Antineoplastic therapy for miscellaneous benign diseases affecting soft tissue and bone
- Thomas F DeLaney, MD
Thomas F DeLaney, MD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Radiation Oncology
- Harvard Medical School
The majority of benign soft tissue masses are usually non-neoplastic and of little clinical consequence. However, some locally aggressive fibromatoses (desmoid tumors), infiltrating lipomas, neurofibromas, and vertebral hemangiomas may cause significant morbidity and, in some cases, mortality.
Radiation therapy for a variety of benign diseases affecting soft tissue and bone will be reviewed here. These include Gorham's disease, desmoplastic fibroma of bone, pigmented villonodular synovitis (tenosynovial giant cell tumor), and symptomatic vertebral hemangiomas. Radiation therapy for other conditions affecting soft tissue, such as desmoid tumors, keloid, ganglia, and Dupuytren's contracture, is discussed separately. (See "Desmoid tumors: Epidemiology, risk factors, molecular pathogenesis, clinical presentation, diagnosis, and local therapy" and "Keloids and hypertrophic scars" and "Ganglion cysts of the wrist and hand" and "Dupuytren's contracture", section on 'Other interventions'.)
Gorham's disease (also known as Gorham-Stout disease, disappearing bone disease, or essential osteolysis) is a rare disorder characterized by relatively painless bone destruction with massive osteolysis due to progression of lymphangiomatous tissue . There may also be significant osteoclast activation. Why this occurs is not clear, but increased levels of interleukin-6 may contribute to the osteolysis .
The pattern is that of lytic destruction of cortical bone and extension across joints to the adjacent bone. There is usually little or no soft tissue mass.
Treatment — Treatment consists of resection, when this is feasible from the standpoints of the medical condition of the patient, the technical aspects of the procedure, and the likelihood of retained function. There is anecdotal evidence that radiation therapy (RT) also may be effective [3-5]:
- Manisali M, Ozaksoy D. Gorham disease: correlation of MR findings with histopathologic changes. Eur Radiol 1998; 8:1647.
- Devlin RD, Bone HG 3rd, Roodman GD. Interleukin-6: a potential mediator of the massive osteolysis in patients with Gorham-Stout disease. J Clin Endocrinol Metab 1996; 81:1893.
- Mawk JR, Obukhov SK, Nichols WD, et al. Successful conservative management of Gorham disease of the skull base and cervical spine. Childs Nerv Syst 1997; 13:622.
- Dunbar SF, Rosenberg A, Mankin H, et al. Gorham's massive osteolysis: the role of radiation therapy and a review of the literature. Int J Radiat Oncol Biol Phys 1993; 26:491.
- Heyd R, Micke O, Surholt C, et al. Radiation therapy for Gorham-Stout syndrome: results of a national patterns-of-care study and literature review. Int J Radiat Oncol Biol Phys 2011; 81:e179.
- Duffy BM, Manon R, Patel RR, Welsh JS. A case of Gorham's disease with chylothorax treated curatively with radiation therapy. Clin Med Res 2005; 3:83.
- Ebert CS Jr, Zdanski C, Ardeshirpour F, et al. Recurrent infantile myofibromatosis: a report of conservative management and discussion of treatment strategies. Ear Nose Throat J 2008; 87:E4.
- Hagberg H, Lamberg K, Aström G. Alpha-2b interferon and oral clodronate for Gorham's disease. Lancet 1997; 350:1822.
- Kuriyama DK, McElligott SC, Glaser DW, Thompson KS. Treatment of Gorham-Stout disease with zoledronic acid and interferon-α: a case report and literature review. J Pediatr Hematol Oncol 2010; 32:579.
- Hammer F, Kenn W, Wesselmann U, et al. Gorham-Stout disease--stabilization during bisphosphonate treatment. J Bone Miner Res 2005; 20:350.
- Rössler J, Saueressig U, Kayser G, et al. Personalized Therapy for Generalized Lymphatic Anomaly/Gorham-Stout Disease With a Combination of Sunitinib and Taxol. J Pediatr Hematol Oncol 2015; 37:e481.
