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鼻窦区域侵袭性砂粒体样骨化性纤维瘤:一组独特的纤维-骨病变的临床病理研究

Aggressive psammomatoid ossifying fibromas of the sinonasal region: a clinicopathologic study of a distinct group of fibro-osseous lesions.

作者信息

Wenig B M, Vinh T N, Smirniotopoulos J G, Fowler C B, Houston G D, Heffner D K

机构信息

Department of Otolaryngic and Endocrine Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.

出版信息

Cancer. 1995 Oct 1;76(7):1155-65. doi: 10.1002/1097-0142(19951001)76:7<1155::aid-cncr2820760710>3.0.co;2-p.

DOI:10.1002/1097-0142(19951001)76:7<1155::aid-cncr2820760710>3.0.co;2-p
PMID:8630892
Abstract

BACKGROUND

Psammomatoid ossifying fibromas represent a unique subset of fibro-osseous lesions of the sinonasal tract. They have distinctive histomorphologic features and a tendency toward locally aggressive behavior, including invasion and destruction of adjacent anatomic structures.

METHODS

Seven cases of psammomatoid ossifying fibromas of the sinonasal tract were identified in the files of the Otolaryngic Tumor Registry at the Armed Forces Institute of Pathology. Medical records, including the clinical history, location of the lesions, radiographs, treatment, and follow-up were reviewed in each case. Follow-up information was available in all of the cases.

RESULTS

Four of the patients were male and three were female. The patient's ages ranged from 5 to 54 years (median age, 33 years). Symptoms included facial swelling, nasal obstruction, pain, sinusitis, headache, and proptosis. Radiographic studies confirmed the presence of an osseous and/or soft tissue mass varying in appearance from well demarcated without invasion or erosion to invasive with bone erosion and intracranial extension. Sites of involvement included the nasal cavity and all paranasal sinuses, particularly the ethmoid and maxillary sinuses. Often, more than one sinus was involved and extension of disease included involvement of the orbit, nasopharynx, palate, and anterior cranial fossa. The histologic appearance was characterized by the presence of small mineralized (psammomatoid) bodies admixed with a cellular stroma with a variable amount of myxomatous material and scattered giant cells. Confusion with other osseous and soft tissue tumors may occur resulting in too limited or too aggressive management. En bloc surgical excision is the treatment of choice and may prove curative. Aggressive behavior with recurrence(s) or invasion into adjacent structures occurred. At the time of this writing, the patients are alive over follow-up periods ranging from 6 months to 7 years.

CONCLUSIONS

Gnathic and midfacial fibro-osseous proliferations are a diverse group of lesions. A subset of these fibro-osseous lesions with predilection for the sinonasal tract were identified. These lesions are characterized by their distinctive histology, including psammomatoid ossicles and their locally aggressive growth. Complete surgical removal is the treatment of choice.

摘要

背景

砂粒样骨化性纤维瘤是鼻窦纤维-骨病变中的一个独特亚型。它们具有独特的组织形态学特征,并有局部侵袭性行为倾向,包括侵犯和破坏相邻的解剖结构。

方法

在武装部队病理研究所耳鼻喉肿瘤登记处的档案中识别出7例鼻窦砂粒样骨化性纤维瘤。对每个病例的病历进行了回顾,包括临床病史、病变部位、X线片、治疗和随访情况。所有病例均有随访信息。

结果

4例患者为男性,3例为女性。患者年龄范围为5至54岁(中位年龄33岁)。症状包括面部肿胀、鼻塞、疼痛、鼻窦炎、头痛和眼球突出。影像学研究证实存在骨和/或软组织肿块,其外观从边界清晰无侵犯或侵蚀到有骨侵蚀和颅内扩展的侵袭性表现不等。受累部位包括鼻腔和所有鼻窦,尤其是筛窦和上颌窦。通常,不止一个鼻窦受累,疾病扩展包括眼眶、鼻咽、腭和前颅窝受累。组织学表现的特征是存在小的矿化(砂粒样)体,与含有不同量黏液样物质和散在巨细胞的细胞性基质混合。可能会与其他骨和软组织肿瘤混淆,导致治疗过于保守或激进。整块手术切除是首选治疗方法,可能治愈。出现了侵袭性行为并复发或侵犯相邻结构。在撰写本文时,患者在6个月至7年的随访期内均存活。

结论

颌骨和面中部纤维-骨增殖是一组多样的病变。已识别出这些纤维-骨病变中倾向于鼻窦的一个亚型。这些病变的特征是其独特的组织学,包括砂粒样小骨和局部侵袭性生长。完整的手术切除是首选治疗方法。

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