Pediatric Dermatology Vol. 7 No. 1 57-59
Dermatofibrosarcoma Protuberans in a 9-Year-Old Child: Treatment by MOHS Micrographic Surgery David J. Goldberg, M.D.,* and Martha Maso, M.D., M.P.H.t "Department of Dermatologic Surgery, University of Medicine and Dentistry-New Jersey Medical School, Newark, New Jersey; and fDepartment of Dermatology, Columbia University College of Physicians and Surgeons, New York, New York
Abstract: Dermatofibrosarcoma protuberans (DFSP) is an uncommon, iocaliy and deepiy recurring dermal neoplasm that, when it occurs in cosmetically sensitive areas, can be devastating, particuiarly in children. MOHS micrographic surgery is now recognized as the treatment of choice for DFSP. A iesion of DFSP near the breast of a 9-year~old girl was treated by this technique. To our knowiedge, this is the first case of DFSP in a chiid in which MOHS micrographic surgery was used as the first-tine treatment modaitty.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, indolent, locally recurring neoplasm that can occur in children as well as adults. We present a case of DFSP in a 9-year-old child treated by MOHS micrographic surgery, which we suggest is the treatment of choice for this condition. CASE REPORT
A 9-year-old girl had a one-year history of an asymptomatic, nodulocystic lesion superior to the left breast. She was referred to a plastic surgeon for an incisional biopsy, which revealed DFSP (Fig. 1). Subsequently the patient was referred to the section of Dermatologic Surgery at the New Jersey Medical School, where the neoplasm was removed in five stages of the MOHS technique. The referring plastic surgeon repaired the wound with a split-thickness skin graft. One year after surgery, repeat biopsies of the hypertrophic scar revealed no histologic evidence of recurrence (Fig. 2).
HISTOPATHOLOGY
Incisional biopsy revealed a large neoplasm confined to the dermis and subcutaneous fat. This growth showed short fascicles of spindle-shaped cells in a weave pattern, with the neoplastic cells having oval and wavy nuclei. These findings are diagnostic for DFSP. DISCUSSION
Dermatofibrosarcoma protuberans often remains for years as an unchanging fibrous plaque before culminating in a multinodulated, rapidly growing mass. The tumor can take on a yellow, fleshcolored, red, blue, or brown hue (1), and may or may not be painful or ulcerate (2). Distant metastases, albeit rare, have been reported (3,4) and tend to arise by hematogenous spread (4). The condition occurs equally in both males and females. Lesions most frequently appear on the trunk and proximal extremities, but may also be
Address correspondence to David J. Goldberg, M.D.. 400 0*d Hook Road. Westwood, NJ 07675.
57
58
Pediatric Dermatology Vol. 7 No. 1 March 1990
Figure 1. Short fascicles of spindle shaped-cells in a weave pattern are diagnostic of DFSP.
Figure 2. One year after MOHS surgery. Hypertrophic scarring is present at wound edges. No clinical or histologic evidence of recurrent DFSP was found.
