CLINICAL CASES   

Gazzetta Medica Italiana Archivio per le Scienze Mediche 2000 June;159(3):107-9

Copyright © 2000 EDIZIONI MINERVA MEDICA

language: Italian

Pancreatic granulocytic sarcoma. A case report

Pellicanò S. ¹, Calzone R. ², Dattola O. L. ¹, Mandica D. ², Terra L. ¹

¹ Regione Calabria - ASL n. 5, Ospedale «S. Giovanni di Dio», Divisione di «Malattie Infettive», Crotone; ² Regione Calabria - ASL n. 5, Ospedale «S. Giovanni di Dio», Dietista

Gra­nu­lo­cyt­ic sar­co­ma (GS) is a ­tumor ­formed by mye­loid pre­cur­sors in an extram­e­dul­lary ­site and ­most fre­quent­ly asso­ciat­ed ­with ­acute myel­o­blas­tic leu­ke­mia or ­with oth­er mye­lop­ro­lif­er­a­tive dis­or­ders. It ­occurs rel­a­tive­ly com­mon­ly in Afri­ca and has ­been report­ed to ­affect 10-25% of ­black chil­dren pre­sent­ing ­with ­acute myel­o­blas­tic leu­ke­mia. ­Bone, ­skin, ­lymph ­nodes and ­soft tis­sue are the ­most com­mon ­site. Involve­ment of oth­er ­organs has ­been rare­ly report­ed includ­ing ­female gen­i­tal ­tract, ­lung, gas­troin­tes­ti­nal ­tract, ­orbit and tes­tis. Gra­nu­lo­cyt­ic sar­co­ma in non-leu­kem­ic ­patients is ­often asymp­to­mat­ic, fre­quent­ly mis­di­ag­nosed or mis­tak­en for malig­nant lym­pho­ma. Some­times it is pre­sent­ed as a reac­ti­va­tion of chron­ic mye­loid leu­ke­mia ­after allo­gen­ic mar­row trans­plan­ta­tion. Uncom­mon mod­ifi­ca­tions of chro­mo­some 5 ­long arm or trans­lo­ca­tion 9-11 and p22-q23 in malig­nant ­cells ­could ­explain the imma­ture eosin­o­phil reac­tion in ­this ­tumor. An extreme­ly ­rare ­case of gra­nu­lo­cyt­ic sar­co­ma of pan­cre­as in the ­absence of demon­strable hemat­o­log­ic dis­ease is ­described. The ­patient was a 39-­year-old man admit­ted ­because of jaun­dice. Ultra­so­nog­ra­phy and com­put­ed tomog­ra­phy scan­ning of the abdo­men dis­closed a hypo­in­tense ­area ­about 3,52 cm in diam­e­ter ­near the ­head of the pan­cre­as. On the ­basis of ­these find­ings car­ci­no­ma was sus­pect­ed and the ­patient was treat­ed ­with pan­creat­ic-cephal­ic-duod­e­nec­to­my but his­to­log­ic diag­no­sis was gra­nu­lo­cyt­ic sar­co­ma. The ­patient had nor­mal ­bone mar­row and periph­er­al ­blood for a fur­ther six ­months ­from the ­onset of ­acute myel­o­blas­tic leu­ke­mia. It is impor­tant to ­include ­this ­entity in the dif­fe­ren­tial diag­no­sis of: gas­troin­tes­ti­nal bleed­ing par­tic­u­lar­ly in a ­patient pre­vi­ous­ly diag­nosed ­with mye­loid leu­ke­mia or pre­leu­ke­mia; ­breast ­tumors, espe­cial­ly in ­tumors ­with dif­fuse pro­life­ra­tion of ­small ­tumor ­cells; scap­u­lar mass­es dur­ing child­hood. Gra­nu­lo­cyt­ic sar­co­ma of pan­cre­as is an uncom­mon ­site; ­from ­this ­report it ­would ­appear ­that it may pre­cede oth­er man­i­fes­ta­tions of leu­ke­mia.