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Pancreas

Tumors

Serous cystadenoma


Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 23 November 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Benign tumor, usually in women, composed of small cystic spaces lined by small cuboidal cells with clear cytoplasm (glycogen) (WHO)
● Also called microcystic adenoma, glycogen rich cystadenoma

Clinical features
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● Mean age 66, 70% women, associated with von Hippel Lindau syndrome
● Symptoms: none, local discomfort/pain, obstruction if in pancreatic head; may cause diabetes if tumor destroys enough islets
● Excision is almost always curative
● One malignant case reported, histologically benign, but metastasized to stomach and liver and invaded spleen (Am J Surg Pathol 1989;13:61)

Case reports
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● 47 year old woman and 53 year old man with coexisting pancreatic endocrine neoplasms (Am J Surg Pathol 1996;20:471, Arch Pathol Lab Med 2003;127:1369)
● Coexisting pancreatic adenocarcinoma (Am J Surg Pathol 1990;14:352)

Gross description
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● Large (mean 11 cm) multiloculated mass, sharply outlined, cysts filed with clear fluid
● Spongy
● Resembles infantile polycystic kidney
● Often has central stellate scar
● Rarely is multicentric, usually in tail or body
● Either macrocystic (megacystic, oligocystic, usually < 10 cysts, Hum Pathol 1992;23:871) or microcystic (1-3 mm)
● Large tumor size and head location predict aggressive behavior

Gross images
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Circumscribed, sponge-like cut surface with central stellate scar


Well demarcated cystic lesion with central stellate scar in tail of pancreas; cysts are filled with watery fluid and separated by thin septa


76 year old man with incidental pancreatic mass - contributed by Dr. Hanni Gulwani, New Delhi (India)


Solid variant

Micro description
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● Small cystic spaces lined by small cuboidal cells with clear cytoplasm (glycogen), minimal mucin, myoepithelial layer present, round hyperchromatic central nuclei
● Minimal papillae, has islets between lobules which may calcify (radiating pattern), tumor is vascular (seen by selective angiography)
● Occasionally has papillary features (Arch Pathol Lab Med 2001;125:1591)
● Rarely oncocytic change; rarely massive cystic degeneration resemble pseudocyst (Am J Surg Pathol 2012;36:726)
● Fluid has low CEA content compared to mucinous cystic neoplasms
● Solid variant: rare, similar microscopically to serous cystadenoma but no cystic spaces; cells arranged in nests, sheets and trabeculae separated by thick fibrous bands; has PAS+ cytoplasm (glycogen); may histologically resemble sugar tumor, clear cell carcinoma, clear cell pancreatic endocrine tumor, renal cell carcinoma, but benign behavior (Am J Surg Pathol 1996;20:1401)

Micro images
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Cuboidal cells with watery clear cytoplasm (due to glycogen), with round dense nuclei but no atypia; sparse stroma between cysts, cystic content has no significant staining


Cysts exhibit marked size variation (larger at periphery, smaller and uniform centrally)


Tumor cell cytoplasm is PAS+ due to glycogen


Intracystic papillary projection is formed by regular cuboidal cells


Cystic lesion is well demarcated from adjoining pancreas by small fibrous band containing some vessels


Tumor cell cytoplasm is PAS+ due to glycogen


Various images


76 year old man with incidental pancreatic mass - contributed by Dr. Hanni Gulwani, New Delhi (India)


Papillary variant


Solid variant


Serous cystadenocarcinoma
Top: 71 year old patient with metastasizing tumor resembling a serous cystadenoma, cysts of variable size, and focal papillary projections
Bottom: Lymph node metastasis of above tumor


Positive stains
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● EMA, low molecular weight keratin, PAS without diastase
● Alpha-inhibin, neuron-specific enolase, and MUC6 (Am J Surg Pathol 2004;28:339)
● Synaptophysin (92%), CD56 (75%, Appl Immunohistochem Mol Morphol 2011;19:141)

Negative stains
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● CEA, trypsin, chromogranin, S100, desmin, vimentin, Factor VIII

Electron microscopy description
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● Prominent microvilli, glycogen granules, epithelial cells connected by occluding junctions and belt desmosomes resting on a basement membrane
● No neurosecretory or zymogen granules

Electron microscopy images
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Cytoplasm has multiple glycogen particles, some mitochondria and multivesicular bodies, but no microvilli are present at the apical surface

Molecular description
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● May be aneuploid (Arch Pathol Lab Med 1991;115:563)

Cystic lesion is well demarcated from adjoining pancreas by small fibrous band containing some vessels

Differential diagnosis
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Lymphangioma: Factor VIII+, lymphocytes present, epithelial cells lack glycogen)
Mucinous cystic neoplasms: larger cystic spaces, CEA+, mucin+
Pancreatic pseudocyst: some serous cystadenomas have focal cystic degeneration resembling a pseudocyst (Am J Surg Pathol 2012;36:726)
Solid pseudopapillary tumor: pseudopapillary features

Additional references
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Am J Surg Pathol 1988;12:251, Am J Surg Pathol 1986;10:365

End of Pancreas > Tumors > Serous cystadenoma


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