Correspondence Clinical Letter
Clinical letter Squamous cell carcinoma arising in hidradenitis suppurativa
DOI: 10.1111/ddg.12279
Dear Editors, Chronic wounds and inflammatory processes persisting for years entail the risk of malignant degeneration. Thus, in chronic hidradenitis suppurativa with recurrent abscesses, fistulas and scarring, the development of squamous cell carcinomas—also known as Marjolin ulcer—is a serious complication. A 52-year-old man presented in our clinic with chronic inflammatory fistulas present since the age of 18, which previously had been treated by surgical colleagues with multiple incisions. Physical examination revealed the clinical picture of hidradenitis suppurativa Hurley stage III with widespread fibrotic scar tissue, draining fistulas, and nodules affecting the sacral and gluteal region (25 × 25 cm) (Figure 1a). Clinically there was no evidence of malignancy. Imaging diagnostics with MRI scan revealed inflammatory changes of the sacrum indicating a possible osteomyelitis. There was no evidence of gastrointestinal involvement. Surgical treatment was performed under general anesthesia and included complete en bloc resection of the affected hidradenitis suppurativa areas with ablation of the altered structures of the sacrum (Figure 1b). The histological examination showed a moderately differentiated squamous cell carcinoma in the center of the resected hidradenitis suppurativa tissue (Figure 1c). It remained uncertain whether the basal margins were tumor free. Thus, after discussing the patient’s case at our tumor board, a second resection was performed by colleagues of the Trauma and Reconstructive Surgery Department and the Department of GI, Thoracic and Vascular Surgery including ablation of the coccyx and further parts of the sacrum. Following vacuum assisted closure therapy (VAC therapy) plastic reconstruction was performed with a gluteus maximus V-Y flap (right side), a subcutaneous transposition flap (left side) and a subsequent mesh-graft transplantation of the remaining defect ( Figure 1d). Histological examination revealed residual tumor in the previous excision margins but tumor-free bony parts (R0 resection). The follow-up 6 months later showed iliac and inguinal lymph node metastases right and a presacral lymph node metastasis left. The iliac and inguinal lymph node metastases were surgically removed. The presacral node could
not be localized intra-operatively and was thus treated with radiotherapy with the field also including the lymphatic pathways. Malignant degeneration in chronic inflammatory processes, such as scars, is supposed to be promoted by free radicals released by inflammatory cells with subsequent DNA damage and neoplastic transformation of the epithelium [1]. The incidence of malignant transformation in hidradenitis suppurativa is estimated to 3.2 % [1, 2]. To date more than 70 cases of squamous cell carcinoma arising in gluteal and sacral hidradenitis suppurativa and pilonidal sinus are documented in the medical database. Men are affected more frequently. The average age at diagnosis is approximately 52 years, and in more than 75 % of the patients hidradenitis suppurativa has been present for more than 10 years [1–6]. The development of malignant tumors in the area of these inflammatory changes is a rare complication, and in the majority of the cases histological investigation reveals squamous cell carcinomas whereas basal cell carcinomas are rarely found [1]. The clinical picture includes vast exophytic tumors of more than 5 cm in diameter and ulcerations (Marjolin ulcer) [1]. Bone infiltration is found in approximately 8 % of the patients and local lymph node metastases may be present at the time of diagnosis [1]. Wide surgical en bloc excision of the affected tissue is the treatment of choice (hidradenitis suppurativa Hurley stage II and higher) which in some cases (bone involvement, i nvolvement of the perianal and rectal region) turns out to be very tricky. A complete surgical excision is recommended under protection of the surrounding healthy tissue. In special cases (for example in order to identify fistulas of the intestine [7, 8]) clinical examination should be completed by ultrasound diagnostics (subcutaneous and endosonography) and MRT scan. Fistula tracts can be detected with probes and staining during surgery [2, 7–10]. Very deep fistulas of the pararectal region may be treated with curettage [11, 12]. Depending on the size of the resulting defect, complete s econdary wound healing or plastic reconstructive wound closure with flaps is possible [1, 2, 7, 10]. Therapy and follow-up of squamous cell carcinoma should be performed in accordance with the specific guideline [13]. Relapses after surgical treatment alone are frequent (44–48 % independent from the level of differentiation) and most commonly occur 9 months post-surgery [1, 6], indicating a relatively high aggressiveness of the tumor (5-year survival rate 61 %). Postoperative radiation therapy may reduce the recurrence rate to 30 % [1]. In our patient we avoided radiation therapy fearing impaired wound healing due to the considerable size of the defect and the complex plastic reconstruction. The role of postoperative chemotherapy is uncertain and controversial but should be considered in case of
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
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Correspondence Clinical Letter
Figure 1 Clinical appearance prior to surgery (a), post-surgical appearance (b), histological specimen (HE; x200) (c), appearance 4 months post-surgery (d).
