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Summary

Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with high propensity for local recurrence. We present a case of a 53-year-old woman with a recurrent DFSP of the clavicular region. The patient underwent extensive local excision including the external cortical bone of the left clavicle. The resulting large defect was reconstructed using pedicled Latissimus Dorsi myocutaneous flap. The histopathological examination confirmed dermatofibrosarcoma protuberans with clear surgical margins and the patient was satisfied with the final aesthetic result.

INTRODUCTION

The dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor that involves the dermis, with a strong tendency to infiltrate surrounding tissues. It is characterized by high propensity for local recurrence but low tendency to metastatic dissemination, being therefore recognized as an intermediate to low degree of malignancy tumor 1. The typical clinical presentation is an irregular multiple mass or else a hard-indurated plaque affecting the skin. It is mostly seen on the trunk and extremities, but it may occur on the head and neck region also, such as the scalp, cheek, supra-clavicular region and the orbit 2,3. The incidence is quite similar in women and men (4.4 vs 4.2% per million people per year) with a relative 5-year survival of 99.2% 4. The most affected age group of DFSP is from 20 to 50 years 5. The exact cause of the onset is not yet clearly understood. Studies have involved a chromosomal translocation, resulting in the fusion protein COL1A1-PDGFB, which promotes tumor growth through the overproduction of platelet-derived growth factor (PDGF). Confirmed diagnosis is obtained through skin biopsy 6. Immunohistochemical analysis should be always utilized to support the diagnosis. Staining for Vimentin and CD34 is commonly employed, and sensitivity has been reported between 84 and 100 percent 7. The gold standard treatment is represented by surgery, since wide excision with at least 2 cm of safety margin should always be performed. The primary surgical goal is clean, tumor-free resection margins 8. Unresectable DFSPs should be treated with radiation therapy and/or targeted therapy. The aim of this paper is to present a case report of a severe DFSP recurrence of the left supraclavicular region, treated with a wide and deep excision followed by reconstruction with a pedicled Latissimus Dorsi (LD) myocutaneous flap. This type of reconstruction allowed us to repair such a large loss of substance with nerve structures and large vessels exposition, and to fill the subcutaneous volume deficiency with the myo-adipose portion of the flap, that could also be enhanced with autologous fat tissue transfer 9.

CASE REPORT

We present a case of a 53-year-old female patient with a recurrent DFSP of the left supraclavicular region. The patient had already undergone several resection procedures of the tumor mass, which had recurred several times. At the time of the visit, the patient showed the result of the last surgery with a further 5.5 x 5.2 cm tumor excision and partial thickness skin graft reconstruction (Fig. 1). The histological examination showed a diagnosis of cicatricial fibrosis with chronic giant cell inflammation with hypercellular areas with morphological and immunophenotypic aspects (CD34+). The report was compatible with extended Dermatofibrosarcoma Protuberans, present at the deep margin of resection. The patient was scheduled for radicalization of the old excision and reconstruction using the pedicled LD myocutaneous flap. A 15 x 9 cm skin paddle over the LD muscle was drawn very low on the back (about 8 cm below the tip of the scapula) in order to ensure a proper transposition of the flap and to allow a tension-free reconstruction (Fig. 2). The patient was positioned on right lateral decubitus and an en-bloc excision was performed, including the muscular component, i.e. part of the clavicular portion of the pectoralis major muscle and clavicular fibers of the deltoid muscle, and the external clavicular cortical bone. We reached macroscopically disease-free margins, carefully identifying and preserving the external jugular vein and the branches of the brachial plexus (Fig. 3). Simultaneously, the LD flap was harvested from the back, basing it on pre-operative drawing. Once the skin paddle was isolated on the LD, the whole muscle was dissected from its iliac insertion to its tendinous origin at the humerus, which has been resected in order to ensure a tension-free transposition. We left the thoracolumbar fascia on the back intact to avoid a potential lumbar hernia 10. The neurovascular pedicle was identified from below and the nerve was not divided. The flap was then rotated through the axilla to the supraclavicular region and the donor area was closed, leaving one suction drain per surgical site. No postoperative complications occurred. Histopathological studies on the surgical specimen identified multiple foci of spindle cell neoplasia with morphological and immunophenotypic characters (CD34+) compatible with the diagnosis already known in the history of the patient of DFSP involving the skin, the subcutaneous tissue and the clavicular fibers of pectoralis major and deltoid muscles. The whole mass with overlying skin was excised with adequate safety margins.

