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Dermatofibrosarcoma Protuberans of the Head and Neck: A Report of 16 Cases

Dermatofibrosarcoma Protuberans of the Head and Neck: A Report of 16 Cases Abstract • Dermatofibrosarcoma protuberans of the head and neck is a rare neoplasm. Overall, sarcomas account for less than 1% of all head and neck malignancies. Of 229 patients with sarcomas of the head and neck seen at our institution between 1955 and 1988, 14 (7%) were assigned the diagnosis of dermatofibrosarcoma protuberans after pathologic review. Two additional cases have been seen since 1988, bringing the total to 16. All cases were low-grade lesions. Follow-up ranged from 36 to 198 months, with a median of 114 months. Fifteen patients were managed with surgery alone, and nine (60%) developed local recurrence. Eight were salvaged with further surgery. There were no regional lymph node recurrences or distant metastases. One patient was judged to be a poor surgical candidate and received primary radiation therapy. He died disease free 3 years after treatment. At last follow-up, 15 (94%) of 16 patients were ultimately disease free after salvage treatment. The overall 5-year survival rate was 93% (13/14). We conclude from this series, and from a review of the literature, that wide surgical resection achieving good margins offers excellent probability of cure, and that radiation therapy is a reasonable alternative in patients who have unresectable lesions or who are medically inoperable. (Arch Otolaryngol Head Neck Surg. 1993;119:891-896) References 1. Figueiredo MTA, Marques LA, Campos-Filho N. Soft tissue sarcomas of the head and neck in adults and children: experience at a single institution and a review of the literature . Int J Cancer . 1988;42:198-200.Crossref 2. Tran L, Mark R, Calcaterra TC, Parker RG. Adult sarcomas of the head and neck: prognostic factors and treatment strategies . Cancer . 1992;70:169-177.Crossref 3. Darier J, Ferrand M. Dermatofibromes progressifs et recidivants ou fibrosarcomes de la peau . Ann Dermatol Venereol . 1924;5:545-562. 4. Hoffman E. Über das Knollentreibende Fibrosarkom der Haut (Dermatofibrosarcoma protuberans) . Derm Z . 1925;43:1-28.Crossref 5. Enzinger FM, Weiss SW. Soft Tissue Tumors . St Louis, Mo: Mosby—Year Book; 1988:252-268. 6. Pack GT, Tabah EJ. Dermatofibrosarcoma protuberans: a report of 39 cases . Arch Surg . 1951;62:391-411.Crossref 7. Taylor HB, Helwig EB. Dermatofibrosarcoma protuberans: a study of 115 cases . Cancer . 1962;15:717-725.Crossref 8. Bendix-Hansen K, Myhre-Jensen O, Kaae S. Dermatofibrosarcoma protuberans: a clinicopathological study of nineteen cases and a review of the world literature . Scand J Plast Reconstr Surg . 1983;17:247-252.Crossref 9. McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: an analysis of 86 cases, five with metastasis . Ann Surg . 1967;166:803-816.Crossref 10. Barnes L, Coleman J, Johnson J. Dermatofibrosarcoma protuberans of the head and neck . Arch Otolaryngol . 1984;10:398-404.Crossref 11. Burkhardt BR, Soule EH, Winkelman RK, Ivins JC. Dermatofibrosarcoma protuberans: a study of fifty-six cases . Am J Surg . 1966;111:638-644.Crossref 12. Roses DR, Valensi Q, La Trenta G, Harris MN. Surgical treatment of dermatofibrosarcoma protuberans . Surg Gynecol Obstet . 1986;162:449-452. 13. Weber PJ, Gretzula JC, Hevia O, Garland LB, Barquin P, Gould E. Dermatofibrosarcoma protuberans . J Dermatol Surg Oncol . 1988;14:555-558.Crossref 14. Junaid TA, Ani AN, Ejeckam GC. Dermatofibrosarcoma protuberans in the parotid gland: a case report . Br J Oral Surg . 