Correspondence to
inoperability and metastasis in accordance with the specific guideline [13]. In conclusion, patients suffering from chronic inflammatory processes in the perianal, perineal and sacral region should be treated with wide and radical surgical excision due to the risk of malignant degeneration and development of aggressive squamous cell carcinomas with poor outcome.
Dr. med. Solveig Herschel Department of Dermatology Medical Faculty Carl Gustav Carus Technical University Dresden Fetscherstraße 74 01307 Dresden, Germany E-mail: [email protected]
Solveig Herschel, Jörg Laske, Annette Stein Department of Dermatology, Medical Faculty Carl Gustav Carus, Technical University Dresden
Conflict of interest None.
418
References 1
2
de Bree E, Zoetmulder FAN, Christodoulakis M et al. Treatment of malignancy arising in pilonidal disease. Ann Surg Oncol 2001; 8(1): 60–4. Stein A, Sebastian G. Acne inversa. Hautarzt 2003; 54: 173–87.
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
Correspondence Clinical Letter
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5 6
7
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MacLean GM, Colemann DJ. Three fatal cases of squamous cell carcinoma arising in chronic perineal hidradenitis suppurativa. Ann R Coll Surg Engl 2007; (89): 709–12. Williams ST, Busby RC, DeMuth RJ, Nelson H. Perineal hidradenitis suppurativa: presentation of two unusual complications and a review. Ann Plast Surg 1991; 26(5): 456–62. Fimmel S, Zouboulis CC. Comorbidities of hidradenitis suppurativa (acne inversa). Dermatoendocrinol 2010; 2(1): 9–16. Constantinoi C, Widom K, Desantis J, Obmann M. Hidradenitis suppurativa complicated by squamous cell carcinoma. Am Surg 2008; 74(12): 1177–81. Zouboulis CC, Bechara FG, Fritz K et al. S1-Leitlinie zur Therapie der Hidradenitis suppurativa/Acne inversa. J Dtsch Dermatol Ges 2012; Suppl. 5; 1–31. Revuz J. Hidradenitis suppurativa. JEADV 2009; 23: 985–98.
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Büyükasik O, Hasdemir AO, Kahramansoy N et al. Surgical pproach to extensive hidradentis suppurativa. Dermatol Surg a 2011; 37: 835–42. 10 Breuninger H. Therapie des Pilonidalsinus und der Acne inversa. Hautarzt 2004; 55: 254–258. 11 Bocchini SF, Habr-Gama A, Desiderio RK et al. Gluteal and perianal hidradenitis suppurativa. Surgical treatment by wide excision. Dis Colon Rectum 2003; 46: 944–9. 12 Balik E, Eren T, Bulut T et al. Surgical approach to extensive hidradenitis suppurativa in the perineal/perianal und gluteal regions. World J Surg 2009. 33: 481–7. 13 Breuninger H, Eigentler T, Bootz F et al. S2k Kurzleitlinie – Plattenepithelkarzinom der Haut. J Dtsch Dermatol Ges 2013; Suppl. 3; 39–47.