- Miettinen M, Fetsch JF. Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts. Hum Pathol 1998; 29:676.
- Bernal K, Nelson M, Neff JR, et al. Translocation (2;11)(q31;q12) is recurrent in collagenous fibroma (desmoplastic fibroblastoma). Cancer Genet Cytogenet 2004; 149:161.
- Hauben EI, Jundt G, Cleton-Jansen AM, et al. Desmoplastic fibroma of bone: an immunohistochemical study including beta-catenin expression and mutational analysis for beta-catenin. Hum Pathol 2005; 36:1025.
- Sanfilippo NJ, Wang GJ, Larner JM. Desmoplastic fibroma: a role for radiotherapy? South Med J 1995; 88:1267.
- Nag HL, Kumar R, Bhan S, et al. Radiotherapy for desmoplastic fibroma of bone: a case report. J Orthop Surg (Hong Kong) 2003; 11:90.
- Darling JM, Goldring SR, Harada Y, et al. Multinucleated cells in pigmented villonodular synovitis and giant cell tumor of tendon sheath express features of osteoclasts. Am J Pathol 1997; 150:1383.
- Müller LP, Bitzer M, Degreif J, Rommens PM. Pigmented villonodular synovitis of the shoulder: review and case report. Knee Surg Sports Traumatol Arthrosc 1999; 7:249.
- Mankin H, Trahan C, Hornicek F. Pigmented villonodular synovitis of joints. J Surg Oncol 2011; 103:386.
- Bhimani MA, Wenz JF, Frassica FJ. Pigmented villonodular synovitis: keys to early diagnosis. Clin Orthop Relat Res 2001; :197.
- Dürr HR, Stäbler A, Maier M, Refior HJ. Pigmented villonodular synovitis. Review of 20 cases. J Rheumatol 2001; 28:1620.
- Hamlin BR, Duffy GP, Trousdale RT, Morrey BF. Total knee arthroplasty in patients who have pigmented villonodular synovitis. J Bone Joint Surg Am 1998; 80:76.
- Ogilvie-Harris DJ, Weisleder L. Arthroscopic synovectomy of the knee: is it helpful? Arthroscopy 1995; 11:91.
- Mollon B, Lee A, Busse JW, et al. The effect of surgical synovectomy and radiotherapy on the rate of recurrence of pigmented villonodular synovitis of the knee: an individual patient meta-analysis. Bone Joint J 2015; 97-B:550.
- Horoschak M, Tran PT, Bachireddy P, et al. External beam radiation therapy enhances local control in pigmented villonodular synovitis. Int J Radiat Oncol Biol Phys 2009; 75:183.
- Blanco CE, Leon HO, Guthrie TB. Combined partial arthroscopic synovectomy and radiation therapy for diffuse pigmented villonodular synovitis of the knee. Arthroscopy 2001; 17:527.
- Bisbinas I, De Silva U, Grimer RJ. Pigmented villonodular synovitis of the foot and ankle: a 12-year experience from a tertiary orthopedic Oncology Unit. J Foot Ankle Surg 2004; 43:407.
- Chin KR, Barr SJ, Winalski C, et al. Treatment of advanced primary and recurrent diffuse pigmented villonodular synovitis of the knee. J Bone Joint Surg Am 2002; 84-A:2192.
- Berger B, Ganswindt U, Bamberg M, Hehr T. External beam radiotherapy as postoperative treatment of diffuse pigmented villonodular synovitis. Int J Radiat Oncol Biol Phys 2007; 67:1130.
- Heyd R, Micke O, Berger B, et al. Radiation therapy for treatment of pigmented villonodular synovitis: results of a national patterns of care study. Int J Radiat Oncol Biol Phys 2010; 78:199.
- Griffin AM, Ferguson PC, Catton CN, et al. Long-term outcome of the treatment of high-risk tenosynovial giant cell tumor/pigmented villonodular synovitis with radiotherapy and surgery. Cancer 2012; 118:4901.
- Shabat S, Kollender Y, Merimsky O, et al. The use of surgery and yttrium 90 in the management of extensive and diffuse pigmented villonodular synovitis of large joints. Rheumatology (Oxford) 2002; 41:1113.