seen on the distal extremities, head, and neck (2,3,5-8). It is more common in whites than in blacks, the only exception being the pigmented variety (5). Factors predisposing to tumor development have not been identified. Individuals in the second to fifth decades of life appear to be most frequently affected, although DFSP has been reported in individuals of all ages (9). Sixteen cases have thus far been reported in children 10 years of age or younger (Table 1); how-
ever, data regarding exact age at onset, sex ofthe patients, and locations of lesions are available in only a limited number of instances (1,6,7,9^11). This condition is thought to arise in the dermis de novo from cells with an as yet controversial histogenesis. That DFSP derives from fibrocystic (9,12), histiocytic (13), or neuromelanocytic (14) cell lines was proposed variously since its description by Darier (15) in 1924, until Hashimoto et al (6) investigated lesions ultrastructurally. Their data speak against origins from those postulated cell lines. Rather, they imply a development from endoneural or perineural cells based on electron microscopic features that endoneural or perineural cells share with DFSP. Most recently, Dupree et al (5) reported on the existence of three cell types in DFSP. The largest population resembles fibroblasts; the other two are either partially or entirely invested by basal lamina, with one exhibiting long and thin cellular processes, and the other containing both melanosomes and premelanosomes. Thus, while Dupree et al believed that the most differentiated cells are akin to perineural cells, the precise histogenesis of DFSP remains to be elucidated. Less controversial are the condition's salient histologic features (9), which consist of the invariably pervasive cartwheel or whirligig configurations of radially oriented, plump spindle cells with round nuclei that appear to emanate from a central hub, are most dense centrally, and become less cellular peripherally. Mitotic figures tend to be infrequent, even in rapidly proliferating tumors and in cellular foci. Other variable features include myxomatous changes, particularly notable in recurrent tumors, as well as dilated vascular spaces and multinucleated giant cells. An atrophic epidermis with flattened rete ridges tends to overlie the dermal tumor (9). Surgical excision has been the traditional mainstay of treatment for DFSP. Both the proclivity of the neoplasm for local tissue invasion and high recurrence rate derive from the numerous microscopic projections of cells that appear to extend at
TABLE 1. Dermatofibrosarcoma Protuberans in Children up to 10 Years of Age Reference 9
7 6
io
n1
This report
No. of Patients
Sex
Location
Treatment Method
11
* *
* * Left costal margin Right cheek Face * Chest
Wide excision Wide excision Wide excision Resected at age 24 yrs by MOHS ' Wide excision MOHS after two prior wide excisions MOHS primary method
1
1 1 1 I 1
Information unavailable.
F
M M F F
Goldberg and Maso: DFSP treated by MOHS Micrographic Surgery 59
least 3 cm beyond the tumor core in all directions (1,2). Unless histologic examination at the time of excision includes lateral and deep margins, tumor fascicles undoubtedly will evade excision and contribute to high rates of regional recurrence. These range from 49% to 54% of patients treated by local excision (4,9) and 10% to 20% of those treated by wide excision of at least 3 cm of uninvolved skin and underlying fascia (3,16). In addition, wide excision margins may be impossible to achieve without disfigurement. In contrast to surgical excision, treatment of DFSP with MOHS micrographic surgery, as of this writing, has been associated with an absence of recurrence, with as much as five years of postsurgical follow-up (1,10,17-20). These findings are particularly noteworthy given that up to 80% of DFSPs recur within three years of removal (4). Thus MOHS technique clearly offers a better cure rate than surgical excision. In children, DFSP is rare. Thus it is not surprising that there is a dearth of published material related to its treatment in children by MOHS micrographic surgery. Hobbs et a! (I) reported on one individual who underwent two excisions of the neoplasm before receiving MOHS surgery at 5 years of age. The location of our patient's lesion near the breast called for both a highly curative as well as tissue-sparing approach to treatment. She is, to our knowledge, the first child to undergo the MOHS procedure as the primary treatment for DFSP.
REFERENCES 1. 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. 2. Pack GT, Tabh EJ. Dermatofibrosarcoma protuberans. A report of thirty nine cases. Arch Surg 3. McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: an analysis of 86 cases—5 with metastasis. Ann Surg 1967;166:803-816.