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
419
Clinical letter Squamous cell carcinoma arising in hidradenitis suppurativa
DOI: 10.1111/ddg.12279
Dear Editors, Chronic wounds and inflammatory processes persisting for years entail the risk of malignant degeneration. Thus, in chronic hidradenitis suppurativa with recurrent abscesses, fistulas and scarring, the development of squamous cell carcinomas—also known as Marjolin ulcer—is a serious complication. A 52-year-old man presented in our clinic with chronic inflammatory fistulas present since the age of 18, which previously had been treated by surgical colleagues with multiple incisions. Physical examination revealed the clinical picture of hidradenitis suppurativa Hurley stage III with widespread fibrotic scar tissue, draining fistulas, and nodules affecting the sacral and gluteal region (25 × 25 cm) (Figure 1a). Clinically there was no evidence of malignancy. Imaging diagnostics with MRI scan revealed inflammatory changes of the sacrum indicating a possible osteomyelitis. There was no evidence of gastrointestinal involvement. Surgical treatment was performed under general anesthesia and included complete en bloc resection of the affected hidradenitis suppurativa areas with ablation of the altered structures of the sacrum (Figure 1b). The histological examination showed a moderately differentiated squamous cell carcinoma in the center of the resected hidradenitis suppurativa tissue (Figure 1c). It remained uncertain whether the basal margins were tumor free. Thus, after discussing the patient’s case at our tumor board, a second resection was performed by colleagues of the Trauma and Reconstructive Surgery Department and the Department of GI, Thoracic and Vascular Surgery including ablation of the coccyx and further parts of the sacrum. Following vacuum assisted closure therapy (VAC therapy) plastic reconstruction was performed with a gluteus maximus V-Y flap (right side), a subcutaneous transposition flap (left side) and a subsequent mesh-graft transplantation of the remaining defect ( Figure 1d). Histological examination revealed residual tumor in the previous excision margins but tumor-free bony parts (R0 resection). The follow-up 6 months later showed iliac and inguinal lymph node metastases right and a presacral lymph node metastasis left. The iliac and inguinal lymph node metastases were surgically removed. The presacral node could
not be localized intra-operatively and was thus treated with radiotherapy with the field also including the lymphatic pathways. Malignant degeneration in chronic inflammatory processes, such as scars, is supposed to be promoted by free radicals released by inflammatory cells with subsequent DNA damage and neoplastic transformation of the epithelium [1]. The incidence of malignant transformation in hidradenitis suppurativa is estimated to 3.2 % [1, 2]. To date more than 70 cases of squamous cell carcinoma arising in gluteal and sacral hidradenitis suppurativa and pilonidal sinus are documented in the medical database. Men are affected more frequently. The average age at diagnosis is approximately 52 years, and in more than 75 % of the patients hidradenitis suppurativa has been present for more than 10 years [1–6]. The development of malignant tumors in the area of these inflammatory changes is a rare complication, and in the majority of the cases histological investigation reveals squamous cell carcinomas whereas basal cell carcinomas are rarely found [1]. The clinical picture includes vast exophytic tumors of more than 5 cm in diameter and ulcerations (Marjolin ulcer) [1]. Bone infiltration is found in approximately 8 % of the patients and local lymph node metastases may be present at the time of diagnosis [1]. Wide surgical en bloc excision of the affected tissue is the treatment of choice (hidradenitis suppurativa Hurley stage II and higher) which in some cases (bone involvement, i nvolvement of the perianal and rectal region) turns out to be very tricky. A complete surgical excision is recommended under protection of the surrounding healthy tissue. In special cases (for example in order to identify fistulas of the intestine [7, 8]) clinical examination should be completed by ultrasound diagnostics (subcutaneous and endosonography) and MRT scan. Fistula tracts can be detected with probes and staining during surgery [2, 7–10]. Very deep fistulas of the pararectal region may be treated with curettage [11, 12]. Depending on the size of the resulting defect, complete s econdary wound healing or plastic reconstructive wound closure with flaps is possible [1, 2, 7, 10]. Therapy and follow-up of squamous cell carcinoma should be performed in accordance with the specific guideline [13]. Relapses after surgical treatment alone are frequent (44–48 % independent from the level of differentiation) and most commonly occur 9 months post-surgery [1, 6], indicating a relatively high aggressiveness of the tumor (5-year survival rate 61 %). Postoperative radiation therapy may reduce the recurrence rate to 30 % [1]. In our patient we avoided radiation therapy fearing impaired wound healing due to the considerable size of the defect and the complex plastic reconstruction. The role of postoperative chemotherapy is uncertain and controversial but should be considered in case of
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
417
Correspondence Clinical Letter
Figure 1 Clinical appearance prior to surgery (a), post-surgical appearance (b), histological specimen (HE; x200) (c), appearance 4 months post-surgery (d).
