Author/Editor		Štabuc-Šilih, Mirna; Lamovec, Janez; Štabuc, Borut
Title		Vnetni psevdotumor orbite z malignim potekom
Translated title		Inflammatory orbital pseudotumor with a malignant course
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 71, št. Suppl 2
Publication year		2002
Volume		str. II-71-4
Language		slo
Abstract		Background. Inflammatory pseudotumor or inflammatory myofibroblastic tumor is a benign, tumor forming proliferation of myofibroblasts with a potential for recurrence and persistent local growth, in some respects similar to fibromatosis. The lesion does not metastasize except exceedingly rarely. Though inflammatory pseudotumor is not a malignant lesion, it may occasionally behave aggressively, like fibromatosis or fibrosarcoma, and threatens patient's life. Patients and methods. Patient D. E., born in 1974, in the second month of pregnancy, was admitted to our Clinic in 1996 because of the protrusion of the left eye. She has often had evening headaches for the last 5 years. She has previously been treated for epistaxis. CT showed a tumorous formation in the ethmoidal labyrinth, which grew into adjacent structures, destroyed the neighboring bones and extended into extradural space of cranial cavity. The patient was operated upon at the ENT clinic where a tumor of a "woman's fist" size was excised. It showed expansive, non-infiltrative growth. Microscopically, it was an ifjlammatory pseudotumor. For one year after the operation the patient was in good health, without protrusion. After that period, she had repeated recurrences, reoperations, she was irradiated and received chemotherapy. In spite of all treatment, the tumor locally progressed and the patient died in December 2000. Conclusions. Although repeated histopathologic examinations showed a benign inflammatory pseudotumor, because of the proximity of vital structures the course of disease was malignant just like in a malignant neoplasm.
Summary		Izhodišča. Vnetni (inflamatorni) psevdotumor ali inflamatorni miofibroblastni tumor je benigno proliferiranje miofibroblastov, ki tvori tumor. Po operaciji lahko lokalno recidivira in neustavljivo raste, a ne metastazira, razen v najbolj izjemnih primerih. V tem oziru je podoben fibromatozi. Zraste kjer koli v telesu. Čeprav gre za benigno tvorbo, lahko zaradi agresivne rasti, podobne fibromatozi ali sarkomu, ogrozi bolnikovo življenje. Bolniki in metode. Bolnica D. E., rojena 1974, v drugem mesecu nosečnosti, je bila sprejeta na našo kliniko 1.1996 zaradi protruzije levega očesa. Navajala je, da ima že 5 let glavobol, ki se je pojavljal ob večerih. Poprej se je na otološki kliniki zdravila zaradi epistakse. Preiskava obnosnih votlin z računalniško tomografijo je pokazala obsežno tumorsko tvorbo v predelu etmoidnega labirinta, ki je vraščala v sosednje strukture, maličila okolne kosti in vraščala tudi ekstraduralno v lobanjsko votlino. Bolnico so nato operirali na otološki kliniki in ji odstranili za "žensko pest" velik tumor ekspanzivnega, neinfiltrativnega tipa. Patohistološko je šlo za vnetni (inflamatorni) psevdotumor. Po operaciji je protruzija zrkla izginila. Kakšno leto dni se je dobro počutila. Po tem obdobju pa je imela številne lokalne recidive. Ponovno so jo operirali in nato obsevali. Dobivala pa je tudi kemoterapijo. Kjub vsemu zdravljenju je bolezen lokalno napredovala. Bolnica je umrla decembra 2000. Zaključki. Kljub temu da je šlo pri tej bolnici za benigen proces, tj. za vnetni (inflamatorni) psevdotumor, je bil potek bolezni zaradi bližine vitalnih struktur takšen kot pri malignih tumorjih.
Descriptors		ORBITAL NEOPLASMS MYOFIBROMATOSIS NEOPLASMS, MUSCLE TISSUE ADULT