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  Table of Contents    
CASE REPORT  
Year : 2015  |  Volume : 58  |  Issue : 1  |  Page : 93-95
Pancreatoblastoma in an adult


Department of Pathology, The First Affiliated Hospital and College of Basic Medical Sciences of China Medical University, Shenyang, China

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Date of Web Publication11-Feb-2015
 

   Abstract 

Pancreatoblastoma is a malignant pancreatic tumor that rarely occurs in adults. We report a case of an adult female with pancreatoblastoma. A mass was detected in the pancreatic head using computed tomography and ultrasonography. The clinical diagnosis was a solid-pseudopapillary neoplasm of the pancreas. However, after the operation, the final diagnosis was pancreatoblastoma, which showed two lines of differentiation: Acinar differentiation and squamoid corpuscles. The patient is currently in good condition.

Keywords: Differentiation, immunohistochemistry, pancreatoblastoma

How to cite this article:
Zhang D, Tang N, Liu Y, Wang EH. Pancreatoblastoma in an adult. Indian J Pathol Microbiol 2015;58:93-5

How to cite this URL:
Zhang D, Tang N, Liu Y, Wang EH. Pancreatoblastoma in an adult. Indian J Pathol Microbiol [serial online] 2015 [cited 2020 Apr 9];58:93-5. Available from: http://www.ijpmonline.org/text.asp?2015/58/1/93/151199



   Introduction Top


Pancreatoblastoma is an uncommon malignant epithelial tumor, mainly occurring in children. It was formerly known as infantile carcinoma of the pancreas and accounts for approximately 25% of tumors in children <10 years of age. The male-to-female ratio is 1.3:1. The tumor is very rare in adults, and only 27 cases have been reported thus far (USA, 14 cases; Spain, 1 case; Belgium, 1 case; Italy, 4 cases; France, 2 cases; Germany, 2 cases; Japan, 2cases; and China, 1 case). [1],[2],[3],[4],[5],[6],[7],[8]

We present a case of an adult female who was hospitalized because of abdominal pain. Vital signs were normal, and the levels of serum tumor markers were within reference ranges. A mass was detected in the pancreatic head on imaging studies. A preoperative diagnosis of a solid-pseudopapillary neoplasm was made, but the pathological result indicated pancreatoblastoma. Postoperative chemotherapy was performed. The patient is in good condition at present.


   Case Report Top


A 30-year-old woman was hospitalized because of abdominal pain, nausea, and vomiting, but no jaundice. Defecation was recorded. No abdominal mass was noted palpation.

The patient's vital signs on admission were, as follows: Temperature, 36.5°C; pulse, 74/min; and blood pressure, 120/80 mmHg. The results of the serological examination were, as follows: alpha-feto protein (AFP) level, 4.23 ng/mL (reference, 0.00-35.00); carcinoembryonic antigen (CEA) level, 1.50 ng/mL (reference, 0.00-4.00); cancer antigen 125 (CA125) level, 17.74 ng/mL (reference, 0.00-35.00); and cancer antigen 19-9 (CA19-9) level, 24.11 ng/mL (reference, 0.00~30.00).

Abdominal ultrasonography showed around mass with low-to-moderate echogenicity in the pancreatic head beside the superior mesenteric vein. The mass was 2.5 cm in diameter. Computed tomography showed an uncinate lesion (approximately 2.5 cm), which was a solitary nodule [Figure 1].
Figure 1: Computed tomography shows a primary lesion (white circle) in the pancreatic head


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Comprehensive analysis of the imaging and clinical results indicated a preoperative diagnosis of solid-pseudopapillary neoplasm of the pancreas.

The patient underwent exploratory laparotomy, but no nodules were found in the liver, abdominal wall, or pelvic cavity. Bile duct dilatation was not detected. The mass (diameter, 2.5 cm) located in the pancreatic uncinate was soft, well circumscribed, but encapsulated. Pancreatic tissue covering the tumor was cut, and then the integral tumor was resected. The frozen sections of the resected tumor specimen indicated a diagnosis of solid-pseudopapillary neoplasm.

Grossly, the cut surface of the tumor was gray and separated into small nodules by fibrous bands. A small amount of necrotic tissue was visible locally and a cyst measuring 1.5 cm in diameter was located in the tumor. The cystic wall was dark red and had some areas of ulcerations.

