Primary Gallbladder Neuroendocrine Tumors: Insights into a Rare Histology Using a Large National Database

Ayabe, Reed I.; Wach, Michael M.; Ruff, Samantha M.; Martin, Sean P.; Diggs, Laurence P.; Wiemken, Timothy; Hinyard, Leslie; Davis, Jeremy L.; Luu, Carrie; Hernandez, Jonathan M.

doi:10.1245/s10434-019-07440-6

Cited by 36 publications

(50 citation statements)

References 28 publications

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“…The extrahepatic bile ducts and the ampullary region are more commonly observed in a GB-NEC of high malignancy, poor prognosis and little to no pathological differentiation (20). The occurrence of early metastasis is mainly identified through local infiltration and lymph node metastasis (21).…”

Section: Pathological Classificationmentioning

confidence: 99%

“…Advantages and disadvantages of the various treatment options discussed are listed in Table I: Due to its complexity, gallbladder cancer must be treated surgically by experienced biliary tract physicians and pathologists (42). GB-NEC is characterized by high malignancy, early lymphatic metastasis (the N2 lymph node metastasis rate is significantly higher compared with that of patients with adenocarcinoma in the same period) and a poor prognosis when compared with all other types of gallbladder cancer (20). Radical resection is considered to be the most effective and preferred method of surgical treatment for patients with GB-NEC (43).…”

Section: Treatment Optionsmentioning

confidence: 99%

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Neuroendocrine tumors of the gallbladder (Review)

et al. 2020

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A gallbladder neuroendocrine neoplasm (GB-NEN) is a unique heterogeneous neoplasm, originating from neuroendocrine cells and able to secrete peptides as its neurotransmitter. Among all the neuroendocrine tumors (NETs), the GB-NEN prevalence is 0.5%, which accounts for ~2.1% of all gallbladder tumors. The current study aimed to present an up-to-date review of the clinical features, diagnosis and treatment of NETs of the gallbladder. A systematic literature search was performed in order to identify all relevant studies published. Thus far, the etiology and pathogenesis of GB-NEN remains unclear. Moreover, an ambiguous clinical manifestation exists as observed during laboratory examinations. As GB-NEN is a rare gallbladder lesion and is seldom seen in clinical practice, there is a limited availability of review reports, and it is therefore often considered only as a case study. It is difficult to distinguish GB-NEN from other gallbladder diseases using imaging diagnostic techniques. Although chemotherapy treatment provides prolonged progression-free survival, surgery is considered the best option. Thus, to overcome the inherent risks or shortfalls of traditional surgery; early detection, diagnosis and treatment of GB-NEN are required to improve patient longevity.

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Section: Pathological Classificationmentioning

confidence: 99%

Section: Treatment Optionsmentioning

confidence: 99%

Neuroendocrine tumors of the gallbladder (Review)

et al. 2020

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“…Nearly half of the patients presented with stage IV disease, the median OS for this cohort was 25 months. They also reported that older age, large-cell histology, positive surgical margins were independently associated with decreased survival [15].…”

Section: Discussionmentioning

confidence: 94%

Mixed neuroendocrine–non-neuroendocrine neoplasms of the gallbladder: a case report

Ishikawa¹,

Nakano²,

Osaka³

et al. 2021

surg case rep

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Background Primary neuroendocrine tumors of the gallbladder (GB-NETs) are rare, accounting for 0.5% of all NETs and 2.1% of all gallbladder cancers. Among GB-NETs, mixed neuroendocrine–non-neuroendocrine neoplasms of the gallbladder (GB-MiNENs) are extremely rare. Case presentation We present the case of a 66-year-old woman who was referred to us for the management of a gallbladder tumor (incidentally found during abdominal ultrasonography indicated for gallbladder stones). The patient had no history of abdominal pain or fever, and the findings on a physical examination were unremarkable. Blood tests showed normal levels of tumor markers. Imaging studies revealed a mass of approximately 10 mm in diameter (with no invasion of the gallbladder bed) located at the fundus of the gallbladder. A gallbladder cancer was suspected. Therefore, an open whole-layer cholecystectomy with regional lymph nodes dissection was performed. The postoperative course was uneventful, and she was discharged on postoperative day 6. Pathological findings showed GB-MiNENs with invasion of the subserosal layer and no lymph node invasion (classified T2aN0M0 pStage IIA according to the Union for International Cancer Control, 8th edition staging system). Analysis of the neuroendocrine markers revealed positive chromogranin A and synaptophysin, and a Ki-67 index above 95%. Fourteen months after the operation, a local recurrence was detected, and she was referred to another hospital for chemotherapy. Conclusions GB-MiNENs are extremely aggressive tumors despite their tumor size. Optimal therapy should be chosen for each patient.

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“…In Algeria, gallbladder cancer represents 4% of women's incident cancers [4], Primary GBNEC are rare and represent 2,3% of all gallbladder neoplasm and less than 0,5% of all gastrointestinal NEC [5].…”

Section: Discussionmentioning

confidence: 99%

Pure large cell neuroendocrine carcinoma of the gallbladder, is surgical relentlessness beneficial? A case report and literature review

et al. 2021

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Pure large cell neuroendocrine carcinoma of the gallbladder is a rare disease. However, the prognosis of this aggressive tumor is poor with short survival after diagnosis. We are describing in this manuscript a case of pure large cell neuroendocrine carcinoma in which survival exceeded 26 months, after performing two curative surgeries. We are reporting the case of a 68-year-old woman with a history of recovered right breast carcinoma and operated 6 years later for a completely asymptomatic gallbladder tumor of 31 mm. In this case, curative surgery was performed allowing monobloc resection of the gallbladder and the hepatic segments IVb and V, a lymph node dissection was performed to. The histological examination of the specimen and immunohistochemistry confirms that the tumor was a grade 3 pure large cell neuroendocrine carcinoma of gallbladder with lymph node invasion, the hepatic and biliary surgical margins were free. Postoperative adjuvant chemotherapy was administered and the evolution was eventless until the discovery at 20 months of a lymph node considered being metastatic recurrence. A second surgery was performed allowing removal of three lymph nodes. This time, a different protocol of chemotherapy was administered to our patient who remains alive and without recurrence at 26 months from her first surgery. Surgical relentlessness with free margins resections associated with appropriate chemotherapy probably improves the survival of patients suffering from this rare and aggressive tumor.

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Primary Gallbladder Neuroendocrine Tumors: Insights into a Rare Histology Using a Large National Database

Cited by 36 publications

References 28 publications

Neuroendocrine tumors of the gallbladder (Review)

Neuroendocrine tumors of the gallbladder (Review)

Mixed neuroendocrine–non-neuroendocrine neoplasms of the gallbladder: a case report

Pure large cell neuroendocrine carcinoma of the gallbladder, is surgical relentlessness beneficial? A case report and literature review

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