GYNECOLOGIC
ONCOLOGY
41, 245-249
(1991)
CASE REPORT Dermatofibrosarcoma Protuberans of the Vulva JONATHAN *Department
F. LEAKE, M.D.,* of Gynecology
JOSEPHBUSCEMA, M.D.,*
and Obstetrics
and TDepartment
KATHLEEN
of Pathology,
R. CHO, M.D. ,t AND JOHN L. CURRIE, M.D.*
The Johns
Hopkins
Hospital,
Baltimore,
Maryland
21205
ReceivedOctober30, 1990 Dermatofibrosarcoma protuberansof the vulva is an ucnommon low-gradesarcomaof dermalorigin. Although widelocal excision is the treatment of choice, microscopictumor projectionsbeyond the central tumor noduleexplain the tumors’ propensityfor local recurrence.Frozen sectionsof marginsmay be useful to ensure completeresection.The following report contributes two additional patients with this uncommonneoplasm.Notably, one of thesetwo had a fibrosarcomatousarea within the dermatofibrosarcomaprotuberans.This is the secondreported caseof a fibrosarcomaarising in a dermatofibrosarcoma protuberansof the vulva. 0 1991 Academic Press, Inc.
INTRODUCTION Vulvar sarcomas are infrequently encountered in gynecologic oncology, comprising only 3.0% of all primary malignancies at this site [l]. Leiomyosarcoma, the most common type, accounts for 33 [2] to 42% [l] of all vulvar sarcomas. In contrast, dermatofibrosarcoma protuberans (DFSP) is rarely encountered. A review of the literature reveals only six reported cases of DFSP arising at this site [2-71. An additional two patients with this uncommon neoplasm are reported herein. One of these patients had a fibrosarcomatous area within the DFSP. This represents the second reported case of a fibrosarcoma arising in a vulvar DFSP [7].
CASE REPORT Patient 1 A 37-year-old Caucasian female, para 0, presented with a 2-year history of a lesion of the left mons pubis. Initially, the mass was of 1.5 cm diameter; however, over the ensuing 2 years it slowly enlarged. Medical evaluation was sought; incision and simple enucleation of a 6 x 4.5 x 2.5cm mass of the left mons pubis and labium majus
was performed. Histologic evaluation revealed dermatofibrosarcoma protuberans with involved surgical margins. The patient was referred to The Johns Hopkins Hospital, where examination revealed a firm, mobile, wellcircumscribed 2 x 2-cm nodule of the left mons pubis and a second 0.5 x 0.5-cm nodule at the mid aspect of the left labium majus. There were no clinically suspicious inguinal lymph nodes and the remainder of the physical examination was normal. Wide radical excision, with 2cm peripheral margins, coupled with deep resection to the urogenital diaphragm was performed. Frozen section demonstrated microscopic involvement of the original margins, prompting further excision to obtain tumor-free limits of resection. Histologic evaluation revealed extensive dermatofibrosarcoma protuberans with clear surgical margins. The postoperative course was uncomplicated and the patient has been clinically free of disease for 15 months following surgery. Microscopically, the neoplasm diffusely infiltrated the dermis and subcutaneous tissue (Fig. 1). The central portion of the tumor was composed of fusiform cells arranged in a woven (storiform) pattern around an inconspicuous vasculature (Fig. 2). In the deep regions, the tumor interdigitated with lobules of subcutaneous fat. Examination of the tumor periphery revealed microscopic projections of tumor extending well beyond the macroscopically recognizable central portion of the neoplasm. Tumor nuclei were oval and without significant pleomorphism. Mitotic activity averaged two mototic figures per 10 highpowered fields (HPF). Cellular atypia, necrosis, and myxoid areas were not identified. Patient 2 A 61-year-old Black female was referred to The Johns Hopkins Hospital with the diagnosis of a recurrent vulvar
245 All
0090-8258/91 $1.50 Copyright 0 1991 by Academic Press, Inc. rights of reproduction in any form reserved.
