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恶性磷尿性间叶肿瘤,舌部混合性结缔组织型

Malignant phosphaturic mesenchymal tumor, mixed connective tissue variant of the tongue.

作者信息

Uramoto Naoki, Furukawa Mitsuru, Yoshizaki Tomokazu

机构信息

Division of Otolaryngology, Graduate School of Medical Science, Kanazawa University, 13-1 Takaramachi, Kanazawa 920-8641, Japan.

出版信息

Auris Nasus Larynx. 2009 Feb;36(1):104-5. doi: 10.1016/j.anl.2008.01.003. Epub 2008 Mar 7.

DOI:10.1016/j.anl.2008.01.003
PMID:18329207
Abstract

The majority of the oncogenic osteomalacia-associated mesenchymal tumors are considered to belong to the category of phosphaturic mesenchymal tumors, mixed connective tissue (PMTMCT) variant, of which malignant cases are very rare. Here we report a case of a recurrent malignant PMTMCT variant which arose in the tongue. The patient was treated with surgery at an initial treatment and the first recurrence. In accordance with the tumor recurrence and resection, the hypophosphatemia progressed and improved. However, hypophosphatemia did not progress after receiving radiation therapy at the second recurrence even though the recurrent tumor gradually increased its size. These results suggest clinical feature of malignant PMTMCT could be changed by radiation therapy. Thus, this report could add an insight to the nature of PMTMCT.

摘要

大多数与致癌性骨软化相关的间充质肿瘤被认为属于磷酸尿性间充质肿瘤、混合结缔组织(PMTMCT)变体类别,其中恶性病例非常罕见。在此,我们报告一例发生于舌部的复发性恶性PMTMCT变体病例。该患者在初始治疗及首次复发时接受了手术治疗。随着肿瘤的复发和切除,低磷血症有进展和改善。然而,在第二次复发接受放射治疗后,尽管复发性肿瘤逐渐增大,但低磷血症并未进展。这些结果提示放射治疗可能改变恶性PMTMCT的临床特征。因此,本报告可为PMTMCT的本质提供新的见解。

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