4. Hajdu SI. Pathology of soft tissue tumors. Philadelphia: Lea & Febiger, 1979:60, 83. 5. Dupree WB, Lanloss JM, Weiss SW. Pigmented dermatofibrosarcoma protuberans (Bednar tumor). Am J Surg Pathol 1985;9:630-639. 6. Hashimoto K, Brownstein MH, Jakobiec FA. Dermatofibrosarcoma protuberans. A tumor with perineural and endoneural cell features. Arch Dermatol 1974;110:874-885. 7. Burkhardt BR. Soule EH, Winkellman RK, Ivins JC. Dermatofibrosarcoma protuberans. Study of fifty-six cases. Am J Surg 1966;U 1:638-644. 8. Bendix-Hansen K, Myhre-Jensen O, Kane S. Dermatofibrosarcoma protuberans. A clinicopathologic study of nineteen cases and review of world literature. Scand J Plast Reconstr Surg 1983;17:247-252. 9. Taylor HB, Heiwig EB. Dermatofibrosarcoma protuberans. A study of 115 cases. Cancer i%2;l 15:717724. 10. Peters CW, Hanke CW, Pasarell HA, Bennett JE. Chemosurgical reports. Dermatofibrosarcoma protuberans of the face. J Dermatol Surg Oncol 1982; 8:823-826. 11 Sagi A. Ben-Yakar Y, Mahler D. A ten-year-old boy with dermatofibrosarcoma protuberans of the face. J Dermatol Surg Oncol I987;13:82-83. 12. Shneidman D, Belizaine R. Arsenic exposure followed by the development of dermatofibrosarcoma protuberans. Cancer 1986;58:1585-1587. 13. Stout AP, Lattes R. Tumors of the soft tissues, second series, part 1. Armed Forces Institute of Pathology, 1967:44. 14. Bednar B. Storiform neurofibromas of the skin, pigmented and non-pigmented. Cancer 1957; 10:368-376. 15. Darier J. Dermatofibromes progressifs et recidivants; ou fibrosarcomes de la peau. Ann Dermatol Syph i924;5:545-562. 16. Roses DF, Valensi Q, La Trenta G, Hanis MN. Surgical treatment of dermatofibrosarcoma protuberans. Surg Gynecol Obstet l986;162:449-452. 17. Robinson JK. Dermatofibrosarcoma protuberans resected by Mohs' surgery. J Am Acad Dermatol 1985;12;1093-1098. 18. Hess KA. Hanke CW, Estes NC. Shideler SJ. Chemosurgical reports: myxoid dermatofibrosarcoma protuberans. J Dermatol Surg Oncol 1985; 11:268-271. 19. Mohs FE. Chemosurgery: microscopically controlled surgery for skin cancer. Springfield, IL: Charles C Thomas, 1978:255. 20. Mikhail GR, Lynn BR. Dermatofibrosarcoma protuberans. J Dermatol Surg Oncol 1978;4:81-84.
Dermatofibrosarcoma Protuberans in a 9-Year-Old Child: Treatment by MOHS Micrographic Surgery David J. Goldberg, M.D.,* and Martha Maso, M.D., M.P.H.t "Department of Dermatologic Surgery, University of Medicine and Dentistry-New Jersey Medical School, Newark, New Jersey; and fDepartment of Dermatology, Columbia University College of Physicians and Surgeons, New York, New York
Abstract: Dermatofibrosarcoma protuberans (DFSP) is an uncommon, iocaliy and deepiy recurring dermal neoplasm that, when it occurs in cosmetically sensitive areas, can be devastating, particuiarly in children. MOHS micrographic surgery is now recognized as the treatment of choice for DFSP. A iesion of DFSP near the breast of a 9-year~old girl was treated by this technique. To our knowiedge, this is the first case of DFSP in a chiid in which MOHS micrographic surgery was used as the first-tine treatment modaitty.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, indolent, locally recurring neoplasm that can occur in children as well as adults. We present a case of DFSP in a 9-year-old child treated by MOHS micrographic surgery, which we suggest is the treatment of choice for this condition. CASE REPORT
A 9-year-old girl had a one-year history of an asymptomatic, nodulocystic lesion superior to the left breast. She was referred to a plastic surgeon for an incisional biopsy, which revealed DFSP (Fig. 1). Subsequently the patient was referred to the section of Dermatologic Surgery at the New Jersey Medical School, where the neoplasm was removed in five stages of the MOHS technique. The referring plastic surgeon repaired the wound with a split-thickness skin graft. One year after surgery, repeat biopsies of the hypertrophic scar revealed no histologic evidence of recurrence (Fig. 2).
