Gaeta Raffaele, Aringhieri Giacomo, Zampa Virna, D'Arienzo Antonio, Andreani Lorenzo, Capanna Rodolfo, Zavaglia Katia, Franchi Alessandro
9310Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy.
Department of Diagnostics and Imaging, Pisa University Hospital, Pisa, Italy.
Int J Surg Pathol. 2021 Dec;29(8):892-898. doi: 10.1177/10668969211010221. Epub 2021 Apr 13.
Dedifferentiated chondrosarcoma is a well-recognized entity, but its occurrence in the distal extremities is exceedingly rare. We present the case of a 49-year-old woman who experienced local recurrence of an "enchondroma" of the proximal phalanx of the fourth finger of the left hand, which had been initially treated with intralesional curettage at another hospital 4 years before, and 1 year before for a local recurrence. The imaging findings indicated an aggressive behavior, and an incisional biopsy showed a highly cellular proliferation of spindle and pleomorphic elements without evidence of matrix production intermixed with few fragments of a well-differentiated cartilaginous neoplasm with bland cellular atypia, focal nuclear hyperchromatism, and binucleation. An isocitrate dehydrogenase 2 R172S mutation was detected. The final diagnosis was dedifferentiated chondrosarcoma. Despite amputation of the fourth finger, the patient developed lung metastases and further local relapse. Recurrent cartilaginous tumors of the extremities should not be underestimated and should be followed in view of the possible acquisition of aggressive clinical behavior.
去分化软骨肉瘤是一种公认的疾病实体，但其在四肢远端的发生极为罕见。我们报告一例49岁女性病例，其左手无名指近节指骨的“内生软骨瘤”出现局部复发，该内生软骨瘤最初在4年前于另一家医院接受病灶内刮除术治疗，1年前因局部复发再次治疗。影像学检查结果显示具有侵袭性行为，切开活检显示梭形和多形性细胞高度增殖，无基质产生迹象，其间混杂有少量分化良好的软骨性肿瘤碎片，细胞异型性温和，局灶性核深染及双核。检测到异柠檬酸脱氢酶2 R172S突变。最终诊断为去分化软骨肉瘤。尽管对该无名指进行了截肢，但患者仍发生了肺转移及进一步的局部复发。四肢复发性软骨肿瘤不应被低估，鉴于其可能出现侵袭性临床行为，应予以密切随访。