DISCUSSION

The DFSP is a rare mesenchymal tumor that accounts for 1% of soft tissue malignancies and 0, 1% of all other malignancies 11. It has a strong local invasion and high recurrence rate (10-60%), due to the highly irregular shape and finger protrusions of the tumor. However, it rarely causes regional or distant metastases 12,13. Although the etiology is unclear, some authors reported that it may develop from trauma, burn or surgery scar tissues 14. Microscopically, it extends far beyond the assessed clinical margins, spreading in the dermis and subcutaneous tissue 15. The high incidence of misdiagnosis of this cancer highlights the importance for pathological examination of early skin lesions to clarify any doubt 16,17. The most common misdiagnoses are sebaceous cysts, keloid, scar, lipoma, dermatofibroma, morphea, neurofibroma, basal cell carcinoma, desmoid tumor, Kaposi sarcoma, nodular fasciitis, and sarcoidosis. Histopathological examination with immunohistochemistry remains the gold standard to confirm an accurate diagnosis 18,19. Treatment options include complete surgical excision, Mohs micrographic surgery, radiation, and imatinib mesylate 20-22. Standard therapeutic approach is wide and deep tumor excision, achieving negative resection margins and simultaneously preserving the uninvolved tissue from resection. Series on outcomes of DFSP, demonstrated that wide local excision with adequate reconstruction can guarantee disease control in nearly 90% of the cases 23. Other authors suggest the use of Mohs micrographic surgery 24. Anyhow, reaching negative surgical margins is the primary endpoint of treatment 25. There is not a consensus on the extension of safe surgical margins to obtain local control. The recurrence rate after surgical treatment with 2-3 cm surgical margins is 0-30%. When 5 cm of free surgical margins are obtained, recurrence rate is reported as 0-5% 26,27. However, is strongly recommended to remove the tumor with the skin, subcutaneous tissue and the muscle fascia all together 28. Since primary closure is not always practicable, reconstructive surgery may be required using either skin graft, local flaps or free flaps.

In our clinical case, skin, subcutaneous tissue, muscle and external cortical bone of the left clavicle were resected en-bloc. Negative surgical margins were obtained in the histopathological examinations and tissue defect was reconstructed with a LD myocutaneous flap, using the skin paddle over the muscle to replace the loss of skin substance. An MRI performed 6 months after surgery showed no signs of recurrence and the patient was satisfied with the final aesthetic result (Figs. 4-6).

Similar cases are described in literature, using a wide range of reconstructions. Mishra GS et al. reported the case of a 30-year-old Hindu woman with recurrent DFSP of the clavicular region treated with complete excision and reconstruction with Pectoralis Major Myocutaneous flap 29. The Pectoralis Major myocutaneous flap is a good reconstructive option, but the strong limitation is represented by the impossibility of guaranteeing a skin paddle as large as that of the LD flap. Moreover, the anterior scar result in a worse cosmetic result, particularly in women. LD myocutaneous flap is a valid option since it permits the harvesting of a large muscle flap with a reliable skin paddle 30-32. It allows us to repair wide defects with exposure of nerve structures and vessels also, obtaining the replacement of the skin envelope and volume 33. The use of radiotherapy in the treatment of DFSP has been investigated in many studies 34. It is particularly encouraged if resection is inadequate but there is limited objective data to support its routine use currently 35,36. However, successful application and recommendations have been reported in very few small series 37. Imatinib mesylate was designed to treat Philadelphia chromosome positive leukemia (chronic myelogenous leukemia). The application of imatinib for DFSP has been limited, in cases where surgery is not favorable or in locally advanced cases the use of systemic tyrosine kinas receptor blockers (imatinib mesilat) has hopeful results 23, but its precise role in DFSP is currently under investigation in several clinical trials 38.