246
LEAKE
FIG. 1. Low-power
ET AL.
view demonstrating the infiltration of dermis and subcutaneous tissue by DFSP.
mass. On routine examination 2 years earlier, a 5 x 2cm subcutaneous nodule was noted in the right labium majus. The nodule was excised and on histopathologic examination was identified as a fibrosarcoma arising in a dermatofibrosarcoma protuberans. The lateral margins were involved with DFSP; however, the patient refused
FIG. 2. High-power
further wide resection. At age 61, she was noted to have a similar nodule in the same location and was referred for further therapy. Examination revealed a firm, mobile, well-circumscribed 8 x 5 x 4-cm mass of the right labium majus. There were no clinically suspicious inguinal lymph nodes and the remainder of the physical examination was
view demonstrating fusiform cells arranged in a storiform pattern.
247
CASE REPORT
FIG. 3. High-power
view of fibrosarcomatous element demonstrating fusiform cells arranged in a herringbone
pattern.
with fusiform nuclei; scattered rough endoplasmic reticnormal. A right radical hemivulvectomy with Z-plasty cytoplasmic propedicle flap reconstruction was performed. Frozen sec- ulum; and long, slender, interdigitating jections. In many areas abundant collagen fibrils sepations of all margins were free of tumor. Histopathologic rated the tumor cells. An incomplete basal lamina was examination revealed a fibrosarcoma arising in a DFSP. The postoperative course was uncomplicated and the pa- focally noted and rare intercellular junctions were identified (Fig. 4). tient is clinically free of disease 18 months following surgery. Microscopically, the neoplasm infiltrated the dermis DISCUSSION and subcutaneous tissues. The DFSP component was comprised of fusiform cells arranged in a woven (storiAlthough the term dermatofibrosarcoma protuberans form) pattern infiltrating the subcutaneous tissue and sur- was first employed by Hoffman [8] in 1925, this uncomrounding fat cells. The tumor cell nuclei were oval and mon fibrous tumor was originally described by Darier and without significant pleomorphism. The mitotic rate was 1 Ferrand [9] in 1924. It is a rare, low-grade sarcoma of per 10 HPF. There was a gradual transition between the dermal origin which characteristically presents during DFSP and the fibrosarcomatous portion of the neoplasm. early or mid-adult life as a firm, well-circumscribed noThe fibrosarcomatous elements were concentrated in the dular mass, fixed to the overlying skin, but movable over subcutis; these displayed long, interlacing fascicles of fu- the deeper tissues [lo-121. The majority of patients are siform cells creating a herringbone pattern (Fig. 3). A asymptomatic; however, pain, tenderness, and ulceration moderate amount of stromal collagen was identified. In may develop with tumors in an accelerated growth phase. comparison with the DFSP, slightly increased cellularity Although these neoplasms may occur at almost any site and cellular atypia were noted and the mitotic activity except the palms and soles [lo], they are most frequently was greater (8 per 10 HFP). The microscopic findings reported to involve the trunk and proximal extremities were consistent with a grade l-2 fibrosarcoma. [10,12]. Radiation appears to be without benefit as priImmunohistochemical and ultrastructural studies were mary or adjuvant therapy [10,12,14] and wide local exconsistent with a fibroblastic tumor. Immunohistochemcision of primary and recurrent tumors is the treatment ical stains for markers of smooth muscle (muscle-specific of choice [lo-161, resulting in 91 to 100% survival. The actin and desmin) and neural tissues (S-100) were neg- low incidence of regional lymph node metastasis (O-l%) ative. Electron microscopy demonstrated elongated cells argues against routine lymph node dissection [lo-14,171.
248
LEAKE
FIG. 4. Electron micrograph of DFSP demonstrating
ET AL.
fusiform cells with slender, interdigitating
Dermatofibrosarcoma protuberans is characterized by its local infiltration, slow progression, and propensity for local recurrence. Approximately 80% of local recurrences occur within 3 years [lo] and recurrence rates of 20.5 [ll] to 49% [12] are common following wide excision. Despite locally aggressive behavior, distant metastases are extremely uncommon (O-6%) [lo-141 and typically occur after a history of multiple recurrences [10,12]. Approximately three-fourths of patients with metastases manifest hematogenous spread, predominantly to the lung, and one-fourth manifest lymphatic spread to regional lymph node [17]. Although their use has been predominantly anecdotal, radiation and methotrexate, cytoxan, and actinomycin D combination chemotherapy do not appear to be efficacious in the treatment of metastases [10,18] and resection of isolated pulmonary metastases has been advocated due to the low-grade biologic behavior of the tumor [19]. Predicting which tumors will metastasize is quite difficult. Lesions which metastasize display no histologic features that reliably distinguish them from nonmetastatic lesions [lo]. Nonetheless, metastatic tumors share some common clinical features; they are usually large lesions
cytoplasmic projections.
with a lag time between diagnosis and metastasis of several years and they typically demonstrate local recurrence
[lOI.
