Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient who, eight years prior, had a Heller operation for achalasia developed hypertrophic osteoarthropathy possibly secondary to a leiomyoma of the esophagus with sarcomatous changes. No local invasion or metastases were found. Total esophagectomy completely cured the esophageal symptoms and relieved the swelling and pain of her knees and other joints. This case illustrates the hypertrophic osteoarthropathy syndrome, its marked diagnostic significance and its occurrence secondary to an esophageal leiomyoma and leiomyosarcoma.
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PMID:Hypertrophic osteoarthropathy with myogenic tumors of the esophagus. 742 77

Esophageal replacement remains a challenge. Colon and jejunum provide alternative conduits to replace the lower esophagus when stomach is not suitable. Between 1971 and 1991, 41 patients underwent short-segment interposition of the esophagus with jejunum or colon. Indications were failed antireflux procedures (n = 21), nondilatable stricture (n = 9), achalasia (n = 2), moniliasis (n = 2), Barrett's esophagus with carcinoma in situ (n = 2), hemorrhagic esophagitis after esophagogastrectomy (n = 1), motility disorder (n = 1), instrumental perforation (n = 1), carcinoma (n = 1), and leiomyosarcoma (n = 1). Thirty-one patients (75.6%) had prior surgical procedures. Interposition with colon was performed in 22 patients and with jejunum in 19. Major complications occurred in 45% after colon interposition (10/22) and hospital mortality was 4.5% (1/22). Major complications after jejunal interposition occurred in 31% (6/19) and hospital mortality was 10.5% (2/19). A contained anastomotic leak occurred in 1 patient, perforation of a colon segment in 1, and jejunal graft necrosis in a third. Late functional results in 34 patients with a mean follow-up of 87 months were excellent or good in 26, fair in 5, and poor in 1. Colon interposition failed to improve symptoms in 2 patients with gastrointestinal motility disorders. Six patients underwent manometry and barium food provocation study. Two colon segments and 3 jejunal interpositions were hypoperistaltic or aperistaltic according to manometry. There was 1 case of aperistaltic jejunum with a distended afferent loop. When stomach is not available, successful palliation of swallowing can be accomplished with either jejunum or colon. Surgeons involved in the management of esophageal disease should be familiar with the technical details of both procedures.
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PMID:Short-segment intestinal interposition of the distal esophagus. 823 Dec 8

Carcinosarcoma of the esophagus is a rare malignant neoplasm that consists of both carcinomatous and sarcomatous elements. The histogenesis of the sarcomatous component is generally considered to result from metaplasia of carcinomatous cells toward mesenchymal differentiation. True carcinosarcoma, characterized as a collision between a carcinoma and a sarcoma, is extremely rare. We describe a patient with primary achalasia who developed a true carcinosarcoma of the esophagus in which clonal differences between carcinomatous and sarcomatous elements were genetically and immunohistochemically demonstrated. A polypoid tumor located in the middle third of the esophagus developed in a 51-year-old man with longstanding achalasia. The tumor was predominantly composed of spindle-shaped sarcomatous cells. Squamous cell carcinoma in situ and islands of well-differentiated squamous cell carcinoma in the sarcomatous element were histologically observed. The sarcomatous element was immunoreactive for both mesenchymal and myoid markers. The carcinomatous component expressed type I and type II cytokeratins as well as epithelial membrane antigen. Analysis for chromosomal loss of heterozygosity performed in multiple microdissected samples of each sarcomatous and carcinomatous element revealed distinct genetic clonalities. These differences in immunohistochemical and genetic clonalities suggest that the tumor composed of squamous cell carcinoma and leiomyosarcoma originated separately from epithelial and mesenchymal precursors.
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PMID:Esophageal carcinosarcoma: a genetic analysis. 928 91

The classic teaching in performance and interpretation of diagnostic studies is that "children are not small adults." The purpose of this article is to show a spectrum of "adult" disease entities that can occur, but are not usually considered in the pediatric population: cricopharyngeal achalasia, Schatzki's ring, achalasia, Helicobacter pylori, pancreatic carcinoma, adenocarcinoma of the colon, nasopharyngeal carcinoma, thyroid carcinoma, malignant melanoma, hydatidiform mole, renal cell carcinoma, leiomyosarcoma of the ovary and sarcoidosis. Radiologists interpreting pediatric imaging should recognize these entities and perform an appropriate diagnostic workup.
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PMID:Adult diseases in children. 1019 1

A case of diffuse esophageal leiomyomatosis is presented, with emphasis on the imaging findings across multiple contemporary diagnostic modalities. This entity represents a rare presentation of uncommon benign smooth muscle tumors of the esophagus. The characteristic clinical, histologic, and multimodality imaging findings may distinguish this benign tumor from its malignant counterpart leiomyosarcoma and from achalasia.
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PMID:Multimodality diagnostic imaging of diffuse esophageal leiomyomatosis. 1636 82