A case of multicentric pancreatic mixed acinar-ductal carcinoma diagnosed by a yogurt-like cell clump flowing from the papilla of Vater
© The Author(s). 2017
Received: 25 March 2016
Accepted: 16 January 2017
Published: 23 January 2017
Histological confirmation is needed when the pancreatic lesions is uncertain for neoplastic or nonneoplastic. Current case with multicentric pancreatic carcinomas showing indefinite clinical images was successfully diagnosed by a biopsy of a novel object expelled from the papilla.
A 71-year-old male was referred because of elevated serum pancreatic enzymes. Computed tomography revealed an unclear low-density area in the pancreatic body without evidence of tumor and mild dilation of the upstream main pancreatic duct (MPD). Other images, including abdominal ultrasound, endoscopic ultrasound, and magnetic resonance imaging, did not demonstrate cancerous findings. Endoscopic retrograde cholangiopancreatography showed a crab-claw-like obstruction in the MPD. Surprisingly, the component constituting the obstruction was moved by contrast injection and spilled out of the papilla orifice as a yogurt-like white object. Biopsy of this object by histology revealed a cancer cell clump. Pancreatectomy was performed, and pathology of the resected pancreas showed multiple nodular tumors replacing the acini and extending into the MPD. These neoplasms histologically resembled mixed acinar-ductal carcinoma.
Current report presented a rare tumor with multicentric pancreatic lesions, preoperatively diagnosed by a biopsy of an uncommon substance.
KeywordsPancreas Mixed Acinar cell carcinoma Ductal adenocarcinoma Endoscopic retrograde pancreatography Biopsy Case report
In the diagnosis of solid pancreatic tumors, non-invasive image examinations, including ultrasonography (US), endoscopic ultrasonography (EUS), computed tomography (CT), and magnetic resonance imaging (MRI), are effective [1–3], but they have limitations for definitive diagnosis . EUS-guided fine needle aspiration (EUS-FNA) is an excellent tool to obtain histological materials, but it is effective only when the target is visible by EUS . Endoscopic retrograde cholangiopancreatography (ERCP) is also a useful diagnostic tool that enables procurement of pathological samples by aspirated or brush cytology and biopsy . These histological samples are usually pancreatic juice, mucin, brushing materials, lavage fluid, and biopsied pancreatic ductal tissues.
We present a rare case of pancreatic poorly differentiated carcinoma that could not be diagnosed by imaging examinations but could be diagnosed by a yogurt-like white substance that spilled out from the papilla of Vater during endoscopic retrograde pancreatography (ERP). The substance demonstrated unique histologic features.
Histology of another solitary tumor at the Pt was mostly the same as that of the tumor in the Pb (Fig. 3a). It was contained in a component of neuroendocrine tumor arranged in a ribbon-like pattern, with diffuse expression of synaptophysin and chromogranin A, and negative for Ki-67 (<2%) (Figs. 3e). Thus it was considered that the tumor metastasized in the neuroendocrine tumor in the Pt. The size of this tumor was 17 mm.
In addition to the above two lesions, multiple neoplastic cells nests, replacing the acini and creeping into the small duct, were microscopically detected in the proximal section of the specimen (Fig. 3a). Immunostaining of CK19 was positive within the neoplastic cells and progressed through the excretory ducts. These foci were not evident in the preoperative images, by rapid pathological diagnosis, or even in the macroscopic view of the sliced pancreatic specimens.
The overall pathological diagnosis by UICC criteria was mixed acinar-ductal carcinoma, pT2N0M0 pStage IB, with partial endocrine differentiation. Lymphatic permeation was positive, but no venous invasion or lymph nodal metastasis (0/17) were recognized within the resected materials. Neoplastic cells were floating in the pancreatic duct at the cut end, but the cells were not connective with the basal membrane. The patient received adjuvant chemotherapy with S-1 (120 mg/day) for 6 months and was followed up because of his advanced age.
