Pathology Department, Arrixaca Hospital, Murcia, Spain.
Ann Diagn Pathol. 2010 Feb;14(1):44-9. doi: 10.1016/j.anndiagpath.2009.03.009. Epub 2009 Oct 31.
Desmoplastic spindle cell tumor of the liver is a recently described and extremely unusual neoplasm that affects children and young adults. We report 1 case in a 33-year-old man. The patient had abdominal pain and dyspepsia. Abdominal examination showed that the liver was enlarged and palpable until umbilical region. Laboratory studies demonstrated positive serologic markers to hepatitis B virus. All other analytical studies were irrelevant. Computed tomography revealed a large tumor mass in left hepatic lobe showing heterogeneous densities, with hyperdense peripheral areas, as multiple nodular calcifications of less than 1 cm. In the central part of the mass, a big hypodense area was observed. There was no evidence of extrahepatic disease. Grossly, the tumor was well circumscribed with multiple nodular calcifications. The tumor was characterized by the presence of cohesive nests of bland spindle cells arranged in short fascicles and surrounded by desmoplastic stroma, intermixed with epithelioid cells. Mitotic activity was very low. Extensive osteoid formation was seen inside the cell nests. The tumor cells showed cytoplasmic reactivity for vimentin and pan-cytokeratin. The cells of desmoplastic stroma were immunoreactive for actin. The biologic behavior is still uncertain with only 5 published cases, but current information suggests that they are low-grade tumors with an indolent course. The clinical and morphologic features of this tumor are very similar to those of tumors previously reported as "nested stromal-epithelial tumor of liver" and "ossifying stromal-epithelial tumor of liver." We describe the histologic, immunohistochemical, and molecular genetic features of a case of desmoplastic spindle cell tumor of the liver and review the literature.
肝促结缔组织增生性 spindle 细胞瘤是一种新近描述的、极其罕见的肿瘤，主要影响儿童和青年。我们报告了 1 例 33 岁男性患者。患者有腹痛和消化不良。腹部检查发现肝脏肿大且可触及，直至脐部。实验室研究显示乙型肝炎病毒血清学标志物阳性。其他所有分析研究均无相关性。计算机断层扫描显示左肝叶有一个大的肿瘤肿块，呈不均匀密度，周边区域呈高密度，有多发性小于 1cm 的结节状钙化。肿块中央区域观察到一个大的低密度区。无肝外疾病的证据。大体上，肿瘤边界清楚，有多个结节状钙化。肿瘤的特征是存在由排列成短束的温和 spindle 细胞组成的凝聚巢，周围环绕着促结缔组织增生的基质，混有上皮样细胞。有丝分裂活性非常低。在细胞巢内可见广泛的骨样形成。肿瘤细胞对波形蛋白和细胞角蛋白 pan 呈细胞质反应。促结缔组织增生的基质细胞对肌动蛋白呈免疫反应性。由于仅发表了 5 例病例，其生物学行为仍不确定，但目前的信息表明它们是低度恶性肿瘤，具有惰性病程。该肿瘤的临床和形态特征与先前报道的“肝巢状间质上皮瘤”和“肝骨化性间质上皮瘤”非常相似。我们描述了肝促结缔组织增生性 spindle 细胞瘤的组织学、免疫组织化学和分子遗传学特征，并复习了文献。
