Osteosarcoma and Langerhans Cell Histiocytosis in a Pediatric Patient with Lynch Syndrome: A Case Report.

Q4 Medicine JBJS case connector Pub Date : 2024-10-03 eCollection Date: 2024-10-01 DOI:10.2106/JBJS.CC.24.00200
Soheil Sabzevari, Carol D Morris, Meera R Hameed, Daniel E Prince
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Abstract

Case: Lynch syndrome (hereditary nonpolyposis colorectal cancer) is associated with extracolonic manifestations, but skeletal tumors are rare. Our patient, a 12-year-old boy with Lynch syndrome, developed osteosarcoma of the left femur. Treatment included cytotoxic chemotherapy, wide resection, and pembrolizumab. Two years later, he developed an aggressive lesion in the contralateral femur that was thought to be metastatic osteosarcoma but which histology revealed to be Langerhans cell histiocytosis.

Conclusion: This case underscores the importance of advanced testing in patients with osteosarcoma and poor response to chemotherapy, and of tissue sampling when patients with a primary malignancy develop new bone lesions.

Level of evidence: IV.

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一名林奇综合征小儿患者的骨肉瘤和朗格汉斯细胞组织细胞增生症:病例报告。
病例林奇综合征(遗传性非息肉病结直肠癌)与结肠外表现有关,但骨骼肿瘤却很罕见。我们的患者是一名患有林奇综合征的 12 岁男孩,他患上了左股骨骨肉瘤。治疗包括细胞毒化疗、广泛切除术和 pembrolizumab。两年后,他的对侧股骨出现侵袭性病变,原以为是转移性骨肉瘤,但组织学检查发现是朗格汉斯细胞组织细胞增生症:本病例强调了对化疗反应不佳的骨肉瘤患者进行晚期检测的重要性,以及在原发性恶性肿瘤患者出现新的骨病变时进行组织取样的重要性:证据等级:IV。
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来源期刊
JBJS case connector
JBJS case connector Medicine-Medicine (all)
CiteScore
0.60
自引率
0.00%
发文量
319
期刊介绍: JBJS Case Connector helps improve patient care by providing the medical community with a journal that harnesses technology to provide information tools for discovery and reporting of unusual musculoskeletal problems, findings, treatment, and outcomes. Co-edited by Thomas W. Bauer, MD, PhD, and Ronald W. Lindsey, MD, JBJS Case Connector assists orthopaedic surgeons in the search for precedents, connections, and trends in their efforts to improve patient care. Using this unique journal, surgeons can find the commonalities between cases, benefit from the experience of their peers, and filter case information by many important variables in order to provide the best possible care for orthopaedic patients. This cross-referenced online journal includes thousands of orthopaedic case reports. It compiles symptoms, conditions, and demographic details to empower surgeons to find cases similar to theirs. Surgeons can mine the database to reveal emerging trends and identify patterns, distinguishing between truly rare cases and repeated, related single instances of a larger problem. The JBJS Case Connector Image Quiz feature provides interactive quizzes based on images from content published by JBJS and includes a discussion area for further exploration of ideas and concepts. The JBJS Image Quiz app for iPad and iPhone is available in the App Store. Contributions to JBJS Case Connector are welcomed from anywhere in the world and are considered on their merits. Articles must be written in English and should be submitted as outlined in the Instructions to Authors. All authors must abide by the JBJS ethics policies and all submissions to JBJS Case Connector are covered by the JBJS embargo policy.
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