1975;12:298-301.Crossref 15. Hess KA, Hanke WC, Estes NC, Shideler SJ. Chemosurgical reports: Myxoid dermatofibrosarcoma protuberans . J Dermatol Surg Oncol . 1985; 11:268-271.Crossref 16. Marks LB, Suit HD, Rosenberg AE, Wood WC. Dermatofibrosarcoma protuberans treated with radiation therapy . Int J Radiat Oncol Biol Phys . 1989;17:379-384.Crossref 17. McGregor JK. Role of surgery in the management of dermatofibrosarcoma protuberans . Ann Surg . 1961;154:255-258.Crossref 18. Rinck PA, Hebermalz HJ, Lobeck H. Effective radiotherapy in one case of dermatofibrosarcoma protuberans . Strahlentherapie . 1982;158:681-685. 19. American Joint Committee on Cancer. Soft tissues . In: Manual for Staging of Cancer . 3rd ed. Philadelphia, Pa: JB Lippincott; 1988:127-132. 20. Brenner W, Schaefler K, Chabra H, et al. Dermatofibrosarcoma protuberans metastatic to a regional lymph node: report of a case and review of the literature . Cancer . 1975;36:1897-1902.Crossref 21. Hobbs ER, Wheeland RG, Bailin PL, Ratz JL, Yetman RJ, Zins JE. Treatment of dermatofibrosarcoma protuberans with Mohs micrographic surgery . Ann Surg . 1988;207:102-107.Crossref 22. Simstein N, Tuthill RJ, Spesman EE, et al. Dermatofibrosarcoma protuberans: case reports and review of the literature . South Med J . 1977;70: 487-489.Crossref 23. Morman MR, Lin RY, Petrozzi JW. Dermatofibrosarcoma protuberans arising in a site of multiple immunizations . Arch Dermatol . 1979;115:1453.Crossref 24. Melezer M, Dvorsky C. Acanthosis nigricans bei Dermatofibrosarcoma protuberans mit multiplen Hautmetastasen . Hautarzt . 1957;8:54. 25. Schneidman D, Belizaire R. Arsenic exposure followed by the development of dermatofibrosarcoma protuberans . Cancer . 1986;58:1585-1587.Crossref 26. Shelley WB. Malignant melanoma and dermatofibrosarcoma protuberans in a 60-year-old patient with lifelong acrodermatitis enterapathica . J Am Acad Dermatol . 1982;6:63.Crossref 27. Robinson JK. Dermatofibrosarcoma protuberans resected by Moh's surgery (chemosurgery): a 5-year prospective study . J Am Acad Dermatol . 1985;12:1093-1098.Crossref 28. Rowsell AR, Poole MD, Godfrey AM. Dermatofibrosarcoma protuberans: the problems of surgical management . Br J Plast Surg . 1986;39:262-264.Crossref 29. Mikhail GR, Lynn BH. Dermatofibrosarcoma protuberans . J Dermatol Surg Oncol . 1978;4:81-84.Crossref 30. Giuliano A, Eilber FR. The rationale for planned reoperation after unplanned total excision of soft tissue sarcomas . J Clin Oncol . 1985;3:1344-1348. 31. Du Boulay CEH. Immunohistochemistry of soft tissue tumours: a review . J Pathol . 1985;146:77-81.Crossref 32. Kindblom LG, Jacobsen GK, Jacobsen M. Immunohistochemical investigations of tumors of supposed fibroblastic-histiocytic origin . Hum Pathol . 1982;13:834-840.Crossref 33. Fletcher CDM, Evans BJ, MacArtney JC, et al. Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochemical study with a review of the literature . Histopathology . 1985;9:921-938.Crossref 34. O'Dowd J, Laidler P. Progression of dermatofibrosarcoma protuberans to malignant fibrous histiocytoma: report of a case with implications for tumor histogenesis . Hum Pathol . 1988;19:368-370.Crossref 35. Sagi A, Ben-Yakar Y, Mahler J. A ten-year-old boy with dermatofibrosarcoma protuberans of the face . J Dermatol Surg Oncol . 1987;13:82-83.Crossref 36. Friedman RJ, Rigel DS, Kopf AW, Harris MN, Baker D, eds. Cancer of the Skin . Philadelphia, Pa: WB Saunders Co; 1991:266-268. 37. Tamoney HJ. Dermatofibrosarcoma protuberans: a review of 12 cases . Conn Med . 1971;35:674-679. 38. Farhood A, Hadju S, Shiu M, Strong E. Soft tissue sarcomas of the head and neck in adults . Am J Surg . 1990;160:365-369.Crossref 39. Potter DA, Glenn J, Kinsella T, et al. Patterns of recurrence in patients with high-grade soft-tissue sarcomas . J Clin Oncol . 