Dermatofibrosarcoma P. M. McLaughlin,
protuberans of the scalp
M. Girach.
G. A. Wood
of‘Oral and Ma.~illojuciul
Department
Surgery, Glun Chyd
Hospital,
Bodel\v~~ddun, Chyd
S EMMA R Y. Dermatotibrosarcoma protuberans is an uncommon tumour of the dermis and rarely occurs on the scalp; there are few known predisposing factors. A case is described of such a tumour that occurred at the site of radiotherapy for a basal cell carcinoma. Histological characteristics, bchaviour and management of dermatofibrosarcoma protuberans are discussed.
INTRODUCTION
those of a dcrmatofibrosarcoma protuberans. Excision was reported as incomplete on the deep margin (Figure). The patient was returned to thcatrc. whcrc under local anacsthesia and intravenous sedation, further local excision was carried out with a 3 cm excision margin. and en bloc removal of the underlying subcutis including fascia. The defect was reconstructed using a split skin graft. Excision was reported as complete. and the patient remains recurrence free to date.
Dermatolibrosarcoma protuberans (DFSP) is an uncommon tumour that arises in the dermis. It is characteriscd by slow invasive growth, local recurrence after excision. and infrequent metastasis. The lesion is approximately four times as common in men:
has
a peak
incidence
in the third
decade,
and
may occur at sites of previous trauma. The tumour occurs most commonly on the trunk and proximal extremities (Taylor & Helwig, 1962). It also appears on the head and neck, but less than 5% of all lesions are located on the scalp (Barnes & Coleman, 1984). A case is reported of DFSP of the scalp following radiotherapy, such an incidence WC believe previously unreported. The characteristics and management of thcsc tumours are discussed.
DISCUSSION The term introduced
dermatofibrosarcoma protuberans was by Holfman in 1925, although the tumour
Case report A 76-year-old lady was rcferrcd to the dermatology dcpartmcnt at Glan Clwyd hospital in June 1988 with a lesion clinically diagnosed as a basal cell carcinoma on the left temporal skin. This lesion was removed under local anacsthetic and shown histologically to be complctcly excised, the clinical diagnosis being confirmed. At a follow up visit in February 1989, a recurrence was noted at the excision site, shown by punch biopsy to be a further basal cell carcinoma and a referral made to the local radiotherapy unit. Treatment was given in the form of external beam radiotherapy as a 2OcGy single dose. resulting in complete remission of the lesion. In August 1991, the patient was referred to the Dcpartmcnt of Oral and Maxillofacial Surgery with a further lesion at the original excision site on the scalp. A raised nodule. 5 mm in diamctcr, was found on the left temporal skin, and a provisional diagnosis of recurrent basal cell carcinoma was made. Excision under local anacsthcsia and sedation was carried out, taking a 3 mm margin of clearance around the lesion. and primary closure obtained. IIowevcr histology of the exiscd specimen showed the superficial and deep dermis replaced by a fibroblastic infiltrate of spindle and stellate cells with interweaving bundles of fibrocollagen and focal myxoid degeneration. This myxoid stroma extended into the subcutis and incorporated fibroblastic cells. scattered mitoscs were seen. These appearances were
IGgure - Photomicrograph of the excision sPecimcn showing a typical cartwheel arrangement of tibroblasts (H&E. Original magnification x I 15).
