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Year : 2017  |  Volume : 30  |  Issue : 4  |  Page : 1254-1256

Acinar cell carcinoma of pancreas mimicking as pseudocyst – Report of an interesting case

1 Department of Pathology, Sagar Dutta Medical College, Kolkata, India
2 Department of Pathology, Bankura Sammilani Medical College, Bankura, India
3 Department of Pathology, North Bengal Medical College, Darjeeling, West Bengal, India

Date of Submission14-Apr-2017
Date of Acceptance19-Jul-2017
Date of Web Publication04-Apr-2018

Correspondence Address:
Adhikari Anindya
Vil-Basudevpur, P.O. Banipur, P.S. Sankrail 711304, Howrah, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mmj.mmj_295_17

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Acinar cell carcinoma of the pancreas is a rare and aggressive malignancy, comprising less than 5% of all pancreatic neoplasms. Middle-aged men are commonly affected. In our case, a 35-year-old man presented with epigastric fullness, anorexia, and weight loss for 3 months. Computed tomogram scan of the abdomen revealed a cystic lesion (93 × 83 × 62 mm) over the head of pancreas, suggestive of pancreatic pseudocyst. Serum amylase and lipase levels were 25 710 and 292.50 U/l, respectively. Excision followed by histopathological examination revealed acinar cell carcinoma of pancreas. The patient developed pancreatic fistula postoperatively. Chemotherapy with 5-fluorouracil was initiated. There was no residual tumor. The patient remained disease free for 8 months postoperatively.

Keywords: acinar cell carcinoma, pancreas, pseudocyst

How to cite this article:
Kumar MP, Anindya A, Datta PR, Kalyan K. Acinar cell carcinoma of pancreas mimicking as pseudocyst – Report of an interesting case. Menoufia Med J 2017;30:1254-6

How to cite this URL:
Kumar MP, Anindya A, Datta PR, Kalyan K. Acinar cell carcinoma of pancreas mimicking as pseudocyst – Report of an interesting case. Menoufia Med J [serial online] 2017 [cited 2020 Apr 2];30:1254-6. Available from: http://www.mmj.eg.net/text.asp?2017/30/4/1254/229214

  Introduction Top

Pancreatic carcinoma with acinar cell differentiation is rare, comprising less than 5% of all pancreatic neoplasms[1]. Although children may be affected, middle-aged or elderly males are mainly victimized[2]. Usually the patient presents with abdominal mass, icterus, and weight loss. Acinar cell carcinomas (ACCs) are associated with increased serum lipase level and may manifest in the classic presentation as the Schmid triad of subcutaneous fat necrosis, polyarthritis, and eosinophilia[3]. ACC is associated with increased levels of tumor markers such as α-fetoprotein and carcinoembryonic antigen[4]. These are aggressive tumors with early metastasis to the regional lymph nodes and liver. However, at times, it has a benign appearance on histology as well as on radiology. This case is being reported as it presented as pancreatic pseudocyst radiologically and serologically.

  Case History Top

A 35-year-old man presented with upper abdominal distension, anorexia, and weight loss for 3 months. On palpation, a firm mass with ill-defined borders was felt in the epigastric region. Ultrasonography showed a cystic mass over the head of pancreas. Computed tomography scan of the abdomen [Figure 1]a revealed a cystic lesion (93 × 83 × 62 mm) over the head of pancreas, suggestive of pancreatic pseudocyst. Hemogram report was within normal limits. His serum amylase level was 25 710 U/l (normal range: 22–80 U/l) and lipase level was 292.50 U/l (normal range: ≤64 U/l); both levels were markedly raised. The cyst was excised and measured 10 × 8 × 4 cm on grossing [Figure 1]b. On cut section, a partly solid cystic growth with papillary excrescences was noted. Histopathological examination of the mass [Figure 2] showed a well-circumscribed tumor with cells arranged in organoid fashion. Those cells were slightly pleomorphic, were round to oval in shape, had granular cytoplasm, and had basally placed nuclei with clumped chromatin. Clear cell changes were also noted focally. The tumor also showed a varied histopathological pattern of acinar, solid, trabecular, and papillary structures. Some of the cells showed prominent eosinophilic nucleoli. There were focal areas of hemorrhage and necrosis. Lymphovascular invasion of tumor cells was noted. The clear cells were positive for periodic acid–Schiff stain. Immunohistochemistry showed positivity for cytokeratin, whereas chromogranin A and synaptophysin were negative. The patient developed postoperative pancreatic fistula for which he underwent distal pancreatectomy with splenectomy. In total, two lymph nodes dissected were free from tumor. Chemotherapy with five cycles of 5-fluorouracil was given over a total period of 8 weeks. No residual tumor could be found. The patient remained disease free till 8 months postoperatively.
Figure 1: (a) CT scan image of the tumor. (b) Gross appearance of the tumor.

