“Pure” large cell neuroendocrine carcinoma of the gallbladder. Report of a case and review of the literature

Abstract

a b s t r a c tPrimary Neuroendocrine Tumours (NETs) of the gallbladder are rare. Among all NETs of the gallbladder, large cell neuroendocrine carcinoma (LCNEC) is exceedingly rare. In most of the cases LCNECs are combined with other histological components. We reviewed clinical presentation and management of all patients with "pure" LCNEC from published literature since the first case was published in 2000, as well as one patient from our experience. Only 7 cases of "pure" LCNEC has been described in the last 15 years, our case is the eighth. The diagnosis of gallbladder NETs is rarely made preoperatively since the presentation generally consists of non-specific symptoms including upper abdominal pain, discomfort, jaundice, weight loss. The majority of patients are identified incidentally at the time of cholecystectomy for cholelithiasis. It is not possible to differentiate preoperatively between gallbladder adenocarcinoma and gallbladder neuroendocrine carcinoma (NEC) with imaging techniques. The only curative therapeutic modality for LCNECs is a complete en bloc surgical resection, including regional lymph node clearances and hepatic lobectomy, but only in patients without multiple metastasis. LCNECs benefit from an aggressive surgical resection in combination with chemotherapy, if resectability is possible. If the tumour is non-resectable, the primary management is therefore medical and not surgical. All patients with LCNEC presented a poor prognosis with a median survival of 10 months after the initial diagnosis. Only in five patients (62.5%) a wide surgical excision was performed, while in the other cases the tumour was non-resectable or multiple liver metastases were present at diagnosis.

A 0.8-cm clear cell neuroendocrine tumor G1 of the gallbladder with lymph node metastasis: a case report

“…Preoperative diagnosis of NETs of the gallbladder is rare because, in general, the patients present with non-specific symptoms such as upper abdominal pain, discomfort, jaundice, and weight loss [ 18 ]. Previous studies showed that few cases of gallbladder NETs manifested with carcinoid syndrome [ 16 , 19 ].…”

Combined Adenosquamous and Large Cell Neuroendocrine Carcinoma of the Gallbladder

“…ACs have a relatively favorable prognosis, whereas tumors with squamous or neuroendocrine differentiation have a poor outcome (mean survival time, 50 vs 23 or 10 months, respectively) [ 2 , 6 ]. LCNEC has been known to exhibit aggressive behavior and early metastasis [ 4 ]. Among the previously reported LCNEC cases, 53% had directly invaded the liver at the time of diagnosis, and 47% had been identified with locoregional or distant metastases involving bone, liver or lymph nodes [ 3 ].…”

“…There are also differences in chemotherapeutic agents between conventional AC and combined tumor of the gallbladder. Conventional AC is usually treated with gemcitabine and cisplantin, while combined ASC or NEC is treated with FOLFOX (leucovorin, fluorouracil, and oxaliplatin) or etoposide [ 4 , 16 ].…”

“…The prognosis of this tumor is poor and early metastasis is common [ 2 ]. To date, 18 cases of gallbladder LCNEC have been reported [ 3 , 4 ]. LCNEC of the gallbladder is usually combined with other types of malignancies, mostly adenocarcinoma.…”

Ectopic ACTH-producing large cell neuroendocrine Pancoast tumour presenting as Horner syndrome

“…LCNEC is a type of neuroendocrine tumour that can affect lungs, gall bladder,7 urinary bladder,8 head and neck,9 prostate,10 ovary,11 uterine cervix,12 vagina,13 endometrium,14 breast,15 colon,16 ileum,17 rectum,18 bile ducts,19 stomach,20 renal pelvis,21 skin,22 thymus,23 etc. Pulmonary neuroendocrine tumours make up to 20% of all lung cancers of which LCNEC are 10% (ie, 2–3% of all lung cancers).…”