J Gastrointest Canc DOI 10.1007/s12029-015-9757-5

CASE REPORT

Gallbladder Neuroendocrine Neoplasms: A Case Report of Gallbladder Small Cell Carcinoma Dalal M. Nemenqani 1,2 & Jyotsna Fuloria 3 & Rehab A. Karam 4,5 & Haneen Hammadi 5

# Springer Science+Business Media New York 2015

Introduction Gallbladder (GB) carcinoma is the fifth most common gastrointestinal malignancy with 7000 new cases diagnosed in the USA each year [1]. The majority of these tumors are adenocarcinomas [2, 3]. Other histologic types of GB cancers include squamous, adenosquamous, and neuroendocrine carcinoma (NEC). The overall 5-year survival rate for gallbladder cancer is approximately 5 % [3]. Neuroendocrine tumors (NETs) commonly arise in gastrointestinal tracts and bronchi but rarely occur in the gallbladder (GB) [4]. Depending upon biological behavior, NETs are broadly classified as well-differentiated NETs (classical carcinoid tumors) or neuroendocrine tumor grade 1 (G1); welldifferentiated neuroendocrine carcinomas (atypical carcinoid) or grade 2 (G2); and poorly differentiated neuroendocrine carcinoma (large cell/small cell carcinoma or high-grade neuroendocrine carcinomas) or grade 3 (G3). High-grade neuroendocrine carcinomas are distinguished from typical carcinoids (20/10 hpf) [5]. * Rehab A. Karam [email protected]

Small cell carcinoma (SCC) of the gallbladder is a rare disease, with Surveillance, Epidemiology and End Results (SEER) data suggesting an incidence of approximately 0.5 % of all gallbladder cancers [6] and 0.2 % of all gastrointestinal carcinoid [7]. First SCC of gallbladder was reported in 1981 by AlboresSaavedra et al. [8]. Gallbladder neuroendocrine tumors may be functioning with symptoms arising due to the production of biologically active peptides (carcinoid syndrome 20 mitoses/2 mm2 and small cell cytological features. The cellularity is typically very high with hyperchromatic nuclei, absent or very small nucleoli with scant cytoplasm, a high nuclear to cytoplasmic ratio, and a round or fusiform shape. The mitotic rate is very high [3, 7]. Immunohistochemical staining shows strong positive staining for neuroendocrine markers (chromogranin A and synaptophysin) [7]. In comparison to NETs which usually are diffusely positive for neuroendocrine markers, SCCs show more focal staining [17]. Similarly, the neoplastic cells in the reported case were positive for CD56 and synaptophysin indicating a neuroendocrine origin. Surgical excision (ranging from cholecystectomy alone to extensive surgical resections, including regional lymph node clearances and hepatic lobectomy) remains the best curative

Fig. 3 Immunohistochemical staining of gallbladder specimen biopsy, positive for synaptophysin, consistent with small cell carcinoma (antisynaptophysin ×400)

Fig. 4 Immunohistochemical staining of gallbladder specimen biopsy, positive for cell proliferation marker Ki 67 (anti-Ki 67×400)

J Gastrointest Canc Conflict of Interest The authors declare that they have no competing interests.

References 1. 2.

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Fig. 5 Postchemotherapy abdominal CT revealed reduction of gallbladder tumor size measuring 2.9×1.4 cm

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option. However, therapeutic options for this pathology are often limited due to the advanced nature of the disease at diagnosis, and the necessity of a radical resection and the extent of surrounding tissue removed are debatable [18]. If the tumor is nonresectable, the primary management in this setting is therefore medical (chemotherapy) rather than surgical. Different regimens are described, and the chemotherapeutic agents of choice are cisplatin, etoposide, and 5-flurouracil [19, 20]. Patients treated with surgical excision and adjuvant chemotherapy have an increased median survival from 4.5 to 13 months [9]. The current patient was managed as a locally advanced small cell carcinoma of the gallbladder and was started on neoadjuvant chemotherapeutic regimen in the form of 3 cycles of carboplatin and etoposide followed by surgical resection, then followed postoperatively with 3 other cycles of chemotherapy.

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Conclusion

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SCC of the gallbladder is a relatively rare and aggressive tumor with poor prognosis. Early diagnosis with prompt surgical intervention provides the patient with the best long-term outcome. Combinations of chemotherapy along with radical resection improve the prognosis. More prospective studies on a larger scale should be conducted to further illuminate the pathogenesis of different stages of SCC. It is hoped that with advances in multimodality therapy the prognosis would improve.

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Gallbladder Neuroendocrine Neoplasms: A Case Report of Gallbladder Small Cell Carcinoma.

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