1985;3:353-357. 40. Suit HD, Mankin HJ, Wood WC, et al. Treatment of the patient with stage M-O soft tissue sarcoma . J Clin Oncol. 1988;6:854-861. 41. Huth JF, Eilber FR. Patterns of metastatic spread following resection of extremity soft-tissue sarcomas and strategies for treatment . Semin Surg Oncol . 1988;4:20-25.Crossref 42. Perez CA, Brady LW. Soft tissue sarcomas . In: Principles and Practice of Radiation Oncology . Philadelphia, Pa: JB Lippincott; 1992:1399-1412. 43. Lindberg RD, Martin RG, Romsdahl MM, et al. Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas . Cancer . 1981;47:2391-2397.Crossref 44. Posner MC, Shiu MH, Newsome JL, Hadju SI, Gaynor JJ, Brennan MF. The desmoid tumor . Arch Surg . 1989;124:191-196.Crossref 45. Assad WA, Nori D, Hilaris BS, Shiu MH, Hajdu SI. Role of brachytherapy in the management of desmoid tumors . Int J Radiat Oncol Biol Phys . 1986;12:901-906.Crossref 46. Greenberg HM, Goebel R, Weichselbaum RR, Greenberger JS, Chaffey JT, Cassady JR. Radiation therapy in the treatment of aggressive fibromatosis . Int J Radiat Oncol Biol Phys . 1981;7:305-310.Crossref 47. Keil KD, Suit HD. Radiation therapy in the treatment of agressive fibromatosis (desmoid tumors) . Cancer . 1984;54:2051-2055.Crossref 48. Leibel SA, Wara WM, Hill DR, et al. Desmoid tumors: local control and patterns of relapse following radiation therapy . Int J Radiat Oncol Biol Phys . 1983;9:1167-1171.Crossref 49. Elias AD, Antman KH. Adjuvant chemotherapy for soft tissue sarcoma: an approach in search of an effective regimen . Semin Oncol . 1989;16:305-311. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Otolaryngology - Head & Neck Surgery American Medical Association

Dermatofibrosarcoma Protuberans of the Head and Neck: A Report of 16 Cases

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American Medical Association
Copyright
Copyright © 1993 American Medical Association. All Rights Reserved.
ISSN
0886-4470
eISSN
1538-361X
DOI
10.1001/archotol.1993.01880200097014
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Abstract

Abstract • Dermatofibrosarcoma protuberans of the head and neck is a rare neoplasm. Overall, sarcomas account for less than 1% of all head and neck malignancies. Of 229 patients with sarcomas of the head and neck seen at our institution between 1955 and 1988, 14 (7%) were assigned the diagnosis of dermatofibrosarcoma protuberans after pathologic review. Two additional cases have been seen since 1988, bringing the total to 16. All cases were low-grade lesions. Follow-up ranged from 36 to 198 months, with a median of 114 months. Fifteen patients were managed with surgery alone, and nine (60%) developed local recurrence. Eight were salvaged with further surgery. There were no regional lymph node recurrences or distant metastases. One patient was judged to be a poor surgical candidate and received primary radiation therapy. He died disease free 3 years after treatment. At last follow-up, 15 (94%) of 16 patients were ultimately disease free after salvage treatment. The overall 5-year survival rate was 93% (13/14). We conclude from this series, and from a review of the literature, that wide surgical resection achieving good margins offers excellent probability of cure, and that radiation therapy is a reasonable alternative in patients who have unresectable lesions or who are medically inoperable. (Arch Otolaryngol Head Neck Surg. 1993;119:891-896) References 1. Figueiredo MTA, Marques LA, Campos-Filho N. Soft tissue sarcomas of the head and neck in adults and children: experience at a single institution and a review of the literature . Int J Cancer . 