When fibrosarcoma develops in a dermatofibrosarcoma protuberans, the age distribution and clinical presentation of the tumor is similar to that of pure DFSP. The anatomic location is usually truncal, in contrast to the marked predilection of pure fibrosarcoma for the extremities [20]. Although fibrosarcomatous changes typically occur after a history of repeated recurrence [21], 42% may be present at the time of initial excision [7]. In all reported cases, the fibrosarcomatous areas have been grade 1 or 2 [7]. The clinical behavior of a fibrosarcoma in a DFSP is similar to that of typical DFSP; there is a marked predilection for local recurrence and metastases of fibrosarcoma are observed in only 8.5% [7]. This contrasts to 46% metastasis associated with pure fibrosarcoma [20]. The infrequent metastasis of fibrosarcoma associated with DFSP may be secondary to its superficial location, low cytologic grade, and small size [7]. The treatment of choice, therefore, remains wide local excision, as with pure DFSP. Although wide local excision is the treatment of choice
CASE REPORT
for dermatofibrosarcoma protuberans, the risk of recurrence appears to inversely correlate with the extent of wide excision. Roses et al. [16] reported a 20% recurrence rate with surgical margins of 3 cm or more from the palpable tumor mass; this is compared to 41% if the margins were 2 cm or less. While this neoplasm clinically appears to be well circumscribed, microscopic projections of tumor often extend well beyond the central nodule. These projections explain the difficulty in obtaining tumor-free margins and the relatively high local recurrence rate. Current recommendations call for a 3-cm or greater margin of excision [5,10,16]; however, margins of this size may at times be difficult to obtain on the vulva while still maintaining cosmesis. Although myocutaneous flaps and Z-plasty reconstruction may yield good cosmetic results, a more conservative excision (margins
ONCOLOGY
41, 245-249
(1991)
CASE REPORT Dermatofibrosarcoma Protuberans of the Vulva JONATHAN *Department
F. LEAKE, M.D.,* of Gynecology
JOSEPHBUSCEMA, M.D.,*
and Obstetrics
and TDepartment
KATHLEEN
of Pathology,
R. CHO, M.D. ,t AND JOHN L. CURRIE, M.D.*
The Johns
Hopkins
Hospital,
Baltimore,
Maryland
21205
ReceivedOctober30, 1990 Dermatofibrosarcoma protuberansof the vulva is an ucnommon low-gradesarcomaof dermalorigin. Although widelocal excision is the treatment of choice, microscopictumor projectionsbeyond the central tumor noduleexplain the tumors’ propensityfor local recurrence.Frozen sectionsof marginsmay be useful to ensure completeresection.The following report contributes two additional patients with this uncommonneoplasm.Notably, one of thesetwo had a fibrosarcomatousarea within the dermatofibrosarcomaprotuberans.This is the secondreported caseof a fibrosarcomaarising in a dermatofibrosarcoma protuberansof the vulva. 0 1991 Academic Press, Inc.
INTRODUCTION Vulvar sarcomas are infrequently encountered in gynecologic oncology, comprising only 3.0% of all primary malignancies at this site [l]. Leiomyosarcoma, the most common type, accounts for 33 [2] to 42% [l] of all vulvar sarcomas. In contrast, dermatofibrosarcoma protuberans (DFSP) is rarely encountered. A review of the literature reveals only six reported cases of DFSP arising at this site [2-71. An additional two patients with this uncommon neoplasm are reported herein. One of these patients had a fibrosarcomatous area within the DFSP. This represents the second reported case of a fibrosarcoma arising in a vulvar DFSP [7].
