Dermatofibrosarcoma Protuberans GEORGE R. MIKHAIL, M.D. AND BRUCE H. LYNN, M.D.
Dermatofibrosarcoma protuberans is a well-differentiated sarcoma that rarely metastasizes but is characterized by a high recurrence rate. This is due to clinically undetectable, microscopic extensions into the surrounding dermis. Chemosurgery (Mohs) is effective in the ablation of this tumor because the extensions can be identified and eradi cated.
is a well-differ entiated sarcoma of the dermis. Characteristically, the lesion begins as a firm, purplish nodule or plaque which may change little for a number of years, but eventually enters a phase of rapid, nodular growth and possibly ulceration. The trunk is the site o f predilection, but no anatomic site, with the exception of the hand, is exempt. In the majority of reported cases, the process appeared before age 30, but again no age is exempt. All races have been affected.12 3 Early lesions o f dermatofibrosarcoma protuberans may be misinterpreted by the clinician as histiocytomas (dermatofibromas), keloids, or metastatic malignancies. For histologic diagnosis an ample specimen that includes central and peripheral portions of the tumor and subcutaneous fat is required. The main difficulty is differentiation from simple histiocytoma. The micro scopic hallmark of dermatofibrosarcoma protuberans is the cartwheel or storiform configuration o f the neoplastic cells.2 This feature may also be seen in some histiocytomas, although not as distinctly as in the malig nant condition.3 The following points are helpful in the diagnosis o f histiocytoma: (a) absence of a shelf-like mass extending laterally from the main part of the tumor into the surrounding dermis; (b) presence of a perivas cular round-cell infiltrate at the periphery of the lesion; (c) presence o f hemosiderin or foam cells, and (d) absence o f involvement o f the subcutaneous fat. There is
D e r m a to fib r o sa r c o m a p r o tu b e r a n s
From the Chemosurgery Unit, D epartm ent o f Dermatology, Henry Ford Hospital, Detroit, Michigan. Address reprint requests to Dr. Mikhail, D epartm ent o f D erm atol ogy, Henry Ford Hospital, 2799 W. Grand Blvd., Detroit, Michigan 48202.
a rare cellular variant of histiocytoma which may present particular difficulty. Such a lesion may grow to a considerable size, may involve the adipose tissue, and may present a cartwheel arrangement.4 In dermatofibrosarcoma protuberans, there is exten sive infiltration by spindle cells into the surrounding dermis in a shelf-like fashion. This shelf may extend for several centimeters beyond the palpable tumor and accounts for frequent recurrences after attempted conventional surgical excision.4 5 6 For this reason, McPeak and his associates recommend that a margin of at least 3 cm of normal-appearing tissue together with underlying deep fascia be excised.5 Considering these anatomic features of this malignancy, it appears likely that the microscopically monitored excision technique of Mohs should be reliable and effective in eradicating it.7’8 Two cases of dermatofibrosarcoma protuberans treated by Mohs’ techniques are here presented. The original fixed-tissue method was applied in the first case, and the fresh-tissue modification was used in the second. CASE REPORTS
Case 1. A 19-year-old girl attended a dermatologic clinic on December 26, 1963 because of a “bruise” below the right clavicle that had been present for 4 years. An indurated, nodular lesion, 2 cm in diameter was excised, and the histology was interpreted as representing a dermatofibroma of the cellular type (Fig. 1A). The tumor recurred and was reexcised with a wide margin 6 months later. This time the surgical specimen was read as dermatofibrosarcoma protuberans. A fibrocellular
J. Dermatol. Surg. Oncol. 4:1 January 1978
81
DERM ATOFIBROSARCOM A PROTUBERANS
F IG U R E 1C. The second recurrence o f the malignancy in the right infraclavicular fossa.
H
j
Pili M 2*»/■•t ■ lü, *•.
,*
FIG U R E 1A. Dermatofibrosarcoma protuberans mistaken fo r dermatofibroma. The cartwheel pattern is absent but the subcu taneous fa t is invaded by the neoplasm.
