Serous cystadenoma, NOS

Serous cystadenoma, NOS
Pancreas

Definition

Serous cystic pancreatic tumours are cystic epithelial neoplasms composed of glycogen-rich, ductular-type epithelial cells that produce a watery fluid similar to serum. Most are benign (serous cystadenomas), either serous microcystic adenoma or serous oligocystic adenoma. Only very rare cases exhibit signs of malignancy (serous cystadenocarcinoma)

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Bosman FT, Carneiro F, Hruban RH, Theise ND
WHO Classification of Tumours of the Digestive System
4th Edition
International Agency for Research on Cancer (IARC): Lyon 2010

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Serous microcystic adenoma is a rare neoplasm

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Morohoshi T, Held G, Klöppel G (1983)
Exocrine pancreatic tumours and their histological classification. A study based on 167 autopsy and 97 surgical cases.
Histopathology 7: 645-61

, the mean age at presentation is 66 years with a predominance in women (70%)

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Solcia E, Capella C, Kloppel G
Tumors of the Pancreas
3rd ed.
Armed Forces Institute of Pathology: Washington 1997

. The aetiology and pathogenesis of the neoplasm are unknown. The striking predilection for women suggests that sex hormones or genetic factors may play a role. An association with Von Hippel-Lindau disease

has been confirmed by recent genetic molecular investigations

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Vortmeyer AO, Lubensky IA, Fogt F, Linehan WM, Khettry U, Zhuang Z (1997)
Allelic deletion and mutation of the von Hippel-Lindau (VHL) tumor suppressor gene in pancreatic microcystic adenomas.
Am J Pathol 151: 951-6

. Approximately two thirds of patients exhibit symptoms related to local mass effects, including abdominal pain, palpable mass, nausea and vomiting, and weight loss

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Pyke CM, van Heerden JA, Colby TV, Sarr MG, Weaver AL (1992)
The spectrum of serous cystadenoma of the pancreas. Clinical, pathologic, and surgical aspects.
Ann Surg 215: 132-9

. The prognosis of patients with this neoplasm is excellent, since there is only a minimal risk of malignant transformation

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Mao C, Guvendi M, Domenico DR, Kim K, Thomford NR, Howard JM (1995)
Papillary cystic and solid tumors of the pancreas: a pancreatic embryonic tumor? Studies of three cases and cumulative review of the world's literature.
Surgery 118: 821-8

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Serous oligocystic adenoma is a benign neoplasm composed of few, relatively large cysts, lined by uniform glycogen-rich cuboidal epithelial cells. This neoplasm is very rare and there is no sex predilection. The aetiology of this neoplasm is not known. The most common symptom was upper abdominal discomfort or pain

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Solcia E, Capella C, Kloppel G
Tumors of the Pancreas
3rd ed.
Armed Forces Institute of Pathology: Washington 1997

. Other symptoms included jaundice and steatorrhoea. There is no evidence of malignant potential

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Egawa N, Maillet B, Schröder S, Mukai K, Klöppel G (1994)
Serous oligocystic and ill-demarcated adenoma of the pancreas: a variant of serous cystic adenoma.
Virchows Arch 424: 13-7

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Serous cystadenocarcinoma is a rare malignant cystic epithelial neoplasm composed of glycogen-rich cells. Serous cystadenocarcinomas are slowly growing neoplasms and palliative resection may be helpful even in advanced stages

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Yoshimi N, Sugie S, Tanaka T, Aijin W, Bunai Y, Tatematsu A, Okada T, Mori H (1992)
A rare case of serous cystadenocarcinoma of the pancreas.
Cancer 69: 2449-53