Surgical management of congenital dermatofibrosarcoma protuberans

DERMATOLOGIC SURGERY

Surgical management of congenital dermatofibrosarcoma protuberans W. Elliot Love, DO,a,c Susan A. Keiler, MD,a,c Joan E. Tamburro, DO,a,c Kord Honda, MD,a,c Arun K. Gosain, MD,b,c and Jeremy S. Bordeaux, MD, MPHa,c Cleveland, Ohio Congenital dermatofibrosarcoma protuberans (DFSP) is a rare tumor with varying clinical presentations that is commonly misdiagnosed. Treatment of congenital DFSP is complicated by delays in diagnosis and its propensity for subclinical spread. Of 61 reported cases, 11 (18%) were treated with Mohs micrographic surgery (MMS) and 46 (75%) were treated with wide local excision (WLE). One case was treated with imatinib, and the remaining 3 did not differentiate between receiving MMS or WLE. In the cases of congenital DFSP treated with MMS the clearance rate was 100% with an average follow-up of 4.3 years. The clearance rate seen with WLE was 89% with an average follow-up period of 1.9 years. The average margins taken during MMS (1.7 cm) were smaller than those taken with WLE (2.8 cm). Fifty percent of cases with available follow-up undergoing WLE required multiple surgeries. Based on superior cure rates with longterm follow-up, smaller surgical margins, and fewer surgical sessions, MMS should be considered as firstline treatment for congenital DFSP. ( J Am Acad Dermatol 2009;61:1014-23.) Key words: congenital dermatofibrosarcoma protuberans; Mohs micrographic surgery.

D

ermatofibrosarcoma protuberans (DFSP) is an intermediate-grade malignancy that may be locally aggressive and seldom metastasizes. It is a rare tumor constituting less than 0.1% of cutaneous malignancies in adults.1 Histologically DFSP is a CD341 spindle cell proliferation arranged in a storiform pattern that infiltrates deep into the dermis and adipose layers in a fascicular growth pattern. A characteristic genetic translocation fusing the collagen type 1a-1 gene on chromosome 17 and the platelet-derived growth factor b (PDGFB) gene on chromosome 22 may be present.2 DFSP has a local recurrence rate up to 60% to 75%3,4 after wide local excision (WLE). The preferred treatment for DFSP is Mohs micrographic surgery (MMS) offering clearance rates of 93.4% to 100%.4-7

From the Department of Dermatology, University Hospitals Case Medical Centera; Department of Plastic Surgery, Rainbow Babies and Children’s Hospitalb; and Case Western Reserve University School of Medicine.c Funding sources: Dr Bordeaux is supported by the Dermatology Foundation Clinical Career Development Award in Dermatologic Surgery. Conflicts of interest: None declared. Reprint requests: Jeremy S. Bordeaux, MD, MPH, Department of Dermatology, University Hospitals Case Medical Center, 11100 Euclid Ave, Lakeside 3500, Cleveland, OH 44124. E-mail: Jeremy. [email protected]. 0190-9622/$36.00 ª 2009 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2009.05.028

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Abbreviations used: DFSP: MMS: PDGFB: WLE:

dermatofibrosarcoma protuberans Mohs micrographic surgery platelet-derived growth factor b wide local excision

Congenital DFSP are present at birth and the first case was reported by Borrie8 in 1952; including our case our literature search revealed 61 cases.2,8-40 Varying clinical presentations frequently lead to misdiagnosis (Tables I and II). Because of the locally aggressive nature of the tumor and frequent delay in the diagnosis, treatment with WLE may result in large surgical defects and multiple surgeries. Although the lowest recurrence rates for adult DFSP are seen when treated with MMS, congenital DFSP has typically been treated with WLE.

CASE REPORT A 10-month-old girl presented to our pediatric dermatology clinic for a second opinion regarding a large ‘‘cyst’’ on the mid aspect of her back present since birth. The lesion started as a 4-mm skin-colored papule that had rapidly enlarged with increased redness during a 7-month period. On physical examination, on the central mid aspect of her back, there was a 3.2- 3 3-cm light erythematous tumor containing a small area of terminal hairs (Fig 1, A and B). Magnetic resonance imaging of the lower thoracic spine revealed a 3.0- 3 3.7- 3 0.6-cm soft tissue mass

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extending into the subcutaneous fat without intraevolution of congenital DFSP in 10% of spinal extension. Ultrasound showed no evidence of cases.2,14,18,25,31,38 Congenital DFSP has a female predominance muscle invasion. A punch biopsy specimen revealed (52% vs 38% in boys and 10% unreported). They a dermal and subcutaneous proliferation of spindled occur most often on the trunk (49%), distal extremcells within a myxoid stroma forming intersecting ities (15%), proximal extremities (13%), head and fascicles. The spindle cells lacked significant atypia neck (7%), and buttocks (5%); the remaining sites and extended into the subcutaneous fat in a honeywere unreported. Clinical comb pattern (Fig 1, C ). presentation of congenital Immunohistochemistry was CAPSULE SUMMARY DFSP is highly variable strongly positive for CD34 and includes: (1) pink or (Fig 1, D), whereas CD31, This article compares all cases of erythematous-to-violaceous S-100, smooth muscle actin, congenital dermatofibrosarcoma nodular plaque (most comand factor XIIIa were protuberans treated with wide local mon) (2) solitary tumor, (3) negative, consistent with DFSP. excision versus Mohs micrographic erythematous, violaceous, or The collagen type 1a-1surgery and illustrates that because of dyschromic patch (4) atrophic PDGFB translocation was higher cure rates with long-term followplaque, or (5) sclerotic nodunot present. up, smaller surgical margins, and fewer lar plaque. Lesions are often The patient underwent numbers of surgeries Mohs micrographic mistaken clinically for a vasMMS under general anesthesurgery should be the treatment of cular malformation or tumor, sia. A series of 0.5-cm periphchoice. infantile fibromatosis/myofieral margins were drawn To our knowledge this article contains all bromatosis, fibrosarcoma, or surrounding the tumor to dereported cases of congenital fibrous hamartoma (Tables I termine the final margins dermatofibrosarcoma protuberans and and II). The misdiagnosis taken after she was microdescribes all clinical variants to assist the of congenital DFSP may comscopically clear (Fig 2, A). clinician in making this difficult plicate later definitive surgical The tumor was debulked diagnosis. treatment because of scarand one stage (1-cm margin) ring from prior inappropriate was necessary to achieve tuThis article contains up-to-date treatments or enlargement of mor-free margins. The epidemiology of congenital the original tumor. wound was closed via a comdermatofibrosarcoma protuberans. Diagnosis of congenital plex linear closure and DFSP is based on its charachealed well without compliteristic histology demonstrating intersecting fascicles cation (Fig 2, B and C ). There was no evidence of of spindled cells in a mucinous stroma arranged in a recurrence at 1 year and she will continue to be storiform pattern. The spindle cells lack significant monitored indefinitely. atypia and frequently fill the papillary and reticular dermis with extension into the subcutaneous fat. The spindle cells stain positive for antibodies to CD34 but DISCUSSION fail to stain with antibodies to CD31, S-100, muscleThe estimated incidence of DFSP is 0.8 cases per specific actin, or factor XIIIa. Fibrosarcomatous million. It most commonly occurs between the ages change in DFSP may result in misdiagnosis as a of 20 to 50 years and children younger than 16 years fibrosarcoma, especially in partially sampled lesions. represent only 6% of all cases.22,26 Congenital cases Long fascicles of spindled cells with increased mitotic are even less common and many of the reported activity and often a herringbone pattern characterize cases lack supportive documentation at the time of these areas. Focal necrosis, uncommon in the storibirth. However, the frequency of congenital and form components of DFSP, may be present in these childhood cases may be underestimated as a result of fibrosarcomatous areas. Furthermore, in 50% of cases subtle initial clinical findings. The delay in diagnosis with fibrosarcomatous regions, the fibrosarcomatous of congenital DFSP is striking with the average age of regions do not stain with antibodies against CD34, diagnosis being 14 years. Furthermore, there is a whereas the storiform areas retain this staining pat5-year average delay after the initial presentation to a tern.41 DFSP with fibrosarcomatous change may have clinician and the final diagnosis (Tables I and II). a higher incidence of metastasis although it is unclear The cause of congenital DFSP is unknown. what percent of the tumor must display this change to Trauma has been associated with up to 20% of confer an increased metastatic potential. A true childhood and adult DFSP.1 Trauma was also fibrosarcoma would not stain with antibodies against reported to be associated with enlargement or d

d

d

Age, sex 2

Location

Price et al

6 mo/F

Leg

Borrie8

52 y/M

Shoulder

Topar et al9

26 y/F

Abdomen

Lee et al11

9 mo/F

Buttock

Muniesa et al12

10 y/M

Leg

*Stebut and Bra¨uninger13

3 y/F

*Maire et al14

6 mo/F

Upper aspect of back

Clinical appearance

3- 3 3-cm Purple mass, grew to 5 3 5 cm 10- 3 8-cm Pink firm nodular plaque 5- 3 5-cm Tender immobile red-brown mass with 3 surrounding brown plaques 3- 3 4-cm Red-violaceous plaque 9- 3 7.5-cm Fixed indurated plaque with peripheral atrophy and two 1-cm brown-yellow central nodules 0.5-cm Firm livid nodule

2 y/F

Lower aspect of back Lower aspect of back Scalp

10- 3 6-cm Erythematous infiltrating plaque 4-cm Hypopigmented plaque 2- 3 6-cm Pink fibrous plaque

10 y/F 3 y/F

Foot Thigh

0.5-cm Small blue papule 10-cm Angiomatous plaque

3 y/F 11 y/M

Follow-up

Hemangioma

Imatinib

Unclear

Hemangioma

1.3-cm WLE

Not available

Nevoid lesion

3-cm WLE with fascia

3 y Clear

None stated

WLE, margins unknown, with FTSG WLE, margins unknown

6 mo Clear

None stated

Lymphocytoma

Not available

Not available

WLE, margins unknown; recurred age 28 y, re-excised, diagnosed histiocytoma; recurred age 33 y, diagnosed DFSP, re-excised with 2-cm margin WLE, margins unknown

7 mo Clear

WLE, margins unknown

Not available

DFSP

WLE, margins unknown

Not available

Congenital aplasia cutis vs fibrous hamartoma vs infantile fibromatosis Difficult to characterize Angioma

WLE age 7 y; several incomplete excisions

Not available

WLE, margins unknown None (parents refused)

Not available Not available

Tufted hemangioma vs infantile myofibromatosis Difficult to characterize

DECEMBER 2009

8-cm Angiomatous firm plaque

Course

J AM ACAD DERMATOL

Buttock

Initial diagnosis

1016 Love et al

Table I. Non-Mohs micrographic surgeryetreated congenital dermatofibrosarcoma

4- 3 1-cm Erythematous patch with rapid evolution into fibromatous nodular plaque

2 y/M

Thorax

*Reddy et al15

10 mo/M 5 y/M

Sacral Back

4.5- 3 2-cm Blue-violet subcutaneous nodule 8-cm Plaque 1.5-cm Nodule

Fidalgo et al16

5 y/M

Forearm

Gu et al17

29 y/M

Pubic

*Weinstein et al19

2 mo/M

Scalp

3 y/F

Ankle

6 y/M

Back

1- 3 2-cm Red-blue plaque 1- 3 1.5-cm Atrophic plaque with black nodular center

1.5 y/F

Neck

Two small red nodules

Ill-defined firm red-brown nodular plaque 5-cm Firm blue-black nodular plaque with ulcer 0.8-cm Flesh-colored subcutaneous nodule with surrounding hair

Fibromatosis vs xanthomatous hamartoma vs congenital cutaneous aplasia vs infantile fibrosarcoma Fibrosarcoma vs angioma vs mastocytoma Sacrococcygeal teratoma Fibrous histiocytoma, infantile myofibromatosis

Congenital hemangioma Birthmark

Dermoid cyst vs glioma vs cephalocele vs hamartoma Myofibroma, vascular malformation Vascular malformation vs juvenile fibromatosis vs histiocytoma

Not available

1.2-cm WLE, with 3-cm re-excisiony Limited excision Excised age 5 y; recurred age 14 y, re-excised; recurred 4 mo, 2-cm WLE with STSG; recurred 4 y, 3-cm WLE 2-cm WLE

Not available 6 mo Clear 18 mo Clear

3 y Clear

WLE, margins unknown; repair with rectus flap

12 mo Clear

Incomplete excision; 3-cm WLE, tissue expansion, primary closure Incomplete excision; 1-cm WLE Original excision age 6 y, returned age 14 y and diagnosed as DFSP, WLE and graft, recurred 1 y at medial graft excised, recurred 18 y with further WLE and skin graft Excisional biopsy, recurred 5 mo later and treated with WLE; recurred age 3.5 y

12 mo Clear

Not available 6 y Clear

Not available

Continued

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Insect bites vs hemangioma

WLE, margins unknown; with re-excision 6 mo latery

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Trunk

VOLUME 61, NUMBER 6

15 y/F

Age, sex

Terrier-Lancombe et al20

Location

WLE, margins unknown

Not available

WLE, margins unknown

3 y Clear

2 cm 1.8- 3 1-cm purple patch progressed to blue-black plaque 4.5- 3 2.5-cm Soft skin-colored nodule 2- 3 3-cm Thin plaque

Unknown Hemangioma

WLE, margins unknown 2-cm WLE

Not available 9 mo Clear

Unknown

WLE, margins unknown

18 mo Clear

Hemangioma

3-cm WLE

2 y Clear

Small blue macule Nodular firm plaque 3- 3 4-cm Firm red-violet plaque

Hematoma vs trauma Morphea Unclear

3 y Clear Not available 12 mo Clear

6-cm Exophytic brown firm tumor Unknown Unknown 3- 3 4-cm Nodule

Verrucous pigmented nevus Unknown Unknown Congenital nevus

4-cm WLE with FTSG WLE, margins unknown Original excision unknown; recurred, re-excised with 3-cm margin WLE, margins unknown

9 mo Clear 2.5 y Clear Not available

Hemangioma

WLE, margins unknown WLE, margins unknown Two incomplete excisions; mastectomy WLE, margins unknown

Birthmark

Unknown

Not available

Unknown

WLE, margins unknown

Not available

Unknown Unknown Unknown Unknown

WLE, WLE, WLE, WLE,

12 mo Clear 1 mo Clear 3.1 y Clear 2.8 y Clear

6 mo/F

Thigh

0.7- 3 1.2-cm Atrophic hypopigmented plaque 3 cm

Thigh Back

Maeda et al23

1.5 y/F

Chest

Marcus et al24

7 mo/F

Martin et al25 *Annessi et al29

13 y/M 3 y/F 16 y/F

Lower aspect of back Leg Abdomen Abdomen

Gorczyca et al30

30 y/M

Trunk

McKee and Fletcher31 *Chapman et al33

6 y/M 5 y/M 25 y/F

Shoulder Back Breast

Schvarcz34

2 y/M

Hand Unknown

Taylor and Helwig36

4 patients; age/sex unknown 35 y/M 33 y/F 49 y/F 42 y/M

Unknown

Abdomen Abdomen Chest Thigh

Unknown Unknown Unknown Unknown

margins margins margins margins

unknown unknown unknown unknown

6 mo Clear

DECEMBER 2009

Unknown

Not available

J AM ACAD DERMATOL

Burkhardt et al35

Firm multinodular ulcerated tumor Red-brown nodular plaque Unknown

Pack and Tabah37

Follow-up

3 y Clear

1- 3 3-cm Red-black nodule

Buttock

Course

Incomplete excision; MMS at age unknown

Back

M

Initial diagnosis

Subcutaneous fat necrosis vs myofibroma vs tufted angioma vs fibrous hamartoma Aplasia cutis vs trauma vs hemangioma Unknown

7 wk/M

F 4 y/F

Checketts et al22

Clinical appearance

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Table I. Cont’d

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DFSP, Dermatofibrosarcoma protuberans; F, female; FTSG, full-thickness skin graft; M, male; MMS, Mohs micrographic surgery; NA, not available; STSG, split-thickness skin graft; WLE, wide local excision *Recurrent or incompletely excised tumor. y Re-excised after tumor was CD341. z Case series did not differentiate between cases treated by WLE and MMS; these cases were not included in data analysis for treatment or follow-up.

2-15 y Clear for series Unknownz Abdomen 3 mo/NA

3 mo/M

Lower extremity Lower extremity 14 y/F Jafarian et al39

Indurated plaque

Morphea, sarcoma, infantile fibromatosis, infantile myofibroma DFSP, myofibroma, rhabdomyosarcoma

Unknownz

2-15 y Clear for series 2-15 y Clear for series Unknownz DFSP

4 y Clear 12 y/F Gerlini et al38

Leg

Erythematous plaque; nodule formed after trauma Depressed popular and nodular plaque Atrophic indurated papule

Hemangioma

2-cm WLE with skin graft

VOLUME 61, NUMBER 6

CD34 and would not possess the translocation specific for DFSP. Diagnosis may be confirmed with the presence of a reciprocal translocation of chromosomes 17 and 22 generating a fusion between the gene encoding type 1 collagen and the gene for PDGFB. This translocation was reported positive in 69% of congenital DFSP tested (9 of 13).2,14,17,25 Imatinib mesylate (inhibitor of PDGFB tyrosine kinase) may be used if the expression of the collagen type 1a-1-PDGFB translocation is present.42,43 Imatinib has shown varying efficacy in the treatment of metastatic and unresectable DFSP in adult patients.44 Clinical improvement was also seen in an 18-month-old child treated with imatinib for unresectable disease.2 The role of imatinib in adult and congenital DFSP is not yet defined. We recommend its use for unresectable or metastatic disease, preferably in the setting of a clinical trial. Currently two phase II prospective trials evaluating imatinib use for DFSP are underway but children younger than 18 years are excluded.45 In preparation for treatment of congenital DFSP, imaging studies such as magnetic resonance imaging and ultrasound are paramount for proper surgical planning. Tumor margins can be grossly delineated to determine subclinical extent. Imaging may also be useful in planning wound closure, particularly if underlying structures are involved.18 The treatment of choice for adult DFSP is MMS. Not only does it offer the lowest recurrence rates, but it also allows for smaller defect sizes and thus less complicated repairs with an improved cosmetic result.4,46 WLE of congenital DFSP is still recommended in recent literature as the treatment of choice.9,15 Only 11 (18%) of the 61 reported cases of congenital DFSP reported using MMS as the primary treatment. MMS for congenital DFSP was first reported by Weber et al32 in 1988 on an adult woman. It was first reported on a child with a congenital DFSP in 1994,28 approximately 16 years after MMS was first described for DFSP.47,48 Advantages of MMS over WLE include higher clearance rates with longer follow-up, smaller excised margins, and fewer surgeries. The overall reported clearance rate for congenital DFSP treated with MMS is 100% with an average follow-up of 4.3 years, ranging from 9 months to 13.5 years (Table II). The overall reported clearance rate for congenital DFSP treated with WLE is 89% with an average reported follow-up of 1.9 years and ranging from 1 month to 6 years (Table I). There have been no reports of metastatic congenital DFSP, but local recurrence is thought to be a major cause in the increased metastatic potential of adult DFSP.26 Excised margins were smaller with MMS (1.7 vs 2.8

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Table II. Congenital dermatofibrosarcoma treated with Mohs micrographic surgery Age, sex

Location

Our patient

10 mo/F Back

Malerich et al10

6 wk/NA Scalp

Thornton et al18 15 y/F

Chest

3 y/M

Chest

16 y/F

Abdomen

13 y/F

Shoulder

2 y/M

Back

Marini et al21 Garcia et al26

Weber et al32

Feramisco et al40

Clinical appearance

3- 3 3.2-cm Erythematous tumor 6-cm Soft violaceous mass 2- 3 2.5-cm Violaceous indurated nodular plaque 0.8- 3 0.9-cm Violaceous plaque 4- 3 5-cm Firm erythematous plaque 1- 3 2-cm Indurated plaque

4- 3 4.5-cm Violaceous patch with central bullae 16 y/F Leg 6- 3 8-cm Atrophic plaque 13 y/F Breast 3- 3 4-cm Hyperpigmented nodular plaque 69 y/F Lower aspect 11- 3 14-cm Brown of back indurated nodular plaque 7 mo/M Inguinal 1.6- 3 0.7-cm Erythematous depressed linear plaque

Initial diagnosis

Course

Follow-up

DFSP

1-cm Margin MMS, 1 stage

1 y Clear

None stated

MMS, 3 stages, involved periosteum, margins unknown; bilateral rotation 2.5-cm Margin MMS, 1 stage; primary closure

3 y Clear*

Birthmark

Birthmark

1.5-cm Margin MMS, 1 stage; advancement flap Birthmark 2-cm Margin MMS, 2 stages; advancement flap Granuloma 2-cm Margin annulare MMS, 1 stage; rotation flap and FTSG Hemangioma 2-cm Margin MMS, 2 stages; advancement flap Congenital MMS, margin and fibroma closure unknown None stated MMS, 4 stages, margin unknown None stated

DFSP

MMS, 3 stages, final defect 13 3 20 cm; secondary intent 1-cm Margin MMS, 1 stage; advancement flap

13.5 y Cleary

5.9 y Cleary

4.8 y Cleary

4.5 y Cleary

4.3 y Cleary

9 mo Clear NA (4.4 y average for series) NA

11 mo Clear

DFSP, Dermatofibrosarcoma protuberans; F, female; FTSG, full-thickness skin graft; M, male; MMS, Mohs micrographic surgery; NA, not available. *Follow-up obtained through written communication with Victor Marks, MD, of Geisinger Medical Center, Danville, PA, December 9, 2008. y Follow-up obtained through written communication with Allison Vidimos, RPh, MD, of the Cleveland Clinic, Cleveland, OH, February 2, 2009.

cm with WLE) with an average of two stages required to produce tumor-free margins. The smaller margins required by MMS produce a postoperative wound that may require less complicated closure and allow for better cosmesis and function. This holds true for our patient, who would have required a skin flap or graft to close the defect had it been excised with 3-cm margins. Furthermore, 50% of cases treated with WLE that provided follow-up required multiple surgeries. MMS allows for one surgical session with tumor-free margins before initial reconstruction. A disadvantage to performing MMS on congenital DFSP is the possibility for extended operating times as

a result of tissue processing and multiple stages. Tissue processing of large tumors can be expedited by using multiple cryostats to section tissue. In our case two cryostats were used simultaneously and tissue processing was completed in 30 minutes. Another disadvantage to performing MMS for congenital DFSP is the requirement for appropriate facilities and coordination among many specialties. The Mohs micrographic surgeon must have access to an operating room and sufficient time must be planned to perform the procedure. Lastly a Mohs micrographic surgeon must be available to perform the procedure. Many hospitals do not have a Mohs micrographic

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Fig 1. A and B, Congenital dermatofibrosarcoma protuberans. C, Biopsy specimen showing spindle cells within myxoid stroma extended into subcutaneous fat. D, Spindle cells diffusely highlighted by CD34 (C and D, Hematoxylin-eosin stain; original magnifications: 34.)

surgeon on staff. Even in the academic setting Mohs micrographic surgeons may not have privileges at the free-standing children’s hospital because MMS is rarely performed on children. In this situation consideration should be given to traveling to an area where MMS is available for congenital DFSP.

CONCLUSION Congenital DFSP is a rare, locally aggressive tumor with a variable clinical presentation. Prompt diagnosis and treatment are essential to maximize functional and aesthetic outcomes. Radiographic imaging is beneficial in the workup and surgical planning of these tumors. Treatment with MMS offers higher clearance rates with longer follow-up, smaller margins, and fewer surgeries. It is therefore recommended that MMS be used as the treatment of choice for congenital DFSP when appropriate resources are available. The authors would like to thank Drs Victor Marks and Allison Vidimos for providing long-term patient follow-up for this review.

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Fig 2. Operative defect after initial tumor debulking with 0.5-cm margin (A) and after first Mohs layer (0.5 cm) (B). C, Postoperative defect repaired by complex linear closure.

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