F IG U R E IB. The shelf-like band o f the process in the dermis.
FIG U R E
IE. (Right) Appearance o f the wound following
chemosurgery.
82
J. Dermatol Surg. Oncol. 4:1 January 1978
F IG U R E ID . 77ie shelf-like component o f the neoplasm has
myxoid features.
MIKHAIL AN D LYNN
F IG U R E IF . Clinical appearance after healing by secondary
intention.
band was said to extend laterally from the main tumor into the adjoining dermis (Fig. IB). A second recurrence appeared 10 years later. A dusky red, nontender mass, again 2 cm in diameter, was found at the medial end o f a scar in the right infraclavicular fossa (Fig. 1C). A biopsy specimen o f it showed shelf-like growth in the dermis
F IG U R E 2A. The cartwheel pattern o f dermatofibrosarcoma
protuberans.
FIG U R E 2B. The site o f the residual process in left breast.
that had myxoid features (Fig. ID), a picture not uncommonly seen in recurrent lesions of this malignan cy.2 The lesion was managed by Mohs’ fixed-tissue technique. Complete microscopic ablation required the excision of 4 layers o f tissue. The immediate wound measured 5.7 X 5 cm (Fig. IE). It took 7 weeks to heal by secondary intention in the form o f a soft scar measuring 3.5 X 1.7 cm (Fig IF). No recurrence or metastasis has appeared after 3 years o f follow-up. Case 2. A 19-year-old woman consulted a dermatolo gist in February 1974 because o f a blue-red mass which had been present in the left breast for 4 months. A punch biopsy was read as histiocytoma. Because the growth continued to enlarge, the patient was referred to a plastic surgeon who performed a wide and deep excision 4 months later. The tissue was examined by the same pathologist who read the original biopsy and who now reported the surgical specimen as an incompletely excised dermatofibrosarcoma protuberans (Fig. 2A). The patient was then referred to us for chemosurgical extirpation of the lesion. Examination revealed a surgical scar 3.2 X 1.5 cm in length and breadth on the medial aspect o f the breast (Fig. 2B). The residual
FIG U R E 2C. The fibrocellular shelf o f the malignancy at the periphery o f the lesion.
F IG U R E 2D. The clinical appearance afterfresh-tissue ablation followed by closure o f the wound.
DERM A TO FIBROSARCOM A PROTUBERANS
tumor was ablated 2 months later in 2 sessions by the Mohs fresh-tissue method. The tumor shelf identified during chemosurgery was fibrocellular (Fig. 2C). The widely gaping wound, 8.5 cm in its vertical diameter, was closed after careful hemostasis by approximating the skin margins with 3-0 silk sutures and Steri-Strips®, and dressed with a pressure bandage. Buried sutures were intentionally omitted in order to avoid confusion should a foreign body granuloma arise. Healing was uneventful (Fig. 2D). When seen 13 months after surgery, there was no recurrence or palpable mass in the breast and there were no signs o f retraction when the pectoral muscles were tensed by raising o f the arms above the head.
Although the disease is insidious in its course and rarely metastasizes, it tends to be subtly an aggressive tumor by sending out clinically undetectable extensions that account for its high recurrence rate when incompletely extirpated. Increased awareness by clinicians, pathol ogists and surgeons of the insidious nature o f this lesion is important in its definitive management. A CK N O W L ED G M E N T This work was supported in part by the N ational Institutes o f Health Biomedical Research support G rant No. 5S07 PR 05490-14 and the Ford Foundation through the Henry Ford Hospital grants R 10501, R35786 and R12016.
