A rare case of liposarcoma in a young male located on the neck in region 6. Case report and literature review

Autori:

Iva Franković, Tena Šimunjak, Boris Šimunjak, Marica Žižić

Sažetak
Liposarkomi su učestali tumori mekih tkiva u odraslih, međutim u regiji glave i vrata predstavljaju samo 2–9% malignih mezenhimalnih tumora. Najčešće su to liposarkomi subkutanog tkiva lica, vlasišta, dubokih mekih tkiva vrata, parafaringealnog i retrofaringealnog prostora te aerodigestivnog trakta (larinksa, orofarinksa i hipofarinksa). Opisani su i liposarkomi žlijezda slinovnica i štitne žlijezde. Prikazani bolesnik je u trenutku dijagnoze imao samo 32 godine, što je iznimna rijetkost, jer se dob bolesnika sa liposarkomima prema podatcima iz dostupne literature kreće u rasponu od 40 do 60 godina. Lokalizacija tumora u prednjoj regiji vrata suprasternalno također predstavlja rijetko sijelo koje do sada nije opisano u dostupnoj literaturi. Liposarkomi su zloćudni tumori koji najčešće nastaju de novo. U prikazanog pacijenta tumor je smješten u regiji 6 na vratu, citološki dijagnosticiran kao lipom, uz redovite kontrole ultrazvukom praćen u vremenskom intervalu od tri godine. Zbog kliničke slike ubrzanog rasta i početnih znakova volumne kompresije postavljena je sumnja na mogućnost postojanja sarkoma podrijetla masnog tkiva. Zbog nepovoljne lokalizacije i nemogućnosti postizanja širokih resekcijskih rubova, u bolesnika je učinjena potpuna kirurška resekcija tumora s resekcijskim rubovima diktiranim anatomskim smještajem vitalnih struktura vrata i medijastinuma. Kako je u patohistološkom nalazu reseciranog tumora bio opisan proboj čahure, u dogovoru s onkologom provedena je postoperativna adjuvantna radioterapija.
Summary

Liposarcoma is a common soft tissue tumor in adults, however, in the head and neck region liposarcomas represent only 2–9% of malignant mesenchymal tumors. The most common are liposarcomas of the subcutaneous tissue of the face, scalp, deep soft tissues of the neck, parapharyngeal and retropharyngeal space, and the aerodigestive tract (larynx, oral cavity and pharynx). Liposarcomas of the salivary and thyroid glands have also been described. Liposarcomas generally occur between the ages of 40 and 60, but our patient was only 32 years old when he was diagnosed with liposarcoma in the region 6 of the neck, which is also uncommon and has not been described in the available literature. Liposarcomas are malignant tumors which usually occur de novo. The patient from our case report was diagnosed with lipoma by means of ultrasonography and fine needle aspiration biopsy at the exact location of liposarcoma, and ultrasonographically followed in the period of three years. Because of the acceleration in the tumor growth and signs of volume compression, a possibility of the existence of malignant tumor of fat tissue origin was suspected. Because of the inconvenient tumor localisation and inability of securing wide resection margins, the tumor was resected with margins that were dictated by anatomical
relations among the tumor and vital neurovascular structures of the lower neck and mediastinum. As the penetration of the tumor capsule by the tumor tissue was present in the definitive histopathological finding, adjuvant radiotherapy was applied in agreement with the oncologist.