1988;42:198-200.Crossref 2. Tran L, Mark R, Calcaterra TC, Parker RG. Adult sarcomas of the head and neck: prognostic factors and treatment strategies . Cancer . 1992;70:169-177.Crossref 3. Darier J, Ferrand M. Dermatofibromes progressifs et recidivants ou fibrosarcomes de la peau . Ann Dermatol Venereol . 1924;5:545-562. 4. Hoffman E. Über das Knollentreibende Fibrosarkom der Haut (Dermatofibrosarcoma protuberans) . Derm Z . 1925;43:1-28.Crossref 5. Enzinger FM, Weiss SW. Soft Tissue Tumors . St Louis, Mo: Mosby—Year Book; 1988:252-268. 6. Pack GT, Tabah EJ. Dermatofibrosarcoma protuberans: a report of 39 cases . Arch Surg . 1951;62:391-411.Crossref 7. Taylor HB, Helwig EB. Dermatofibrosarcoma protuberans: a study of 115 cases . Cancer . 1962;15:717-725.Crossref 8. Bendix-Hansen K, Myhre-Jensen O, Kaae S. Dermatofibrosarcoma protuberans: a clinicopathological study of nineteen cases and a review of the world literature . Scand J Plast Reconstr Surg . 1983;17:247-252.Crossref 9. McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: an analysis of 86 cases, five with metastasis . Ann Surg . 1967;166:803-816.Crossref 10. Barnes L, Coleman J, Johnson J. Dermatofibrosarcoma protuberans of the head and neck . Arch Otolaryngol . 1984;10:398-404.Crossref 11. Burkhardt BR, Soule EH, Winkelman RK, Ivins JC. Dermatofibrosarcoma protuberans: a study of fifty-six cases . Am J Surg . 1966;111:638-644.Crossref 12. Roses DR, Valensi Q, La Trenta G, Harris MN. Surgical treatment of dermatofibrosarcoma protuberans . Surg Gynecol Obstet . 1986;162:449-452. 13. Weber PJ, Gretzula JC, Hevia O, Garland LB, Barquin P, Gould E. Dermatofibrosarcoma protuberans . J Dermatol Surg Oncol . 1988;14:555-558.Crossref 14. Junaid TA, Ani AN, Ejeckam GC. Dermatofibrosarcoma protuberans in the parotid gland: a case report . Br J Oral Surg . 1975;12:298-301.Crossref 15. Hess KA, Hanke WC, Estes NC, Shideler SJ. Chemosurgical reports: Myxoid dermatofibrosarcoma protuberans . J Dermatol Surg Oncol . 1985; 11:268-271.Crossref 16. Marks LB, Suit HD, Rosenberg AE, Wood WC. Dermatofibrosarcoma protuberans treated with radiation therapy . Int J Radiat Oncol Biol Phys . 1989;17:379-384.Crossref 17. McGregor JK. Role of surgery in the management of dermatofibrosarcoma protuberans . Ann Surg . 1961;154:255-258.Crossref 18. Rinck PA, Hebermalz HJ, Lobeck H. Effective radiotherapy in one case of dermatofibrosarcoma protuberans . Strahlentherapie . 1982;158:681-685. 19. American Joint Committee on Cancer. Soft tissues . In: Manual for Staging of Cancer . 3rd ed. Philadelphia, Pa: JB Lippincott; 1988:127-132. 20. Brenner W, Schaefler K, Chabra H, et al. Dermatofibrosarcoma protuberans metastatic to a regional lymph node: report of a case and review of the literature . Cancer . 1975;36:1897-1902.Crossref 21. Hobbs ER, Wheeland RG, Bailin PL, Ratz JL, Yetman RJ, Zins JE. Treatment of dermatofibrosarcoma protuberans with Mohs micrographic surgery . Ann Surg . 1988;207:102-107.Crossref 22. Simstein N, Tuthill RJ, Spesman EE, et al. Dermatofibrosarcoma protuberans: case reports and review of the literature . South Med J . 1977;70: 487-489.Crossref 23. Morman MR, Lin RY, Petrozzi JW. Dermatofibrosarcoma protuberans arising in a site of multiple immunizations . Arch Dermatol . 1979;115:1453.Crossref 24. Melezer M, Dvorsky C. Acanthosis nigricans bei Dermatofibrosarcoma protuberans mit multiplen Hautmetastasen . Hautarzt . 1957;8:54. 25. Schneidman D, Belizaire R. Arsenic exposure followed by the development of dermatofibrosarcoma protuberans . Cancer . 1986;58:1585-1587.Crossref 26. Shelley WB. Malignant melanoma and dermatofibrosarcoma protuberans in a 60-year-old patient with lifelong acrodermatitis enterapathica . J Am Acad Dermatol . 