-2 :: Case - 7020 :: - Mucinous Cystic Neoplasm of the Pancreas
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Tx and Followup:
Imaging guided biopsy of the lesion was considered, and declined secondary to inaccessibility. Patient will decide whether to undergo laproscopy.

Additional Discussion:
Finding of such markedly increased CA 19-9, along with pathologic evidence of metastatic disease nearly seal the diagnosis. The mucinous cystic pancreas tumors favor the tail. Ductal neoplasms tend to favor the head and body, and contain more soft tissue component. Imaging features in this case are highly suggestive of the diagnosis. There is no surrounding inflammation to suggest pseudocyst.

Discussion Author: Andrew E Simays, MD

Mucinous Csytic Neoplasm of the Pancreas; AKA-macrocystic adenoma, mucinous cystadenoma, cystadenocarcinoma.

Frequency- 10% of pancreatic cysts, 1% of pancreatic neoplasms

Mean Age- 50 years ( 20-95 ), 50% between 40-60 years old
M:F 1:19

Pathology: Large smooth round/lobulated multiloculated cystic mass encapsulated by a layer of fibrous connective tissue

Histology: similar to biliary and ovarian mucinous tumors; cysts lined by tall columnar, mucin-producing cells subtended by a densely cellular mesenchymal stroma (reminiscent of ovarian stroma), often in papillary arrangement

Location: often in the tail (90%), body, infrequently in head

-well-demarcated thick-walled mass, mean 10-12 cm
-multi/unilocular large cyss >2 cm with thin septa <2 mm
* a tumor with <6 cysts of >2 cm in diameter is in 93-95% a mucinous cystic neoplasm
-amorphous discontinuous peripheral mural calcifications (10-15%)
-solid papillary excresences protrude into the interior of the tumor
-hypovascular mass with sparse neovascularity
-great propensity for invasion of adjacent organs
U/S: -cysts may contain low level echoes; echogenic mural calcifications if present
CT: -internal septations may not be seen without contrast enhancement; cysts with HU of water, different cysts with different HU's; enhance of cyst walls
MRI: -high T2 from mucinous component
Angio: -predominantly avascular mass, small area of blush in cyst wall and solid components, mass effect on adjacent arteries and veins by cysts
Mets: -round thick walled cystic lesions in the liver
Prognosis: -invariable transformation into cystadenocarcinoma
Rx: -complete surgical excision, 5 yr survival is 74-90%
DDx: pseudocyst, lymphangioma/hemangioma, variants of ductal adenocarcinoma, solid and cystic papillary epithelioid neoplasm, cystic islet cell tumor, cystic met, atypical serous cystadenoma, sarcoma, infection (amebiasis, echinococcus0

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