The coexistence of a pancreatic papillary cystic tumor with hairy cell leuk
emia is reported, To the best of our knowledge this association has never b
een published. A 41-year-old man diagnosed with hairy cell leukemia develop
ed a second malignancy that corresponded to a papillary cystic pancreatic t
umor. The patient underwent splenectomy and a tumoral surgical resection, a
nd is currently well at 21 months followup. A pathogenetic relationship bet
ween the two malignancies was not demonstrated. Hairy cell leukemia has bee
n reported to be associated to a great number of different second malignanc
ies. In contrast, only two papillary cystic tumors of the pancreas have bee
n described associated to a second neoplasm, a papillary thyroid carcinoma
and a colonic carcinoma, This unusual benign or low-grade malignant pancrea
tic tumor more commonly occurs in the tail of the pancreas of young women.
We want to stress the unusual presentation of this pancreatic tumor affecti
ng the head of the gland in a male patient as well as its coexistence with
a hairy cell leukemia.