- Franssen MJ, Boerbooms AM, Karthaus RP, et al. Treatment of pigmented villonodular synovitis of the knee with yttrium-90 silicate: prospective evaluations by arthroscopy, histology, and 99mTc pertechnetate uptake measurements. Ann Rheum Dis 1989; 48:1007.
- Kroot EJ, Kraan MC, Smeets TJ, et al. Tumour necrosis factor alpha blockade in treatment resistant pigmented villonodular synovitis. Ann Rheum Dis 2005; 64:497.
- West RB, Rubin BP, Miller MA, et al. A landscape effect in tenosynovial giant-cell tumor from activation of CSF1 expression by a translocation in a minority of tumor cells. Proc Natl Acad Sci U S A 2006; 103:690.
- Blay JY, El Sayadi H, Thiesse P, et al. Complete response to imatinib in relapsing pigmented villonodular synovitis/tenosynovial giant cell tumor (PVNS/TGCT). Ann Oncol 2008; 19:821.
- Gelderblom H, Perol D, Chevreau C, et al. An open-label international multicentric phase II study of nilotinib in progressive pigmented villo-noduylar synovotos (PVNS) not amenable to conservative surgical treatment (abstract). J Clin Oncol 31, 2013 (suppl; abstr 10516). http://meetinglibrary.asco.org/content/117842-132 (Accessed on June 11, 2013).
- Cassier PA, Gelderblom H, Stacchiotti S, et al. Efficacy of imatinib mesylate for the treatment of locally advanced and/or metastatic tenosynovial giant cell tumor/pigmented villonodular synovitis. Cancer 2012; 118:1649.
- Gelderblom H, Perol D, Chevreau C, et al. An open-label international multicentric phase II study of nilotinib in progressive pigmented villo-nodular synovitis (PVNS) not amenable to a conservative surgical treatmen (abstract). J Clin Oncol 31, 2013 (suppl; abstr 10516). http://meetinglibrary.asco.org/content/117842-132 (Accessed on June 18, 2013).
- Tap WD, Wainberg ZA, Anthony SP, et al. Structure-Guided Blockade of CSF1R Kinase in Tenosynovial Giant-Cell Tumor. N Engl J Med 2015; 373:428.
- Cassier PA, Italiano A, Gomez-Roca CA, et al. CSF1R inhibition with emactuzumab in locally advanced diffuse-type tenosynovial giant cell tumours of the soft tissue: a dose-escalation and dose-expansion phase 1 study. Lancet Oncol 2015; 16:949.
- Acosta FL Jr, Dowd CF, Chin C, et al. Current treatment strategies and outcomes in the management of symptomatic vertebral hemangiomas. Neurosurgery 2006; 58:287.
- Pastushyn AI, Slin'ko EI, Mirzoyeva GM. Vertebral hemangiomas: diagnosis, management, natural history and clinicopathological correlates in 86 patients. Surg Neurol 1998; 50:535.
- Bartels RH, Grotenhuis JA, Van Der Spek JA. Symptomatic vertebral hemangiomas. J Neurosurg Sci 1991; 35:187.
- Brunot S, Berge J, Barreau X, et al. [Long term clinical follow up of vertebral hemangiomas treated by percutaneous vertebroplasty]. J Radiol 2005; 86:41.
- Persaud T. The polka-dot sign. Radiology 2008; 246:980.
- Heyd R, Seegenschmiedt MH, Rades D, et al. Radiotherapy for symptomatic vertebral hemangiomas: results of a multicenter study and literature review. Int J Radiat Oncol Biol Phys 2010; 77:217.
- Rades D, Bajrovic A, Alberti W, Rudat V. Is there a dose-effect relationship for the treatment of symptomatic vertebral hemangioma? Int J Radiat Oncol Biol Phys 2003; 55:178.
- Miszczyk L, Tukiendorf A. Radiotherapy of painful vertebral hemangiomas: the single center retrospective analysis of 137 cases. Int J Radiat Oncol Biol Phys 2012; 82:e173.