Eccrine porocarcinoma (malignant eccrine poroma) - A clinicopathologic study of 69 cases
Eccrine porocarcinoma (malignant eccrine poroma) - A clinicopathologic study of 69 cases
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DOI：
10.1097/00000478-200106000-00002
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发表时间：
2001-06-01
影响因子：
5.6
通讯作者：
Calonje, E
中科院分区：
文献类型：
--
作者：
Robson, A;Greene, J;Calonje, E
本文报告69例小汗腺汗孔癌的临床病理特点。其中包括七例纯粹原位疾病的病例。年龄29 ~ 91岁（平均73岁）。下肢是最常见的部位（44%）。其他常见部位为躯干（15例，24%）和头部（11例，18%）。EP的组织学诊断是基于一个不规则的肿瘤，至少部分由特征性的多孔基底样上皮细胞形成，显示导管分化和显著的细胞学异常。47例肿瘤（68%）含有成熟的、成形良好的外分泌管，具有嗜酸性的腔角质层，其余肿瘤含有小的、成形不良的导管和/或胞浆内腔。所有导管通过光学显微镜可辨别，并且在49个病例中用DPAS染色和/或CEA/EMA免疫细胞化学突出显示。一个具有广泛的肿瘤边缘和明显的核多形性的变体与增殖性鲍恩样发育不良有一些相似之处。在11例（18%）的肿瘤出现在连续的良性先前存在的汗孔瘤。各种组织学模式显示，包括明确的，鳞状细胞和梭形细胞分化，粘液细胞化生，黑素细胞定植。血管侵犯9例（15%）。3例表现为恶性细胞向表皮性的pagetoid扩展，并表现为多灶性。54例患者（78%）进行了随访，其中9例（17%）发生局部复发，10例发生淋巴结转移（19%），6例（11%）发生远处转移或死亡。有丝分裂、淋巴血管浸润和肿瘤深度>7 mm与预后较差相关。将肿瘤分为边缘推进型或浸润型也可预测预后，后者局部复发风险增加。这份报告是迄今为止最大的EP系列，表明攻击性行为的发生率比普遍认为的要低。此外，EP可以显示各种各样的组织学模式，可能导致诊断错误的疏忽。本系列中的大量病例使预后参数的可靠评估成为可能。侵袭性更强的临床病程可能表现为每个高倍视野核分裂超过14个（死亡风险比[HR] 17.0，95%置信区间[CI] 2.71-107），肿瘤侵犯淋巴管（IIR 4.41，CI 1.13-17.2），深度>7 mm（HR 5.49，CI 1.0-30.3）。因此，核分裂，淋巴管浸润，肿瘤深度应在这些肿瘤进行评估。我们还建议，肿瘤表现为“浸润性”的进展边缘特别容易局部复发，需要广泛切除，并密切关注手术边缘的病理报告。
The clinicopathologic characteristics of 69 cases of eccrine porocarcinoma (EP) have been studied. Seven cases of purely in situ disease are included. Forty patients were female, 29 male with ages ranging from 29 to 91 years (mean 73 years). The lower extremity represented the single most common site (44%). Other common sites were the trunk (15 cases, 24%) and head (11 cases, 18%). The histologic diagnosis of EP was predicated on the basis of an irregular tumor at least partly formed of characteristic poromatous basaloid epithelial cells displaying ductal differentiation, and significant cytologic atypia. Forty-seven tumors (68%) contained mature well-formed eccrine ducts having an eosinophilic luminal cuticle, with the remaining tumors containing small ill-formed ducts and/or intracytoplasmic lumina. All ducts were discernible via light microscopy and in 49 cases were highlighted with DPAS stain and/or CEA/EMA immunocytochemistry. A variant with a broad pushing tumor margin and marked nuclear pleomorphism showed some resemblance to proliferative bowenoid dysplasia. In 11 cases (18%) the tumors appeared to arise in continuity with a benign preexistent poroma. A variety of histologic patterns were displayed including clear, squamous, and spindle cell differentiation, mucus cell metaplasia, and colonization by melanocytes. Lymphovascular invasion was present in 9 cases (15%). Three cases showed pagetoid extension of malignant cells (epidermotropism) and appeared to be multifocal. Follow-up was available in 54 patients (78%) with 9 (17%) experiencing local recurrence, 10 developing lymph node metastases (19%), and 6 (11%) experiencing distant metastases or death. Mitoses, the presence of lymphovascular invasion, and tumor depth >7 mm were associated with a poorer prognosis. Dividing tumors into those with a "pushing" or "infiltrating" advancing margin was also predictive of outcome with the latter having an increased risk of local recurrence. This report, the largest series of EP to date, suggests that the incidence of aggressive behavior is less than popularly believed. Furthermore, EP can display a wide variety of histologic patterns that may lead to diagnostic error in the unwary. The large number of cases in this series enables a reliable evaluation of prognostic parameters. A more aggressive clinical course may be indicated by more than 14 mitoses per high power field (hazard ratio [HR] for death 17.0, 95% confidence interval [CI] 2.71-107), lymphovascular invasion by tumor (IIR 4.41, CI 1.13-17.2), and depth >7 mm (HR 5.49, CI 1.0-30.3). Thus, mitoses, lymphovascular invasion, and tumor depth should be evaluated in these tumors. We also suggest that tumors presenting an "infiltrative" advancing margin are particularly prone to local recurrence and require wide excision with close attention to the surgical margins by the reporting pathologist.