Onagi Hiroko, Horimoto Yoshiya, Arai Takashi, Terukina Hiroyuki, Asai Tohru, Arakawa Atsushi, Saito Tsuyoshi
Department of Human Pathology, Juntendo University School of Medicine, Tokyo, Japan.
Department of Breast Oncology, Juntendo University School of Medicine, Tokyo, Japan.
Case Rep Oncol. 2021 Mar 3;14(1):318-324. doi: 10.1159/000514051. eCollection 2021 Jan-Apr.
Intimal sarcoma arising from the tunica intima of both systemic and pulmonary circulations is a rare disorder, whereas intimal sarcoma with chondroblastic osteosarcomatous differentiation (ISCOS) is even rarer. We present the case of a 25-year-old woman with ISCOS of the pulmonary artery (PA) where the patient went through surgical treatment after careful imaging assessment under a rather emergent situation. A 25-year-old Japanese female presented to our hospital with the chief complaints of dyspnea and palpitations on exertion. Upon arrival, she had systolic murmur, moderate tricuspid regurgitation, and possible pulmonary hypertension. A contrast-enhanced chest computed tomography (CT) showed dilatation of the main PA, filled with a hypodense area with calcification adjacent to the right and left PA. The calcified lesions within the tumor were the key findings suggesting osteoid-forming sarcoma, differentiating it from pulmonary embolism. Due to presence of critical symptomatic obliteration of the pulmonary circulation, an emergency surgery was performed. A whitish shiny mass filled the lumens from the main PA to the bilateral main PAs. The tumor was not attached to the surrounding intima, except for a slight attachment to the left interlobar PA, and could be completely removed from the vessel lumen. Based on the pathological findings, it was diagnosed as a primary ISCOS of the PA, which correlated with the findings of the CT, namely intratumoral calcification. Although the diagnosis-making is quite challenging, multidisciplinary collaboration between clinicians, radiologists, and pathologists is crucial for reaching the correct diagnosis.
起源于体循环和肺循环内膜的内膜肉瘤是一种罕见的疾病，而具有软骨母细胞性骨肉瘤分化的内膜肉瘤（ISCOS）则更为罕见。我们报告一例25岁患有肺动脉（PA）ISCOS的女性病例，该患者在相当紧急的情况下经过仔细的影像学评估后接受了手术治疗。一名25岁的日本女性因劳力性呼吸困难和心悸为主诉前来我院就诊。入院时，她有收缩期杂音、中度三尖瓣反流以及可能的肺动脉高压。胸部增强计算机断层扫描（CT）显示主肺动脉扩张，在左右肺动脉旁有一个低密度区并伴有钙化。肿瘤内的钙化灶是提示成骨样肉瘤的关键发现，使其与肺栓塞相鉴别。由于存在危及生命的肺循环症状性闭塞，遂进行了急诊手术。一个灰白色有光泽的肿物充满了从主肺动脉到双侧主肺动脉的管腔。肿瘤除了与左叶间肺动脉有轻微附着外，未附着于周围内膜，可从血管腔内完全切除。根据病理结果，诊断为PA原发性ISCOS，这与CT表现即肿瘤内钙化相关。尽管诊断颇具挑战性，但临床医生、放射科医生和病理科医生之间的多学科协作对于做出正确诊断至关重要。