CONCLUSIONS

In making the differential diagnosis of the benign lesions of the skin, DFSP must be considered. This awareness will minimize the delay in the diagnosis process and will promote an adequate surgical approach from the outset, avoiding patient’s incomplete resection surgery and subsequent re-operations. In our case, the use of pedicled LD myocutaneous flap allowed us to restore a wide loss of substance and to cover noble structures such as jugular vessels and cervical nerves at the same time, with a satisfactory aesthetic result.

ACKNOWLEDGEMENTS

None.

CONFLICT OF INTEREST STATEMENT

The Authors declare no conflict of interest.

FUNDING

We, hereby certify, that to the best of our knowledge no financial support or benefits have been received by author or any co-author, by any member of our immediate family or any individual or entity with whom or with which we have a significant relationship from any commercial source which is related directly or indirectly to the scientific work which is reported on in the article. None of the Authors has a financial interest in any of the products, devices, or drugs mentioned in this manuscript.

AUTHORS’ CONTRIBUTIONS

Benedetto Longo: A, W

Gennaro D'orsi: D, W

Giada Orlando: D, W

Martina Giacalone: D, DT

Valerio Cervelli: A, W

ETHICAL CONSIDERATION

The research was conducted ethically, with all study procedures being performed in accordance with the requirements of the World Medical Association’s Declaration of Helsinki.

Written informed consent was obtained from each participant/patient for study participation and data publication.

Figures and tables

Figure 1. Preo-perative photograph in which the previous partial thickness skin graft is appreciated.

Figure 2. Pre-operative markings of the LD flap. It can be seen that the skin paddle was drawn very low to allow a tension-free transposition up to the defect in the clavicular area.

Figure 3. Intra-operative detail after wide excision including the external clavicular cortical bone, jugular vessels and cervical nerves.

Figure 4. Post-operative result 9 months after surgical procedure: frontal view.

Figure 5. Post-operative result 9 months after surgical procedure: oblique view.

Figure 6. Post-operative result 9 months after surgical procedure: lateral view.

