Primary Osseous Low-grade Myxofibrosarcoma of Metatarsal Masquerading As Enchondroma: A Case Report

Overview

Journal J Orthop Case Rep

Specialty Orthopedics

Date 2023 Jan 20

PMID 36660161

Authors

Sanjay Desai

Jayeta Choudhury

Kevin Varghese

Tushar Kapoor

Affiliations

Soon will be listed here.

Abstract

Introduction: Low-grade myxofibrosarcoma (LGMFS) is a neoplasm of soft tissues. According to the World Health Organization, LGMFS is a malignant myofibroblastic tumor arising from deep soft tissues with potential for recurrence and late metastatic spread. The incidence estimates are 0.18/million, accounting for 0.6% of all soft-tissue sarcomas. It can directly invade the bone and metastasize to bone; however, primary osseous low-grade myxofibrosarcoma is a rare entity. Thus, recognizing atypical presentations of uncommon neoplasms are a pertinent skill for the radiologist due to significant implications for management.

Case Report: A 40-year-old male with complaints of midfoot pain was referred to radiology department for imaging. Radiographs of the foot showed a lytic mildly expansile lesion in the first metacarpal with wide zone of transition and no sclerotic margin or matrix calcification. Magnetic resource imaging (MRI) and computed tomography (CT) examination demonstrated well defined lobulated lesion which appeared heterogeneously hyperintense on T2W images with cortical breach, extraosseous soft-tissue component, and early rapid progressive enhancement. The radiological diagnosis of enchondroma with pathological fracture was considered. Atypical findings of early progressive enhancement and extraosseous soft-tissue component were, however, incongruous with enchondroma and possibility of an aggressive/malignant etiology was also considered. Hence, the lesion was biopsied and diagnosis of LGMFS was made and subsequently confirmed on excised specimen. Follow-up CT scan post 6 months of surgical resection demonstrated no recurrence.

Conclusion: Primary osseous LGMFS is a rare entity and radiologically mimics enchondromas. Both LGMFS and enchondromas show T2W hyperintensity. MRI features that distinguish LGMFS from enchondroma include low apparent diffusion coefficient values and slow progressive enhancement in dynamic contrast-enhanced MRI.

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References

Kayser D, Walton Z, Bruner E, Chapin R . Myxofibrosarcoma: First report of myxofibrosarcoma of bone arising at a bone infarct. Skeletal Radiol. 2017; 46(8):1143-1147. DOI: 10.1007/s00256-017-2651-4. View

Goel K, Slipak A, Ercolano L, Silverman J, Tang B . Primary Osseous Low-Grade Myxofibrosarcoma of Clavicle Presenting With Multiple Skeletal Metastases. Cureus. 2020; 12(8):e10170. PMC: 7529489. DOI: 10.7759/cureus.10170. View

Romeo S, Bovee J, Kroon H, Tirabosco R, Natali C, Zanatta L . Malignant fibrous histiocytoma and fibrosarcoma of bone: a re-assessment in the light of currently employed morphological, immunohistochemical and molecular approaches. Virchows Arch. 2012; 461(5):561-70. DOI: 10.1007/s00428-012-1306-z. View

Wang L, Li L, Chen D, Yang L, Li S, Cheng C . Low-grade Myofibroblastic sarcoma: clinical and imaging findings. BMC Med Imaging. 2019; 19(1):36. PMC: 6498682. DOI: 10.1186/s12880-018-0287-z. View

Li W, Li D, Zhu X, Lu S, He C, Yang Q . Low-grade myxofibrosarcoma following a metal implantation in femur: a case report. Diagn Pathol. 2014; 9:6. PMC: 3926977. DOI: 10.1186/1746-1596-9-6. View

Park S, Kim H, Lee J, Ko Y . Malignant fibrous histiocytoma of the head and neck: CT and MR imaging findings. AJNR Am J Neuroradiol. 2008; 30(1):71-6. PMC: 7051724. DOI: 10.3174/ajnr.A1317. View