Microscopy showed abundant epithelioid tumor cells, arranged in well-defined islands, separated by fibrous bands. Then, a "geographic" appearance was observed. Tumor cells exhibited two lines of specialized differentiation: Acinar differentiation and squamoid corpuscles [Figure 2]. The tumor cells with acinar differentiation were polygonal, similar in size and shape, and formed solid nests or surrounded small luminal spaces. The nucleoli were prominent. Squamoid corpuscles were scattered and formed large islands of epithelioid cells. Keratinization was present, and nucleoli were inconspicuous.
Figure 2: Histopathology. The tumor cells with acinar differentiation formed solid nests (a) or surrounded small luminal spaces (b) the other specialized differentiation squamoid corpuscles (c) fibrous bands visible (d)


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Immunohistochemical staining showed a positive reaction for epithelial markers cytokeratin, α-1-antichymotrypsin, AFP and the neuroendocrine marker chromogranin A (CgA) [Figure 3], but the negative reaction for vimentin and CEA. The Ki-67 (MIB-1) labeling index was approximately 5%.
Figure 3: Immunohistochemistry. Positivestaining for cytokeratin (a) α-1-antichymotrypsin (b) alpha-feto protein (c) and chromogranin A (d)


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The final pathologic diagnosis was pancreatoblastoma.

The patient was treated with plus vinblastine plus bleomycin (PVB) chemotherapy (cisplatin, vincristine, and bleomycin) postoperatively. The patient has been followed up and was in good condition without a recurrent tumor or metastases.


   Discussion Top


Pancreatoblastoma was first proposed by Horie et al. in 1977. [9] The majority of cases occur in children under the age of 10 years (median age, 4 years). Pancreatoblastoma comprises only 0.5% of pancreatic nonendocrine tumors occurring in children. This tumor is more common in Asians than in the Caucasian population. [10] Very few lesions occur in adults. [1],[4] To our knowledge, only 27 cases of pancreatoblastoma in adults have been reported thus date. Among these, there were 14 cases in the United States, 10 in Europe, and only three in Asia, which is different from the geographical distribution of pancreatoblastoma in children. We suspect that this distribution does not represent the actual prevalence of adult pancreatoblastoma, but may be caused by potential misdiagnosis owing to the lack of expertise among doctors in Asia.

Pancreatoblastoma can arise in any part of the pancreas. Approximately half of the tumorsare located in the pancreatic head. [6]

The clinical features of pancreatoblastoma are nonspecific. Common symptoms and signs include abdominal pain, weight loss, vomiting, and diarrhea; jaundice occasionally occurs .

Alpha-feto protein is a common tumor marker for the diagnosis of pancreatoblastoma. [4] A large proportion of patients have elevated levels of serum AFP, but higher levels in neonates may be normal. The level of serum AFP was not elevated in this case. [5]

Pancreatoblastoma should be distinguished from other pancreatic tumors (e.g., acinar cell carcinoma and solid-pseudopapillary neoplasm). The ultimate diagnosis of pancreatoblastoma depends on pathologic examination.

Pancreatoblastoma grows slowly, and its size varies from 1.5 cm to 20 cm. The tumor is a well circumscribed, solitary solid nodule, which is separated by fibrous bands, and usually very large at the time of diagnosis. [4] Necrosis, calcification, and cystic degeneration may be found. [4]

Microscopically, the epithelial elements are highly cellular, arrange in a well-defined island separated by fibrous bands, and show a "geographic" appearance.

The tumor cells show two lines of differentiation: Acinar differentiation and squamoid corpuscles. [8] Tumor cells with acinar cell differentiation are polygonal and constitute solid areas or acinar-like structures. The nucleus with a single prominent nucleolus shows moderate atypia. The tumor cells with acinar differentiation are difficult to distinguish from acinar cell carcinoma or a solid-pseudopapillary neoplasm. Therefore, squamoid corpuscles are the characteristic feature for the identification of pancreatoblastoma and acinar cell carcinoma (or a solid-pseudopapillary neoplasm). Large epithelioid cells islands or whorled nests of spindled cells, as well as keratosis, may be visible. In contrast to the surrounding cells, cells in the squamoid corpuscle has large oval nuclei, with inconspicuous nucleoli.