CASE REPORT Patient 1 A 37-year-old Caucasian female, para 0, presented with a 2-year history of a lesion of the left mons pubis. Initially, the mass was of 1.5 cm diameter; however, over the ensuing 2 years it slowly enlarged. Medical evaluation was sought; incision and simple enucleation of a 6 x 4.5 x 2.5cm mass of the left mons pubis and labium majus
was performed. Histologic evaluation revealed dermatofibrosarcoma protuberans with involved surgical margins. The patient was referred to The Johns Hopkins Hospital, where examination revealed a firm, mobile, wellcircumscribed 2 x 2-cm nodule of the left mons pubis and a second 0.5 x 0.5-cm nodule at the mid aspect of the left labium majus. There were no clinically suspicious inguinal lymph nodes and the remainder of the physical examination was normal. Wide radical excision, with 2cm peripheral margins, coupled with deep resection to the urogenital diaphragm was performed. Frozen section demonstrated microscopic involvement of the original margins, prompting further excision to obtain tumor-free limits of resection. Histologic evaluation revealed extensive dermatofibrosarcoma protuberans with clear surgical margins. The postoperative course was uncomplicated and the patient has been clinically free of disease for 15 months following surgery. Microscopically, the neoplasm diffusely infiltrated the dermis and subcutaneous tissue (Fig. 1). The central portion of the tumor was composed of fusiform cells arranged in a woven (storiform) pattern around an inconspicuous vasculature (Fig. 2). In the deep regions, the tumor interdigitated with lobules of subcutaneous fat. Examination of the tumor periphery revealed microscopic projections of tumor extending well beyond the macroscopically recognizable central portion of the neoplasm. Tumor nuclei were oval and without significant pleomorphism. Mitotic activity averaged two mototic figures per 10 highpowered fields (HPF). Cellular atypia, necrosis, and myxoid areas were not identified. Patient 2 A 61-year-old Black female was referred to The Johns Hopkins Hospital with the diagnosis of a recurrent vulvar
245 All
0090-8258/91 $1.50 Copyright 0 1991 by Academic Press, Inc. rights of reproduction in any form reserved.
246
LEAKE
FIG. 1. Low-power
ET AL.
view demonstrating the infiltration of dermis and subcutaneous tissue by DFSP.
mass. On routine examination 2 years earlier, a 5 x 2cm subcutaneous nodule was noted in the right labium majus. The nodule was excised and on histopathologic examination was identified as a fibrosarcoma arising in a dermatofibrosarcoma protuberans. The lateral margins were involved with DFSP; however, the patient refused
FIG. 2. High-power
further wide resection. At age 61, she was noted to have a similar nodule in the same location and was referred for further therapy. Examination revealed a firm, mobile, well-circumscribed 8 x 5 x 4-cm mass of the right labium majus. There were no clinically suspicious inguinal lymph nodes and the remainder of the physical examination was
view demonstrating fusiform cells arranged in a storiform pattern.
247
CASE REPORT
FIG. 3. High-power
view of fibrosarcomatous element demonstrating fusiform cells arranged in a herringbone
pattern.
with fusiform nuclei; scattered rough endoplasmic reticnormal. A right radical hemivulvectomy with Z-plasty cytoplasmic propedicle flap reconstruction was performed. Frozen sec- ulum; and long, slender, interdigitating jections. In many areas abundant collagen fibrils sepations of all margins were free of tumor. Histopathologic rated the tumor cells. An incomplete basal lamina was examination revealed a fibrosarcoma arising in a DFSP. The postoperative course was uncomplicated and the pa- focally noted and rare intercellular junctions were identified (Fig. 4). tient is clinically free of disease 18 months following surgery. Microscopically, the neoplasm infiltrated the dermis DISCUSSION and subcutaneous tissues. The DFSP component was comprised of fusiform cells arranged in a woven (storiAlthough the term dermatofibrosarcoma protuberans form) pattern infiltrating the subcutaneous tissue and sur- was first employed by Hoffman [8] in 1925, this uncomrounding fat cells. The tumor cell nuclei were oval and mon fibrous tumor was originally described by Darier and without significant pleomorphism. The mitotic rate was 1 Ferrand [9] in 1924. It is a rare, low-grade sarcoma of per 10 HPF. There was a gradual transition between the dermal origin which characteristically presents during DFSP and the fibrosarcomatous portion of the neoplasm. early or mid-adult life as a firm, well-circumscribed noThe fibrosarcomatous elements were concentrated in the dular mass, fixed to the overlying skin, but movable over subcutis; these displayed long, interlacing fascicles of fu- the deeper tissues [lo-121. The majority of patients are siform cells creating a herringbone pattern (Fig. 3). A asymptomatic; however, pain, tenderness, and ulceration moderate amount of stromal collagen was identified. In may develop with tumors in an accelerated growth phase. comparison with the DFSP, slightly increased cellularity Although these neoplasms may occur at almost any site and cellular atypia were noted and the mitotic activity except the palms and soles [lo], they are most frequently was greater (8 per 10 HFP). The microscopic findings reported to involve the trunk and proximal extremities were consistent with a grade l-2 fibrosarcoma. [10,12]. Radiation appears to be without benefit as priImmunohistochemical and ultrastructural studies were mary or adjuvant therapy [10,12,14] and wide local exconsistent with a fibroblastic tumor. Immunohistochemcision of primary and recurrent tumors is the treatment ical stains for markers of smooth muscle (muscle-specific of choice [lo-161, resulting in 91 to 100% survival. The actin and desmin) and neural tissues (S-100) were neg- low incidence of regional lymph node metastasis (O-l%) ative. Electron microscopy demonstrated elongated cells argues against routine lymph node dissection [lo-14,171.