Tsuchie H, Kaya M, Nagasawa H, Emori M, Murahashi Y, Mizushima E . Distant metastasis in patients with myxofibrosarcoma. Ups J Med Sci. 2017; 122(3):190-193. PMC: 5649325. DOI: 10.1080/03009734.2017.1356404. View

Kurisaki-Arakawa A, Suehara Y, Arakawa A, Takagi T, Takahashi M, Mitani K . Deeply located low-grade fibromyxoid sarcoma with FUS-CREB3L2 gene fusion in a 5-year-old boy with review of literature. Diagn Pathol. 2014; 9:163. PMC: 4167136. DOI: 10.1186/s13000-014-0163-2. View

Doyle L, Moller E, Dal Cin P, Fletcher C, Mertens F, Hornick J . MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. Am J Surg Pathol. 2011; 35(5):733-41. DOI: 10.1097/PAS.0b013e318210c268. View

10.

Frassica F, Sim F, Wold L . Case report 462: Grade 2 myxoid fibrosarcoma of femur. Skeletal Radiol. 1988; 17(1):77-80. DOI: 10.1007/BF00361461. View

11.

Jo V, Fletcher C . WHO classification of soft tissue tumours: an update based on the 2013 (4th) edition. Pathology. 2014; 46(2):95-104. DOI: 10.1097/PAT.0000000000000050. View

12.

Murphey M, Gross T, Rosenthal H . From the archives of the AFIP. Musculoskeletal malignant fibrous histiocytoma: radiologic-pathologic correlation. Radiographics. 1994; 14(4):807-26; quiz 827-8. DOI: 10.1148/radiographics.14.4.7938770. View

13.

Maretty-Nielsen K, Baerentzen S, Keller J, Buvarp Dyrop H, Safwat A . Low-Grade Fibromyxoid Sarcoma: Incidence, Treatment Strategy of Metastases, and Clinical Significance of the FUS Gene. Sarcoma. 2013; 2013:256280. PMC: 3683502. DOI: 10.1155/2013/256280. View

14.

Douis H, Saifuddin A . The imaging of cartilaginous bone tumours. I. Benign lesions. Skeletal Radiol. 2012; 41(10):1195-212. DOI: 10.1007/s00256-012-1427-0. View

15.

Woodhams R, Kan S, Iwabuchi K, Oka H, Sagiuchi T, Hayakawa K . A case of malignant fibrous histiocytoma (MFH) in the skull bone presenting extensive permeative osteolysis. Radiat Med. 2002; 20(3):141-5. View

16.

Finos L, Righi A, Frisoni T, Gambarotti M, Ghinelli C, Benini S . Primary extraskeletal myxoid chondrosarcoma of bone: Report of three cases and review of the literature. Pathol Res Pract. 2017; 213(5):461-466. DOI: 10.1016/j.prp.2017.02.008. View

17.

Nakayama K, Nemoto Y, Inoue Y, Mochizuki T, Soares Jr S, Ohata K . Malignant fibrous histiocytoma of the temporal bone with endocranial extension. AJNR Am J Neuroradiol. 1997; 18(2):331-4. PMC: 8338575. View

18.

Walden M, Murphey M, Vidal J . Incidental enchondromas of the knee. AJR Am J Roentgenol. 2008; 190(6):1611-5. DOI: 10.2214/AJR.07.2796. View

19.

Sargar K, Kao S, Spunt S, Hawkins D, Parham D, Coffin C . MRI and CT of Low-Grade Fibromyxoid Sarcoma in Children: A Report From Children's Oncology Group Study ARST0332. AJR Am J Roentgenol. 2015; 205(2):414-20. PMC: 4570741. DOI: 10.2214/AJR.14.13972. View

20.

Antonescu C, Argani P, Erlandson R, Healey J, Ladanyi M, Huvos A . Skeletal and extraskeletal myxoid chondrosarcoma: a comparative clinicopathologic, ultrastructural, and molecular study. Cancer. 1998; 83(8):1504-21. DOI: 10.1002/(sici)1097-0142(19981015)83:8<1504::aid-cncr5>3.0.co;2-b. View