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June 1984, Vol 110, No. 6 >
ARTICLE | June 1984

Dermatofibrosarcoma Protuberans of the Head and Neck FREE

Leon Barnes, MD; Jack A. Coleman, Jr, MD; Jonas T. Johnson, MD
[+] Author Affiliations

Accepted for publication Dec 31, 1983.

Read in part before the Pennsylvania Academy of Ophthalmology and Otolaryngology, Hershey, March 27, 1983, and the American Academy of Otolaryngology, Anaheim, Calif, Oct 25, 1983.

Reprint requests to the Department of Pathology, Presbyterian-University Hospital, DeSoto and O'Hara streets, Pittsburgh, PA 15213 (Dr Barnes).


Arch Otolaryngol Head Neck Surg. 1984;110(6):398-404. doi:10.1001/archotol.1984.00800320052012
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References
Comments

• To elucidate the natural history of dermatofibrosarcoma protuberans of the head and neck, 17 cases were reviewed and compared with 92 others contained within the English literature. The prototypic case is seen as a firm, solitary, slowly enlarging, asymptomatic, cutaneous nodule between 2 and 5 cm in size and of two years' duration. It is more common in men and in persons aged between 30 and 40 years. The scalp and neck-supraclavicular fossa are the most common sites, accounting for half of the cases. Approximately 50% to 75% of the patients experience local recurrence after initial surgery and, of these, half to three fourths manifest within three years of treatment. Metastases, seen in no more than 6% of all cases, are almost invariably preceded by two or more local recurrences and occur primarily through the bloodstream to the lungs and bones and less often to regional lymph nodes. Prognostic factors, differential diagnosis, and histogenesis are included.

(Arch Otolaryngol 1984;110:398-404)