401
402
British Journal
of Oral and
Maxillocdcial
Surgcr);
was first recognised as a clinicopathologic entity by Daricr and Fcrrand in 1924. Dl:SP is a tumour of so-called fibrohistiocytic origin. although thcrc is insufficient cvidcncc to support cithcr a histiocytic or neuroectodermal origin for this tumour (Fletcher et al., 1985). Other tumours classified similarly are: fibrous histiocytoma. sclerosing haemangioma, atypical fibroxanthoma and malignant fibrous histiocytoma. While the last is now well dcscribcd (Kcarney et al., 1980), much of the published work on DFSP antedates its original description (Kempson & Kyriakos, 1972). Consequently much of the older litcrainvolving descriptions of aetiology and ture clinicopathological bchaviour is misleading, as many of the reported examples would today be reclassified as either atypical fibroxanthoma or malignant fibrous histiocytoma. In the more recent litcraturc, therefore: DFSP is considered to have no precipitating cause. and in most case reports no comment is made on possible aetiological factors. A history of prior trauma is most often mentioned and this has been reported on the scalp (Rockley et 01.~ 1989), a case following BCG vaccination has also been described (McLelland & Chu, 1988). We find no report of DFSP being induced by ionizing radiation, but this could bc considered as a possible aetiological factor in the case described. DFSP is an unusual tumour, the incidcncc of which has been estimated at 0.8 cases per million persons per year (Bendix-Hansen ef al., 1983). Clinically the lesion appears initially as a dusky indurated plaque. The tumour may bc flesh-coloured. bluish-red, rcddish brown, or yellow in colour. In time, the tumour develops a nodular or multinodular quality and may ulcerate. In advanced stages the tumour may invade fascia and muscle, and adjacent underlying structures such as calvarium, dura and brain (Rockley et [II., 1989). While metastases to regional lymph nodes (Hirabayashi et a/., 1989) and lungs (Kahn et al., 1978) do occur in DFSP, such bchaviour is rare. Histologically. DFSP is typified by a storiform pattern created by spindle cells arranged in cartwhccllike arrays about collagenous centres (Taylor & Helwig, 1962). The interlacing bands and whorls of fibroblasts may fill the entire reticular dermis and cxtcnd into subcutaneous fat. Large numbers of mitotic figures arc usually not seen, nor is cell atypia usually found. The nodular nature of DFSP gives an erroneous impression of circumscription. The main body of the tumour gives off multiple microscopic projections of neoplastic cells, which may extend 3 centimctres or more beyond the tumour proper. Failure to excise all of these projections is likely to be the main reason for the high rate of recurrence noted with this tumour (Pack & Tabah, 1951). Taylor and Helwig (1962) in a series of 98 cases, returned a 49”/0 recurrence rate, 40% recurring within 1 year and 75% of the recurrences within 3 years following surgery. Rowsell et al. (1986) reported five cases: all of which required additional surgery following incomplete excision. Two of these patients needed two additional procedures, and one patient underwent
three procedures. in spite of the authors’ technique of taking an avcragc 2.7 cm margin of clinically uninvolved skin. and subcutaneous tissue, at the first re-excision. Roses et al. (1986) used a 3 cm margin in IO patients and reported a 20% recurrence rate, while Bendix-Hansen et al. (1983) took a 3 cm margin in 7 patients with no recurrences. Jambhekar and Chinoy (1988) rcportcd a 25% rccurrcncc ralc in 47 cases where treatment was ‘mainly surgical’, howcvcr, they also employed radiotherapy as both primary and adjuvant therapy. Although DFSP is primarily a surgically managed lesion a regimen of electron and photon radiation has been reported; in six patients so treated, no local recurrences were noted with follow-up periods from 15. 105 months (Schullcr et ul., 1990). IO patients with Hobbs et al. (1988) reported rccurrcncc of DFSP following conventional excision, treated with Mohs micrographic surgery. The average duration of follow-up in the series was 43 months (range 15-91 months)? with no evidence of rccurrcncc in any of the cases.
References Barnes. L. & Coleman. J. A. (1984). Dermatolibrosarcoma protuberans of the head and neck. /irchives q/O~o/rzr~t~go/og. 110, 39x. Bendix-llansen, K., Myhrc-Jcnscn. 0. & Kaae. S. (1983). Dermatolihrosarcoma protubcrans. A clinicopathological study of nineteen cases and rcvicw of world literature. Srandinaviun 17,247.
Jotrrncrl of Plus!ic
and Reconsrrucrice
Surgery.
