Langenbecks Arch Surg (2008) 393:111–114 DOI 10.1007/s00423-007-0209-y
CASE MANAGEMENT AND CLINICAL CONSEQUENCES
Verrucous carcinoma (Buschke–Lowenstein) arising in a sacrococcygeal pilonidal sinus tract: report of a case Oner Mentes & Metin Akbulut & Mahir Bagci
Received: 10 August 2006 / Accepted: 14 June 2007 / Published online: 3 July 2007 # Springer-Verlag 2007
Abstract Purpose Pilonidal sinus disease is usually located in the sacrococcygeal region. Verrucous carcinoma (Buschke– Lowenstein) is a rare variant of squamous cell carcinoma with distinct clinical and histopathologic features, with a favorable prognosis. Malignant degeneration is an infrequent complication, which is associated with a high recurrence rate and poor prognosis. Materials and methods A case report is presented. Results We report a 48-year-old man with a 10-year history of recurrent pilonidal sinus disease presented with a sacrococcygeal verrucous carcinoma arising in the pilonidal sinus tract and associated with good prognosis. After operation, a 5-year disease-free follow-up was observed. Conclusion Careful inspection of the pilonidal area in all chronic and long-standing inflammatory processes is important and should be evaluated for malignant transformation.
Introduction
Keywords Buschke–Lowenstein . Verrucous carcinoma . Pilonidal sinus disease
A 48-year-old man presented himself to our hospital, with at least a 10-year history of recurrent pilonidal disease located in the sacrococcygeal area. His physical examination revealed a pilonidal sinus tract with a circumscribed, elevated vegetative tumor with an exophytic and some endophytic growth. The tumor was about 5×5×5 cm in diameter and had been present for 1 year (Fig. 1). Inguinal lymph nodes were not involved. A computed tomography (CT) scan of the abdomen and the pelvis localized the mass and showed no extension to the bone and the rectum. Chest X-ray and rectosigmoidoscopy were normal. Wide oblique excision and primary closure was performed, and the tumor was resected totally. There was no infiltration to the sacrum. The patient was discharged from the hospital on postoperative day 5 with out any complication. Histopathological examination of the operation specimen revealed
O. Mentes : M. Bagci Department of General Surgery, Etimesgut Military Hospital, Ankara, Turkey M. Akbulut Department of Pathology, Etimesgut Military Hospital, Ankara, Turkey O. Mentes (*) GATA Genel Cerrahi AD, Etlik, 06018 Ankara, Turkey e-mail:
[email protected]
The term of verrucous carcinoma was first introduced by Ackerman in 1948 in the oral cavity and was designated as a variant of squamous cell carcinoma with distinct features including well-differentiated, slow locally invasive growth and verrucous appearance. Although, as initially described, the oral cavity is the most common site. It has become evident that similar lesions can also be recognized in the skin, upper aerodigestive tract, and anogenital sites. Verrucous carcinoma of the pilonidal sinus is considered an extremely rare lesion because only a few cases have been reported in the literature [1, 2]. We report a unique case occurring in the pilonidal sinus tract.
Case report
112
Fig. 1 Vegetan tumor with an exophytic and some endophytic growth arising in a sacrococcygeal pilonidal sinus tract (macroscopiccally surgical margins were free of tumor)
verrucous carcinoma, which had been completely excised. The tumor is composed of an exophytic lesion comprised of thickened, acanthotic papillary squamous epithelium and also an endophytic growth of lobules of very welldifferentiated mature squamous epithelium with minimal cytologic atypia and scant mitotic activity. The tumor lobules have broad and blunt pushing margins, which are not deeply invasive and with no infiltration of the stroma and deeper structures by neoplastic cells. The lamina propria contains minimal lymphoplasmocytic inflammatory cell infiltrate. The surgical margins were microscopically free of tumor (Fig. 2). There were no clinical and radiological signs of local recurrence and lymph node or distant metastases, and no further treatment was given to the patient. Five years after the initial treatment, the patient was in good condition and free of disease.