A number of immunohistochemical features are associated with pancreatoblastomas. It expresses cytokeratin, and tumor cells with acinar differentiation are focally positive for trypsin, chymotrypsin, and esterase; and the staining is, usually, limited to the apical cytoplasm. Tumor cells show positive staining for markers of endocrine differentiation (CgA or synaptophysin) in more than two-thirds of cases, and express markers of ductal differentiation (CEA, DUPAN-2, or B72.3) in approximately half of the cases. In cases with elevated serum AFP levels, immunohistochemical staining of AFP may be positive. However, the above indicators are not specific for pancreatoblastoma.

The optimal treatment of pancreatoblastomais surgical resection. [6] The surgical plan should be based on tumor location, size, and distant metastasis. Pancreatoblastoma is sensitive to chemotherapy, and the PVB regimen or cisplatin plus idarubicin should be used.

Young patients have a good prognosis, with a long-term survival after surgical resection. In contrast, adult patients have poor prognosis [2] and the overall survival rate is approximately 50%.

Pancreatoblastoma may invade adjacent organs, and metastasis is detected in 17-35% of patients at the time of diagnosis. The liver is the most common site of metastases, [2] followed by lymph nodes and lungs. [3]

Pancreatoblastoma can potentially be cured. After early diagnosis and effective treatment, the prognosis for pancreatoblastoma is significantly better than that of pancreatic cancer.


   Acknowledgment Top


This work was supported by the National Natural Science Foundation of China (no.81101779 to Di Zhang, MD; no. 30870977 and 81071905 to En-HuaWang, MD).

 
   References Top

1.
Salman B, Brat G, Yoon YS, Hruban RH, Singhi AD, Fishman EK, et al. The diagnosis and surgical treatment of pancreatoblastoma in adults: A case series and review of the literature. J Gastrointest Surg 2013;17:2153-61.  Back to cited text no. 1
    
2.
Hammer ST, Owens SR. Pancreatoblastoma: A rare, adult pancreatic tumor with many faces. Arch Pathol Lab Med 2013;137:1224-6.  Back to cited text no. 2
    
3.
Balasundaram C, Luthra M, Chavalitdhamrong D, Chow J, Khan H, Endres PJ. Pancreatoblastoma: A rare tumor still evolving in clinical presentation and histology. JOP 2012;13:301-3.  Back to cited text no. 3
    
4.
Savastano S, d'Amore ES, Zuccarotto D, Banzato O, Beghetto M, Famengo B. Pancreatoblastoma in an adult patient. A case report. JOP 2009;10:192-5.  Back to cited text no. 4
    
5.
Cavallini A, Falconi M, Bortesi L, Crippa S, Barugola G, Butturini G. Pancreatoblastoma in adults: a review of the literature. Pancreatology 2009;9:73-80.  Back to cited text no. 5
    
6.
Charlton-Ouw KM, Kaiser CL, Tong GX, Allendorf JD, Chabot JA. Revisiting metastatic adult pancreatoblastoma. A case and review of the literature. JOP 2008;9:733-8.  Back to cited text no. 6
    
7.
Rajpal S, Warren RS, Alexander M, Yeh BM, Grenert JP, Hintzen S, et al. Pancreatoblastoma in an adult: Case report and review of the literature. J Gastrointest Surg 2006;10:829-36.  Back to cited text no. 7
    
8.
Klimstra DS. Nonductal neoplasms of the pancreas. Mod Pathol 2007;20 Suppl 1:S94-112.  Back to cited text no. 8
    
9.
Horie A, Yano Y, Kotoo Y, Miwa A. Morphogenesis of pancreatoblastoma, infantile carcinoma of the pancreas: Report of two cases. Cancer 1977;39:247-54.  Back to cited text no. 9
    
10.
Saif MW. Pancreatoblastoma. JOP 2007;8:55-63.  Back to cited text no. 10
    

Top
Correspondence Address:
Prof. En-Hua Wang
Department of Pathology, The First Affiliated Hospital and College of Basic Medical Sciences of China Medical University, Shenyang
China
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Source of Support: This work was supported by the National Natural Science Foundation of China (no.81101779 to Di Zhang, MD; no.30870977 and 81071905 to En-HuaWang, MD)., Conflict of Interest: None


DOI: 10.4103/0377-4929.151199

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    Abstract
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