R E FEREN CES
COM M ENT
Although dermatofibrosarcoma protuberans is rare, history and clinical findings should alert the physician to the possibility of this condition and should prompt an adequate biopsy. The most reliable features for histolog ical diagnosis o f dermatofibrosarcoma protuberans are 1) involvement of subcutaneous fat and 2) lateral extensions o f the process into the surrounding dermis. The initial diagnosis in the above 2 cases was histiocy toma perhaps because the biopsy specimen was insuffi cient. The initiaTbiopsy from Case 1 did not show the cartwheel configuration. However, the invasion o f the adipose tissue should have aroused suspicion (Fig. 1A) and a larger biopsy might have demonstrated the shelf like extension shown in Figs. IB and ID. As in other neoplastic conditions, the prognosis o f a particular case of dermatofibrosarcoma protuberans depends upon the stage at which the diagnosis is made.
84
J. Derm atol Surg. Oncol 4:1 January 1978
1.
M opper, C., and Pinkus, H. Derm atofibrosarcom a protuberans. Am. J. Clin. Pathol. 20:171-176, 1950. 2. Taylor, H.B., and Helwig, E.B. Derm atofibrosarcom a protuber ans: A study of 115 cases. Cancer 15:717-725, 1962. 3. Shapiro, L., and Brownstein, M.H. Derm atofibrosarcom a protu berans. In: A ndrade, A., Gum port, S.L., Popkin, G.L., and Rees, T.D., eds. C ancer o f the Skin. Philadelphia, W.B. Saunders Co., 1976, pp. 1069-1078. 4. Pinkus, H., and M ehregan, A.H. A Guide to D erm atohistopathology. 2nd ed. New York, Appleton-Century-Crofts, 1976, pp. 597-599. 5. McPeak, C.J., Cruz, T., and Nicastri, A.D. Derm atofibrosarcom a protuberans: An analysis o f 86 cases—five with metastasis. Ann. Surg. 166:803-816, 1967. 6. Smith, J.L., Jr. Tumors o f the corium . In: Helwig, E.B., and Mostofi, F.K., eds. The Skin. Baltimore, W illiams and Wilkins Co., 1971, pp. 533-577. 7. Mohs, F.E. Chemosurgery in Cancer, G angrene and Infections. Springfield, Illinois, Charles C Thomas, 1956. 8. Mohs, F.E. Chemosurgery for skin cancer: Fixed tissue and fresh tissue technique. Arch. Dermatol. 112: 211-215, 1976.
Dermatofibrosarcoma protuberans is a well-differentiated sarcoma that rarely metastasizes but is characterized by a high recurrence rate. This is due to clinically undetectable, microscopic extensions into the surrounding dermis. Chemosurgery (Mohs) is effective in the ablation of this tumor because the extensions can be identified and eradi cated.
is a well-differ entiated sarcoma of the dermis. Characteristically, the lesion begins as a firm, purplish nodule or plaque which may change little for a number of years, but eventually enters a phase of rapid, nodular growth and possibly ulceration. The trunk is the site o f predilection, but no anatomic site, with the exception of the hand, is exempt. In the majority of reported cases, the process appeared before age 30, but again no age is exempt. All races have been affected.12 3 Early lesions o f dermatofibrosarcoma protuberans may be misinterpreted by the clinician as histiocytomas (dermatofibromas), keloids, or metastatic malignancies. For histologic diagnosis an ample specimen that includes central and peripheral portions of the tumor and subcutaneous fat is required. The main difficulty is differentiation from simple histiocytoma. The micro scopic hallmark of dermatofibrosarcoma protuberans is the cartwheel or storiform configuration o f the neoplastic cells.2 This feature may also be seen in some histiocytomas, although not as distinctly as in the malig nant condition.3 The following points are helpful in the diagnosis o f histiocytoma: (a) absence of a shelf-like mass extending laterally from the main part of the tumor into the surrounding dermis; (b) presence of a perivas cular round-cell infiltrate at the periphery of the lesion; (c) presence o f hemosiderin or foam cells, and (d) absence o f involvement o f the subcutaneous fat. There is
D e r m a to fib r o sa r c o m a p r o tu b e r a n s
From the Chemosurgery Unit, D epartm ent o f Dermatology, Henry Ford Hospital, Detroit, Michigan. Address reprint requests to Dr. Mikhail, D epartm ent o f D erm atol ogy, Henry Ford Hospital, 2799 W. Grand Blvd., Detroit, Michigan 48202.