Darricr. J. & Fcrrand. M. (1924). Dermatotibromas progrcssifs ct de recidivants ou fibrosarcomcs de la pcau. Ann&s Derma/olopie et S~~philographie, 5, 545. Flctchcr, C. D. M.. Evans, B. J.. Macartney, J. C.. Smith. N.. Wilson Jones. I!. & McKee, P. II. (1985). Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochcmical study with a review of the literature. Hisropathology,
9.921.
Hirabayashi. S., Kajikawa. A.. Kana7awa. K. & Mimoto. K. (1989). Dermatofibrosarcoma protubcrans with regional lymph node metastasis: a cast report. Head utzd Feck Surger,v. 11,562. IIobbs. E. R.. Whceland. R. G.. Bailin. P. L.. Ratz. J. L., Yetman, R. J. & Zins, J. E. (1988). Treatment of dermatofibrosarcoma protubcrans with Mobs micrographic surgery. Annuls of Surgery.
207, 102.
Hoffman. F.. (1925). Ubcr das knollentreibendc haut (dcrmatofibrosarkoma protubcrdns). Zeirschr~fi.
43,
librosarkom
der
Lkrmutologische
I.
Jambhekar. S. A. & Chinoy, R. C. (1988). Dermatofibrosarcoma protuberans, experience at a cancer hospital in India. Indiun Journul
qf Cancer,2594.
Kahn. L. B., Saxe. N. &Gordon. W. (1978). Dermatolibrosarcoma protubcrans with lymph node and pulmonary metastasis. Arc/rives of Dermafology, 114,8X. Kearney. Mi. M., Soule, E. II. & Ivins, J. C. (1980). Malignant fibrous histiocytoma. A retrospcctivc study of 167 casts. Cuncer, 45, 167. Kcmpson. R. L. & Kyriakos: M. (1972). Fibroxanthosarcoma of the soft tissues. A type of malignant fibrous histiocytoma. Cuncer. 29, 961. McLelland, J. & Chu. T. (198X). Dermatofibrosarcoma protubcrans arising in a BCG vaccination scar. Archiws of Dermarolog~~:
124,496.
Pack, G. T. & Tabah, I?. J. (1951). Dermatofibrosarcoma protuberans: a report of thirty-nine casts. Archives qf’Sur,oery. 63,391.
Dcrmatotibrosarcoma Rockley. P. F., Robinson. J. K., Magid. M. & Goldblatt, D. (1989). Dcrmatofibrosarcolna protubcrans of the scalp: a series of cases, Journal of/he American Acoden?,; of lkrmarolog~.
21, 27X.
Roses, D. F.; Valcnsi. Q.. 1.a Trcnta, G. & Harris. M. N. (1086). Surgical tredrmcnt of derrnatolibrosarcoma protuberans. Srtrgcry, Ciyr~colog & Obstcrrks, 162: 449. Roswell. A. R., Poole. M. D. & Godfrey, A. M. (1986). Dcrmatotihrosarcoma protubcrans: the problem of surgical managcmcnt. &iris/i Journal ofP/usric Surgery. 39, 262. Schuller. D. E., Snyderman, C. II. 8; Quivcy, J. MM.(1990). Dcrmatotibrosarcoma protubcrans. Head& Neck Surgery, 12, 178. Taylor. H. B. & Ilelwig, E. B. (1962). Dermatofibrosarcoma protuberans. A study of I I5 casts. Cuncer. 15, 717.
