A tüdőartériák intimájából kiinduló sarcoma = Intimal sarcoma of pulmonary arteries

Absztrakt: A pulmonalis arterialis intimalis sarcoma ritka, magas mortalitasu daganat, mely lokalizacioja miatt a pulmonalis embolia, valamint a tudőgyulladas tunettanat utanozhatja. A diagnozis felallitasa es a megfelelő kezeles kivalasztasa korul szamos kerdes merulhet fel. Egy 46 eves, korabban hereseminoma, pulmonalis embolia, asztma, valamint pollenallergia miatt kezeles alatt allo ferfi kontroll mellkasi CT-felvetelen bal oldalon a mediastinalis pleurat infiltralo, az arteria pulmonalist teljesen elzaro, osszessegeben 7–8 cm-es terfoglalo folyamatot irtak le. A horgőbiopszias mintabol orsosejtes tumor diagnozisa szuletett, illetve leiomyoma, leiomyomatosus hyperplasia, illetve leiomyosarcoma lehetőseget vetettek fel. A fennallo obstrukcio miatt a beteg bal oldali tudőeltavolitason esett at. A szovettani vizsgalat a pulmonalis arteriakban terjedő, orsosejtes daganatot korismezett, immunhisztokemiailag diffuz SMA- es fokalis MDM2-pozitivitassal, valamint magas proliferacios aktivitassal. A h-caldesmon, az S100 protein, az ERG, valamint a pancitokeratin-immunfestesek negativnak bizonyultak. Fluoreszcens in situ hibridizacioval a tumorsejtek mintegy 10%-aban polysomiat, illetve MDM2-amplifikaciot lehetett igazolni, aminek alapjan a „high-grade” pulmonalis arterialis intimalis sarcoma diagnozisa megerősitesre kerult. Feleves kovetes alatt a betegseg nem ujult ki. A pulmonalis arterialis intimalis sarcoma pontos incidenciaja nem ismert. Egyes forrasok szerint a kronikus pulmonalis hypertonias betegek 1–4%-anal fordul elő. A tunetek kozul a fogyas kelti fel a leginkabb a daganatos betegseg gyanujat. A kepalkoto es a korszovettani vizsgalatoknak kulcsszerepuk van a diagnozis felallitasaban. A daganat rossz korjoslata miatt a minel korabbi felismeres es a kemoterapiaval kombinalt sebeszi kezeles javithatja a tulelesi lehetősegeket. Orv Hetil. 2020; 161(6): 232–236. | Abstract: Pulmonary arterial intimal sarcoma is a rare tumour with high mortality. Due to its localisation, the symptoms can mimic pulmonary thromboembolism and pneumonia, therefore assessing the diagnosis and choosing the adequate therapy is never easy. Its therapy mainly consists of surgery combined with radiochemotherapy. A 46-year-old male patient with testicular seminoma, pulmonary embolism, bronchial asthma and pollen allergy in his history had a follow-up thoracic CT. On the left side of the lung, a pleura-infiltrating 7–8 cm lesion, which occluded the pulmonary artery, was described. The first biopsy specimen showed fragments of spindle cell tumour. The primary diagnosis was leiomyoma, leiomyomatous hyperplasia, yet the presence of leiomyosarcoma could not have been ruled out. Due to the arterial obstruction, the patient underwent left sided pulmonectomy. Histological examination showed a tumour mostly composed of spindle cells that were diffusely positive with SMA, focally diffuse with MDM2 immunohistochemistry together with high proliferation activity. h-Caldesmon, S-100, ERG and pancytokeratin expressions were not detected. With fluorescent in situ hybridization 10% of tumour cells showed polysomy and MDM2 amplification. According to the results, high-grade pulmonary arterial intimal sarcoma diagnosis has been made. The precise incidence of pulmonary arterial intimal sarcoma is unknown. Some literature data suggest it can be the cause of chronic pulmonary hypertension in 1–4% of the cases. Weight loss can draw attention to the malignant nature of the disease. Imaging techniques and histology are the gold standard in setting the diagnosis. The prognosis is poor. Early recognition and surgery combined with chemotherapy can prolong survival. Orv Hetil. 2020; 161(6): 232–236.