Goblet cell carcinoid neoplasm of the appendix: Clinical and CT features

Lee, K.S.; Tang, Laura H.; Shia, Jinru; Paty, Philip B.; Weiser, Martin R.; Guillem, José G.; Temple, Larissa K.; Nash, Garrett M.; Reidy, Diane Lauren; Saltz, Leonard B.; Gollub, Marc J.

doi:10.1016/j.ejrad.2012.05.038

Cited by 23 publications

(18 citation statements)

References 13 publications

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“…This female preponderance has also been noted by others. 3,22 Although Warkel et al 7 in a study of ordinary goblet cell carcinoids at the Armed Forces Institute of Pathology found a 2:1 male predominance, they attributed this to predominant enlistment of males in the military. Hristov’s study not only highlighted the gynecologic pathologists’ perspective on these high-grade examples (hence their exclusive female cohort) but it also demonstrates another intriguing aspect of this tumor, and that is its propensity for transcoelomic pelvic dissemination and gynecologic tract involvement, 14,15,22 which is similar to our findings with 58% of the cases showing gynecologic tract involvement, sometimes as the first sign of disease.…”

Section: Discussionmentioning

confidence: 99%

Adenocarcinoma ex-goblet cell carcinoid (appendiceal-type crypt cell adenocarcinoma) is a morphologically distinct entity with highly aggressive behavior and frequent association with peritoneal/intra-abdominal dissemination: an analysis of 77 cases

et al. 2016

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High-grade versions of appendiceal goblet cell carcinoids (‘adenocarcinoma ex-goblet cell carcinoids’) are poorly characterized. We herein document 77 examples. Tumors occurred predominantly in females (74%), mean age 55 years (29–84), most with disseminated abdominal (77% peritoneal, 58% gynecologic tract involvement) and stage IV (65%) disease. Many presented to gynecologic oncologists, and nine had a working diagnosis of ovarian carcinoma. Metastases to liver (n =3) and lung (n =1) were uncommon and none arose in adenomatous lesions. Tumors had various histologic patterns, in variable combinations, most of which were fairly specific, making them recognizable as appendiceal in origin, even at metastatic sites: I: Ordinary goblet cell carcinoid/crypt pattern (rounded, non-luminal acini with well-oriented goblet cells), in variable amounts in all cases. II: Poorly cohesive goblet cell pattern (diffusely infiltrative cords/single files of signet ring-like/goblet cells). III: Poorly cohesive non-mucinous cell (diffuse-infiltrative growth of non-mucinous cells). IV: Microglandular (rosette-like glandular) pattern without goblet cells. V: Mixed ‘other’ carcinoma foci (including ordinary intestinal/mucinous). VI: goblet cell carcinoid pattern with high-grade morphology (marked nuclear atypia). VII: Solid sheet-like pattern punctuated by goblet cells/microglandular units. Ordinary nested/trabecular (‘carcinoid pattern’) was very uncommon. In total, 33(52%) died of disease, with median overall survival 38 months and 5-year survival 32%. On multivariate analysis perineural invasion and younger age (<55) were independently associated with worse outcome while lymph-vascular invasion, stage, and nodal status trended toward, but failed to reach, statistical significance. Worse behavior in younger patients combined with female predilection and ovarian-affinity raise the possibility of hormone-assisted tumor progression. In conclusion, ‘adenocarcinoma ex-goblet cell carcinoid’ is an appendix-specific, high-grade malignant neoplasm with distinctive morphology that is recognizable at metastatic sites and recapitulates crypt cells (appendiceal crypt cell adenocarcinoma). Unlike intestinal-type adenocarcinoma, it occurs predominantly in women, is disguised as gynecologic malignancy, and spreads along peritoneal surfaces with only rare hematogenous metastasis. It appears to be significantly more aggressive than appendiceal mucinous neoplasms.

