Neuro-endocrine carcinoma of the gallbladder: About two cases

S.Ben Slama | H.Romdhane | Z.Mzoughi | R.Ennaifer | A.Lahmar |

La tunisie chirurgicale - 2018 ; Vol 2017

Resumé

Neuroendocrine tumors of the gallbladder are very rare, they represent less than 1% of all neuroendocrine tumors and less than 5% of gallbladder cancers. They can occur at any age (peak between 75-79 years) with a clear female predominance. The symptomatology is nonspecific and the discovery is often incidental on a piece of cholecystectomy .

Mots Clés

Gallbladder, Carcinoma, Neuroendocrine tumours

Introduction :

Neuroendocrine tumors of the gallbladder are very rare, they represent less than 1% of all neuroendocrine tumors and less than 5% of gallbladder cancers [1]. They can occur at any age (peak between 75-79 years) with a clear female predominance [2]. The symptomatology is nonspecific and the discovery is often incidental on a piece of cholecystectomy [1]. As defined by the WHO, neuroendocrine carcinoma corresponds to an undifferentiated carcinoma, made up of large cells with a high histological grade, mitotic index of more than 20 mitoses / 2 mm² and / or a proliferation index evaluated by the Ki67 to more than 20% [3]. The prognosis of these carcinomas is rather poor with an average survival not exceeding 10-12 months, given the rapid onset of metastases [4].

We report two cases of neuroendocrine carcinoma with large cells of the gallbladder.

Article

Observation N°1:

A 39-year-old woman with no medical history was admitted with a suspicion of cholecystitis as she complained of an upper right abdominal pain for a week. The patient underwent cholecystectomy. On gross examination, the gallbladder was indurated and focally necrotic. The histological examination showed a small differentiated carcinoma, with large cells, infiltrating all the parietal plans with a mitotic index exceeding 25 mitoses / 2mm² (Figure 1). There were many perineural invasion. Immunohistochemistry study confirmed the neuroendocrine nature as Anti-synaptophysin (Novacastra®) And anti-chromogranin (Novacastra®) were positive. The proliferation index Ki-67 (Novacastra®) was 50% positive. A thoraco-abdomino-pelvic CT scan did not reveal any other localization. The patient refused any further treatment and she was lost to follow-up.

Observation N°2:

A hypertensive 68-year-old woman was hospitalized for a painful syndrome of the right hypochondrium with jaundice and dark urine.

There was no fever or vesicular lithiasis on ultrasound. Cholecystectomy was performed and study of the specimen showed an indurated and retracted lesion of 2 × 2cm at level of vesicular body. The histological examination led to the diagnosis of neuroendocrine carcinoma with large cells (of a WHO Grade 3) with a mitotic index at 35 mitoses / 2 mm². Immunohistochemistry was performed revealing positive tumor cells with anti-Synaptophysine and anti-CD56 (Novacastra®) (Figure 2). The lesion infiltrated all the parietal plans. The search for another tumor localization was negative. The patient died after a month of complications related to her high blood pressure.

Conclusion:

There is no consensus for the treatment of these neuroendocrine gallbladder carcinomas. Nevertheless, the main treatment is surgical with a wide excision and lymph node dissection and neoadjuvant chemotherapy depending on the tumor stage [5]. The prognosis remains poor.

 

Figure 1A: Poorly differentiated proliferation infiltrating the gallbladder (HEx10).

 

 

Figure 1B: High mitotic rate (HEx40).

 

 

Figure 2 : Positive immunostains for CD56 (HEx40)

 

 

Références

1- Buscemi S, Orlando E, Damiano G et al. “Pure” large cell neuroendocrine carcinoma of the gallbladder. Report of a case and review of the literature. Int J Surg 2016;28:128-32.

2- Yao JC, Hassan M, Phan A et al. One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008;26:3063-72.

3- Bosman FT, Carneiro F, Hruban RH, Theise ND. WHO classification of tumours of the digestive system. 4th Edition. Lyon: International Agency for Research on Cancer (IARC) 2010.

4- Eltawil KM, Gustafsson BI, Kidd M, Modlin IM. Neuroendocrine tumors of the gallbladder: an evaluation and reassessment of management strategy. J Clin Gastroenterol 2010;44:687-95.

5- Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD. Current status of gastrointestinal carcinoids. Gastroenterology 2005;128:1717-51.