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Rationale: Primary leiomyosarcoma of the bone (LMSB) is a rare aggressive sarcoma with limited treatment options. Histopathologic and immunohistochemical features are similar to their more common uterine and soft tissue counterparts. However, its broader spectrum of histopathologic features and rarity make diagnostic challenges., Patient Concerns: We present a case of LMSB in a 20-year-old female who presented with left shoulder aching pain for 3 months. An osteolytic intramedullary lesion was found in the left proximal humeral epi-metaphysis., Diagnoses: Initial open biopsy showed a giant cell tumor of bone with aneurysmal bone cyst (ABC)-like changes. However, an open biopsy followed by extended curettage showed LMSB with ABC-like changes., Interventions: Wide excision of the lesion and bipolar hemiarthroplasty followed by concomitant chemoradiation therapy was conducted. The mass was completely removed without significant problems., Outcomes: Complete mass excision and symptomatic improvements were achieved, and no subsequent relapses were observed., Lessons: The authors encountered a rare case of LMSB. Most occurrences are in the lower extremity and trunk, respectively. ABC-like changes in bone tumors can lead to misdiagnosis. In this case, the ABC-like changes developed from the underlying LMSB as a secondary alteration. A careful examination of the underlying bone tumor is crucial to avoid misdiagnosing it as ABC or exhibiting ABC-like changes. Moreover, there has been no case report of LMSB with secondary ABC-like changes in bone., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Giant cell-rich lesions are a diverse group of lesions that usually occur in bone and contain varying numbers of reactive osteoclastic-type multinucleate giant cells. These lesions present a challenge in pathologic diagnosis, often requiring a combination of clinical, radiographic, and histopathological assessments. The present retrospective observational study aims to provide a concise diagnostic criterion by combining all these parameters, which will aid in effective diagnosis and targeted treatment planning in the future., Material and Method: Previously diagnosed cases of these lesions were taken from the archives and categorized as Central Giant Cell Granuloma (CGCG), CGCG with secondary Aneurysmal Bone Cyst (ABC), primary ABC, and Brown's Tumour. Their demographic characteristics along with clinical, radiological, and histological data were retrieved and compiled into the table. The data was then analyzed and classified into aggressive and non-aggressive CGCG according to the criteria set in the study., Result: 10 reported cases were of isolated CGCG, 5 were CGCG with secondary ABC, 5 of Brown's tumor and 3 were that of conventional ABC. Out of these, the lesions showing extensive size along with an increased number of giant cells were categorized under aggressive CGCG, whereas those with less aggressive characteristics were categorized under non-aggressive CGCG. The aggressive category comprised 5 cases of isolated CGCG, 2 cases of CGCG with secondary ABC, 3 cases of primary ABC, and 5 of brown tumor, whilst the rest of the cases were categorized under non-aggressive CGCG., Conclusion: Since all these share overlapping features, thereby this type of concise categorization is the dire need so that the lesions can have a precise diagnosis with treatment and follow-up intervals for aggressive lesions., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction: A soft tissue aneurysmal bone cyst is an extremely rare tumor. The objective of the article is to present the clinical, radiological, and histopathological features of a very unusual neoplasm of soft tissues., Case Report: A 13-year-old male patient presented a painful, mobile, and rapidly growing mass on the posteromedial aspect of his left knee. Imaging studies revealed a mass that arose from the medial surface of the distal sartorius muscle, with extension to the subcutaneous fat tissue. It was a well-circumscribed solid tumor with a peripheral rim calcification on plain film, computerized tomography, and ultrasound (zonal phenomenon). On magnetic resonance imaging, a heterogenous mass on T1-weighted images (WI) and T2-WI was seen, with a peripheral hypointense rim in both sequences. An outstanding edema on T2-WI extending to the soft tissue and muscles of the medial compartment of the knee was detected. The mass was resected, and the "tumoral mimickers" histopathological and molecular (next-generation sequencing) diagnoses confirmed a soft tissue aneurysmal bone cyst. A follow-up showed that the patient was free of disease 12 months after surgery., Conclusion: Soft tissue aneurysmal bone cyst is a rare tumor. Appropriate clinical and radiological correlation should be performed to differentiate it from other tumor mimickers., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

An aneurysmal bone cyst (ABC) is a benign bone neoplasm that typically occurs during the first and second decades of life. ABC usually presents as a rapidly growing intramedullary expansile mass with multiple blood-filled cysts in the metaphysis of the long tubular bones. Here, we report a case of a periosteal solid ABC that was initially diagnosed as a high-grade surface osteosarcoma. A 10-year-old male was referred to our hospital for swelling and tenderness of the left upper arm. Radiography revealed periosteal mass without fluid-fluid levels. On performing open biopsy, the tumor showed hypercellular proliferation of uniform spindle to epithelioid cells with brisk mitotic activity (up to 12/2 mm 2 ) and lace-like osteoid formation, which was diagnosed as a high-grade surface osteosarcoma. After one course of chemotherapy using adriamycin and cisplatin, peripheral sclerosis was conspicuous, which led to pathological review and revision of diagnosis as "possibly osteoblastoma." The patient was disease-free for 4 years after marginal resection and curettage. Retrospective nanopore DNA sequencing unexpectedly detected a PAFAH1B1::USP6 rearrangement. The fusion gene was further validated using reverse transcription-polymerase chain reaction and the diagnosis was revised to ABC. Chromothripsis involving chromosome 17 has also been identified. Methylation analysis classified the present tumor as an ABC or non-ossifying fibroma using t-distributed stochastic neighbor embedding and unsupervised hierarchical clustering. This case report highlights the utility of nanopore DNA sequencing for soft tissue and bone tumor diagnosis., (© 2024 Wiley Periodicals LLC.)

Background: Juvenile Psammomatoid Ossifying Fibroma (JPOF) is a type of noncancerous bone tumor that usually affects adolescents in the craniomaxillofacial area. Clinical manifestations are usually symptoms caused by the tumor's invasive compression of surrounding tissues. Aneurysmal Bone Cyst (ABC) is also a benign bone tumor, and it typically occurs in long bones and the spine. Only 2% to 3% of cases occur in the head and neck. Due to the rarity of this combination of clinical cases, clinicians face difficulties in comprehensively understanding this complex lesion. Therefore, a comprehensive review of the clinical manifestations and characteristic imaging findings is necessary for surgeons., Case Presentations: On April 6, 2019, a 13-year-old boy presented with left maxillofacial bulge and pain for 1 month. Magnetic resonance imaging of the paranasal sinuses showed an irregular hive-like mass signal in the left maxillary sinus, and cystic changes with fluid levels were seen in the lesion. After the initial diagnosis of JPOF with primary ABC, we decided to perform a facial mid-facial resection of maxillary sinus tumor to remove the tumor tissue. Finally, after 3 recurrences and 4 operations, there was no tumor recurrence for 20 months after the last operation, and the patient was still under continuous follow-up., Conclusions: This case provided a reference for the diagnosis and treatment of JPOF combined with ABC. In particular, a new understanding of the association between the two diseases and the management of recurrence were proposed, which had the potential to improve clinical understanding of this complicated condition., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

This case report describes an aneurysmal bone cyst in the maxilla of a young dog. It describes the clinical presentation, diagnostics, management, and successful outcome of this highly unusual case. Bone cysts are described as benign, cavitated lesions within bone that are lined by reactive tissues. There is no epithelial lining in the lesions. Bone cysts usually contain hemorrhage or serosanguinous fluid. They usually appear in the long bones, and present as a swelling with or without pain., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.