Fibrous Dysplasia Masquerading as Sternal Malignancy: A Rare and Challenging Presentation

被引:0
|
作者
Devarkonda, Vishal [1 ]
Gaddam, Shiva [2 ]
Morisetti, Manasa [3 ]
Arora, Kshitij [4 ]
Beedupalli, Kavitha [5 ]
机构
[1] Louisiana State Univ Hlth Sci Ctr, Internal Med, Shreveport, LA USA
[2] Louisiana State Univ Hlth Sci Ctr, Hematol & Med Oncol, Shreveport, LA USA
[3] Louisiana State Univ Hlth Sci Ctr, Pathol & Lab Med, Shreveport, LA USA
[4] Louisiana State Univ Hlth Sci Ctr, Pathol, Shreveport, LA USA
[5] Louisiana State Univ Hlth Sci Ctr, Hematol & Oncol, Shreveport, LA USA
关键词
albright syndrome; mazabraud syndrome; sternal malignancy; mccune-albright syndrome; fibrous dysplasia (fd); TUMORS; BONE;
D O I
10.7759/cureus.50833
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
This case report presents a rare and challenging manifestation of polyostotic fibrous dysplasia (FD), a skeletal developmental anomaly characterized by the proliferation of fibrous connective tissue intermingled with irregular bony trabeculae. While monostotic FD is more common, polyostotic FD can occur in the context of McCune-Albright syndrome, a multisystem developmental disorder. Our patient, a 55-year-old female with a history of diabetes, hypothyroidism, and dyslipidemia, presented with progressively worsening dysphagia, sternal pain, and swelling over three years. Clinical examination revealed a tender and hard swelling in the upper sternal area, prompting further evaluation. Laboratory results, including bone turnover markers, were unremarkable. Imaging studies unveiled a sizable anterior mediastinal lesion with heterogeneous enhancement and coarse calcifications, initially raising concerns of malignancy. Subsequent positron emission tomography scan findings confirmed FD involvement in both the sternum and right femur. Histopathology of the mediastinal mass revealed a spindle cell neoplasm with bony metaplasia, consistent with FD, supported by immunohistochemistry. A multidisciplinary team affirmed the diagnosis of polyostotic FD, and follow-up imaging after one year demonstrated no significant change in lesion size, confirming a benign etiology. While bisphosphonate therapy was planned, regrettably, the patient was lost to follow-up. This case underscores the importance of a comprehensive, multidisciplinary approach in diagnosing and managing complex presentations of FD, ultimately contributing to improved patient care and outcomes in such instances.
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页数:8
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