Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural. The published English language literature from 1966 to 2009 was retrieved via PubMed and reviewed. Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted. The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation. GCCs present in the fifth to sixth decade and show no definite sex predominance. The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass. Fifty percent of the female patients present with ovarian metastases. The histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy. Atypia is usually minimal, but carcinomatous growth patterns may be seen. These may be of signet ring cell type or poorly differentiated adenocarcinoma. Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q). The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix. The 5-year overall survival is 76%. The most important prognostic factor is the stage of disease. Appendectomy and right hemicolectomy are the main modalities of treatment, followed by adjuvant chemotherapy in select cases. There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.
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PMID:Goblet cell carcinoid tumors of the appendix: An overview. 2116 Jun 37

Goblet cell carcinoid (GCC) tumors are a rare subgroup of neuroendocrine tumors almost exclusively originating in the appendix. The tumor most often presents in the fifth or sixth decade with a clinical picture of appendicitis or in advanced cases an abdominal mass associated with abdominal pain. Histologically tumors are most often positive for chromogranin A and synaptophysin, however, less homogenous than for classic appendix carcinoids. The malignant potential is higher than that for the classic appendix carcinoids due to local spread and distant metastases at diagnosis and the proliferation markers (Ki67 index) may determine prognosis. Octreotide receptor scintigraphy is usually negative while CT/MRI scans may be useful. Chromogranin A is usually negative and other biomarkers related to the mucinous component or the tumor (CEA, CA-19-9, and CA-125) may be used. Surgery is the main treatment with appendectomy and right hemicolectomy while patients with disseminated disease should be treated with chemotherapy. Overall 5-year survival is approximately 75%. The diagnosis and treatment of GCC tumors should be restricted to high volume NET centers in order to accumulate knowledge and improve survival in GCC NET patients. The aim of this paper is to update on epidemiology, clinical presentation, and diagnostic markers including Ki67 index, treatment, and survival.
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PMID:Goblet cell carcinoids of the appendix. 2336 45

Goblet cell carcinoid tumor is a rare form of carcinoid tumor of the appendix. It behaves more aggressively than classical carcinoid tumor of the appendix. Thus, special attention must be given to its disease course and treatment. Our case presentation is of a 68-year-old female who presented with abdominal pain. Computed tomography of her abdomen and pelvis showed a perforated appendicitis. She underwent an ileocectomy with pathological report showing a goblet cell carcinoid tumor of the appendix with negative lymph nodes. A subsequent colonoscopy done 5 months later showed no synchronous lesions and a healthy anastomosis. Given the limited amount of data available about goblet cell carcinoid tumors of the appendix, it is important to report all findings in an effort to improve our understanding and treatment approaches of this rare disease.
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PMID:Perforation of appendiceal adenocarcinoma ex goblet cell carcinoid: a rare case. 3025 32

A 68 year old male presented to our Emergency Department with a one-day history of right sided abdominal pain, distention and vomiting on a background of no previous abdominal surgery. Abdominal CT demonstrated a high grade, closed loop small bowel obstruction involving the terminal segment of the ileum. Also of significance was alow-density appendiceal nodule. A subsequent laparoscopy revealed the tip of the appendix adherent to the mesosigmoid colon, forming a tight band and consequent mechanical bowel obstruction. Furthermore, the meso-appendix was embedded with crystal deposits and extruding mucin. The decision was made to convert to laparotomy and perform a caecectomy. Immunohistochemistry demonstrated reactivity to synaptophysin, chromogranin A and CD56, confirming the diagnosis of Goblet Cell Carcinoid. A staging CT after this initial surgery revealed no metastasis. After discussion at our oncology MDT, the patient went on to receive a completion right hemicolectomy which revealed no further malignancy on histology. The patient otherwise progressed well, and made a good post-operative recovery.
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PMID:Goblet Cell Carcinoid (GCC) of the Appendix presenting as a Small Bowel Obstruction. 3261 27

Goblet cell carcinoid (GCC) of the perforated appendix is rare, and its pathological features and prognosis remain poorly described. A 71-year-old woman was admitted to our hospital for right lower abdominal pain, vomiting, and high-grade fever. She was diagnosed with acute appendicitis and underwent emergency laparoscopic appendectomy. Intraoperative examination revealed an enlarged and perforated appendix. Histopathological examination revealed GCC of the appendix with subserosal invasion. She underwent laparoscopic ileocecal resection with lymph node dissection (D3) following appendectomy. Histopathological findings showed no residual tumor or lymph node metastases. To the best of our knowledge, this report is a valuable addition to the GCC literature, describing a case of GCC of the appendix presenting as perforated appendix.
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PMID:Perforated Goblet Cell Carcinoid of the Appendix. 3288 10