REFERENCES

1 +
Darier J, Ferrand M:  Dermatofibromes progressifs et recidivants ou fibrosarcomes de la peau . Ann Dermatol Venereol 1924;;5:545-562.
2 +
Binkley GW:  Dermatofibrosarcoma protuberans: Report of six cases . Arch Dermatol 1939;;40:578-594.
3 +
Sherwell S:  Morphea . Arch Dermatol 1890;; 8:72.
4 +
Taylor RW:  Sarcomatous tumors resembling in some respects keloids . Arch Dermatol 1890;;8:384-387.
5 +
Hoffmann E:  Ueber das knollentribende Fibrosarkom der Haut (dermatofibrosarcoma protuberans) . Dermatol Z 1925;;43:1-28.
6 +
Burkhardt BR, Soule EH, Winkelman RK, et al:  Dermatofibrosarcoma protuberans: Study of 56 cases . Am J Surg 1966;;111:638-644.
7 +
Pack GT, Tabah EJ:  Dermatofibrosarcoma protuberans: A report of 39 cases . Arch Surg 1951;;62:391-411.
8 +
Simstein N, Tuthill RJ, Spesman EE, et al:  Dermatofibrosarcoma protuberans: Case reports and review of the literature . South Med J 1977;; 70:487-489.
9 +
Farr HW:  Soft parts sarcomas of the head and neck . Semin Oncol 1981;;8:185-189.
10 +
McPeak CJ, Cruz T, Nicastri AD:  Dermatofibrosarcoma protuberans: An analysis of 86 cases—five with metastasis . Ann Surg 1967;; 166:803-816.
11 +
Smith JL Jr:  Tumors of the corium , in Helwig EB, Mostofi FK (eds): The Skin , International Academy of Pathology Monograph No. 10. Baltimore, Williams & Wilkins Co, 1971;, pp 533-557.
12 +
Costa OG:  Progressive recurrent dermatofibroma (Darier-Ferrand): Anatomoclinical study . Arch Dermatol 1946;;54:432-454.
13 +
Bednar B:  Storiform neurofibromas of the skin, pigmented and nonpigmented . Cancer 1957;; 10:368-376.
14 +
Przybora LA, Wojnerowicz C:  Malignancy of dermatofibrosarcoma protuberans and report of two cases with lymph gland metastases . Oncologia 1959;;12:236-254.
15 +
Taylor HB, Helwig EB:  Dermatofibrosarcoma protuberans: A study of 115 cases . Cancer 1962;;15:717-725.
16 +
Phelan JT, Juardo J:  Dermatofibrosarcoma protuberans . Am J Surg 1963;;106:943-948.
17 +
O'Brien JE, Stout AP:  Malignant fibrous xanthomas . Cancer 1964;;17:1445-1455.
18 +
Sauter LS, DeFeo CP:  Dermatofibrosarcoma protuberans of the face . Arch Dermatol 1971;;104:671-673.
19 +
Tamoney HJ:  Dermatofibrosarcoma protuberans: A review of 12 cases . Conn Med 1971;; 35:674-679.
20 +
Hashimoto K, Brownstein MH, Jakobiec FA:  Dermatofibrosarcoma protuberans: A tumor with perineural and endoneural cell features . Arch Dermatol 1974;;110:874-885.
21 +
Junaid TA, Ani AN, Ejeckam GC:  Dermatofibrosarcoma protuberans in the parotid gland: A case report . Br J Oral Surg 1975;;12:298-301.
22 +
Jacyk WK, Pasnikowski T:  Dermatofibrosarcoma of the face: Report of two cases from Nigeria . Dermatologica 1977;;155:45-49.
23 +
Hausner RJ, Vargas-Cortes F, Alexander RW:  Dermatofibrosarcoma protuberans with lymph node involvement . Arch Dermatol 1978;; 114:88-91.
24 +
Weiss KS, Markus NJ, Hazenfield HN:  Dermatofibrosarcoma protuberans of the nose . Ear Nose Throat J 1979;;58:68-70.
