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Diagnosing Benign Soft Tissue Tumors: A Visual Guideline from Presentation through Treatment

March 01, 2017

Contributors: Ian O'Connor, BS; James C Wittig, MD; Alexander P Decilveo; Alexander P Decilveo

Benign soft tissue tumors are much more common than bone and soft tissue sarcomas. Approximately 500,000 benign soft tissue tumors are diagnosed annually in the United States. The most common benign soft tissue tumors are lipoma, hemangioma, fibromatosis/desmoplastic fibroma, myxoma, schwannoma/neurilemmoma, neurofibroma, giant cell tumor of tendon sheath (GCTTS). These tumors primarily affect adolescents and middle-aged adults. Benign soft tissue tumors originate from the muscles, ligaments, nerves, and blood vessels and can affect nearly any anatomical compartment, most commonly the extremities and trunk. Some tumors have a predilection for certain anatomical locations such as GCTTS for the volar aspect of the fingers and hand. Clinically, soft tissue tumors vary in appearance and behavior. These masses may be slow growing, asymptomatic, and painless (i.e. lipoma) or symptomatic and painful (i.e. schwannoma, GCTTS, and fibromatosis). Some tumors may be locally aggressive or differentiate into a malignant variety. For example, the diffuse form of GCTTS is considered a locally aggressive and destructive lesion that has a high rate of recurrence following excision. Desmoplastic fibroma may differentiate into a grade 1 fibrosarcoma and all retroperitoneal fatty tumors are considered malignant by default due to anatomical location. The aforementioned tumors must be monitored closely for malignant transformations. Radiological imaging of benign soft tissue tumors is generally non-specific, however certain signals may be characteristic. Histopathological studies are diverse among soft tissue masses, yet help greatly when differentiating between benign tumors and their malignant counterparts. Treatment ranges from observation to marginal excision to wide excision. Some lesions can be observed if asymptomatic, however excision is the treatment of choice if the lesion is painful, large (> 5cm), or deep seated. Depending on the entity, chemotherapy and/or radiation may be recommended, yet their use is controversial. If a benign soft tissue tumor is suspected, the patient should be referred to an orthopaedic oncologist for treatment. References: 1. Adani R, Tarallo L, Mugnai R, Colopi S. Schwannomas of the upper extremity: analysis of 34 cases. Acta Neurochir (Wien). 2014 Dec;156(12):2325-30. 2. Baheti AD, Tirumani SH, Rosenthal MH, Howard SA, Shinagare AB, Ramaiya NH, Jagannathan JP. Myxoid soft-tissue neoplasms: comprehensive update of the taxonomy and MRI features. AJR Am J Roentgenol. 2015 Feb;204(2):374-85. 3. Balach T, Stacy GS, Haydon RC (2011) The clinical evaluation of soft tissue tumors. Radiol Clin North Am 49(6):1185–1196. 4. Di Grazia S, Succi G, Fragetta F, Perrotta RE. Giant cell tumor of tendon sheath: study of 64 cases and review of literature. G Chir. 2013 May-Jun;34(5-6):149-52. Review. 5. Erwteman AS, Balach T. Clinical evaluation and management of benign soft tissue tumors of the extremities. Cancer Treat Res. 2014;162:171-202. 6. Gartner L, Pearce CJ, Saifuddin A (2009) The role of the plain radiograph in the characterisation of soft tissue tumours. Skeletal Radiol 38(6):549–558. 7. Kransdorf MJ, Murphey MD (2000) Radiologic evaluation of soft-tissue masses: a current perspective. AJR Am J Roentgenol 175(3):575–587. 8. Mallinson PI, Chou H, Forster BB, Munk PL. Radiology of soft tissue tumors. Surg Oncol Clin N Am. 2014 Oct;23(4):911-36. 9. Rydholm A, Berg NO (1983) Size, site and clinical incidence of lipoma. Factors in the differential diagnosis of lipoma and sarcoma. Acta Orthop Scand 54(6):929–934. 10. Salah MB, Mekni A, Azouz H, Blel A, Kharrat S, Kchir N, Haouet S, Zitouna M. Infantile desmoplastic fibroma of the maxilla. Tunis Med. 2008 Aug;86(8):749-51. 11. Schneider M, Zimmermann AC, Depprich RA, Kübler NR, Engers R, Naujoks CD, Handschel J. Desmoplastic fibroma of the mandible--review of the literature and presentation of a rare case. Head Face Med. 2009 Nov 24;5:25. 12. Slattery WH. Neurofibromatosis type 2. Otolaryngol Clin North Am. 2015 Jun;48(3):443-60. 13. Subhashraj K, Balanand S, Pajaniammalle S. Ancient schwannoma arising from mental nerve. A case report and review. Med Oral Patol Oral Cir Bucal. 2009 Jan 1;14(1):E12-4. 14. Vanhoenacker FM, Hauben E, De Beuckeleer LH, Willemen D, Van Marck E, De Schepper AM. Desmoplastic fibroma of bone: MRI features. Skeletal Radiol. 2000 Mar;29(3):171-5. 15. Wang CS, Duan Q, Xue YJ, Huang XM, Wang LL, Chen ZY, Chen JH, Sun B. Giant cell tumour of tendon sheath with bone invasion in extremities: analysis of clinical and imaging findings. Radiol Med. 2015 Aug;120(8):745-52. 16. Zhang Y, Huang J, Ma X, Wang X, Zhang C, Chen L. Giant cell tumor of the tendon sheath in the foot and ankle: case series and review of the literature. J Foot Ankle Surg. 2013 Jan-Feb;52(1):24-7.

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