The Authors I’. Mclnughlin Registrar
FDSRCS,
ARCS
protuberans
M. Girach BDS Senior House Oficcr G. Wood FDSRCPS, FRCS Consultant Department of Oral and Maxillofacial Glan Clwyd Hospital Bodelwyddan Clwyd LLl8 51JJ
Correspondence and requests Mr P. MM.McLaughlin
Paper received 9 January 1992 Accepted 30 March 1992
Surgery
for olfprints
to
of the scalp
403
protuberans of the scalp
M. Girach.
G. A. Wood
of‘Oral and Ma.~illojuciul
Department
Surgery, Glun Chyd
Hospital,
Bodel\v~~ddun, Chyd
S EMMA R Y. Dermatotibrosarcoma protuberans is an uncommon tumour of the dermis and rarely occurs on the scalp; there are few known predisposing factors. A case is described of such a tumour that occurred at the site of radiotherapy for a basal cell carcinoma. Histological characteristics, bchaviour and management of dermatofibrosarcoma protuberans are discussed.
INTRODUCTION
those of a dcrmatofibrosarcoma protuberans. Excision was reported as incomplete on the deep margin (Figure). The patient was returned to thcatrc. whcrc under local anacsthesia and intravenous sedation, further local excision was carried out with a 3 cm excision margin. and en bloc removal of the underlying subcutis including fascia. The defect was reconstructed using a split skin graft. Excision was reported as complete. and the patient remains recurrence free to date.
Dermatolibrosarcoma protuberans (DFSP) is an uncommon tumour that arises in the dermis. It is characteriscd by slow invasive growth, local recurrence after excision. and infrequent metastasis. The lesion is approximately four times as common in men:
has
a peak
incidence
in the third
decade,
and
may occur at sites of previous trauma. The tumour occurs most commonly on the trunk and proximal extremities (Taylor & Helwig, 1962). It also appears on the head and neck, but less than 5% of all lesions are located on the scalp (Barnes & Coleman, 1984). A case is reported of DFSP of the scalp following radiotherapy, such an incidence WC believe previously unreported. The characteristics and management of thcsc tumours are discussed.
DISCUSSION The term introduced
dermatofibrosarcoma protuberans was by Holfman in 1925, although the tumour
Case report A 76-year-old lady was rcferrcd to the dermatology dcpartmcnt at Glan Clwyd hospital in June 1988 with a lesion clinically diagnosed as a basal cell carcinoma on the left temporal skin. This lesion was removed under local anacsthetic and shown histologically to be complctcly excised, the clinical diagnosis being confirmed. At a follow up visit in February 1989, a recurrence was noted at the excision site, shown by punch biopsy to be a further basal cell carcinoma and a referral made to the local radiotherapy unit. Treatment was given in the form of external beam radiotherapy as a 2OcGy single dose. resulting in complete remission of the lesion. In August 1991, the patient was referred to the Dcpartmcnt of Oral and Maxillofacial Surgery with a further lesion at the original excision site on the scalp. A raised nodule. 5 mm in diamctcr, was found on the left temporal skin, and a provisional diagnosis of recurrent basal cell carcinoma was made. Excision under local anacsthcsia and sedation was carried out, taking a 3 mm margin of clearance around the lesion. and primary closure obtained. IIowevcr histology of the exiscd specimen showed the superficial and deep dermis replaced by a fibroblastic infiltrate of spindle and stellate cells with interweaving bundles of fibrocollagen and focal myxoid degeneration. This myxoid stroma extended into the subcutis and incorporated fibroblastic cells. scattered mitoscs were seen. These appearances were
IGgure - Photomicrograph of the excision sPecimcn showing a typical cartwheel arrangement of tibroblasts (H&E. Original magnification x I 15).
