ABSTRACT
Adenosarcomas are the rarest form of uterine sarcomas, and clinical experience with their management is still limited. Here, we reported 7 patients with uterine adenosarcoma referred to our institution, focusing on main pathologic features, their medical history, and long-term follow-up. Among these patients, we provided a detailed description of the medical history of a 49-year-old woman with advanced uterine adenosarcoma with sarcomatous overgrowth who presented a brilliant radiologic and pathologic response after 3 cycles of epirubicin and ifosfamide, ultimately achieving an extraordinary long-term outcome through an integrated surgical and medical approach. Our single-centre experience would suggest that aggressive uterine adenosarcomas with sarcomatous overgrowth are sensitive to standard epirubicin and ifosfamide and that an integrated approach, both medical and surgical, could be considered in clinical practice, again emphasizing the relevant role of multidisciplinary management for this extremely rare disease.
ABSTRACT
OBJECTIVES: Alterations in lung perfusion are a well-known feature of pulmonary hypertension (PH) seen on nuclear medicine studies. Abnormal radiotracer distribution in patients with PH may be caused by arterial thromboembolic occlusion, like in chronic thromboembolic pulmonary hypertension, by parenchymal destruction as in interstitial lung disease and pulmonary emphysema or by distal arteriopathy, like in idiopathic pulmonary arterial hypertension and other nonembolic forms. The different imaging pattern on radionuclide perfusion studies represents an important element for differential diagnosis. The aim of this study was to evaluate minimum intensity projection (minIP) images as an alternative to perfusion scintigraphy. We compared lung parenchyma attenuation patterns as depicted in minIP reconstructions with scintigraphic findings of lung perfusion in patients affected by pulmonary hypertension from various etiologies. METHODS: One hundred and seven consecutive patients affected by PH of different etiology (37 of those had chronic thromboembolic pulmonary hypertension) who had undergone both multidetector computed tomography pulmonary angiography and 99mTc-MAA perfusion scan were included. Five-millimeter thickness contiguous axial, coronal, and sagittal minIP images were reconstructed from the contrast enhanced computed tomography datasets. Two radiologists evaluated the images and qualitatively graded pulmonary attenuation as homogeneous, inhomogeneous with nonsegmental patchy defects, or inhomogeneous with segmental defects. The presence of parenchymal and pleural alterations was recorded. MinIP perfusion grading results were then compared with those of perfusion scintigraphy. RESULTS: In 87 of 107 patients (81.3%), the attenuation pattern seen on minIP images (39 homogeneous patterns, 13 with nonsegmental patchy defects, and 39 with segmental defects) correlated with the nuclear medicine scans. In the remaining 20 patients (18.7%), the imaging pattern was discordant because of 7 false-positive and 2 false-negative thromboembolic patterns at minIP and 11 false-positive thromboembolic patterns at perfusion scan. Air-trapping and parenchymal disease caused false-positive findings at minIP and perfusion scans, respectively. The sensitivity and specificity of minIP in detection of a chronic thromboembolic perfusion pattern were 94.5% and 90%, whereas perfusion scan had 100% sensitivity and 84% specificity. CONCLUSION: MinIP reconstructions can identify different patterns of pulmonary parenchymal attenuation, which show high concordance with perfusion patterns seen on radionuclide studies in patients with pulmonary hypertension. MinIP is a promising technique to evaluate lung perfusion in PH and may be used as an alternative to scintigraphy in the diagnostic work-up of these patients.