HISTOPATHOLOGY
Incisional biopsy revealed a large neoplasm confined to the dermis and subcutaneous fat. This growth showed short fascicles of spindle-shaped cells in a weave pattern, with the neoplastic cells having oval and wavy nuclei. These findings are diagnostic for DFSP. DISCUSSION
Dermatofibrosarcoma protuberans often remains for years as an unchanging fibrous plaque before culminating in a multinodulated, rapidly growing mass. The tumor can take on a yellow, fleshcolored, red, blue, or brown hue (1), and may or may not be painful or ulcerate (2). Distant metastases, albeit rare, have been reported (3,4) and tend to arise by hematogenous spread (4). The condition occurs equally in both males and females. Lesions most frequently appear on the trunk and proximal extremities, but may also be
Address correspondence to David J. Goldberg, M.D.. 400 0*d Hook Road. Westwood, NJ 07675.
57
58
Pediatric Dermatology Vol. 7 No. 1 March 1990
Figure 1. Short fascicles of spindle shaped-cells in a weave pattern are diagnostic of DFSP.
Figure 2. One year after MOHS surgery. Hypertrophic scarring is present at wound edges. No clinical or histologic evidence of recurrent DFSP was found.
seen on the distal extremities, head, and neck (2,3,5-8). It is more common in whites than in blacks, the only exception being the pigmented variety (5). Factors predisposing to tumor development have not been identified. Individuals in the second to fifth decades of life appear to be most frequently affected, although DFSP has been reported in individuals of all ages (9). Sixteen cases have thus far been reported in children 10 years of age or younger (Table 1); how-
ever, data regarding exact age at onset, sex ofthe patients, and locations of lesions are available in only a limited number of instances (1,6,7,9^11). This condition is thought to arise in the dermis de novo from cells with an as yet controversial histogenesis. That DFSP derives from fibrocystic (9,12), histiocytic (13), or neuromelanocytic (14) cell lines was proposed variously since its description by Darier (15) in 1924, until Hashimoto et al (6) investigated lesions ultrastructurally. Their data speak against origins from those postulated cell lines. Rather, they imply a development from endoneural or perineural cells based on electron microscopic features that endoneural or perineural cells share with DFSP. Most recently, Dupree et al (5) reported on the existence of three cell types in DFSP. The largest population resembles fibroblasts; the other two are either partially or entirely invested by basal lamina, with one exhibiting long and thin cellular processes, and the other containing both melanosomes and premelanosomes. Thus, while Dupree et al believed that the most differentiated cells are akin to perineural cells, the precise histogenesis of DFSP remains to be elucidated. Less controversial are the condition's salient histologic features (9), which consist of the invariably pervasive cartwheel or whirligig configurations of radially oriented, plump spindle cells with round nuclei that appear to emanate from a central hub, are most dense centrally, and become less cellular peripherally. Mitotic figures tend to be infrequent, even in rapidly proliferating tumors and in cellular foci. Other variable features include myxomatous changes, particularly notable in recurrent tumors, as well as dilated vascular spaces and multinucleated giant cells. An atrophic epidermis with flattened rete ridges tends to overlie the dermal tumor (9). Surgical excision has been the traditional mainstay of treatment for DFSP. Both the proclivity of the neoplasm for local tissue invasion and high recurrence rate derive from the numerous microscopic projections of cells that appear to extend at
TABLE 1. Dermatofibrosarcoma Protuberans in Children up to 10 Years of Age Reference 9
7 6
io
n1
This report
No. of Patients
Sex
Location
Treatment Method
11
* *
* * Left costal margin Right cheek Face * Chest
Wide excision Wide excision Wide excision Resected at age 24 yrs by MOHS ' Wide excision MOHS after two prior wide excisions MOHS primary method
1
1 1 1 I 1
Information unavailable.