a rare cellular variant of histiocytoma which may present particular difficulty. Such a lesion may grow to a considerable size, may involve the adipose tissue, and may present a cartwheel arrangement.4 In dermatofibrosarcoma protuberans, there is exten sive infiltration by spindle cells into the surrounding dermis in a shelf-like fashion. This shelf may extend for several centimeters beyond the palpable tumor and accounts for frequent recurrences after attempted conventional surgical excision.4 5 6 For this reason, McPeak and his associates recommend that a margin of at least 3 cm of normal-appearing tissue together with underlying deep fascia be excised.5 Considering these anatomic features of this malignancy, it appears likely that the microscopically monitored excision technique of Mohs should be reliable and effective in eradicating it.7’8 Two cases of dermatofibrosarcoma protuberans treated by Mohs’ techniques are here presented. The original fixed-tissue method was applied in the first case, and the fresh-tissue modification was used in the second. CASE REPORTS
Case 1. A 19-year-old girl attended a dermatologic clinic on December 26, 1963 because of a “bruise” below the right clavicle that had been present for 4 years. An indurated, nodular lesion, 2 cm in diameter was excised, and the histology was interpreted as representing a dermatofibroma of the cellular type (Fig. 1A). The tumor recurred and was reexcised with a wide margin 6 months later. This time the surgical specimen was read as dermatofibrosarcoma protuberans. A fibrocellular
J. Dermatol. Surg. Oncol. 4:1 January 1978
81
DERM ATOFIBROSARCOM A PROTUBERANS
F IG U R E 1C. The second recurrence o f the malignancy in the right infraclavicular fossa.
H
j
Pili M 2*»/■•t ■ lü, *•.
,*
FIG U R E 1A. Dermatofibrosarcoma protuberans mistaken fo r dermatofibroma. The cartwheel pattern is absent but the subcu taneous fa t is invaded by the neoplasm.
F IG U R E IB. The shelf-like band o f the process in the dermis.
FIG U R E
IE. (Right) Appearance o f the wound following
chemosurgery.
82
J. Dermatol Surg. Oncol. 4:1 January 1978
F IG U R E ID . 77ie shelf-like component o f the neoplasm has
myxoid features.
MIKHAIL AN D LYNN
F IG U R E IF . Clinical appearance after healing by secondary
intention.
band was said to extend laterally from the main tumor into the adjoining dermis (Fig. IB). A second recurrence appeared 10 years later. A dusky red, nontender mass, again 2 cm in diameter, was found at the medial end o f a scar in the right infraclavicular fossa (Fig. 1C). A biopsy specimen o f it showed shelf-like growth in the dermis
F IG U R E 2A. The cartwheel pattern o f dermatofibrosarcoma
protuberans.
FIG U R E 2B. The site o f the residual process in left breast.
that had myxoid features (Fig. ID), a picture not uncommonly seen in recurrent lesions of this malignan cy.2 The lesion was managed by Mohs’ fixed-tissue technique. Complete microscopic ablation required the excision of 4 layers o f tissue. The immediate wound measured 5.7 X 5 cm (Fig. IE). It took 7 weeks to heal by secondary intention in the form o f a soft scar measuring 3.5 X 1.7 cm (Fig IF). No recurrence or metastasis has appeared after 3 years o f follow-up. Case 2. A 19-year-old woman consulted a dermatolo gist in February 1974 because o f a blue-red mass which had been present in the left breast for 4 months. A punch biopsy was read as histiocytoma. Because the growth continued to enlarge, the patient was referred to a plastic surgeon who performed a wide and deep excision 4 months later. The tissue was examined by the same pathologist who read the original biopsy and who now reported the surgical specimen as an incompletely excised dermatofibrosarcoma protuberans (Fig. 2A). The patient was then referred to us for chemosurgical extirpation of the lesion. Examination revealed a surgical scar 3.2 X 1.5 cm in length and breadth on the medial aspect o f the breast (Fig. 2B). The residual
FIG U R E 2C. The fibrocellular shelf o f the malignancy at the periphery o f the lesion.
