Genetics of Colorectal Cancer (PDQ®): Genetics - Health Professional Information [NCI] - Major Genetic Syndromes
Table 6. Spigelman Classification
|Points||Polyp Number||Polyp Size (mm)||Histology||Dysplasia|
|Stage I, 1–4 points; Stage II, 5–6 points; Stage III, 7–8 points; Stage IV, 9–12 points|
A baseline upper endoscopy, including side-viewing duodenoscopy, should be performed between ages 25 and 30 years in FAP patients. The subsequent intervals between endoscopy vary according to the findings of the previous endoscopy, often, based on Spigelman stage. Recommended intervals are based on expert opinion although the relatively liberal intervals for stage 0-II disease are based in part on the natural history data generated by the Dutch/Scandinavian duodenal surveillance trial. Refer to Table 7 for more information.
The main advantages of the Spigelman Classification are its long-standing familiarity to and usage by those in the field, which allows reasonable standardization of outcome comparisons across studies.[63,81] However, there are several limitations on attempted application of the Spigelman Classification:
- Most pathologists do not currently employ the term moderate dysplasia, preferring a simpler low- versus high-grade dysplasia system.
- Because of the villous nature of normal duodenal epithelium, pathologists commonly disagree over the classification of "tubular," "tubulovillous," and "villous."
- Spigelman staging requires biopsy, which is not always essential when only a few small plaques are present; conversely, for larger adenomas, sampling variation leads to understaging.[82,83]
Table 7. Recommended Screening Intervals by Spigelman Stage
|Spigelman Stage||NCCN||Groves et al.|
|CP = chemoprevention; ET = endoscopic therapy; GA = general anesthetic; NCCN = National Comprehensive Cancer Network.|
|Refer to theInterventions/FAPsection in theMajor Genetic Syndromessection of this summary for more information about chemoprevention.|
|Referbelowfor additional information about the use of surgical resection in Spigelman stage IV disease.|
|0 (no polyps)||Endoscopy every 4 y||Endoscopy every 5 y|
|I||Endoscopy every 2–3 y||Endoscopy every 5 y|
|II||Endoscopy every 1–3 y||Endoscopy every 3 y|
|CP + ET|
|III||Endoscopy every 6–12 mo||Endoscopy every 1–2 y|
|CP + ET (+/- GA)|
|IV||Surgical referral||Surgical resection|
|Complete mucosectomy or duodenectomy or Whipple procedure if duodenal papilla is involved|
|Endoscopy every 3–6 mo||Endoscopy every 1–2 y|
|CP + ET (+/- GA)|
Many factors, including severity of polyposis, comorbidities of the patient, patient preferences, and availability of adequately trained physicians, determine whether surgical or endoscopic therapy is selected for polyp management. Endoscopic resection or ablation of large or histologically advanced adenomas appears to be safe and effective in reducing the short-term risk of developing duodenal adenocarcinoma;[77,78,85] however, patients managed with endoscopic resection of adenomas remain at substantial risk of developing recurrent adenomas in the duodenum. The most definitive procedure for reducing the risk of adenocarcinoma is surgical resection of the ampulla and duodenum, though these procedures also have higher morbidity and mortality associated with them than do endoscopic treatments. Duodenotomy and local resection of duodenal polyps or mucosectomy have been reported, but invariably, the polyps recur after these procedures. In a series of 47 patients with FAP and Spigelman stage III or stage IV disease who underwent definitive radical surgery, the local recurrence rate was reported to be 9% at a mean follow-up of 44 months. This local recurrence rate is dramatically lower than any local endoscopic or surgical approach from the same study. Pancreaticoduodenectomy and pancreas-sparing duodenectomy are appropriate surgical therapies that are believed to substantially reduce the risk of developing periampullary adenocarcinoma.[83,86,87,88] If such surgical options are considered, preservation of the pylorus is of particular benefit in this group of patients because most will have undergone a subtotal colectomy with ileorectal anastomosis or total colectomy with ileal pouch anal anastomosis. As noted in a Northern European study, and others,[89,90] the vast majority of patients with duodenal adenomas will not develop cancer and can be followed with endoscopy. However, individuals with advanced adenomas (Spigelman stage III or stage IV disease) generally require endoscopic or surgical treatment of the polyps. Chemoprevention studies for duodenal adenomas in FAP patients are currently under way and may offer an alternate strategy in the future.