25 +
Zina AM, Bundino S:  Dermatofibrosarcoma protuberans: An ultrastructural study of five cases . J Cutan Pathol 1979;;6:265-271.
26 +
Rich JD, Zbylski JR, LaRossa DD:  Dermatofibrosarcoma protuberans of the head and neck . Am Surg 1980;;46:208-215.
27 +
Escalona-Zapata J, Fernandez EA, Escuin FL:  The fibroblastic nature of dermatofibrosarcoma protuberans: A tissue culture and ultrastructural study . Virchows Arch Pathol Anat 1981;;391:165-175.
28 +
Peters CW, Hanke CW, Pasarell HA, et al:  Chemosurgical reports: Dermatofibrosarcoma protuberans of the face . J Dermatol Surg Oncol 1982;;8:823-826.
29 +
Fisher ER, Hellstrom HR:  Dermatofibrosarcoma with metastases simulating Hodgkin's disease and reticulum cell sarcoma . Cancer 1966;; 19:1165-1171.
30 +
Mendoza CB Jr, Gerwig WH Jr, Watne AL:  Dermatofibrosarcoma protuberans with metastases treated with methotrexate . Am J Surg 1970;;120:119-121.
31 +
Holm J:  Dermatofibrosarcoma protuberans: Report of a case with review of the literature . Acta Chir Scand 1968;;134:303-306.
32 +
Brenner W, Schaefler K, Chhabra H, et al:  Dermatofibrosarcoma protuberans metastatic to a regional lymph node: Report of a case and review . Cancer 1975;;36:1897-1902.
33 +
Penner DW:  Metastasizing dermatofibrosarcoma protuberans: A case report . Cancer 1951;;4:1083-1086.
34 +
Woolridge WE:  Dermatofibrosarcoma protuberans: A tumor too lightly considered . Arch Dermatol 1957;;75:132-134.
35 +
Ishii T, Koide O:  An autopsy case of metastasizing protuberant dermatofibrosarcoma . Acta Pathol Jpn 1975;;25:503-515.
36 +
Kahn LB, Saxe N, Gordon W:  Dermatofibrosarcoma protuberans with lymph node and pulmonary metastases . Arch Dermatol 1978;; 114:599-601.
37 +
Bonnabeau RC Jr, Stoughton WB, Armanious AW, et al:  Dermatofibrosarcoma protuberans: Report of a case with pulmonary metastasis and multiple intrathoracic recurrences . Oncology 1974;;29:1-12.
38 +
Enzinger FM, Weiss SW:  Fibrohistiocytic tumors of intermediate malignancy , in Enzinger FM, Weiss SW (eds): Soft-Tissue Tumors . St Louis, CV Mosby Co, 1983;, pp 154-165.
39 +
Ozello L, Hamels J:  The histiocytic nature of dermatofibrosarcoma protuberans: Tissue culture and electron microscopic study . Am J Clin Pathol 1976;;65:136-148.
40 +
Alquacil-Garcia A, Unni KK, Goellner JR:  Histogenesis of dermatofibrosarcoma protuberans: An ultrastructural study . Am J Clin Pathol 1978;;69:427-434.
41 +
Kindblom LG, Jacobsen GK, Jacobsen M:  Immunohistochemical investigations of tumors of supposed fibroblastic-histiocytic origin . Hum Pathol 1982;;13:834-840.
42 +
McMaster PE:  Sarcomatoid fibroma of the skin (progressive and recurring dermatofibroma) . Ann Surg 1934;;99:338-347.
43 +
Morman MR, Lin RY, Petrozzi JW:  Dermatofibrosarcoma protuberans arising in a site of multiple immunizations . Arch Dermatol 1979;; 115:1453.