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Figure 2: (a) Sheets of tumor cells with abundant cytoplasm, H and E, X10, (b) Tumor emboli within blood vessel, H and E, X10, (c) Tumor cells are separated by fibro collagenous tissue, H and E, X10, (d) Tumor cells are in sheets and syncytial pattern, H and E, X40.

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  Discussion Top

ACC is a rare and aggressive pancreatic neoplasm of middle-aged or elderly males which may mimic benign lesion like pancreatic pseudocyst both radiologically and serologically. These tumors may secrete lipase and other digestive enzymes, which may result in widespread subcutaneous fat necrosis and arthralgia[3]. Our patient presented with elevated serum lipase level. Hypoglycemia may occur secondary to tumor secretion of insulin and insulin-like growth factor[5]. Radiographic findings generally identify a solid, well-demarcated, hypovascular mass[6]. In this patient, however, computed tomography scan showed a thick-walled cystic lesion resembling a pancreatic pseudocyst.

The neoplasm has a propensity for the pancreatic head, although rarely multifocal tumors may be present. Grossly, the tumors are usually large but well circumscribed. Microscopically, ACCs are cellular neoplasms that characteristically lack the desmoplastic stroma commonly seen with ductal adenocarcinomas. The most common histologic patterns are acinar and solid, whereas the less common patterns include glandular, trabecular, papillary, or intraductal pattern[7]. The tumor cells are usually periodic acid–Schiff positive, diastase resistant. The neoplastic cells are immunoreactive with α1-antitrypsin, chymotrypsin, amylase, and lipases[1],[8]. A minor population of endocrine cells can be seen scattered throughout the tumor which can be positive for neuroendocrine markers like chromogranin and synaptophysin. Immunohistochemistry results for chromogranin A and synaptophysin were negative in our case.

The best treatment option for localized tumors is surgical resection. However, there is a high rate of recurrence after complete surgical resection, suggesting a role of micrometastases even in localized disease[9]. In cases of unresectable tumor with presence of distant metastases, chemotherapy with or without radiation to the pancreas has been used[10]. In our case, although the tumor was completely resectable, adjuvant chemotherapy was administered to prevent recurrence. Present knowledge regarding the efficiency of chemoradiotherapy for ACC is limited, mainly because of its low incidence. The combination of oxaliplatin-based chemotherapy appears most effective for locally advanced or metastatic forms. For patients with unresectable yet locally confined disease, radiotherapy may be one of the treatment options. ACC is associated with a better prognosis than ductal adenocarcinoma at all stages of disease. The predictors of poor prognosis in ACC include the following: age greater than 60 years, markedly elevated serum lipase level, and tumors larger than 10 cm in diameter.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Rosen PP. Metastases in the breast from non-mammary malignant neoplasms. In: Rosen PP, editor. Rosen's breast pathology. Philadelphia, PA: Lippincott Williams and Wilkins; 2001. 689–701.  Back to cited text no. 4
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Ellis IO, Pinder SE, Lee A. Tumors of the breast. In: Fletcher CDM, editor. Diagnostic histopathology of tumors. 3rd ed. China: Churchill Livingstone Elsevier; 2007. 1. 960.  Back to cited text no. 8
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