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Special Feature
January 1, 2008

Image of the Month—Diagnosis

Arch Surg. 2008;143(1):96. doi:10.1001/archsurg.2007.20-b
Answer: Moderately Differentiated Neuroendocrine Carcinoma of the Gallbladder

Histologic examination of the gallbladder showed a 2.5-cm, moderately differentiated neuroendocrine carcinoma of the gallbladder with infiltration of the wall to the submucosa, not involving the fundus or the common biliary duct (Figure 2). Immunophenotypic test results were focally positive for chromogranin and diffusely positive for synaptofisin.

Figure 2.
Pathological examination revealing tumoral infiltration of the wall to the submucosa.

Pathological examination revealing tumoral infiltration of the wall to the submucosa.

The patient underwent Octreoscan scintigraphy, but no pathological area was found. Plasma chromogranin A and urinary 5-hydroxyindoleacetic acid levels were normal. We did not start any chemotherapeutic protocol and we scheduled the patient for follow-up at 3, 6, and 12 months; 6 months after the operation, the patient was found to be in good shape and free of symptoms.

Carcinoid tumors, first described by Lubarsch in 1888 as carcinomas and later classified by Oberndorfer in 1907, are enigmatic, slow-growing malignancies, originating from different neuroendocrine cell types and representing 1.2% to 1.5% of all gastrointestinal neoplasms, with an incidence of 1.6 to 2.0 per 100 000 per year.1Commonly associated with the gastrointestinal tract and bronchopulmonary system, less than 1% of all carcinoids present in atypical anatomical sites like the Meckel diverticulum, pelvic and otolaryngeal organs, breast, esophagus, pancreas, liver, biliary tract, and gallbladder.2Joel3first described a case of carcinoid tumor of the gallbladder in 1929, and Surveillance, Epidemiology, and End Results (SEER) data report only 42 cases of gallbladder carcinoids, composing 0.2% of all carcinoids, with a predominance in women aged 38 to 81 years. Clinically, these patients most commonly present with jaundice and right upper quadrant pain, but more often, they are diagnosed incidentally on histologic examination of gallbladder specimens after cholecystectomy for cholecystitis or after surgical treatment when a biliary malignancy is suspected.4Characteristic pathological findings with respect to size and metastases predict the prognosis.5Classical carcinoids of the gallbladder have neither a metastatic nor invasive character and exhibit a more propitious prognosis, whereas the ‘‘atypical’’ variants are associated with marked cell atypia and mitosis, as well as a poor prognosis. SEER data report the 5-year survival as 60.8% to 14.8%.2The addition of chemotherapy or radiotherapy or both did not change the survival.6

The biologic behavior of carcinoid tumors in the gallbladder is still unclear because of the lack of data in the literature and poor follow-up. The surgical approach depends on age and entity of pathological features. Careful follow-up is mandatory and more extensive studies are necessary.

Correspondence:Giuseppe Virzì, MD, First Department of Surgery, Pr. Piazza, Ospedale Vittorio Emanuele, via Plebiscito 624, Catania 95124, Italy (giuseppevirzi@hotmail.com).

Accepted for Publication:September 24, 2006.

Author Contributions:Study concept and design: Virzì and Ciaccio. Acquisition of data: Virzì, Ragazzi, and Mascali. Analysis and interpretation of data: Virzì, D’Agati, Caputo, Scaravilli, and Piazza. Drafting of the manuscript: Virzì, Ragazzi, and Ciaccio. Critical revision of the manuscript for important intellectual content: Virzì, D’Agati, Caputo, Scaravilli, and Piazza. Study supervision: Piazza.

Financial Disclosure:None reported.

Additional Contributions:Rosanna Virzì, MD, Department of Anatomopatology Ospedale Vittorio Emanuele, Catania, Italy, provided study supervision.

Läuffer  JMZhang  TModlin  IM Review article: current status of gastrointestinal carcinoids.  Aliment Pharmacol Ther 1999;13 (3) 271- 287PubMedArticle
Modlin  IMLye  KKidd  M A five-decade analysis of 13,715 carcinoid tumors.  Cancer 2003;97 (4) 934- 959PubMedArticle
Joel  W Karzinoidtumor der Gallenblase.  Zentralbl Allg Pathol 1929;461- 4
Modlin  IMShapiro  MDKidd  M An analysis of rare carcinoid tumors: clarifying these clinical conundrums.  World J Surg 2005;29 (1) 92- 101PubMedArticle
Nishigami  TYamada  MNakasho  K  et al.  Carcinoid tumor of the gall bladder.  Intern Med 1996;35 (12) 953- 956PubMedArticle
Machado  MCPenteado  SMontagnini  ALMachado  MA Carcinoid tumor of the gallbladder.  Sao Paulo Med J 1998;116 (3) 1741- 1743PubMedArticle