Article

Ovarian metastases of appendiceal tumors with goblet cell carcinoidlike and signet ring cell patterns: a report of 30 cases.

Department of Pathology, The Johns Hopkins University School of Medicine and Hospital, 401 N. Broadway, Baltimore, MD 21231, USA.
American Journal of Surgical Pathology (impact factor: 4.35). 11/2007; 31(10):1502-11. DOI:10.1097/PAS.0b013e31804f7aa1 pp.1502-11
Source: PubMed

ABSTRACT Synchronous and metachronous ovarian and appendiceal mucinous tumors are often the subject of diagnostic consultations in gynecologic pathology practices to address whether the tumors are independent neoplasms or related as primary and metastasis. Those with goblet cell carcinoidlike patterns have not been extensively evaluated in a large series. Clinicopathologic features of 30 cases were examined. All patients presented with signs or symptoms related to a pelvic/adnexal or abdominal mass. The ovarian tumors were bilateral in 25 of 28 cases with data on both ovaries and were typically large (mean/median: 14 cm, range: 4.5 to 24.0 cm). The appendices were often firm or thickened but usually did not have a discrete measurable tumor and were not notably enlarged; microscopically, transmural invasion was present in all of them. The ovarian and appendiceal tumors exhibited a variety of patterns of differentiation, including signet ring cell and glandular, with all displaying some goblet cell carcinoidlike patterns (nests, islands, cords, or cryptlike tubules with goblet cells); teratomatous elements were not identified in any ovarian tumors. Chromogranin was expressed in 7 of 19 ovarian tumors (mean/median: 6.3%/0%; range: 0% to 20%) and synaptophysin was expressed in 4 of 18 of these (mean/median: 7.8%/0%; range: 0% to 90%). Chromogranin was expressed in 6 of 16 appendiceal tumors (mean/median: 11.9%/0%; range: 0% to 70%) and synaptophysin was expressed in 6 of 15 of these (mean/median: 16.7%/0%; range: 0% to 90%). Follow-up was available for 25 patients: 17 died of disease at intervals ranging from 4 to 47 months (mean/median: 18/16) and 8 were alive with disease at 1 to 25 months (mean/median: 11/10); median survival was 19 months and the 1-year and 2-year survival rates were 63% and 34%, respectively. The clinicopathologic features of these ovarian tumors indicate they should be labeled as metastatic appendiceal adenocarcinomas rather than as goblet cell carcinoid tumors both to reflect their behavior and help distinguish them from the rare true primary ovarian goblet cell carcinoid tumors. As the ovarian tumors have appreciable components of signet ring cells they qualify as Krukenberg tumors. In cases in which the primary tumor is not already evident, their "goblet cell carcinoidlike" patterns should direct attention to the appendix as a possible source.

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    Article: Nonneoplastic signet-ring cells in the gallbladder and uterine cervix. A potential source of overdiagnosis.
    Moira Ragazzi, Carolina Carbonara, Juan Rosai
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    ABSTRACT: We describe 3 cases of nonneoplastic signet-ring cell change in ulcerated mucosa, 2 of them in the gallbladder and 1 in an endocervical polyp. In the gallbladder cases, there were focal collections of signet-ring cells both on the mucosal surface and within the lumen of tubules, whereas in the endocervical polyp, the signet-ring cell aggregates were entirely confined to the mucosal surface. In all 3 cases, the signet-ring cells were positive for Mayer's mucicarmine and immunoreactive for keratin AE1/AE3. The lack of nuclear atypicality, the arrangement in superficial and intraluminal nests, and the admixture with histiocytes and other inflammatory cells are in keeping with the interpretation that the signet-ring cells are disrupted mucosal goblet cells exhibiting hyperplastic and degenerative changes. A review of the literature disclosed only other 2 previously reported cases of benign signet-ring cell changes in the gallbladder and none--to the best of our knowledge--in an endocervical polyp. Awareness of this phenomenon is of importance to avoid a potential overdiagnosis of signet-ring cell adenocarcinoma.
    Human pathology 11/2008; 40(3):326-31. · 3.03 Impact Factor
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    Article: Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.
    Laura H Tang, Jinru Shia, Robert A Soslow, Deepti Dhall, W Douglas Wong, Eileen O'Reilly, Jing Qin, Philip Paty, Martin R Weiser, Jose Guillem, Larissa Temple, Leslie H Sobin, David S Klimstra
    [show abstract] [hide abstract]
    ABSTRACT: Appendiceal tumors exhibiting both neuroendocrine and glandular differentiation are uncommon and have caused difficulty in pathologic classification, prediction of prognosis, and clinical management. Previously, such lesions have been variously designated as adenocarcinoid, goblet cell carcinoid (GCC), and mixed adenocarcinoma carcinoid. In this study, we undertook a retrospective investigation of 63 such cases and classified them as typical GCC (group A) and adenocarcinoma ex GCC on the basis of the histologic features of the tumor at the primary site. The adenocarcinoma ex GCC group was further divided into signet ring cell type (group B) and poorly differentiated adenocarcinoma type (group C). The clinical characteristics and prognosis were compared within these groups and with conventional de novo appendiceal adenocarcinomas. Both groups A and B tumors shared a similar immunoprofile, which included generally focal immunoreactivity for neuroendocrine markers, and a normal intestinal type mucin glycoprotein profile (negative MUC1 expression and preserved MUC2 immunoreactivity). The proliferative index was relatively low in these tumors and slightly increased from groups A to B tumors (11% to 16%). Both beta-catenin and E-cadherin exhibited a normal membranous staining pattern in groups A and B tumors. The poorly differentiated adenocarcinomas ex GCC (group C) demonstrated abnormal p53 and beta-catenin immunoreactivity. The mean follow-up time was 49+/-5 (SE) months. The overall disease-specific survival for all subtypes was 77%, with 46% of patients without evidence of disease and 31% alive with disease. The mean survival was 43+/-7 months. All the patients with clinical stage of I or IIA disease had a favorable outcome after appropriate surgery with or without chemotherapy. Although most patients (63%) with GCC presented at an advanced clinical stage, their clinical outcome could be differentiated by subclassification of tumors. The stage IV-matched 5-year survival was 100%, 38%, and 0% for groups A, B, and C, respectively. In conclusion, GCC is a distinctive appendiceal neoplasm that exhibits unique pathologic features and clinical behavior. They display a spectrum of histologic features and possess the potential to transform to an adenocarcinoma phenotype of either signet ring cell or poorly differentiated adenocarcinoma types. Careful evaluation of the morphologic features of GCCs and appropriate pathologic classification are crucial for clinical management and prediction of outcome. Surgical management with right hemicolectomy is recommended after appendectomy for most cases, particularly those with an adenocarcinoma component (groups B and C).
    The American journal of surgical pathology 09/2008; 32(10):1429-43. · 4.06 Impact Factor
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Keywords

16 appendiceal tumors
 
19 ovarian tumors
 
2-year survival rates
 
appendiceal mucinous tumors
 
appendiceal tumors exhibited
 
clinicopathologic features
 
discrete measurable tumor
 
goblet cell carcinoid tumors
 
goblet cell carcinoidlike
 
goblet cell carcinoidlike patterns
 
goblet cells
 
gynecologic pathology practices
 
Krukenberg tumors
 
metastatic appendiceal adenocarcinomas
 
ovarian tumors
 
possible source
 
primary tumor
 
rare true primary ovarian goblet cell carcinoid tumors
 
signet ring cell
 
signet ring cells