F
M M F F
Goldberg and Maso: DFSP treated by MOHS Micrographic Surgery 59
least 3 cm beyond the tumor core in all directions (1,2). Unless histologic examination at the time of excision includes lateral and deep margins, tumor fascicles undoubtedly will evade excision and contribute to high rates of regional recurrence. These range from 49% to 54% of patients treated by local excision (4,9) and 10% to 20% of those treated by wide excision of at least 3 cm of uninvolved skin and underlying fascia (3,16). In addition, wide excision margins may be impossible to achieve without disfigurement. In contrast to surgical excision, treatment of DFSP with MOHS micrographic surgery, as of this writing, has been associated with an absence of recurrence, with as much as five years of postsurgical follow-up (1,10,17-20). These findings are particularly noteworthy given that up to 80% of DFSPs recur within three years of removal (4). Thus MOHS technique clearly offers a better cure rate than surgical excision. In children, DFSP is rare. Thus it is not surprising that there is a dearth of published material related to its treatment in children by MOHS micrographic surgery. Hobbs et a! (I) reported on one individual who underwent two excisions of the neoplasm before receiving MOHS surgery at 5 years of age. The location of our patient's lesion near the breast called for both a highly curative as well as tissue-sparing approach to treatment. She is, to our knowledge, the first child to undergo the MOHS procedure as the primary treatment for DFSP.
REFERENCES 1. 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. 2. Pack GT, Tabh EJ. Dermatofibrosarcoma protuberans. A report of thirty nine cases. Arch Surg 3. McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: an analysis of 86 cases—5 with metastasis. Ann Surg 1967;166:803-816.
4. Hajdu SI. Pathology of soft tissue tumors. Philadelphia: Lea & Febiger, 1979:60, 83. 5. Dupree WB, Lanloss JM, Weiss SW. Pigmented dermatofibrosarcoma protuberans (Bednar tumor). Am J Surg Pathol 1985;9:630-639. 6. Hashimoto K, Brownstein MH, Jakobiec FA. Dermatofibrosarcoma protuberans. A tumor with perineural and endoneural cell features. Arch Dermatol 1974;110:874-885. 7. Burkhardt BR. Soule EH, Winkellman RK, Ivins JC. Dermatofibrosarcoma protuberans. Study of fifty-six cases. Am J Surg 1966;U 1:638-644. 8. Bendix-Hansen K, Myhre-Jensen O, Kane S. Dermatofibrosarcoma protuberans. A clinicopathologic study of nineteen cases and review of world literature. Scand J Plast Reconstr Surg 1983;17:247-252. 9. Taylor HB, Heiwig EB. Dermatofibrosarcoma protuberans. A study of 115 cases. Cancer i%2;l 15:717724. 10. Peters CW, Hanke CW, Pasarell HA, Bennett JE. Chemosurgical reports. Dermatofibrosarcoma protuberans of the face. J Dermatol Surg Oncol 1982; 8:823-826. 11 Sagi A. Ben-Yakar Y, Mahler D. A ten-year-old boy with dermatofibrosarcoma protuberans of the face. J Dermatol Surg Oncol I987;13:82-83. 12. Shneidman D, Belizaine R. Arsenic exposure followed by the development of dermatofibrosarcoma protuberans. Cancer 1986;58:1585-1587. 13. Stout AP, Lattes R. Tumors of the soft tissues, second series, part 1. Armed Forces Institute of Pathology, 1967:44. 14. Bednar B. Storiform neurofibromas of the skin, pigmented and non-pigmented. Cancer 1957; 10:368-376. 15. Darier J. Dermatofibromes progressifs et recidivants; ou fibrosarcomes de la peau. Ann Dermatol Syph i924;5:545-562. 16. Roses DF, Valensi Q, La Trenta G, Hanis MN. Surgical treatment of dermatofibrosarcoma protuberans. Surg Gynecol Obstet l986;162:449-452. 17. Robinson JK. Dermatofibrosarcoma protuberans resected by Mohs' surgery. J Am Acad Dermatol 1985;12;1093-1098. 18. Hess KA. Hanke CW, Estes NC. Shideler SJ. Chemosurgical reports: myxoid dermatofibrosarcoma protuberans. J Dermatol Surg Oncol 1985; 11:268-271. 19. Mohs FE. Chemosurgery: microscopically controlled surgery for skin cancer. Springfield, IL: Charles C Thomas, 1978:255. 20. Mikhail GR, Lynn BR. Dermatofibrosarcoma protuberans. J Dermatol Surg Oncol 1978;4:81-84.