401
402
British Journal
of Oral and
Maxillocdcial
Surgcr);
was first recognised as a clinicopathologic entity by Daricr and Fcrrand in 1924. Dl:SP is a tumour of so-called fibrohistiocytic origin. although thcrc is insufficient cvidcncc to support cithcr a histiocytic or neuroectodermal origin for this tumour (Fletcher et al., 1985). Other tumours classified similarly are: fibrous histiocytoma. sclerosing haemangioma, atypical fibroxanthoma and malignant fibrous histiocytoma. While the last is now well dcscribcd (Kcarney et al., 1980), much of the published work on DFSP antedates its original description (Kempson & Kyriakos, 1972). Consequently much of the older litcrainvolving descriptions of aetiology and ture clinicopathological bchaviour is misleading, as many of the reported examples would today be reclassified as either atypical fibroxanthoma or malignant fibrous histiocytoma. In the more recent litcraturc, therefore: DFSP is considered to have no precipitating cause. and in most case reports no comment is made on possible aetiological factors. A history of prior trauma is most often mentioned and this has been reported on the scalp (Rockley et 01.~ 1989), a case following BCG vaccination has also been described (McLelland & Chu, 1988). We find no report of DFSP being induced by ionizing radiation, but this could bc considered as a possible aetiological factor in the case described. DFSP is an unusual tumour, the incidcncc of which has been estimated at 0.8 cases per million persons per year (Bendix-Hansen ef al., 1983). Clinically the lesion appears initially as a dusky indurated plaque. The tumour may bc flesh-coloured. bluish-red, rcddish brown, or yellow in colour. In time, the tumour develops a nodular or multinodular quality and may ulcerate. In advanced stages the tumour may invade fascia and muscle, and adjacent underlying structures such as calvarium, dura and brain (Rockley et [II., 1989). While metastases to regional lymph nodes (Hirabayashi et a/., 1989) and lungs (Kahn et al., 1978) do occur in DFSP, such bchaviour is rare. Histologically. DFSP is typified by a storiform pattern created by spindle cells arranged in cartwhccllike arrays about collagenous centres (Taylor & Helwig, 1962). The interlacing bands and whorls of fibroblasts may fill the entire reticular dermis and cxtcnd into subcutaneous fat. Large numbers of mitotic figures arc usually not seen, nor is cell atypia usually found. The nodular nature of DFSP gives an erroneous impression of circumscription. The main body of the tumour gives off multiple microscopic projections of neoplastic cells, which may extend 3 centimctres or more beyond the tumour proper. Failure to excise all of these projections is likely to be the main reason for the high rate of recurrence noted with this tumour (Pack & Tabah, 1951). Taylor and Helwig (1962) in a series of 98 cases, returned a 49”/0 recurrence rate, 40% recurring within 1 year and 75% of the recurrences within 3 years following surgery. Rowsell et al. (1986) reported five cases: all of which required additional surgery following incomplete excision. Two of these patients needed two additional procedures, and one patient underwent
three procedures. in spite of the authors’ technique of taking an avcragc 2.7 cm margin of clinically uninvolved skin. and subcutaneous tissue, at the first re-excision. Roses et al. (1986) used a 3 cm margin in IO patients and reported a 20% recurrence rate, while Bendix-Hansen et al. (1983) took a 3 cm margin in 7 patients with no recurrences. Jambhekar and Chinoy (1988) rcportcd a 25% rccurrcncc ralc in 47 cases where treatment was ‘mainly surgical’, howcvcr, they also employed radiotherapy as both primary and adjuvant therapy. Although DFSP is primarily a surgically managed lesion a regimen of electron and photon radiation has been reported; in six patients so treated, no local recurrences were noted with follow-up periods from 15. 105 months (Schullcr et ul., 1990). IO patients with Hobbs et al. (1988) reported rccurrcncc of DFSP following conventional excision, treated with Mohs micrographic surgery. The average duration of follow-up in the series was 43 months (range 15-91 months)? with no evidence of rccurrcncc in any of the cases.
References Barnes. L. & Coleman. J. A. (1984). Dermatolibrosarcoma protuberans of the head and neck. /irchives q/O~o/rzr~t~go/og. 110, 39x. Bendix-llansen, K., Myhrc-Jcnscn. 0. & Kaae. S. (1983). Dermatolihrosarcoma protubcrans. A clinicopathological study of nineteen cases and rcvicw of world literature. Srandinaviun 17,247.
Jotrrncrl of Plus!ic
and Reconsrrucrice
Surgery.
