McDermott M B, Ponder T B, Dehner L P
Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, Barnes-Jewish Hospital, St. Louis, Missouri 63110, USA.
Am J Surg Pathol. 1998 Apr;22(4):425-33. doi: 10.1097/00000478-199804000-00006.
Nasal chondromesenchymal hamartoma is the suggested appellation for a tumefactive process of the nasal passages and contiguous paranasal sinuses in seven children with a detectable mass in the nose. With the exception of one patient who was 7 years of age at diagnosis, the others were 3 months of age or less upon recognition of the mass. Two children were diagnosed in the first 2 weeks of life. Imaging studies showed a complex solid and cystic mass or masses filling the nasal cavity and extending into the ethmoid sinuses in most cases. Erosion of the surrounding bone, including the cribriform plate, resulted in an intracranial component in the four cases. Surgical resection was the treatment of choice despite its technical difficulties that often necessitated a combined intranasal and intracranial approach. Residual disease with continued growth in one case was the clinical outcome in two children, and the remaining five patients have not experienced any further difficulties. The piecemeal fragments of tissue disclosed a collage of histologic features, but the basic morphologic elements were well-demarcated nodules of cartilage with some variation in the cellular density and maturation of the chondrocytes, a myxoid to spindle cell stroma, focal osteoclastlike giant cells in the stroma, and erythrocyte-filled spaces resembling those of the aneurysmal bone cyst. Two of the tumors were less polymorphous or complex in their spectrum of histologic features. These nasal masses have similarities to the so-called chest wall hamartoma or mesenchymal hamartoma of the chest wall in terms of the clinical presentation in infancy and the basic cartilaginous character of both entities. There is a degree of presumption in the designation of these nasal and chest wall tumors as hamartomas because the pathogenesis has not been established for either entity.
鼻软骨间叶性错构瘤是对7例鼻腔内可检测到肿块的儿童鼻腔及相邻鼻窦的一种肿瘤样病变的建议称谓。除1例诊断时7岁的患者外，其他患者在发现肿块时年龄均在3个月及以下。2例患儿在出生后2周内被诊断。影像学研究显示，多数情况下为复杂的实性和囊性肿块，充满鼻腔并延伸至筛窦。包括筛板在内的周围骨质侵蚀导致4例出现颅内成分。尽管手术切除存在技术困难，通常需要鼻内和颅内联合入路，但仍是首选治疗方法。2例患儿出现临床结局为残留病灶且持续生长，其余5例患者未再出现任何问题。组织碎块显示出一系列组织学特征，但基本形态学成分是界限清楚的软骨结节，软骨细胞的细胞密度和成熟度有所不同，有黏液样至梭形细胞基质，基质中有局灶性破骨细胞样巨细胞，还有类似动脉瘤样骨囊肿的充满红细胞的间隙。其中2例肿瘤的组织学特征谱较单一或不复杂。这些鼻腔肿块在婴儿期的临床表现以及二者基本的软骨特性方面，与所谓的胸壁错构瘤或胸壁间叶性错构瘤相似。将这些鼻腔和胸壁肿瘤称为错构瘤有一定推测性，因为二者的发病机制均未明确。
