Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
Histol Histopathol. 2013 Mar;28(3):337-44. doi: 10.14670/HH-28.337.
Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign tumor arising in the sinonasal tract, predominantly involving infants and children. To date, only 27 cases are reported in the international literature and there have been no reported cases of malignant transformation. We present a 40-year-old female patient with nasal obstruction and bloody rhinorrhea. Computed tomography (CT) of the nose and paranasal sinuses confirmed a heterogeneous polypoid soft-tissue mass filling the nasal cavity and extending into the maxillary and ethmoid sinus. The patient underwent a complete radical resection. Histological and immunohistochemical analyses showed a portion of the mass was consistent with typical NCMH. However, some areas of mass exhibited cytological atypia, marked mitotic activity and foci of necrosis. The atypical mesenchymal spindle cells were immunoreactive for vimentin, CD99 and smooth muscle actin (SMA) diffusely. The cartilaginous cells were immunopositive for S-100 protein. Ki-67 index was high in atypical areas, accounting for 50%. A rapid mass recurrence was observed at the original site only 3 months after surgery. The final diagnosis of NCMH with malignant transformation was made. To our knowledge, this is the first report of malignant transformation occurring in an adult with NCMH. Although NCMH commonly develops in the neonate or young infants and exhibits benign histological appearance and favorable prognosis, there is a possibility of malignant transformation in adult patients. Thoroughly histological inspections are suggested to be necessary to accurately diagnose this tumor when it is encountered in adults.
鼻腔软骨间叶性错构瘤（Nasal Chondromesenchymal Hamartoma，NCMH）是一种极为罕见的良性肿瘤，起源于鼻-鼻窦区域，主要发生于婴幼儿。迄今为止，国际文献中仅报道了 27 例，且无恶性转化的报道。我们报告了 1 例 40 岁女性患者，主要表现为鼻塞和血性鼻涕。鼻及鼻窦 CT 证实鼻腔内存在异质性息肉样软组织肿块，并延伸至上颌窦和筛窦。患者接受了完整的根治性切除术。组织学和免疫组织化学分析显示部分肿块符合典型的 NCMH。然而，肿块的某些区域存在细胞学异型性、明显的有丝分裂活性和坏死灶。非典型间叶性梭形细胞弥漫性表达波形蛋白、CD99 和平滑肌肌动蛋白（Smooth Muscle Actin，SMA）。软骨细胞免疫组化 S-100 蛋白阳性。非典型区域的 Ki-67 指数较高，为 50%。术后仅 3 个月，原发病灶处即观察到肿块快速复发。最终诊断为 NCMH 伴恶性转化。据我们所知，这是首例成人 NCMH 发生恶性转化的报道。虽然 NCMH 常见于新生儿或婴幼儿，具有良性组织学表现和良好的预后，但成人患者存在恶性转化的可能。因此，当成人中遇到这种肿瘤时，建议进行彻底的组织学检查以准确诊断。