Darricr. J. & Fcrrand. M. (1924). Dermatotibromas progrcssifs ct de recidivants ou fibrosarcomcs de la pcau. Ann&s Derma/olopie et S~~philographie, 5, 545. Flctchcr, C. D. M.. Evans, B. J.. Macartney, J. C.. Smith. N.. Wilson Jones. I!. & McKee, P. II. (1985). Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochcmical study with a review of the literature. Hisropathology,
9.921.
Hirabayashi. S., Kajikawa. A.. Kana7awa. K. & Mimoto. K. (1989). Dermatofibrosarcoma protubcrans with regional lymph node metastasis: a cast report. Head utzd Feck Surger,v. 11,562. IIobbs. E. R.. Whceland. R. G.. Bailin. P. L.. Ratz. J. L., Yetman, R. J. & Zins, J. E. (1988). Treatment of dermatofibrosarcoma protubcrans with Mobs micrographic surgery. Annuls of Surgery.
207, 102.
Hoffman. F.. (1925). Ubcr das knollentreibendc haut (dcrmatofibrosarkoma protubcrdns). Zeirschr~fi.
43,
librosarkom
der
Lkrmutologische
I.
Jambhekar. S. A. & Chinoy, R. C. (1988). Dermatofibrosarcoma protuberans, experience at a cancer hospital in India. Indiun Journul
qf Cancer,2594.
Kahn. L. B., Saxe. N. &Gordon. W. (1978). Dermatolibrosarcoma protubcrans with lymph node and pulmonary metastasis. Arc/rives of Dermafology, 114,8X. Kearney. Mi. M., Soule, E. II. & Ivins, J. C. (1980). Malignant fibrous histiocytoma. A retrospcctivc study of 167 casts. Cuncer, 45, 167. Kcmpson. R. L. & Kyriakos: M. (1972). Fibroxanthosarcoma of the soft tissues. A type of malignant fibrous histiocytoma. Cuncer. 29, 961. McLelland, J. & Chu. T. (198X). Dermatofibrosarcoma protubcrans arising in a BCG vaccination scar. Archiws of Dermarolog~~:
124,496.
Pack, G. T. & Tabah, I?. J. (1951). Dermatofibrosarcoma protuberans: a report of thirty-nine casts. Archives qf’Sur,oery. 63,391.
Dcrmatotibrosarcoma Rockley. P. F., Robinson. J. K., Magid. M. & Goldblatt, D. (1989). Dcrmatofibrosarcolna protubcrans of the scalp: a series of cases, Journal of/he American Acoden?,; of lkrmarolog~.
21, 27X.
Roses, D. F.; Valcnsi. Q.. 1.a Trcnta, G. & Harris. M. N. (1086). Surgical tredrmcnt of derrnatolibrosarcoma protuberans. Srtrgcry, Ciyr~colog & Obstcrrks, 162: 449. Roswell. A. R., Poole. M. D. & Godfrey, A. M. (1986). Dcrmatotihrosarcoma protubcrans: the problem of surgical managcmcnt. &iris/i Journal ofP/usric Surgery. 39, 262. Schuller. D. E., Snyderman, C. II. 8; Quivcy, J. MM.(1990). Dcrmatotibrosarcoma protubcrans. Head& Neck Surgery, 12, 178. Taylor. H. B. & Ilelwig, E. B. (1962). Dermatofibrosarcoma protuberans. A study of I I5 casts. Cuncer. 15, 717.
The Authors I’. Mclnughlin Registrar
FDSRCS,
ARCS
protuberans
M. Girach BDS Senior House Oficcr G. Wood FDSRCPS, FRCS Consultant Department of Oral and Maxillofacial Glan Clwyd Hospital Bodelwyddan Clwyd LLl8 51JJ
Correspondence and requests Mr P. MM.McLaughlin
Paper received 9 January 1992 Accepted 30 March 1992
Surgery
for olfprints
to
of the scalp
403
