Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P61278 (somatostatin)
 22,083 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We make a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We report a case of Erdheim-Chester disease and review 60 cases from the literature. These cases are consider to have Erdheim-Chester disease when they have either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration with distinctive immunohistochemical phenotype of the non-Langerhans cell histiocytes with positive staining for CD68 and negative staining for S-100 protein and CD1a. Our patient undergoes chemiotherapy according to the LCH-II stratification and therapy plan (Vinblastine, Etoposide and Prednisone) and thereafter receives Carboplatin and Etoposide, and Somatostatin. She is alive and clinically well 33 months after onset of symptoms and the lesions don't appear to progress at imaging examinations. In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, the characteristics radiological pattern of Erdheim-Chester disease together the immunohistochemical phenotype of hystiocytic infiltration supports the theory that Erdheim-Chester disease is a unique disease entity distinct.
 ...
PMID:[Erdheim-Chester disease: a non-Langerhans cell histiocytosis. A clinical-case and review of the literature]. 1534 69

Neuroendocrine tumors of the digestive tract are relatively rare and comprise benign and malignant tumors. WHO classification system is based on recognized differences in morphology, function and clinical behavior. European Neuroendocrine Tumor Society(ENETS)proposed grading system and TNM classification system with disease staging for endocrine tumors of each organ which are both valid tools for prognostic stratification. The only curative therapy is the complete resection of the tumor. Endoscopic submucosal dissection(ESD)or local resection can be performed in low grade and early stage tumors; on the other hand, curative resection with lymph node dissection is recommended for neuroendocrine carcinoma. Complete surgical resection of liver metastases is associated with better long-term survival. Combination chemotherapy, such as Etoposide+Cisplatin/Carboplatin, is useful in treating unresected neuroendocrine carcinomas. Octreotide and Pasireotide (SOM230), somatostatin analogues, are reported to have the benefit of both hormonal symptom control and tumor growth suppression. Development of new effective drug is expected for the treatment of neuroendocrine tumors of the digestive tract.
 ...
PMID:[Treatment of neuroendocrine tumors of the digestive tract]. 1983 17