Correspondence to
inoperability and metastasis in accordance with the specific guideline [13]. In conclusion, patients suffering from chronic inflammatory processes in the perianal, perineal and sacral region should be treated with wide and radical surgical excision due to the risk of malignant degeneration and development of aggressive squamous cell carcinomas with poor outcome.
Dr. med. Solveig Herschel Department of Dermatology Medical Faculty Carl Gustav Carus Technical University Dresden Fetscherstraße 74 01307 Dresden, Germany E-mail: [email protected]
Solveig Herschel, Jörg Laske, Annette Stein Department of Dermatology, Medical Faculty Carl Gustav Carus, Technical University Dresden
Conflict of interest None.
418
References 1
2
de Bree E, Zoetmulder FAN, Christodoulakis M et al. Treatment of malignancy arising in pilonidal disease. Ann Surg Oncol 2001; 8(1): 60–4. Stein A, Sebastian G. Acne inversa. Hautarzt 2003; 54: 173–87.
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
Correspondence Clinical Letter
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4
5 6
7
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MacLean GM, Colemann DJ. Three fatal cases of squamous cell carcinoma arising in chronic perineal hidradenitis suppurativa. Ann R Coll Surg Engl 2007; (89): 709–12. Williams ST, Busby RC, DeMuth RJ, Nelson H. Perineal hidradenitis suppurativa: presentation of two unusual complications and a review. Ann Plast Surg 1991; 26(5): 456–62. Fimmel S, Zouboulis CC. Comorbidities of hidradenitis suppurativa (acne inversa). Dermatoendocrinol 2010; 2(1): 9–16. Constantinoi C, Widom K, Desantis J, Obmann M. Hidradenitis suppurativa complicated by squamous cell carcinoma. Am Surg 2008; 74(12): 1177–81. Zouboulis CC, Bechara FG, Fritz K et al. S1-Leitlinie zur Therapie der Hidradenitis suppurativa/Acne inversa. J Dtsch Dermatol Ges 2012; Suppl. 5; 1–31. Revuz J. Hidradenitis suppurativa. JEADV 2009; 23: 985–98.
9
Büyükasik O, Hasdemir AO, Kahramansoy N et al. Surgical pproach to extensive hidradentis suppurativa. Dermatol Surg a 2011; 37: 835–42. 10 Breuninger H. Therapie des Pilonidalsinus und der Acne inversa. Hautarzt 2004; 55: 254–258. 11 Bocchini SF, Habr-Gama A, Desiderio RK et al. Gluteal and perianal hidradenitis suppurativa. Surgical treatment by wide excision. Dis Colon Rectum 2003; 46: 944–9. 12 Balik E, Eren T, Bulut T et al. Surgical approach to extensive hidradenitis suppurativa in the perineal/perianal und gluteal regions. World J Surg 2009. 33: 481–7. 13 Breuninger H, Eigentler T, Bootz F et al. S2k Kurzleitlinie – Plattenepithelkarzinom der Haut. J Dtsch Dermatol Ges 2013; Suppl. 3; 39–47.
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
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