References

  1. Allen A, Ahn C, Sangüeza O. Dermatofibrosarcoma protuberans. Dermatol Clin. 2019;37:483-488. doi:https://doi.org/10.1016/j.det.2019.05.006
  2. Harati K, Lange K, Goertz O. A single-institutional review of 68 patients with dermatofibrosarcoma protuberans: wide re-excision after inadequate previous surgery results in a high rate of local control. World J Surg Oncol. 2017;15. doi:https://doi.org/10.1186/s12957-016-1075-2
  3. Llombart B, Serra-Guillén C, Monteagudo C. Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 2013;30:13-28. doi:https://doi.org/10.1053/j.semdp.2012.01.002
  4. Criscione V, Weinstock M. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol. 2007;56:968-973. doi:https://doi.org/10.1016/j.jaad.2006.09.006
  5. Amjadzadeh M, Mousavi Ghanavati P. Patient with recurrence dermatofibrosarcoma protuberans: a case report. Clin Case Rep. 2020;8:1192-1194. doi:https://doi.org/10.1002/ccr3.2868
  6. Brooks J, Ramsey ML. Dermatofibrosarcoma protuberans.StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing 2022. 2022
  7. Haycox C, Odland P, Olbricht S. Immunohistochemical characterization of dermatofibrosarcoma protuberans with practical applications for diagnosis and treatment. J Am Acad Dermatol. 1997;37:438-444. doi:https://doi.org/10.1016/s0190-9622(97)70146-4
  8. Farma J, Ammori J, Zager J. Dermatofibrosarcoma protuberans: how wide should we resect?. Ann Surg Oncol. 2010;17:2112-2118. doi:https://doi.org/10.1245/s10434-010-1046-8
  9. Santanelli di Pompeo F, Laporta R, Sorotos M. Latissimus dorsi flap for total autologous immediate breast reconstruction without implants. Plast Reconstr Surg. 2014;134:871e-879e. doi:https://doi.org/10.1097/PRS.0000000000000859
  10. Munhoz A, Montag E, Arruda E. Management of giant inferior triangle lumbar hernia (Petit’s triangle hernia): a rare complication following delayed breast reconstruction with extended latissimus dorsi myocutaneous flap. Int J Surg Case Rep. 2014;5:319-323. doi:https://doi.org/10.1016/j.ijscr.2014.03.026
  11. Stamatakos M, Fyllos A, Siafogianni A. Dermatofibrosarcoma protuberans: a rare entity and review of the literature. J BUON. 2014;19:34-41.
  12. Bhambri S, Desai A, Del Rosso J. Dermatofibrosarcoma protuberans: a case report and review of the literature. J Clin Aesthet Dermatol. 2008;1:34-36.
  13. Gloster H. Dermatofibrosarcoma protuberans [published correction appears in J Am Acad Dermatol 1997;36:526. J Am Acad Dermatol. 1996;35:355-376. doi:https://doi.org/10.1016/s0190-9622(96)90597-6
  14. Felek S, İbas M, Dursun S. Dermatofibrosarcoma protuberans of the neck: a brief review of the literature. Indian J Otolaryngol Head Neck Surg. 2019;71:369-372. doi:https://doi.org/10.1007/s12070-018-1314-7
  15. Snow S, Gordon E, Larson P. Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer. 2004;101:28-38. doi:https://doi.org/10.1002/cncr.20316
  16. Hoesly P, Lowe G, Lohse C. Prognostic impact of fibrosarcomatous transformation in dermatofibrosarcoma protuberans: a cohort study. J Am Acad Dermatol. 2015;72:419-425. doi:https://doi.org/10.1016/j.jaad.2014.11.020
  17. Iorizzo L, Brown M. Atypical fibroxanthoma: a review of the literature. Dermatol Surg. 2011;37:146-157. doi:https://doi.org/10.1111/j.1524-4725.2010.01843.x
  18. Karanian M, Pérot G, Coindre J. Fluorescence in situ hybridization analysis is a helpful test for the diagnosis of dermatofibrosarcoma protuberans. Mod Pathol. 2015;28:230-237. doi:https://doi.org/10.1038/modpathol.2014.97
  19. Saiag P, Grob J, Lebbe C. Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline. Eur J Cancer. 2015;51:2604-2608. doi:https://doi.org/10.1016/j.ejca.2015.06.108
  20. Lemm D, Mügge L, Mentzel T. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol. 2009;135:653-665. doi:https://doi.org/10.1007/s00432-009-0550-3
  21. Stojadinovic A, Karpoff H, Antonescu C. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol. 2000;7:696-704. doi:https://doi.org/10.1007/s10434-000-0696-3
  22. DuBay D, Cimmino V, Lowe L. Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer. 2004;100:1008-1016. doi:https://doi.org/10.1002/cncr.20051
  23. Fiore M, Miceli R, Mussi C. Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol. 2005;23:7669-7675. doi:https://doi.org/10.1200/JCO.2005.02.5122