248
LEAKE
FIG. 4. Electron micrograph of DFSP demonstrating
ET AL.
fusiform cells with slender, interdigitating
Dermatofibrosarcoma protuberans is characterized by its local infiltration, slow progression, and propensity for local recurrence. Approximately 80% of local recurrences occur within 3 years [lo] and recurrence rates of 20.5 [ll] to 49% [12] are common following wide excision. Despite locally aggressive behavior, distant metastases are extremely uncommon (O-6%) [lo-141 and typically occur after a history of multiple recurrences [10,12]. Approximately three-fourths of patients with metastases manifest hematogenous spread, predominantly to the lung, and one-fourth manifest lymphatic spread to regional lymph node [17]. Although their use has been predominantly anecdotal, radiation and methotrexate, cytoxan, and actinomycin D combination chemotherapy do not appear to be efficacious in the treatment of metastases [10,18] and resection of isolated pulmonary metastases has been advocated due to the low-grade biologic behavior of the tumor [19]. Predicting which tumors will metastasize is quite difficult. Lesions which metastasize display no histologic features that reliably distinguish them from nonmetastatic lesions [lo]. Nonetheless, metastatic tumors share some common clinical features; they are usually large lesions
cytoplasmic projections.
with a lag time between diagnosis and metastasis of several years and they typically demonstrate local recurrence
[lOI.
When fibrosarcoma develops in a dermatofibrosarcoma protuberans, the age distribution and clinical presentation of the tumor is similar to that of pure DFSP. The anatomic location is usually truncal, in contrast to the marked predilection of pure fibrosarcoma for the extremities [20]. Although fibrosarcomatous changes typically occur after a history of repeated recurrence [21], 42% may be present at the time of initial excision [7]. In all reported cases, the fibrosarcomatous areas have been grade 1 or 2 [7]. The clinical behavior of a fibrosarcoma in a DFSP is similar to that of typical DFSP; there is a marked predilection for local recurrence and metastases of fibrosarcoma are observed in only 8.5% [7]. This contrasts to 46% metastasis associated with pure fibrosarcoma [20]. The infrequent metastasis of fibrosarcoma associated with DFSP may be secondary to its superficial location, low cytologic grade, and small size [7]. The treatment of choice, therefore, remains wide local excision, as with pure DFSP. Although wide local excision is the treatment of choice
CASE REPORT
for dermatofibrosarcoma protuberans, the risk of recurrence appears to inversely correlate with the extent of wide excision. Roses et al. [16] reported a 20% recurrence rate with surgical margins of 3 cm or more from the palpable tumor mass; this is compared to 41% if the margins were 2 cm or less. While this neoplasm clinically appears to be well circumscribed, microscopic projections of tumor often extend well beyond the central nodule. These projections explain the difficulty in obtaining tumor-free margins and the relatively high local recurrence rate. Current recommendations call for a 3-cm or greater margin of excision [5,10,16]; however, margins of this size may at times be difficult to obtain on the vulva while still maintaining cosmesis. Although myocutaneous flaps and Z-plasty reconstruction may yield good cosmetic results, a more conservative excision (margins
