Adem C, Aubry M C, Tazelaar H D, Myers J L
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.
Arch Pathol Lab Med. 2001 Dec;125(12):1562-5. doi: 10.5858/2001-125-1562-MAMADP.
Diffuse pulmonary hemorrhage is an uncommon presenting manifestation of angiosarcoma.
To review the clinical, radiologic, and pathologic findings of patients with metastatic angiosarcoma who presented with diffuse pulmonary hemorrhage.
Patients fulfilling inclusion criteria were identified from the consultation files. Clinical and radiologic data were obtained from referring pathologists. Histologic slides were reviewed in all patients.
Our patients included 6 men and 1 woman, aged 31 to 73 years; 4 patients were younger than 40 years. Six patients presented with hemoptysis, and all had diffuse abnormalities on radiographic studies. Clinical considerations prior to biopsy included pulmonary hemorrhage syndrome (n = 2), acute respiratory failure (n = 1), and infection (n = 1). Metastatic disease was included in the differential diagnosis in only 1 patient. None had a diagnosis of malignancy prior to lung biopsy. All biopsies showed hemorrhage associated with atypical epithelioid and spindle cells, forming anastomosing vascular channels, distributed along and within lymphatics and arteries. Neoplastic cells were immunoreactive for factor VIII-related protein or CD31 in all cases for which special stains were available. Three patients with complete follow-up died of their disease. Primary sites were discovered in all 3 patients who underwent autopsy examination. Two tumors arose in the heart and 1 in the pelvic soft tissues. One additional patient had a likely primary site identified in the right atrium by cardiac ultrasound and was subsequently lost to follow-up.
Angiosarcoma should be included in the differential diagnosis of diffuse pulmonary hemorrhage, especially in young adults.
弥漫性肺出血是血管肉瘤罕见的临床表现。
回顾以弥漫性肺出血为表现的转移性血管肉瘤患者的临床、放射学和病理学表现。
从会诊档案中确定符合纳入标准的患者。临床和放射学数据来自转诊的病理学家。对所有患者的组织学切片进行复查。
我们的患者包括6名男性和1名女性，年龄31至73岁；4名患者年龄小于40岁。6名患者出现咯血，所有患者的影像学检查均显示弥漫性异常。活检前的临床考虑包括肺出血综合征（n = 2）、急性呼吸衰竭（n = 1）和感染（n = 1）。仅1名患者的鉴别诊断中考虑了转移性疾病。所有患者在肺活检前均未诊断为恶性肿瘤。所有活检均显示出血伴非典型上皮样细胞和梭形细胞，形成吻合的血管通道，沿淋巴管和动脉分布并在其内分布。在所有可进行特殊染色的病例中，肿瘤细胞对VIII因子相关蛋白或CD31呈免疫反应。3名接受完整随访的患者死于该病。在所有3名接受尸检的患者中均发现了原发部位。2例肿瘤起源于心脏，1例起源于盆腔软组织。另外1名患者经心脏超声检查在右心房发现可能的原发部位，随后失访。
血管肉瘤应纳入弥漫性肺出血的鉴别诊断，尤其是在年轻人中。