  24. Rutgers E, Kroon B, Albus-Lutter C. Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol. 1992;18:241-248.
  25. Dagan R, Morris C, Zlotecki R. Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol. 2005;28:537-539. doi:https://doi.org/10.1097/01.coc.0000171278.69291.64
  26. Chang C, Jacobs I, Salti G. Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol. 2004;30:341-345. doi:https://doi.org/10.1016/j.ejso.2003.12.005
  27. Han A, Chen E, Niedt G. Neoadjuvant imatinib therapy for dermatofibrosarcoma protuberans. Arch Dermatol. 2009;145:792-796. doi:https://doi.org/10.1001/archdermatol.2009.140
  28. Cakir B, Misirlioğlu A, Gideroğlu K. Giant fibrosarcoma arising in dermatofibrosarcoma protuberans on the scalp during pregnancy. Dermatol Surg. 2003;29:297-299. doi:https://doi.org/10.1046/j.1524-4725.2003.29066.x
  29. Mishra G, Bhatia A. Dermatofibrosarcoma protuberans: rare cause of head and neck swelling. Indian J Otolaryngol Head Neck Surg. 2007;59:296-297. doi:https://doi.org/10.1007/s12070-007-0086-2
  30. Barrow D, Nahai F, Fleischer A. Use of free Latissimus dorsi musculocutaneous flaps in various neurosurgical disorders. J Neurosurg. 1983;58:252-258. doi:https://doi.org/10.3171/jns.1983.58.2.0252
  31. Robson M, Zachary L, Schmidt D. Reconstruction of large cranial defects in the presence of heavy radiation damage and infection utilizing tissue transferred by microvascular anastomoses. Plast Reconstr Surg. 1989;83:438-442. doi:https://doi.org/10.1097/00006534-198903000-00004
  32. Pennington D, Stern H, Lee K. Free-flap reconstruction of large defects of the scalp and calvarium. Plast Reconstr Surg. 1989;83:655-661. doi:https://doi.org/10.1097/00006534-198904000-00010
  33. Longo B, Paolini G, Belli E. Wide excision and anterolateral thigh perforator flap reconstruction for dermatofibrosarcoma protuberans of the face. J Craniofac Surg. 2013;24:e597-e599. doi:https://doi.org/10.1097/SCS.0b013e3182a238c1
  34. Heuvel S, Suurmeijer A, Pras E. Dermatofibrosarcoma protuberans: recurrence is related to the adequacy of surgical margins. Eur J Surg Oncol. 2010;36:89-94. doi:https://doi.org/10.1016/j.ejso.2009.07.006
  35. Ballo M, Zagars G, Pisters P. The role of radiation therapy in the management of dermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys. 1998;40:823-827. doi:https://doi.org/10.1016/s0360-3016(97)00895-x
  36. O’Brien K, Seroussi E, Dal Cin P. Various regions within the alpha-helical domain of the COL1A1 gene are fused to the second exon of the PDGFB gene in dermatofibrosarcomas and giant-cell fibroblastomas. Genes Chromosomes Cancer. 1998;23:187-193.
  37. Dagan R, Morris C, Zlotecki R. Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol. 2005;28:537-539. doi:https://doi.org/10.1097/01.coc.0000171278.69291.64
  38. Han A, Chen E, Niedt G. Neoadjuvant imatinib therapy for dermatofibrosarcoma protuberans. Arch Dermatol. 2009;145:792-796. doi:https://doi.org/10.1001/archdermatol.2009.140

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Authors

Benedetto Longo - Chair of Plastic Surgery, Department of Surgical Sciences, School of Medicine and Surgery, Tor Vergata University of Rome, Italy

Gennaro D'orsi - Department of Surgical Sciences, School of Medicine and Surgery, PhD program in Medical-Surgical Applied Sciences, Tor Vergata University of Rome, Italy

Giada Orlando - Chair of Plastic Surgery, Department of Surgical Sciences, School of Medicine and Surgery, Tor Vergata University of Rome, Italy

Martina Giacalone - Chair of Plastic Surgery, Department of Surgical Sciences, School of Medicine and Surgery, Tor Vergata University of Rome, Italy

Valerio Cervelli - Chair of Plastic Surgery, Department of Surgical Sciences, School of Medicine and Surgery, Tor Vergata University of Rome, Italy

How to Cite
[1]
Longo, B., D’orsi, G., Orlando, G., Giacalone, M. and Cervelli, V. 2022. Recurrent dermatofibrosarcoma protuberans of the clavicular region: radical excision and reconstruction with Latissimus Dorsi myocutaneous flap. Plastic Reconstructive and Regenerative Surgery. 1, (Jul. 2022), 14–19. DOI:https://doi.org/10.57604/PRRS-002.
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