F IG U R E 2D. The clinical appearance afterfresh-tissue ablation followed by closure o f the wound.
DERM A TO FIBROSARCOM A PROTUBERANS
tumor was ablated 2 months later in 2 sessions by the Mohs fresh-tissue method. The tumor shelf identified during chemosurgery was fibrocellular (Fig. 2C). The widely gaping wound, 8.5 cm in its vertical diameter, was closed after careful hemostasis by approximating the skin margins with 3-0 silk sutures and Steri-Strips®, and dressed with a pressure bandage. Buried sutures were intentionally omitted in order to avoid confusion should a foreign body granuloma arise. Healing was uneventful (Fig. 2D). When seen 13 months after surgery, there was no recurrence or palpable mass in the breast and there were no signs o f retraction when the pectoral muscles were tensed by raising o f the arms above the head.
Although the disease is insidious in its course and rarely metastasizes, it tends to be subtly an aggressive tumor by sending out clinically undetectable extensions that account for its high recurrence rate when incompletely extirpated. Increased awareness by clinicians, pathol ogists and surgeons of the insidious nature o f this lesion is important in its definitive management. A CK N O W L ED G M E N T This work was supported in part by the N ational Institutes o f Health Biomedical Research support G rant No. 5S07 PR 05490-14 and the Ford Foundation through the Henry Ford Hospital grants R 10501, R35786 and R12016.
R E FEREN CES
COM M ENT
Although dermatofibrosarcoma protuberans is rare, history and clinical findings should alert the physician to the possibility of this condition and should prompt an adequate biopsy. The most reliable features for histolog ical diagnosis o f dermatofibrosarcoma protuberans are 1) involvement of subcutaneous fat and 2) lateral extensions o f the process into the surrounding dermis. The initial diagnosis in the above 2 cases was histiocy toma perhaps because the biopsy specimen was insuffi cient. The initiaTbiopsy from Case 1 did not show the cartwheel configuration. However, the invasion o f the adipose tissue should have aroused suspicion (Fig. 1A) and a larger biopsy might have demonstrated the shelf like extension shown in Figs. IB and ID. As in other neoplastic conditions, the prognosis o f a particular case of dermatofibrosarcoma protuberans depends upon the stage at which the diagnosis is made.
84
J. Derm atol Surg. Oncol 4:1 January 1978
1.
M opper, C., and Pinkus, H. Derm atofibrosarcom a protuberans. Am. J. Clin. Pathol. 20:171-176, 1950. 2. Taylor, H.B., and Helwig, E.B. Derm atofibrosarcom a protuber ans: A study of 115 cases. Cancer 15:717-725, 1962. 3. Shapiro, L., and Brownstein, M.H. Derm atofibrosarcom a protu berans. In: A ndrade, A., Gum port, S.L., Popkin, G.L., and Rees, T.D., eds. C ancer o f the Skin. Philadelphia, W.B. Saunders Co., 1976, pp. 1069-1078. 4. Pinkus, H., and M ehregan, A.H. A Guide to D erm atohistopathology. 2nd ed. New York, Appleton-Century-Crofts, 1976, pp. 597-599. 5. McPeak, C.J., Cruz, T., and Nicastri, A.D. Derm atofibrosarcom a protuberans: An analysis o f 86 cases—five with metastasis. Ann. Surg. 166:803-816, 1967. 6. Smith, J.L., Jr. Tumors o f the corium . In: Helwig, E.B., and Mostofi, F.K., eds. The Skin. Baltimore, W illiams and Wilkins Co., 1971, pp. 533-577. 7. Mohs, F.E. Chemosurgery in Cancer, G angrene and Infections. Springfield, Illinois, Charles C Thomas, 1956. 8. Mohs, F.E. Chemosurgery for skin cancer: Fixed tissue and fresh tissue technique. Arch. Dermatol. 112: 211-215, 1976.