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1 +
Darier J, Ferrand M:  Dermatofibromes progressifs et recidivants ou fibrosarcomes de la peau . Ann Dermatol Venereol 1924;;5:545-562.
2 +
Binkley GW:  Dermatofibrosarcoma protuberans: Report of six cases . Arch Dermatol 1939;;40:578-594.
3 +
Sherwell S:  Morphea . Arch Dermatol 1890;; 8:72.
4 +
Taylor RW:  Sarcomatous tumors resembling in some respects keloids . Arch Dermatol 1890;;8:384-387.
5 +
Hoffmann E:  Ueber das knollentribende Fibrosarkom der Haut (dermatofibrosarcoma protuberans) . Dermatol Z 1925;;43:1-28.
6 +
Burkhardt BR, Soule EH, Winkelman RK, et al:  Dermatofibrosarcoma protuberans: Study of 56 cases . Am J Surg 1966;;111:638-644.
7 +
Pack GT, Tabah EJ:  Dermatofibrosarcoma protuberans: A report of 39 cases . Arch Surg 1951;;62:391-411.
8 +
Simstein N, Tuthill RJ, Spesman EE, et al:  Dermatofibrosarcoma protuberans: Case reports and review of the literature . South Med J 1977;; 70:487-489.
9 +
Farr HW:  Soft parts sarcomas of the head and neck . Semin Oncol 1981;;8:185-189.
10 +
McPeak CJ, Cruz T, Nicastri AD:  Dermatofibrosarcoma protuberans: An analysis of 86 cases—five with metastasis . Ann Surg 1967;; 166:803-816.
11 +
Smith JL Jr:  Tumors of the corium , in Helwig EB, Mostofi FK (eds): The Skin , International Academy of Pathology Monograph No. 10. Baltimore, Williams & Wilkins Co, 1971;, pp 533-557.
12 +
Costa OG:  Progressive recurrent dermatofibroma (Darier-Ferrand): Anatomoclinical study . Arch Dermatol 1946;;54:432-454.
13 +
Bednar B:  Storiform neurofibromas of the skin, pigmented and nonpigmented . Cancer 1957;; 10:368-376.
14 +
Przybora LA, Wojnerowicz C:  Malignancy of dermatofibrosarcoma protuberans and report of two cases with lymph gland metastases . Oncologia 1959;;12:236-254.
15 +
Taylor HB, Helwig EB:  Dermatofibrosarcoma protuberans: A study of 115 cases . Cancer 1962;;15:717-725.
16 +
Phelan JT, Juardo J:  Dermatofibrosarcoma protuberans . Am J Surg 1963;;106:943-948.
17 +
O'Brien JE, Stout AP:  Malignant fibrous xanthomas . Cancer 1964;;17:1445-1455.
18 +
Sauter LS, DeFeo CP:  Dermatofibrosarcoma protuberans of the face . Arch Dermatol 1971;;104:671-673.
19 +
Tamoney HJ:  Dermatofibrosarcoma protuberans: A review of 12 cases . Conn Med 1971;; 35:674-679.
20 +
Hashimoto K, Brownstein MH, Jakobiec FA:  Dermatofibrosarcoma protuberans: A tumor with perineural and endoneural cell features . Arch Dermatol 1974;;110:874-885.
21 +
Junaid TA, Ani AN, Ejeckam GC:  Dermatofibrosarcoma protuberans in the parotid gland: A case report . Br J Oral Surg 1975;;12:298-301.
22 +
Jacyk WK, Pasnikowski T:  Dermatofibrosarcoma of the face: Report of two cases from Nigeria . Dermatologica 1977;;155:45-49.
23 +
Hausner RJ, Vargas-Cortes F, Alexander RW:  Dermatofibrosarcoma protuberans with lymph node involvement . Arch Dermatol 1978;; 114:88-91.
24 +
Weiss KS, Markus NJ, Hazenfield HN:  Dermatofibrosarcoma protuberans of the nose . Ear Nose Throat J 1979;;58:68-70.
25 +
Zina AM, Bundino S:  Dermatofibrosarcoma protuberans: An ultrastructural study of five cases . J Cutan Pathol 1979;;6:265-271.
26 +
Rich JD, Zbylski JR, LaRossa DD:  Dermatofibrosarcoma protuberans of the head and neck . Am Surg 1980;;46:208-215.
27 +
Escalona-Zapata J, Fernandez EA, Escuin FL:  The fibroblastic nature of dermatofibrosarcoma protuberans: A tissue culture and ultrastructural study . Virchows Arch Pathol Anat 1981;;391:165-175.
28 +
Peters CW, Hanke CW, Pasarell HA, et al:  Chemosurgical reports: Dermatofibrosarcoma protuberans of the face . J Dermatol Surg Oncol 1982;;8:823-826.
29 +
Fisher ER, Hellstrom HR:  Dermatofibrosarcoma with metastases simulating Hodgkin's disease and reticulum cell sarcoma . Cancer 1966;; 19:1165-1171.
30 +
Mendoza CB Jr, Gerwig WH Jr, Watne AL:  Dermatofibrosarcoma protuberans with metastases treated with methotrexate . Am J Surg 1970;;120:119-121.
31 +
Holm J:  Dermatofibrosarcoma protuberans: Report of a case with review of the literature . Acta Chir Scand 1968;;134:303-306.
32 +
Brenner W, Schaefler K, Chhabra H, et al:  Dermatofibrosarcoma protuberans metastatic to a regional lymph node: Report of a case and review . Cancer 1975;;36:1897-1902.
33 +
Penner DW:  Metastasizing dermatofibrosarcoma protuberans: A case report . Cancer 1951;;4:1083-1086.
34 +
Woolridge WE:  Dermatofibrosarcoma protuberans: A tumor too lightly considered . Arch Dermatol 1957;;75:132-134.
35 +
Ishii T, Koide O:  An autopsy case of metastasizing protuberant dermatofibrosarcoma . Acta Pathol Jpn 1975;;25:503-515.
36 +
Kahn LB, Saxe N, Gordon W:  Dermatofibrosarcoma protuberans with lymph node and pulmonary metastases . Arch Dermatol 1978;; 114:599-601.
37 +
Bonnabeau RC Jr, Stoughton WB, Armanious AW, et al:  Dermatofibrosarcoma protuberans: Report of a case with pulmonary metastasis and multiple intrathoracic recurrences . Oncology 1974;;29:1-12.
38 +
Enzinger FM, Weiss SW:  Fibrohistiocytic tumors of intermediate malignancy , in Enzinger FM, Weiss SW (eds): Soft-Tissue Tumors . St Louis, CV Mosby Co, 1983;, pp 154-165.
39 +
Ozello L, Hamels J:  The histiocytic nature of dermatofibrosarcoma protuberans: Tissue culture and electron microscopic study . Am J Clin Pathol 1976;;65:136-148.
40 +
Alquacil-Garcia A, Unni KK, Goellner JR:  Histogenesis of dermatofibrosarcoma protuberans: An ultrastructural study . Am J Clin Pathol 1978;;69:427-434.
41 +
Kindblom LG, Jacobsen GK, Jacobsen M:  Immunohistochemical investigations of tumors of supposed fibroblastic-histiocytic origin . Hum Pathol 1982;;13:834-840.
42 +
McMaster PE:  Sarcomatoid fibroma of the skin (progressive and recurring dermatofibroma) . Ann Surg 1934;;99:338-347.
43 +
Morman MR, Lin RY, Petrozzi JW:  Dermatofibrosarcoma protuberans arising in a site of multiple immunizations . Arch Dermatol 1979;; 115:1453.

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