Department of Surgery, University of Munich, Campus Grosshadern, Germany.
World J Surg Oncol. 2011 Jun 6;9:62. doi: 10.1186/1477-7819-9-62.
Pulmonary sclerosing hemangioma (SH) is a rare tumor of the lung predominantly affecting Asian women in their fifth decade of life. SH is thought to evolve from primitive respiratory epithelium and mostly shows benign biological behavior; however, cases of lymph node metastases, local recurrence and multiple lesions have been described.
We report the case of a 21-year-old Caucasian male with a history of locally advanced and metastatic rectal carcinoma (UICC IV; pT4, pN1, M1(hep)) that was eventually identified as having hereditary non-polyposis colorectal cancer (HNPCC, Lynch syndrome). After neoadjuvant chemotherapy followed by low anterior resection, adjuvant chemotherapy and metachronous partial hepatectomy, he was admitted for treatment of newly diagnosed bilateral pulmonary metastases. Thoracic computed tomography showed a homogenous, sharply marked nodule in the left lower lobe. We decided in favor of atypical resection followed by systematic lymphadenectomy. Histopathological analysis revealed the diagnosis of SH.
Cases have been published with familial adenomatous polyposis (FAP) and simultaneous SH. FAP, Gardner syndrome and Li-Fraumeni syndrome, however, had been ruled out in the present case. To the best of our knowledge, this is the first report describing SH associated with Lynch syndrome.
肺硬化性血管瘤（SH）是一种罕见的肺部肿瘤，主要影响五十多岁的亚洲女性。SH 被认为起源于原始呼吸上皮，其生物学行为大多为良性；然而，已有淋巴结转移、局部复发和多发病变的病例报道。
我们报告了一例 21 岁的白人男性，他患有局部晚期和转移性直肠腺癌（UICC IV；pT4、pN1、M1（肝）），最终被诊断为遗传性非息肉病性结直肠癌（HNPCC，林奇综合征）。在新辅助化疗后行低位前切除术、辅助化疗和同时性部分肝切除术，他因新诊断的双侧肺转移而入院治疗。胸部计算机断层扫描显示左肺下叶有一个均质、边界清晰的结节。我们决定行非典型切除术加系统淋巴结清扫术。组织病理学分析显示硬化性血管瘤的诊断。
已有家族性腺瘤性息肉病（FAP）和同时性 SH 的病例报道。然而，在本病例中排除了 FAP、Gardner 综合征和 Li-Fraumeni 综合征。据我们所知，这是首例描述与林奇综合征相关的 SH 的病例报告。