1982;6:63.Crossref 27. Robinson JK. Dermatofibrosarcoma protuberans resected by Moh's surgery (chemosurgery): a 5-year prospective study . J Am Acad Dermatol . 1985;12:1093-1098.Crossref 28. Rowsell AR, Poole MD, Godfrey AM. Dermatofibrosarcoma protuberans: the problems of surgical management . Br J Plast Surg . 1986;39:262-264.Crossref 29. Mikhail GR, Lynn BH. Dermatofibrosarcoma protuberans . J Dermatol Surg Oncol . 1978;4:81-84.Crossref 30. Giuliano A, Eilber FR. The rationale for planned reoperation after unplanned total excision of soft tissue sarcomas . J Clin Oncol . 1985;3:1344-1348. 31. Du Boulay CEH. Immunohistochemistry of soft tissue tumours: a review . J Pathol . 1985;146:77-81.Crossref 32. Kindblom LG, Jacobsen GK, Jacobsen M. Immunohistochemical investigations of tumors of supposed fibroblastic-histiocytic origin . Hum Pathol . 1982;13:834-840.Crossref 33. Fletcher CDM, Evans BJ, MacArtney JC, et al. Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochemical study with a review of the literature . Histopathology . 1985;9:921-938.Crossref 34. O'Dowd J, Laidler P. Progression of dermatofibrosarcoma protuberans to malignant fibrous histiocytoma: report of a case with implications for tumor histogenesis . Hum Pathol . 1988;19:368-370.Crossref 35. Sagi A, Ben-Yakar Y, Mahler J. A ten-year-old boy with dermatofibrosarcoma protuberans of the face . J Dermatol Surg Oncol . 1987;13:82-83.Crossref 36. Friedman RJ, Rigel DS, Kopf AW, Harris MN, Baker D, eds. Cancer of the Skin . Philadelphia, Pa: WB Saunders Co; 1991:266-268. 37. Tamoney HJ. Dermatofibrosarcoma protuberans: a review of 12 cases . Conn Med . 1971;35:674-679. 38. Farhood A, Hadju S, Shiu M, Strong E. Soft tissue sarcomas of the head and neck in adults . Am J Surg . 1990;160:365-369.Crossref 39. Potter DA, Glenn J, Kinsella T, et al. Patterns of recurrence in patients with high-grade soft-tissue sarcomas . J Clin Oncol . 1985;3:353-357. 40. Suit HD, Mankin HJ, Wood WC, et al. Treatment of the patient with stage M-O soft tissue sarcoma . J Clin Oncol. 1988;6:854-861. 41. Huth JF, Eilber FR. Patterns of metastatic spread following resection of extremity soft-tissue sarcomas and strategies for treatment . Semin Surg Oncol . 1988;4:20-25.Crossref 42. Perez CA, Brady LW. Soft tissue sarcomas . In: Principles and Practice of Radiation Oncology . Philadelphia, Pa: JB Lippincott; 1992:1399-1412. 43. Lindberg RD, Martin RG, Romsdahl MM, et al. Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas . Cancer . 1981;47:2391-2397.Crossref 44. Posner MC, Shiu MH, Newsome JL, Hadju SI, Gaynor JJ, Brennan MF. The desmoid tumor . Arch Surg . 1989;124:191-196.Crossref 45. Assad WA, Nori D, Hilaris BS, Shiu MH, Hajdu SI. Role of brachytherapy in the management of desmoid tumors . Int J Radiat Oncol Biol Phys . 1986;12:901-906.Crossref 46. Greenberg HM, Goebel R, Weichselbaum RR, Greenberger JS, Chaffey JT, Cassady JR. Radiation therapy in the treatment of aggressive fibromatosis . Int J Radiat Oncol Biol Phys . 1981;7:305-310.Crossref 47. Keil KD, Suit HD. Radiation therapy in the treatment of agressive fibromatosis (desmoid tumors) . Cancer . 1984;54:2051-2055.Crossref 48. Leibel SA, Wara WM, Hill DR, et al. Desmoid tumors: local control and patterns of relapse following radiation therapy . Int J Radiat Oncol Biol Phys . 1983;9:1167-1171.Crossref 49. Elias AD, Antman KH. Adjuvant chemotherapy for soft tissue sarcoma: an approach in search of an effective regimen . Semin Oncol . 1989;16:305-311.

Journal

Archives of Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Aug 1, 1993

References