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Section: Discussionmentioning

confidence: 99%

Adenocarcinoma ex-goblet cell carcinoid (appendiceal-type crypt cell adenocarcinoma) is a morphologically distinct entity with highly aggressive behavior and frequent association with peritoneal/intra-abdominal dissemination: an analysis of 77 cases

et al. 2016

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“…This patient was re-operated upon where right hemicolectomy was undertaken (underwent second stage procedure). Older age seems to be associated with GCC, where mean age of GCC patients is around 52 years [24], and others have reported appendicular GCC in two females (60 and 54 year olds) and a male (>60 years) [25], [26], [27], [28]. Although a case of 31-year-old man has also been described in the literature [29].…”

Section: Discussionmentioning

confidence: 99%

Frequency, characteristics and outcomes of appendicular neuroendocrine tumors: A cross-sectional study from an academic tertiary care hospital

Abdelaal

Ansari

Al‐Bozom

et al. 2017

Annals of Medicine &Amp; Surgery

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BackgroundAppendicular neuroendocrine tumors (NET, Carcinoid tumors) of the appendix are rare and mostly diagnosed incidentally on the post-operative histopathological examination. NET are usually associated with good 5-year survival rates. We aimed to assess our experience for the diagnosis and management of NET over 11 years.MethodIt is a retrospective chart review of all clinically suspected patients with acute appendicitis who underwent emergent appendectomy with intention to treat between January 2004–December 2014, and were clinically followed up until 2016.ResultsDuring the study period, a total of 13641 patients underwent emergency appendectomy, of which 32 were histologically confirmed NET. The mean age of the NET cases was 25.3 ± 7.9 years; 78% were males and all were clinically presented with acute appendicitis. The mean leucocyte was 15 ± 14 × 109 per Liter, and mean tumor size was 4.86 ± 3.18 (ranged 1.5–13) mm. The median length of hospital stay was 4 (2–15) days. One patient had right hemicolectomy; diagnosed with right colonic cancer with NET being an incidental finding as part of histopathological assessment. Another patient required a second stage procedure; he was diagnosed as goblet cell carcinoid with positive margin. None of the patients died 30-day postoperatively and all of them survived on clinical follow-up that ranged between 2 and 13 years.ConclusionCarcinoid tumors of the appendix are rare and typically diagnosed incidentally. Detailed examination of routine appendectomy specimens is the key for diagnosis. Simple appendectomy suffices for tumors <2 cm for adequate clearance. Appendicular carcinoid tumors are associated with good long-term outcomes.

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“…GCCTs tend to occur in older patients (typically sixth decade) and represent less than 15 % of all appendiceal neoplasms. Clinical presentation may include abdominal pain or acute appendicitis [13]. CT findings include infiltrative pattern with diffuse mural thickening.…”

Section: Pathologymentioning

confidence: 99%

“…Metastatic disease to the peritoneum and ovaries may be present [12]. Acute appendicitis can occur secondary to diffuse infiltration, which causes a fibrous proliferation reaction and resultant obstruction of the appendiceal lumen [13]. …”

Section: Pathologymentioning

confidence: 99%

Anatomical variants and pathologies of the vermix

et al. 2014

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The appendix may demonstrate a perplexing range of normal and abnormal appearances on imaging exams. Familiarity with the anatomy and anatomical variants of the appendix is helpful in identifying the appendix on ultrasound, computed tomography, and magnetic resonance imaging. Knowledge of the variety of pathologies afflicting the appendix and of the spectrum of imaging findings may be particularly useful to the emergency radiologist for accurate diagnosis and appropriate guidance regarding clinical and surgical management. In this pictorial essay, we review appendiceal embryology, anatomical variants such as Amyand hernias, and pathologies from appendicitis to carcinoid, mucinous, and nonmucinous epithelial neoplasms.

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Goblet cell carcinoid neoplasm of the appendix: Clinical and CT features

Cited by 23 publications

References 13 publications

Adenocarcinoma ex-goblet cell carcinoid (appendiceal-type crypt cell adenocarcinoma) is a morphologically distinct entity with highly aggressive behavior and frequent association with peritoneal/intra-abdominal dissemination: an analysis of 77 cases

Adenocarcinoma ex-goblet cell carcinoid (appendiceal-type crypt cell adenocarcinoma) is a morphologically distinct entity with highly aggressive behavior and frequent association with peritoneal/intra-abdominal dissemination: an analysis of 77 cases

Frequency, characteristics and outcomes of appendicular neuroendocrine tumors: A cross-sectional study from an academic tertiary care hospital

Anatomical variants and pathologies of the vermix

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