A Case of Small-Cell Neuroendocrine Carcinoma of the Gallbladder in a Background of High-Grade Biliary Intraepithelial Neoplasia

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S72-S72
Author(s):  
B Alkamachi ◽  
B Sharaf Aldeen ◽  
M Alhamar ◽  
H Jaratli

Abstract Introduction/Objective Neuroendocrine carcinoma (NEC) of the gallbladder is a very rare neoplasia comprising 4% of all malignant gallbladder neoplasms. Most of NECs show molecular accumulation of TP53, while KRAS mutations are rare. Approximately 40% of Biliary intraepithelial neoplasia (BilIN) cases are associated by KRAS mutations as an early molecular event, whereas TP53 mutation appears to be a late molecular event. Methods We report a case of a 62-year-old male who presented to emergency department for evaluation of abdominal pain associated with vomiting. Imaging studies demonstrated a distended gallbladder with small amount of pericholecystic fluid along with 5 mm calculus at the gallbladder neck consistent with symptomatic cholelithiasis and acute cholecystitis. No mass lesion was radiologically identified. A laparoscopic cholecystectomy was performed. Grossly, there was a thickened area of mucosa at the distal body-fundus, measuring 5.0 x 4.5 cm. Multiple calculi were present. On histology, thickened area showed sheets and nests of moulded small cells with hyperchromatic nuclei, brisk mitotic activity and confluent necrosis. A diagnosis of small-cell NEC is established, and confirmed by positive immunoreactivity to neuroendocrine markers (CD56, Synaptophysin) and epithelial markers (CK7 and CAM5.2). Multiple foci of high-grade BilIN were noted. Subsequent entire submission of the gallbladder revealed no adenocarcinoma present. The pathologic stage was pT2a pNX. Conclusion In conclusion, this rare gallbladder carcinoma is found incidentally in a patient with acute cholecystitis symptoms and no clinical proof of mass lesion. In addition, there is a background of high-grade BilIN, which shares the molecular pathway of small-cell NEC. Therefore, awareness of such coexistence of these two pathologic entities in the same gallbladder, is essential to alert pathologists to look for the poorly-prognosis small cell NEC, whenever BilIN is encountered in random section of a gallbladder.

Medicina ◽  
2021 ◽  
Vol 57 (12) ◽  
pp. 1338
Author(s):  
Tiberiu-Augustin Georgescu ◽  
Roxana Elena Bohiltea ◽  
Octavian Munteanu ◽  
Florentina Furtunescu ◽  
Antonia-Carmen Lisievici ◽  
...  

Neuroendocrine neoplasms (NENs) are particularly rare in all sites of the gynecological tract and include a variety of neoplasms with variable prognosis, dependent on histologic subtype and site of origin. Following the expert consensus proposal of the International Agency for Research on Cancer (IARC), the approach in the latest World Health Organization (WHO) Classification System of the Female Genital Tumours is to use the same terminology for NENs at all body sites. The main concept of this novel classification framework is to align it to all other body sites and make a clear distinction between well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The previous WHO Classification System of the Female Genital Tumours featured more or less the same principle, but used the terms ‘low-grade neuroendocrine tumor’ and ‘high-grade neuroendocrine carcinoma’. Regardless of the terminology used, each of these two main categories include two distinct morphological subtypes: NETs are represented by typical and atypical carcinoid and NEC are represented by small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC). High-grade NECs, especially small cell neuroendocrine carcinoma tends to be more frequent in the uterine cervix, followed by the endometrium, while low-grade NETs usually occur in the ovary. NENs of the vulva, vagina and fallopian tube are exceptionally rare, with scattered case reports in the scientific literature.


2011 ◽  
Vol 19 (5) ◽  
pp. 395-399 ◽  
Author(s):  
Sherry Thompson ◽  
Maureen Cioffi-Lavina ◽  
Jennifer Chapman-Fredricks ◽  
Carmen Gomez-Fernandez ◽  
Gustavo Fernandez-Castro ◽  
...  

Lung Cancer ◽  
2010 ◽  
Vol 68 (3) ◽  
pp. 438-445 ◽  
Author(s):  
Satoshi Igawa ◽  
Reiko Watanabe ◽  
Ichiro Ito ◽  
Haruyasu Murakami ◽  
Toshiaki Takahashi ◽  
...  

2019 ◽  
Vol 12 (1) ◽  
pp. bcr-2018-228017 ◽  
Author(s):  
Shilpy Jha ◽  
Suvradeep Mitra ◽  
Amit Kumar Adhya ◽  
Prasant Nayak

Urothelial carcinoma with villoglandular differentiation (UCVGD) is a rare aggressive variant of urothelial carcinoma. It is usually associated with high-grade urothelial carcinoma or rarely adenocarcinoma. There is only one other previous report of UCVGD associated with small cell neuroendocrine carcinoma of urinary bladder. We report the second case of UCVGD with small cell neuroendocrine carcinoma of urinary bladder in a 74-year-old non-smoker male patient. The mass was muscle invasive and also invaded the prostate. This entity needs to be confidently diagnosed due to its prognostic and therapeutic implications.


2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
P. Furtado ◽  
M. V. A. Lima ◽  
C. Nogueira ◽  
M. Franco ◽  
F. Tavora

Small cell carcinoma of the prostate is a rare neoplasm, with only a few series hitherto reported. A little less than half of the cases are associated with conventional acinar adenocarcinoma, which are usually high grade. Although consensus has not been reached, the majority of patients with small cell neuroendocrine carcinoma of the prostate have advanced disease at diagnosis and disproportionally low PSA levels compared to patients with conventional acinar adenocarcinoma. Treatment consists mainly of chemotherapy associated with surgery. Radiation therapy is reserved for selected cases. This study reviews the most up-to-date information on small cell carcinomas of the prostate.


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