Twenty-two months after the operation, recurrence occurred in the remaining pancreas. Again, excretion of the yogurt-like substance was recognized at the major papilla. Additional pancreaticoduodenectomy was performed, and the resected specimen showed the same but more poorly differentiated neoplasm spreading into the MPD and branch pancreatic ducts, 45 mm in size (Fig. 3f). A small nodule of hepatic metastasis was also found and removed during the operation. The patient again received S-1 as adjuvant chemotherapy, and is still alive until 13 months after the additional surgery with progression of liver metastasis.
We report here a case of multiple mixed acinar-ductal carcinoma of the pancreas demonstrating mostly atypical images and partially invisible by imaging, which was difficult to diagnose without obtaining histological evidence. The crucial pathological sample was a yogurt-like cell clump that flowed out of the papilla during ERP. This unique phenomenon helped us diagnose and treat this rare pancreatic tumor. To the best of our knowledge, such an occurrence has not yet been reported.
Histological samples from pancreatic cancer, obtained by EUS-FNA, forceps biopsy, aspiration, and/or brushing cytology, are usually solid tissue, liquid, or sometimes mucinous. The current sample, a “yogurt-like” or”gel-like” whitish substance, was distinctive. Presumably, the white color was due to its high cellularity, and the viscosity reflected the intercellular adhesion (Figs. 2d, 3b). Histology of the current tumor resembled pancreatic ACC, which characteristically shows a sheet-like spreading of neoplastic cells without dense interstitial tissues and sometimes accompanies partial differentiation to ductal dilatation (about 30%) [6, 7], neuroendocrine neoplasm (14–27%) [8–12], and intraductal extension (about 10%) . ACC with pancreatic ductal ingrowth typically shows a nodular or papillary progression pattern, similar to that of intraductal papillary mucinous neoplasm , as seen in the current case. This rare but characteristic histology of the MPD may have been reflected in the yogurt-like substance expelled during ERP.
The current case pathologically demonstrated an ACC-like structure by HE staining, but it was negative for trypsin, Bcl-10, and lipase. The sensitivity of this type of immunostaining for ACC is reported to be fairly high: 95–100% for trypsin [12, 14, 15], 82–100% for Bcl-10 [12, 16, 17], and 26–45% for lipase [12, 14, 15]. Our literature survey revealed no cases of ACC showing null expression of Bcl-10, lipase, and trypsin. On the other hand, CK7, CK19, CEA, CA19-9, and MUC1 are highly sensitive for ordinary pancreatic ductal adenocarcinoma (sensitivity: 96%, 100%, 85%, 75%, and 88%, respectively) [10, 18–21], and CK7, CK19, and MUC1 were partially positive in the current tumor. These findings suggested that although HE section indicated ACC, the phenotype of the ACC were vanishing and phenotype of ductal adenocarcinoma appeared in some severe atypicus area. Hence, we pathologically classified the current tumor as mixed acinar-ductal carcinoma.
Although the histology of ACC-like component was quite typical for ACC, excepting the immunohistochemistry, imaging of the current tumor was not typical, as ACC usually forms a huge and exophytic, well-circumscribed, hypovascular mass [22–26]. Toll et al. report a similar case of ACC showing multifocal intraductal extensions and replacements of normal pancreatic parenchyma throughout the entire pancreas . The current case demonstrated the same progression pattern, and it was considered as a reason for the poor tumor definition in the preoperative images.
In summary, we report a rare case of multifocal mixed acinar-ductal carcinoma of the pancreas. The carcinoma showed atypical imaging findings and was diagnosed by pathological examination of a yogurt-like cell clump that flowed out of the papilla during ERP.
Acinar cell carcinoma
Diffusion weighted image
Endoscopic retrograde cholangiopancreatography
Endoscopic retrograde pancreatography
EUS-guided fine needle aspiration
18F-Fluorodeoxyglucose-positron emission tomography
Hematoxylin and eosin
Main pancreatic duct
Magnetic resonance imaging
Availability of data and materials
Data described in this report is not exhibited in public.
YK and HM contributed to the concept and design of this study. YK, HM, KS, SU, SH, KU, and AT contributed to treatment of the patient. YK HM, and HO contributed to writing the manuscript. All authors contributed to the manuscript. All authors have approved the final draft submitting for publication.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.
This study was approved by the Institutional Review Board of Shizuoka Cancer Center, Japan.
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