Metastasizing "Benign" Cutaneous Fibrous Histiocytoma A Clinicopathologic Analysis of 16 Cases
Metastasizing "Benign" Cutaneous Fibrous Histiocytoma A Clinicopathologic Analysis of 16 Cases
复制标题
DOI：
10.1097/pas.0b013e31827070d4
复制
发表时间：
2013-04-01
影响因子：
5.6
通讯作者：
Fletcher, Christopher D. M.
中科院分区：
文献类型：
--
作者：
Doyle, Leona A.;Fletcher, Christopher D. M.
皮肤纤维组织细胞瘤（FH）被认为是一种良性肿瘤;然而，某些类型的FH已被证明有局部复发的倾向，并且有罕见的转移病例报告。在这项研究中，16例形态学良性FH与局部或远处转移的咨询文件中确定。对原发性、复发性和转移性肿瘤的病理特征以及临床结果进行了评价。9例为男性患者，7例为女性患者;平均年龄为42岁（范围：3 - 68岁）。原发肿瘤发生于小腿5例，小腿1例，躯干3例，肩部3例，颈部2例，手指1例。1例病例的原发部位未知。15例原发性肿瘤涉及真皮; 6例延伸至浅表皮下组织。肿瘤大小范围为1至5 cm（中位数为3.2 cm）。在组织学上，原发性肿瘤表现出FH的特征，在大多数情况下，由多形的中性梭形细胞和组织细胞样细胞组成，呈混合的故事状和束状生长模式，混合有泡沫细胞、多核细胞和不同比例的炎性细胞。组织学变异包括11例细胞型（2例具有混合的非典型和细胞特征）、2例囊性、1例非典型和1例上皮样类型。所有肿瘤均可见透明化胶原束包埋。有丝分裂活性范围为< 1至13/10 HPF。1例原发肿瘤出现局灶性坏死。10例患者局部肿瘤复发; 4例患者多次局部复发。至首次复发的时间范围为6周至13年。1例局部复发的肿瘤细胞学检查显示细胞凋亡增加，但复发的肿瘤在其他方面与原发肿瘤相似。转移发生在诊断后0至180个月（中位数17个月），并涉及肺（12例），淋巴结（8），软组织（6）和肝脏（1）。5例患者在原发肿瘤区域出现多个卫星结节。转移瘤在形态上与原发肿瘤相似。到目前为止，6例患者死于疾病，中位死亡时间为64个月（范围，10至168个月）。四名患者患有转移性疾病。2例患者在末次随访时无疾病，1例患者死于不相关疾病。转移的形态良性皮肤FH是一个非常罕见的，但临床上积极的事件。原发性肿瘤往往是大的和细胞，但侵略性的行为不能单独预测的形态学基础上，但是，早期或频繁的局部复发可能需要更密切的临床随访。
Cutaneous fibrous histiocytoma (FH) is considered a benign tumor; however, certain types of FH have been shown to have a tendency for local recurrence, and there are rare reported cases of metastasis. In this study, 16 cases of morphologically benign FH with locoregional or distant metastasis were identified in consult files. Pathologic features of primary, recurrent, and metastatic tumors, as well as clinical outcome, were evaluated. Nine were male and 7 were female patients; mean age was 42 years (range, 3 to 68 y). Primary tumors arose on the leg in 5 patients, buttock in 1, trunk in 3, shoulder in 3, neck in 2, and finger in 1. The primary site in 1 case was unknown. Fifteen primary tumors available for review involved the dermis; 6 extended into the superficial subcutis. Tumor size ranged from 1 to 5 cm (median 3.2 cm). Histologically, primary tumors showed characteristic features of FH, being composed in most cases of a polymorphous population of bland spindle and histiocytoid cells in a mixed storiform and fascicular growth pattern with admixed foam cells, multinucleate cells, and inflammatory cells in varying proportions. Histologic variants included 11 cellular (2 with mixed atypical and cellular features), 2 aneurysmal, 1 atypical, and 1 epithelioid type. All tumors showed entrapment of hyalinized collagen bundles. Mitotic activity ranged from < 1 to 13/10 HPF. Focal necrosis was seen in 1 primary tumor. Ten patients had local tumor recurrence; 4 patients had multiple local recurrences. Time to first recurrence ranged from 6 weeks to 13 years. The local recurrences of 1 tumor showed increased cytologic atypia, but recurrences were otherwise morphologically similar to primary tumors. Metastases occurred 0 to 180 months after diagnosis (median 17 mo) and involved the lungs (12 patients), lymph nodes (8), soft tissues (6), and liver (1). Five patients developed multiple satellite nodules in the region of the primary tumor. Metastases were morphologically similar to the primary tumors. So far, 6 patients died of disease, with a median time to death of 64 months (range, 10 to 168 mo). Four patients are alive with metastatic disease. Two patients are disease free at last follow-up, and 1 patient died of unrelated disease. Metastasis of morphologically benign cutaneous FH is an extremely rare but clinically aggressive event. Primary tumors tend to be large and cellular, but aggressive behavior cannot be predicted on morphologic grounds alone; however, early or frequent local recurrence may warrant closer clinical follow-up.