both tumors were composed of a mixture of spindle cells, epithelial cells and mature adipose tissue. The spindle cells element accounted 85% and 70% each in the two cases. They resembled fibroblasts in morphology and were arranged frequently in fascicular, woven or storiform patterns. Epithelial cells element represented nearly 10% in both cases. Most of the epithelial cells had a non-keratinization squamous appearance. They formed small solid islands and adamantinoma-like "nastomosing cords", or appeared as lining cells in large cystic spaces. In focal areas, glandular differentiation presented as small glands. A transition between the spindle cell and epithelium components could be also identified in some areas. Mature adipose tissue was irregularly distributed in the two tumors, about < 5% and 20% respectively. Immunohistochemically, the epithelial element expressed AE1/AE3, CK5, CK7, CK8 and EMA, whereas the spindle component expressed AE1/AE3, CK5, CK7, CK8, vimentin, CD10, CD34, alpha-SMA, MSA, and calponin. Both elements were negative for CK20, TTF-1, desmin, S-100 protein, CD57, GFAP and CD99.

CONCLUSIONSEHT is a benign tumor that occurs predominantly in the lower neck region of young to middle-aged males. Immunohistochemical study revealed myoepithelial differentiation of the spindle cells, suggesting EHT is a mixed tumor composed of epithelial and myoepithelial cells. EHT possibly originates from the remnants of cervical sinus of His, and therefore, may be renamed as branchial anlage mixed tumor.

Adult ; Anion Exchange Protein 1, Erythrocyte ; metabolism ; Choristoma ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Hamartoma ; Humans ; Keratins, Type II ; metabolism ; Lymphatic Diseases ; metabolism ; pathology ; surgery ; Male ; Mucin-1 ; metabolism ; Thymoma ; metabolism ; pathology ; surgery ; Thymus Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism