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Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction
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Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction Maria Eliza Nedu a,b, Ileana Rodica Matei a,b,∗, Alexandru Valentin Georgescu a,b a b
Department of Plastic Surgery, University of Medicine and Pharmacy “IuliuHatieganu”, Cluj-Napoca, Romania Department of Plastic Surgery Spitalul Clinic de Recuperare, Rehabilitation Clinical Hospital Cluj-Napoca, Str. Viilor 46-50, Cluj Napoca 400347, Romania
a r t i c l e
i n f o
Article history: Available online xxx Keywords: Dermatofibrosarcoma protuberans Skin defect Giant keystone flaps
a b s t r a c t Introduction: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare malignant tumor, with important local aggressiveness. Case report: We present a case of a 59-years-old man with a history of Psoriasis and viral hepatitis C presented in our department with a giant lesion on the posterior trunk, of 24/36 cm, with pus and local bleeding, causing important anemia. The tumor was excised, creating an elliptical defect of around 34 × 42 cm down to fascial level, which was covered by two lateral flaps – modified Keystone type III. Results: The flaps were completely viable and integrated with small dehiscence, which were assisted to heal secondarily. Conclusion: Our paper presents a reliable alternative to skin grafting for a very large defect on the posterior trunk, the modified type III keystone flap, to our knowledge, for the first time presented for such a wide defect and on adult. © 2019 Elsevier Ltd. All rights reserved.
Introduction Dermatofibrosarcoma protuberans (DFSP)is a relatively rare spindle cell tumor of the dermis, with unknown exact etiology, having high local aggressivity by his nodules, which can extend sometimes into the deep soft tissues [1–3]. The correct diagnosis is critical because ofthe malignant character and probability of recurrence, and can be difficult due to its rarity and histological variants [4–7]. Adding to this challenge the location of the defect on the posterior trunk, knowing the paucity of reliable axial pattern flap, reduced elasticity, and the length of recipient vessels in case of microsurgery, makes the overall procedure even more difficult [8]. In case of small or moderately large defects surgeons prefer using local flaps instead of skin grafts and use split skin grafts in large defects [9]. Whenever possible, skin flaps are preferable, especially local flaps, because they respect the “like with like” principle, because of the reduced donor site morbidity [10] and better aesthetic aspect of the regions’ contour. The Keystone island perforator flap allows the coverage of elliptical defects. This flap is based on musclecutaneous or fasciocuta-
∗ Corresponding author at: Department of Plastic Surgery Spitalul Clinic de Recuperare, Rehabilitation Clinical Hospital Cluj-Napoca, Str. Viilor 46-50, Cluj Napoca 400347, Romania E-mail address: [email protected] (I.R. Matei).
neous perforators, which accompanies nerves within dermatomal segments and straddles longitudinal (superficial veins, cutaneous nerves) [10]. We report the use of a modified type III Keystone flap for the coverage of a large defect on the posterior trunk following the removal of a giant plaque-like dermatofibrosarcoma. Case report A 59-years-old man with a history of psoriasis and viral hepatitis C presented to the department of plastic surgery with a lesion on the posterior trunk (Fig. 1). The lesion had an insidious evolution in the last 5 years, with no general symptoms, having however a slow increase in volume in parallel with the development of additional satellite lesions. Thus, 4 years ago a biopsy was performed establishing the diagnosis of dermatofibroma. The subsequent evolution led to a continuous volume increase and 6 months ago appeared necrosis and ulcers, with local infection and bleeding followed by pruritus. The increased lesion size led to impossibility to lie in dorsal decubitus. The patient presented in our service with a large sized tumoral mass (24/36 cm) (Fig. 1a) pruriginous, discharging pus and with local intermittent bleeding (Fig. 1b), associated also with generalized pallor, weight loss and important fatigability. The clinical exam showed a large pink plaque over the mid-lateral aspect of the posterior trunk, from the distal ½ of the thorax to the
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Please cite this article as: M.E. Nedu, I.R. Matei and A.V. Georgescu, Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction, Injury, https://doi.org/10.1016/j.injury.2019.10.041
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Fig. 1. Preoperative clinical appearance of the plaque on the posterior trunk. (a) general view. (b) aspect of one of the ulcerated and infected tumoral masses.
Fig. 3. CT images of the plaque. (a) transversal plane showing the entire height of the plaque. (b) sagittal plane.
Fig. 2. (a,b) CT images - the prefascial position of the nodular lesions (transversal sections, in the lumbar area).
proximal part of the inter-gluteal sulcus. Laterally, the tumor extended more on the right side. The contour was irregular and had on its’ surface multiple sessile nodules with different dimensions, seemingly coalescent. Between them there were several pedicled giant nodular lesions of different dimensions: 9/5/3 cm, 5/5/4 cm with infected ulcerations on the surface and satellite nodules (Fig. 1a and b). The entire tumor plaque formed by the nodules fixed in dermis was mobile to the underlying tissues. The regional lymph nodes were not clinically palpable. The preclinical investigations indicated a severe anemia: RBC 2.81 1012/L, HGB 6.6 g/dL, HCT 21.6%, MCH 23.5 pg, PLT 414, MONO% 13.1, INR 1.32, TQT 14.2, GOT 47 U/L, ferritin 13.9 mg/ml, sideremia 35 μg/dl, GGT 166 U/L, PCR 1.3 mg/dl. The fecal occult blood test was negative, allowing us to eliminate from discussion other bleeding sources, while no other symptoms or conditions were found to explain anemia besides the minor chronic bleeding from tumor. The AcHCV and AgHBS exams were negative. The CT exam revealed a homogeneous, well delimited aspect of the tumor, with pre-fascial location. Neither calcifications nor necrosis were observed (Figs. 2 and 3). On the CT exam, no reaction of lymphatic nodules was identified. Furthermore, no lung or hepatic metastasis were observed (Fig. 3). The symptomatic severe anemia imposed blood transfusion until HGB value was 10.8 gr/dL. The entire “plaque” was widely excised down to fascia level (Fig. 4a), leaving an elliptical defect in the median area of the posterior trunk of around 34 × 42 cm (Fig. 4b). The defect was covered using two lateral flaps – modified Keystone type III - based on musculocutaneous perforators from intercostal arteries, thoracodorsal arteries and lumbar arteries. The flaps dimensions were: one flap of 47 × 12 cm from the level of the right hemithorax and one flap of 49 × 16 cm from the left side (Fig. 5). The island flaps’ edges were undermined for 5–6 cm around to facilitate the ad-
Fig. 4. Intraoperative aspects. (a) premuscular excision of the tumor. (b) remaining defect after excision. (c) excision specimen. (d) design of one of the keystone flaps.
vancement to cover the defect. The middle of the flaps was undermined more, leaving only a 2–3 cm connection to the donor site, in order to facilitate the closure in this skinny patient. After the defect was covered, due to the fact that each flap covered partially the defect with 3/4 of its surface, the donor site was directly sutured. The red-dot sign was present. Active drainage was used. Immediately after finishing the suture on the right flap, in lumbar area a mild red-cyanotic area was observed. However, the evolution was favorable, with a gradual remission within 4 days, followed by the complete integration of the flaps, with normal capillary refill. The drainage was removed 12 days postoperatively and the stiches were partially removed starting with day 14 until day 23 postoperatively. The donor site healed perfectly with a smooth scar, while the flaps presented some minor zones of dehiscence on the scar between flaps in the most tensioned areas: inter-scapular 2/1 cm and below one of 1/1 cm, and in lumbar area two zones of 1.5/1 cm and 2.5/1.5 cm, where small amounts of serous drainage persisted. The dehiscence was assisted and healed spontaneously, without any complication, but with a further reactivation of the psoriasis over some of the scars (Fig. 5d and e). The histopathological exam showed that all excisional margins are free and using immunohistochemistry coloration the final diagnostic was established: dermatofibrosarcoma protuberans the myxoid type.
Please cite this article as: M.E. Nedu, I.R. Matei and A.V. Georgescu, Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction, Injury, https://doi.org/10.1016/j.injury.2019.10.041
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Fig. 5. Postoperative aspect. (a) First day postoperative aspect. (b) Postoperative aspect in the 23rd day: some minor zones of dehiscence on the scar between flaps in the most tensioned areas: inter-scapular 2/1 cm and below one of 1/1 cm, and in lumbar area two zones of 1.5/1 cm and 2.5/1.5 cm. (c) Postoperative aspect after 6 weeks: evident improvement of the small zones of dehiscence. (d,e,f) Postoperative aspect after 3 months: complete healing, but with the reactivation of the psoriasis over some of the scars.
Discussions DFSP is a rare tumor accounting for 2–6% of soft tissue sarcomas and less than 0.01% of all malignancies, with a incidence of 0.8–4.2 per million population [2,5,11–14]. The age distribution is variable between reports [2,3,5,6,11,13,14]. The trunk is the most frequent location of the tumor (40–60%) [5,15], followed by extremities [2]. The clinical appearance of DFSP presents as a red-brown to bluish nodular mass, located superficially, which grows slowly without symptoms. In late stages, ulceration can appear on its surface, like in the case we are presenting, with the nodules fixed on the dermis, but mobile to the deep tissues. The challenge of surgical removal is given by tumor projections in the periphery of the nodules. In addition, our case presented multiple satellite nodules and irregular pink plaque forming the protuberant appearance [11]. On palpation the nodules seem firm. The ulcerations can be infected [16]. They also tend to infiltrate adjacent structures, presenting a local aggressivity [13]. This is the reason why it is recommended to remove the deep fascia to eliminate vertical infiltrating cells [17]. It needs to be differentiated from fibrosarcomatous type, which presents more frequent recurrence after surgical excision of the tumor. Imagistic exams are important to evaluate the depth of the tumor spreading. The radiological display on CT appeared like a solid calcified isodense subcutaneous tumor [2,6,18]. The treatment is surgical excision, depending on the size of the tumor, with safety margins between 3–5 cm [2,17]. However, the patient needs close follow up because of the high rate of local recurrence, up to 20%−30%(1/3 of cases in 5 years) [3–5,19,20]. If surgery is not an option or because of the local invasion, radiotherapy or molecular targeted therapy with imatinibmesylate needs to be added. The radiotherapy has limited results [5,15].
3
DFSP is considered an intermediate malignant tumor, because of its 2–5% rate of metastasis, mostly in lung, more frequently seen after local recurrence [11,12]. However, myxoid type of DFSP rarely metastasizes, but can present fibrosarcomatous degeneration [5,17,21]. The method of reconstruction, following the excision, is influenced not only by the size of the defect, but also by the underlying exposed structures and patient’s characteristics [22]. Although split thickness skin graft seems to have almost the same results in donor site heeling compared with full-thickness grafts, because of the thickness of the dermis in posterior trunk skin, the latter is preferable [23]. However, the limited size of full thickness skin graft that can be harvested, compared to the size of our defect, makes it an unsuitable option. Split thickness skin graft imposes not only two surgical sites [24], but also the possibility of poor graft take, prolonged healing time and poor aesthetic outcome, in terms of color and long term texture/thickness of the dermis, and stability in time [9]. When we take in consideration the split thickness skin graft, we should think also at the bleeding and the amount of hemoglobin we need for healing, knowing the relatively anemic status of the patient. Also, thinking about the possible subsequent post-surgical radiotherapy, the stability of a skin graft is a less acceptable option. Based on all the above data and patient characteristics, the best option remained the use of local flaps, even though the use of local or regional flaps in such cases involves potential flap necrosis or wound dehiscence [9]. Among the options of local flaps, taking into consideration the defect’s size, the best ones are: a bilateral bipedicled fasciocutaneous flap or an island perforator Keystone flap [25]. The first option involves important subfascial dissection, compromising the perforators and possibly the viability of the flap, but is also an important source of bleeding and great tension on the wound edges [26]. We consider the second option as ideal when skin coverage is needed. The location and defect size is important, as some authors consider that keystone flap is not suitable for very large defects, because it can create wound tension [27]. However, this can be avoided by large elliptical excisions, where the larger the arc, the less the tension [10]. The typical Keystone flap has a 1:1 ratio for the width of the defect to the width of the flap while the length is imposed by the length of the defect [28]. The marginal flap undermining permits the advancement of the flap to cover the defect and its direct closure, with reported cases of direct donor site area closure for defects with sizes up to 8–10 cm, depending on the laxity of the back after flap harvest [9]. Another advantage for direct closure of the donor site without excessive tension is positioning the flap along the side of the excision margin which has greater tissue laxity [10]. In our case, we utilized a 3:4 ratio, which reduced the tension even for such a large defect and permitted direct closure of the donor site by suture. Based on the elliptical shape of the excision and the efficient marginal flap undermining, we managed to cover donor sites of 12 cm with direct suture. The best type of Keystone flap in this kind of defect is type III, which involves a double Keystone flap that can be used for larger defects and exploits the laxity of surrounding regions. This seems to be the best alternative to bipedicled fasciocutaneous flap, the tension being distributed all around the flap, not only in the middle part and also, allowing the direct donor site closure [4,29]. The red dot sign and hyperemic flare [28], present also in our flap, is considered to be the vascularization response to the depletion of catecholamine (denervation) or vessel dilatation confirmed by Doppler ultrasound at 14 days, of their perforating vessels [10]. Later on, histological investigations demonstrated the diameter increase for arterioles and venules in island flaps which support the idea of the increased vascularity in this type of flaps [30]. However, islanding contributes to decreased arteriole diameter and
Please cite this article as: M.E. Nedu, I.R. Matei and A.V. Georgescu, Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction, Injury, https://doi.org/10.1016/j.injury.2019.10.041
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presumably decreased inflow, potentially helping to ease congestion and improve flap survival [30]. The Keystone flap has the advantage of negligible postoperative pain due to intraoperative careful dissection of cutaneous branches of the surrounding nerves in the dermis. Another advantage is minimal postoperative edema, despite the island type of flap, explained, somehow, by the increased vascularity of the flap [10,30]. In a study, complete healing came with mild dysesthesia particularly in the large flaps, feature that was not present in our case [10]. Aesthetically and functionally, the principle of replacing “like with like” is respected, implying also shorter hospitalization time, faster recovery and social reinsertion [28]. The disadvantage of the Keystone flap is the temporary numbness of the flaps. Also, a complication found more frequently than the flap necrosis, which is very rare, is the wound dehiscence, reported in a cohort study to be around 18% [24]. This complication was seen also in our case. However this complication is mild considering that the posterior thoracic tissue is relatively thick, allowing it to adhere strongly to underlying layers and making it moderately inelastic, providing also flap integration and secondary healing of the dehiscence wounds [24]. Similar, in our case, immediately postoperatively, venous congestion was observed in the center and lower third of the right flap. On postoperative day 3, the venous congestion started to improve noticeably, and then completely disappeared [25]. The keystone flap has also the advantage that, because of the fast healing and integration of the flap, the patient can benefit from an early adjuvant post-surgical therapy, extremely valuable in malignant cases. DFSP bigger than 10 cm are rare; there were reported tumors of 18 × 13 cm (11) and 6 × 17 × 20 cm6 but even more then 25 cm [31]. To our knowledge, our case represents the biggest plaque of DFSP reported in literature so far. Furthermore, this is a case of myxoid DFSP, a rare type of DFSP. Also, as far as we know, the largest defect covered by a type III keystone flap was of 45/18 cm [32], and involved the use of a larger and a smaller flap, not two similar giant dimensions flaps, as in our case.
[5]
[6]
[7] [8] [9] [10] [11]
[12] [13]
[14] [15]
[16] [17]
[18] [19]
[20]
[21]
[22]
[23]
Conclusions In this paper we presented an alternative to skin grafting for a large defect on the posterior trunk, where the poor laxity of the skin is a drawback in the use of local or regional skin flaps. The modified type III keystone flap acted as a reliable local skin flap, to our knowledge, for the first time presented for such a wide defect and on adult. The histopathology exam of the removed plaque identified it as a myxoid dermatofibrosarcoma protuberans, in which case a follow-up of 5 years is required to observe any possible local recurrence.
[24]
[25]
[26] [27]
[28]
References [1] Belyaeva EA, Elenitsas R, Roth R, Miller C, Gelfand JM. Dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous changes in a patient with psoriasis treated with adalimumab. JAAD Case Reports 2015;1(5):272–3. [Internet]Available from: http://dx.doi.org/10.1016/j.jdcr.2015.06.007. [2] Al Barwani AS, Taif S, Al Mazrouai RA, Al Muzahmi KS, Alrawi A. Dermatofibrosarcoma protuberans: insights into a rare soft tissue tumor. J Clin Imaging Sci 2016;6:16. doi:10.4103/2156-7514.181492. [3] Torreggiani WC, Al-Ismail K, Munk PL, Nicolaou S, O’Connell JX, Knowling MA. Dermatofibrosarcoma protuberans: MR IM- aging features. AJR Am J Roentgenol 2002;178:989–93. [4] Zhang L, Liu Q, Cao Y, Zhong J, Zhang W. Dermatofibrosarcoma protuberans. Med (Baltimore) 2015;94(24):e1001 [Internet]Available from:
[29]
[30]
[31]
[32]
http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:landingpage&an= 0 0 0 05792- 201506030- 0 0 037. Sawke G, Sawke N, Gaur B, Thakur S. A large gradually increasing cutaneous swelling on lower back. J Cutan Aesthet Surg 2010;3(1):45–7. doi:10.4103/ 0974-2077.63300. Xing S, Wang J, Zhai W, Xia T, Wang C. Surgical treatment of enormous recurrent dermatofibrosarcoma protuberans. Thorac Cardiovasc Surg Rep 2015;4(1):2–4. doi:10.1055/s- 0034- 1387822. Al-naami A.Q., Khan L.A., Amin K., Al-naami A.M., Sun Z. Dermatofibrosarcoma protuberans — a rare neoplasm. 2016;6(2):229–30. Mathes DW, Thornton JF, Rohrich RJ. Management of posterior trunk defects. Plast Reconstr Surg. 2006;118:73e–83e. Azizeddin A, Choong PFM, Grinsell D. Reconstructive options for large back free flap donor sites. ANZ J Surg 2017. [Internet]Available fromhttp://doi.wiley.com/. Behan FC. The keystone design perforator island flap in reconstructive surgery. ANZ J Surg 2003;73(3):112–20. Yılmaz A, Çenesizoglu E, Eg˘ ilmez E, Önel S, Mus¸ tu M, Cennet A. Dermatofibrosarcoma protuberans: a case report of a rare, bulky tumor that was managed with surgical therapy. Int J Shoulder Surg 2009;3(1):16–20. doi:10.4103/ 0973-6042.48432. Aziz R, Jamil A. Dermatofibrosarcoma: a rare neoplasm of skin. BMJ Case Rep 2012:9–11. Paramythiotis D, Stavrou G, Panagiotou D, Petrakis G, Michalopoulos A. Dermatofibrosarcoma protuberans: a case report and review of the literature. Hippokratia 2016;20(1):80–3. Malkud S, Dyavannanavar V. Dermatofibrosarcoma protuberans. Ind Dermatol Online J 2017;8(6):495–7. doi:10.4103/idoj.IDOJ_424_16. Bhambri S, Desai A, Del Rosso JQ, Mobini N. Dermatofibrosar- coma protuberans: a case report and review of the literature. J Clin Aesthet Dermatol 2008;1:34–6. McGregor JK. Role of surgery in the management of dermatofibrosarcoma protuberans. Ann Surg 1961;154(2):255–8. Kim BJ, Kim H, Jin US, Minn KW, Chang H. Wide local excision for dermatofibrosarcoma protuberans: a single-center series of 90 patients. Biomed Res Int 2015;2015:642549 Epub 2015 Nov 25. doi:10.1155/2015/642549. Baker PA, O’Dowd GJ, Khan IU. Dermatofibrosarcoma protuberans arising in a decorative tattoo. Sarcoma 2005;9(1–2):37–41. Stivala A, Lombardo GAG, Pompili G, Tarico MS, Fraggetta F, Perrotta RE. Dermatofibrosarcoma protuberans: our experience of 59 cases. Oncol Lett 2012;4(5):1047–55. Larbcharoensub N, Kayankarnnavee J, Sanpaphant S, Kiranantawat K, Wirojtananugoon C, Sirikulchayanonta V. Clinicopathological features of dermatofibrosarcoma protuberans. Oncol Lett 2016;11(1):661–7. Gu W, Ogose A, Kawashima H, Umezu H, Kudo N, Hotta T, et al. Congenital dermatofibrosarcoma protuberans with fibrosarcomatous and myxoid change. J Clin Pathol 2005;58(9):984–6. Sinha S, Yip MJ, Gill S, Pohl MJ, Donahoe SR. A giant fungating metastatic basal cell carcinoma of the back and novel reconstruction using two large keystone design island perforator flaps. J Plast Reconstr Aesthetic Surg 2013;66(7):1015–18. Davis W, Wu C, Sieber D, Vandevender D. A comparison of full and split thickness skin grafts in radial forearm donor sites. J. Hand Microsurg. 2011;3:18–24. Taleb M, Choi L, Kim S. Safety and efficacy of the keystone and rhomboid flaps for immediate reconstruction after wide local excision of non-head and neck melanomas. World J Surg Oncol 2016;14(1):269. [Internet]Available from:http: //wjso.biomedcentral.com/articles/10.1186/s12957- 016- 1019- x. Jamjoom H, Alnoman H, Almadani Y. Closure of a large thoracolumbar myelomeningocele using a modified bilateral keystone flap. Plast Reconstr Surg - Glob Open 2016;4(12):e1114. [Internet]Available from:http://insights. ovid.com/crossref?an=01720096-201612000-00037. Jackson IT. The keystone design perforator island flap in reconstructive surgery. Anz J Surg 2003;73:261. Chen HC. Precautions in using keystone flap. J Plast Reconstr Aesthetic Surg 2010;63(4):720. [Internet]Available fromhttp://dx.doi.org/10.1016/j.bjps. 2009.02.049. Behan FC, Lo CH, Sizeland A. The interpretation of vascular changes observed in keystone island flaps: a hypothesis. J Plast Reconstr Aesthetic Surg 2010;63(2):e215–16. [Internet]Available from http://dx.doi.org/10.1016/j.bjps. 2009.02.063. Malone CH, Goodwin B, Wagner RF, Resto V, Kelly B. Nodular fasciitis complicating a staged surgical excision of dermatofibrosarcoma protuberans. Case Rep Dermatol Med 2016;2016:1–5. [Internet]Available from https://www.hindawi. com/journals/cridm/2016/6074182/. Lo CH, Nottle T, Mills J. Keystone island flap. Plast Reconstr Surg - Glob Open 2016;4(2):e617 [Internet]Available from: http://content.wkhealth.com/ linkback/openurl?sid=WKPTLP:landingpage&an=01720096-201602000-00013. Assassa GS, Siegel ME, Chen DC, Ansari AN. Dermatofibrosarcoma protuberans of the toe: findings on multiple imaging modalities. Clin Nucl Med 1993;18:978–80. Rao Aravind L, Janna Rakesh K. Keystone flap: versatile flap for reconstruction of limb defects. J Clin Diagn Res 2015;9(3):PC05–7.
Please cite this article as: M.E. Nedu, I.R. Matei and A.V. Georgescu, Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction, Injury, https://doi.org/10.1016/j.injury.2019.10.041
JID: JINJ
[m5G;October 31, 2019;21:12]
Injury xxx (xxxx) xxx
Contents lists available at ScienceDirect
Injury journal homepage: www.elsevier.com/locate/injury
Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction Maria Eliza Nedu a,b, Ileana Rodica Matei a,b,∗, Alexandru Valentin Georgescu a,b a b
Department of Plastic Surgery, University of Medicine and Pharmacy “IuliuHatieganu”, Cluj-Napoca, Romania Department of Plastic Surgery Spitalul Clinic de Recuperare, Rehabilitation Clinical Hospital Cluj-Napoca, Str. Viilor 46-50, Cluj Napoca 400347, Romania
a r t i c l e
i n f o
Article history: Available online xxx Keywords: Dermatofibrosarcoma protuberans Skin defect Giant keystone flaps
a b s t r a c t Introduction: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare malignant tumor, with important local aggressiveness. Case report: We present a case of a 59-years-old man with a history of Psoriasis and viral hepatitis C presented in our department with a giant lesion on the posterior trunk, of 24/36 cm, with pus and local bleeding, causing important anemia. The tumor was excised, creating an elliptical defect of around 34 × 42 cm down to fascial level, which was covered by two lateral flaps – modified Keystone type III. Results: The flaps were completely viable and integrated with small dehiscence, which were assisted to heal secondarily. Conclusion: Our paper presents a reliable alternative to skin grafting for a very large defect on the posterior trunk, the modified type III keystone flap, to our knowledge, for the first time presented for such a wide defect and on adult. © 2019 Elsevier Ltd. All rights reserved.
Introduction Dermatofibrosarcoma protuberans (DFSP)is a relatively rare spindle cell tumor of the dermis, with unknown exact etiology, having high local aggressivity by his nodules, which can extend sometimes into the deep soft tissues [1–3]. The correct diagnosis is critical because ofthe malignant character and probability of recurrence, and can be difficult due to its rarity and histological variants [4–7]. Adding to this challenge the location of the defect on the posterior trunk, knowing the paucity of reliable axial pattern flap, reduced elasticity, and the length of recipient vessels in case of microsurgery, makes the overall procedure even more difficult [8]. In case of small or moderately large defects surgeons prefer using local flaps instead of skin grafts and use split skin grafts in large defects [9]. Whenever possible, skin flaps are preferable, especially local flaps, because they respect the “like with like” principle, because of the reduced donor site morbidity [10] and better aesthetic aspect of the regions’ contour. The Keystone island perforator flap allows the coverage of elliptical defects. This flap is based on musclecutaneous or fasciocuta-
∗ Corresponding author at: Department of Plastic Surgery Spitalul Clinic de Recuperare, Rehabilitation Clinical Hospital Cluj-Napoca, Str. Viilor 46-50, Cluj Napoca 400347, Romania E-mail address: [email protected] (I.R. Matei).
neous perforators, which accompanies nerves within dermatomal segments and straddles longitudinal (superficial veins, cutaneous nerves) [10]. We report the use of a modified type III Keystone flap for the coverage of a large defect on the posterior trunk following the removal of a giant plaque-like dermatofibrosarcoma. Case report A 59-years-old man with a history of psoriasis and viral hepatitis C presented to the department of plastic surgery with a lesion on the posterior trunk (Fig. 1). The lesion had an insidious evolution in the last 5 years, with no general symptoms, having however a slow increase in volume in parallel with the development of additional satellite lesions. Thus, 4 years ago a biopsy was performed establishing the diagnosis of dermatofibroma. The subsequent evolution led to a continuous volume increase and 6 months ago appeared necrosis and ulcers, with local infection and bleeding followed by pruritus. The increased lesion size led to impossibility to lie in dorsal decubitus. The patient presented in our service with a large sized tumoral mass (24/36 cm) (Fig. 1a) pruriginous, discharging pus and with local intermittent bleeding (Fig. 1b), associated also with generalized pallor, weight loss and important fatigability. The clinical exam showed a large pink plaque over the mid-lateral aspect of the posterior trunk, from the distal ½ of the thorax to the
https://doi.org/10.1016/j.injury.2019.10.041 0020-1383/© 2019 Elsevier Ltd. All rights reserved.
Please cite this article as: M.E. Nedu, I.R. Matei and A.V. Georgescu, Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction, Injury, https://doi.org/10.1016/j.injury.2019.10.041
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Fig. 1. Preoperative clinical appearance of the plaque on the posterior trunk. (a) general view. (b) aspect of one of the ulcerated and infected tumoral masses.
Fig. 3. CT images of the plaque. (a) transversal plane showing the entire height of the plaque. (b) sagittal plane.
Fig. 2. (a,b) CT images - the prefascial position of the nodular lesions (transversal sections, in the lumbar area).
proximal part of the inter-gluteal sulcus. Laterally, the tumor extended more on the right side. The contour was irregular and had on its’ surface multiple sessile nodules with different dimensions, seemingly coalescent. Between them there were several pedicled giant nodular lesions of different dimensions: 9/5/3 cm, 5/5/4 cm with infected ulcerations on the surface and satellite nodules (Fig. 1a and b). The entire tumor plaque formed by the nodules fixed in dermis was mobile to the underlying tissues. The regional lymph nodes were not clinically palpable. The preclinical investigations indicated a severe anemia: RBC 2.81 1012/L, HGB 6.6 g/dL, HCT 21.6%, MCH 23.5 pg, PLT 414, MONO% 13.1, INR 1.32, TQT 14.2, GOT 47 U/L, ferritin 13.9 mg/ml, sideremia 35 μg/dl, GGT 166 U/L, PCR 1.3 mg/dl. The fecal occult blood test was negative, allowing us to eliminate from discussion other bleeding sources, while no other symptoms or conditions were found to explain anemia besides the minor chronic bleeding from tumor. The AcHCV and AgHBS exams were negative. The CT exam revealed a homogeneous, well delimited aspect of the tumor, with pre-fascial location. Neither calcifications nor necrosis were observed (Figs. 2 and 3). On the CT exam, no reaction of lymphatic nodules was identified. Furthermore, no lung or hepatic metastasis were observed (Fig. 3). The symptomatic severe anemia imposed blood transfusion until HGB value was 10.8 gr/dL. The entire “plaque” was widely excised down to fascia level (Fig. 4a), leaving an elliptical defect in the median area of the posterior trunk of around 34 × 42 cm (Fig. 4b). The defect was covered using two lateral flaps – modified Keystone type III - based on musculocutaneous perforators from intercostal arteries, thoracodorsal arteries and lumbar arteries. The flaps dimensions were: one flap of 47 × 12 cm from the level of the right hemithorax and one flap of 49 × 16 cm from the left side (Fig. 5). The island flaps’ edges were undermined for 5–6 cm around to facilitate the ad-
Fig. 4. Intraoperative aspects. (a) premuscular excision of the tumor. (b) remaining defect after excision. (c) excision specimen. (d) design of one of the keystone flaps.
vancement to cover the defect. The middle of the flaps was undermined more, leaving only a 2–3 cm connection to the donor site, in order to facilitate the closure in this skinny patient. After the defect was covered, due to the fact that each flap covered partially the defect with 3/4 of its surface, the donor site was directly sutured. The red-dot sign was present. Active drainage was used. Immediately after finishing the suture on the right flap, in lumbar area a mild red-cyanotic area was observed. However, the evolution was favorable, with a gradual remission within 4 days, followed by the complete integration of the flaps, with normal capillary refill. The drainage was removed 12 days postoperatively and the stiches were partially removed starting with day 14 until day 23 postoperatively. The donor site healed perfectly with a smooth scar, while the flaps presented some minor zones of dehiscence on the scar between flaps in the most tensioned areas: inter-scapular 2/1 cm and below one of 1/1 cm, and in lumbar area two zones of 1.5/1 cm and 2.5/1.5 cm, where small amounts of serous drainage persisted. The dehiscence was assisted and healed spontaneously, without any complication, but with a further reactivation of the psoriasis over some of the scars (Fig. 5d and e). The histopathological exam showed that all excisional margins are free and using immunohistochemistry coloration the final diagnostic was established: dermatofibrosarcoma protuberans the myxoid type.
Please cite this article as: M.E. Nedu, I.R. Matei and A.V. Georgescu, Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction, Injury, https://doi.org/10.1016/j.injury.2019.10.041
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Fig. 5. Postoperative aspect. (a) First day postoperative aspect. (b) Postoperative aspect in the 23rd day: some minor zones of dehiscence on the scar between flaps in the most tensioned areas: inter-scapular 2/1 cm and below one of 1/1 cm, and in lumbar area two zones of 1.5/1 cm and 2.5/1.5 cm. (c) Postoperative aspect after 6 weeks: evident improvement of the small zones of dehiscence. (d,e,f) Postoperative aspect after 3 months: complete healing, but with the reactivation of the psoriasis over some of the scars.
Discussions DFSP is a rare tumor accounting for 2–6% of soft tissue sarcomas and less than 0.01% of all malignancies, with a incidence of 0.8–4.2 per million population [2,5,11–14]. The age distribution is variable between reports [2,3,5,6,11,13,14]. The trunk is the most frequent location of the tumor (40–60%) [5,15], followed by extremities [2]. The clinical appearance of DFSP presents as a red-brown to bluish nodular mass, located superficially, which grows slowly without symptoms. In late stages, ulceration can appear on its surface, like in the case we are presenting, with the nodules fixed on the dermis, but mobile to the deep tissues. The challenge of surgical removal is given by tumor projections in the periphery of the nodules. In addition, our case presented multiple satellite nodules and irregular pink plaque forming the protuberant appearance [11]. On palpation the nodules seem firm. The ulcerations can be infected [16]. They also tend to infiltrate adjacent structures, presenting a local aggressivity [13]. This is the reason why it is recommended to remove the deep fascia to eliminate vertical infiltrating cells [17]. It needs to be differentiated from fibrosarcomatous type, which presents more frequent recurrence after surgical excision of the tumor. Imagistic exams are important to evaluate the depth of the tumor spreading. The radiological display on CT appeared like a solid calcified isodense subcutaneous tumor [2,6,18]. The treatment is surgical excision, depending on the size of the tumor, with safety margins between 3–5 cm [2,17]. However, the patient needs close follow up because of the high rate of local recurrence, up to 20%−30%(1/3 of cases in 5 years) [3–5,19,20]. If surgery is not an option or because of the local invasion, radiotherapy or molecular targeted therapy with imatinibmesylate needs to be added. The radiotherapy has limited results [5,15].
3
DFSP is considered an intermediate malignant tumor, because of its 2–5% rate of metastasis, mostly in lung, more frequently seen after local recurrence [11,12]. However, myxoid type of DFSP rarely metastasizes, but can present fibrosarcomatous degeneration [5,17,21]. The method of reconstruction, following the excision, is influenced not only by the size of the defect, but also by the underlying exposed structures and patient’s characteristics [22]. Although split thickness skin graft seems to have almost the same results in donor site heeling compared with full-thickness grafts, because of the thickness of the dermis in posterior trunk skin, the latter is preferable [23]. However, the limited size of full thickness skin graft that can be harvested, compared to the size of our defect, makes it an unsuitable option. Split thickness skin graft imposes not only two surgical sites [24], but also the possibility of poor graft take, prolonged healing time and poor aesthetic outcome, in terms of color and long term texture/thickness of the dermis, and stability in time [9]. When we take in consideration the split thickness skin graft, we should think also at the bleeding and the amount of hemoglobin we need for healing, knowing the relatively anemic status of the patient. Also, thinking about the possible subsequent post-surgical radiotherapy, the stability of a skin graft is a less acceptable option. Based on all the above data and patient characteristics, the best option remained the use of local flaps, even though the use of local or regional flaps in such cases involves potential flap necrosis or wound dehiscence [9]. Among the options of local flaps, taking into consideration the defect’s size, the best ones are: a bilateral bipedicled fasciocutaneous flap or an island perforator Keystone flap [25]. The first option involves important subfascial dissection, compromising the perforators and possibly the viability of the flap, but is also an important source of bleeding and great tension on the wound edges [26]. We consider the second option as ideal when skin coverage is needed. The location and defect size is important, as some authors consider that keystone flap is not suitable for very large defects, because it can create wound tension [27]. However, this can be avoided by large elliptical excisions, where the larger the arc, the less the tension [10]. The typical Keystone flap has a 1:1 ratio for the width of the defect to the width of the flap while the length is imposed by the length of the defect [28]. The marginal flap undermining permits the advancement of the flap to cover the defect and its direct closure, with reported cases of direct donor site area closure for defects with sizes up to 8–10 cm, depending on the laxity of the back after flap harvest [9]. Another advantage for direct closure of the donor site without excessive tension is positioning the flap along the side of the excision margin which has greater tissue laxity [10]. In our case, we utilized a 3:4 ratio, which reduced the tension even for such a large defect and permitted direct closure of the donor site by suture. Based on the elliptical shape of the excision and the efficient marginal flap undermining, we managed to cover donor sites of 12 cm with direct suture. The best type of Keystone flap in this kind of defect is type III, which involves a double Keystone flap that can be used for larger defects and exploits the laxity of surrounding regions. This seems to be the best alternative to bipedicled fasciocutaneous flap, the tension being distributed all around the flap, not only in the middle part and also, allowing the direct donor site closure [4,29]. The red dot sign and hyperemic flare [28], present also in our flap, is considered to be the vascularization response to the depletion of catecholamine (denervation) or vessel dilatation confirmed by Doppler ultrasound at 14 days, of their perforating vessels [10]. Later on, histological investigations demonstrated the diameter increase for arterioles and venules in island flaps which support the idea of the increased vascularity in this type of flaps [30]. However, islanding contributes to decreased arteriole diameter and
Please cite this article as: M.E. Nedu, I.R. Matei and A.V. Georgescu, Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction, Injury, https://doi.org/10.1016/j.injury.2019.10.041
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presumably decreased inflow, potentially helping to ease congestion and improve flap survival [30]. The Keystone flap has the advantage of negligible postoperative pain due to intraoperative careful dissection of cutaneous branches of the surrounding nerves in the dermis. Another advantage is minimal postoperative edema, despite the island type of flap, explained, somehow, by the increased vascularity of the flap [10,30]. In a study, complete healing came with mild dysesthesia particularly in the large flaps, feature that was not present in our case [10]. Aesthetically and functionally, the principle of replacing “like with like” is respected, implying also shorter hospitalization time, faster recovery and social reinsertion [28]. The disadvantage of the Keystone flap is the temporary numbness of the flaps. Also, a complication found more frequently than the flap necrosis, which is very rare, is the wound dehiscence, reported in a cohort study to be around 18% [24]. This complication was seen also in our case. However this complication is mild considering that the posterior thoracic tissue is relatively thick, allowing it to adhere strongly to underlying layers and making it moderately inelastic, providing also flap integration and secondary healing of the dehiscence wounds [24]. Similar, in our case, immediately postoperatively, venous congestion was observed in the center and lower third of the right flap. On postoperative day 3, the venous congestion started to improve noticeably, and then completely disappeared [25]. The keystone flap has also the advantage that, because of the fast healing and integration of the flap, the patient can benefit from an early adjuvant post-surgical therapy, extremely valuable in malignant cases. DFSP bigger than 10 cm are rare; there were reported tumors of 18 × 13 cm (11) and 6 × 17 × 20 cm6 but even more then 25 cm [31]. To our knowledge, our case represents the biggest plaque of DFSP reported in literature so far. Furthermore, this is a case of myxoid DFSP, a rare type of DFSP. Also, as far as we know, the largest defect covered by a type III keystone flap was of 45/18 cm [32], and involved the use of a larger and a smaller flap, not two similar giant dimensions flaps, as in our case.
[5]
[6]
[7] [8] [9] [10] [11]
[12] [13]
[14] [15]
[16] [17]
[18] [19]
[20]
[21]
[22]
[23]
Conclusions In this paper we presented an alternative to skin grafting for a large defect on the posterior trunk, where the poor laxity of the skin is a drawback in the use of local or regional skin flaps. The modified type III keystone flap acted as a reliable local skin flap, to our knowledge, for the first time presented for such a wide defect and on adult. The histopathology exam of the removed plaque identified it as a myxoid dermatofibrosarcoma protuberans, in which case a follow-up of 5 years is required to observe any possible local recurrence.
[24]
[25]
[26] [27]
[28]
References [1] Belyaeva EA, Elenitsas R, Roth R, Miller C, Gelfand JM. Dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous changes in a patient with psoriasis treated with adalimumab. JAAD Case Reports 2015;1(5):272–3. [Internet]Available from: http://dx.doi.org/10.1016/j.jdcr.2015.06.007. [2] Al Barwani AS, Taif S, Al Mazrouai RA, Al Muzahmi KS, Alrawi A. Dermatofibrosarcoma protuberans: insights into a rare soft tissue tumor. J Clin Imaging Sci 2016;6:16. doi:10.4103/2156-7514.181492. [3] Torreggiani WC, Al-Ismail K, Munk PL, Nicolaou S, O’Connell JX, Knowling MA. Dermatofibrosarcoma protuberans: MR IM- aging features. AJR Am J Roentgenol 2002;178:989–93. [4] Zhang L, Liu Q, Cao Y, Zhong J, Zhang W. Dermatofibrosarcoma protuberans. Med (Baltimore) 2015;94(24):e1001 [Internet]Available from:
[29]
[30]
[31]
[32]
http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:landingpage&an= 0 0 0 05792- 201506030- 0 0 037. Sawke G, Sawke N, Gaur B, Thakur S. A large gradually increasing cutaneous swelling on lower back. J Cutan Aesthet Surg 2010;3(1):45–7. doi:10.4103/ 0974-2077.63300. Xing S, Wang J, Zhai W, Xia T, Wang C. Surgical treatment of enormous recurrent dermatofibrosarcoma protuberans. Thorac Cardiovasc Surg Rep 2015;4(1):2–4. doi:10.1055/s- 0034- 1387822. Al-naami A.Q., Khan L.A., Amin K., Al-naami A.M., Sun Z. Dermatofibrosarcoma protuberans — a rare neoplasm. 2016;6(2):229–30. Mathes DW, Thornton JF, Rohrich RJ. Management of posterior trunk defects. Plast Reconstr Surg. 2006;118:73e–83e. Azizeddin A, Choong PFM, Grinsell D. Reconstructive options for large back free flap donor sites. ANZ J Surg 2017. [Internet]Available fromhttp://doi.wiley.com/. Behan FC. The keystone design perforator island flap in reconstructive surgery. ANZ J Surg 2003;73(3):112–20. Yılmaz A, Çenesizoglu E, Eg˘ ilmez E, Önel S, Mus¸ tu M, Cennet A. Dermatofibrosarcoma protuberans: a case report of a rare, bulky tumor that was managed with surgical therapy. Int J Shoulder Surg 2009;3(1):16–20. doi:10.4103/ 0973-6042.48432. Aziz R, Jamil A. Dermatofibrosarcoma: a rare neoplasm of skin. BMJ Case Rep 2012:9–11. Paramythiotis D, Stavrou G, Panagiotou D, Petrakis G, Michalopoulos A. Dermatofibrosarcoma protuberans: a case report and review of the literature. Hippokratia 2016;20(1):80–3. Malkud S, Dyavannanavar V. Dermatofibrosarcoma protuberans. Ind Dermatol Online J 2017;8(6):495–7. doi:10.4103/idoj.IDOJ_424_16. Bhambri S, Desai A, Del Rosso JQ, Mobini N. Dermatofibrosar- coma protuberans: a case report and review of the literature. J Clin Aesthet Dermatol 2008;1:34–6. McGregor JK. Role of surgery in the management of dermatofibrosarcoma protuberans. Ann Surg 1961;154(2):255–8. Kim BJ, Kim H, Jin US, Minn KW, Chang H. Wide local excision for dermatofibrosarcoma protuberans: a single-center series of 90 patients. Biomed Res Int 2015;2015:642549 Epub 2015 Nov 25. doi:10.1155/2015/642549. Baker PA, O’Dowd GJ, Khan IU. Dermatofibrosarcoma protuberans arising in a decorative tattoo. Sarcoma 2005;9(1–2):37–41. Stivala A, Lombardo GAG, Pompili G, Tarico MS, Fraggetta F, Perrotta RE. Dermatofibrosarcoma protuberans: our experience of 59 cases. Oncol Lett 2012;4(5):1047–55. Larbcharoensub N, Kayankarnnavee J, Sanpaphant S, Kiranantawat K, Wirojtananugoon C, Sirikulchayanonta V. Clinicopathological features of dermatofibrosarcoma protuberans. Oncol Lett 2016;11(1):661–7. Gu W, Ogose A, Kawashima H, Umezu H, Kudo N, Hotta T, et al. Congenital dermatofibrosarcoma protuberans with fibrosarcomatous and myxoid change. J Clin Pathol 2005;58(9):984–6. Sinha S, Yip MJ, Gill S, Pohl MJ, Donahoe SR. A giant fungating metastatic basal cell carcinoma of the back and novel reconstruction using two large keystone design island perforator flaps. J Plast Reconstr Aesthetic Surg 2013;66(7):1015–18. Davis W, Wu C, Sieber D, Vandevender D. A comparison of full and split thickness skin grafts in radial forearm donor sites. J. Hand Microsurg. 2011;3:18–24. Taleb M, Choi L, Kim S. Safety and efficacy of the keystone and rhomboid flaps for immediate reconstruction after wide local excision of non-head and neck melanomas. World J Surg Oncol 2016;14(1):269. [Internet]Available from:http: //wjso.biomedcentral.com/articles/10.1186/s12957- 016- 1019- x. Jamjoom H, Alnoman H, Almadani Y. Closure of a large thoracolumbar myelomeningocele using a modified bilateral keystone flap. Plast Reconstr Surg - Glob Open 2016;4(12):e1114. [Internet]Available from:http://insights. ovid.com/crossref?an=01720096-201612000-00037. Jackson IT. The keystone design perforator island flap in reconstructive surgery. Anz J Surg 2003;73:261. Chen HC. Precautions in using keystone flap. J Plast Reconstr Aesthetic Surg 2010;63(4):720. [Internet]Available fromhttp://dx.doi.org/10.1016/j.bjps. 2009.02.049. Behan FC, Lo CH, Sizeland A. The interpretation of vascular changes observed in keystone island flaps: a hypothesis. J Plast Reconstr Aesthetic Surg 2010;63(2):e215–16. [Internet]Available from http://dx.doi.org/10.1016/j.bjps. 2009.02.063. Malone CH, Goodwin B, Wagner RF, Resto V, Kelly B. Nodular fasciitis complicating a staged surgical excision of dermatofibrosarcoma protuberans. Case Rep Dermatol Med 2016;2016:1–5. [Internet]Available from https://www.hindawi. com/journals/cridm/2016/6074182/. Lo CH, Nottle T, Mills J. Keystone island flap. Plast Reconstr Surg - Glob Open 2016;4(2):e617 [Internet]Available from: http://content.wkhealth.com/ linkback/openurl?sid=WKPTLP:landingpage&an=01720096-201602000-00013. Assassa GS, Siegel ME, Chen DC, Ansari AN. Dermatofibrosarcoma protuberans of the toe: findings on multiple imaging modalities. Clin Nucl Med 1993;18:978–80. Rao Aravind L, Janna Rakesh K. Keystone flap: versatile flap for reconstruction of limb defects. J Clin Diagn Res 2015;9(3):PC05–7.
Please cite this article as: M.E. Nedu, I.R. Matei and A.V. Georgescu, Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction, Injury, https://doi.org/10.1016/j.injury.2019.10.041