Discussion Pilonidal disease is a common disease of young men that can result in abscess and draining sinus tracts [3]. Common complications of pilonidal disease are infection, abscess formation, and recurrent sinus disease after surgery. Less common complications include sacral osteomyelit and meningitis [4]. Malignant transformation is a rare but well-known complication of pilonidal disease and occurs in approximately 0.1% of patients with recurrent pilonidal disease and most commonly in men [5–8]. Most frequently malignancies were observed in patients with long-standing recurrent disease, and a large majority of the malignancies associated with pilonidal disease are squamous cell carcinomas [7–12]. Other malignancies are
Langenbecks Arch Surg (2008) 393:111–114
Fig. 2 The tumor lobules have a broad, pushing margin and are not deeply invasive and very well-differentiated mature squamous epithelium with minimal cytologic atypia and scant mitotic activity (H&E, 20×)
extremely rare, but basal cell carcinomas, adenocarcinomas, and verrucous carcinomas were reported [1, 13]. To our knowledge, there have been only a few cases with similar histological features located in a pilonidal sinus. All cases occurred in the setting of long-standing pilonidal disease. The mean duration of antecedent disease is being 23 years. Men were most often affected (80%). Mean age at diagnosis was 50 years [14]. In contrast to other case reports in the literature, our patient had a considerably shorter antecedent period (10 years) of pilonidal disease before malignant degeneration was detected. Verrucous carcinomas represent a distinct, well-differentiated variant of squamous cell carcinoma, characterized by a slow and locally invasive growth pattern. Grossly, they present as exophytic, fungating masses, but verrucous carcinomas with an inverted growth were also reported [1, 13]. The gross appearance is generally a bulky tumor suggesting an aggressive behavior, whereas histopathology reveals a relatively low-grade malignancy. The tumor may even have a benign appearance; therefore, diagnosis of verrucous carcinoma may be difficult because of the lack of cytological features of malignancy and particularly if biopsy specimen involves only the surface epithelium. Large and deep biopsies are thus mandatory. Microscopic features are notoriously similar to those of a viral wart, but in a large biopsy or excisional specimen, it can be differentiated by its characteristic “pushing” endophytic-invasive margin [15]. There seems to be much confusion regarding the terminology of this rare tumor; it was variously described as giant condyloma acuminata (Buschke–Lowenstein tumor), squamous papillomatosis, condyloma acuminata with malignant transformation, and well-differentiated squamous
Langenbecks Arch Surg (2008) 393:111–114
cell carcinoma. However, verrucous carcinoma presents as a distinct entity with exoendophytic growth pattern (in contrast to condyloma accuminata) of squamous cells showing mild atypia with pushing margins (in contrast to the invasive character of well-differentiated squamous carcinoma). It is characterized by negligible cellular atypia and a low mitotic rate. These are reflected in slow-growing lesions, locally invasive behavior, and very infrequent metastatic spread. However, the lymph node enlargement may show the presence of coexisting foci of squamous cell carcinoma. Enlargement of regional lymph nodes does not mean always metastasis disease. Furthermore, trauma or infection may lead to the enlargement of lymph nodes. Although several etiologic factors are implicated in the malignant transformation, the etiology of verrucous carcinoma is not known. The role of human papillomavirus (HPV) in the etiology of verrucous carcinoma has not yet been clarified. The role of HPV in verrucous carcinoma is likely but has to be demonstrated more specifically to determine whether HPV presence is a coincidence or a cofactor in carcinogenesis [16]. Generally, it is believed that malignant transformation is caused by the release of free oxygen radicals by activated inflammatory cells, inducing genetic damage and neoplastic transformation [17, 18]. The literature regarding pilonidal sinus verrucous carcinoma is comprised of reports containing very small number of cases with insufficient results. Preoperative evaluation of patients suspicious of carcinoma of pilonidal disease should include physical examination of the inguinal lymph node, perineum, and anus. Rectosigmoidoscopy should be done to rule out extension into the lower gastrointestinal tract. Abdomino-pelvic CT or magnetic resonance imaging is indicated to demonstrate local extent and to detect intra-abdominal metastases, including the spread to iliac and para-aortic lymph nodes [19]. Verrucous carcinoma of the skin and mucosa is an uncommon type of squamous cell carcinoma. It is clinically characterized by a slowly but relentlessly enlarging tumor. Histopathologically, it shows minimal atypia, and biologically, it is typified by a low incidence of metastasis [20]. Lesions in the pilonidal sinus are usually large tumors and frequently more than 5 cm in diameter [21]. Whereas, surgery still remains as the treatment of choice for small verrucous carcinoma. Therefore, radiotheraphy should be considered part of the treatment in large tumors, and the underlying bone is involved. The aim of the radiotheraphy is to reduce local recurrence rate and also to improve the survival rate. Concomitant radiotherapy and chemotherapy is proven to be superior to radiotherapy alone in locally advanced metastasis [22]. Inguinal lymph node metastasis is a very bad prognostic sin, and regional lymphadenopathy
113
has been treated by lymph node dissection [21]. The prognosis of verrucous carcinoma is relatively good if wide local excision or radical surgery is performed.
Conclusion Malignancies of this location often have a more aggressive clinical course than squamous cell carcinoma of other skin locations; therefore, careful inspection of the pilonidal sinus disease in all chronic and long-standing inflammatory processes is important and should be evaluated for malignant transformation.
References 1. Anscombe AM, Isaacson P (1983) An unusual variant of squamous cell carcinoma (inverted verrucous carcinoma) arising in a pilonidal sinus. Histopathology 7:123–127 2. Norris CS (1983) Giant condyloma acuminatum (Buschke– Lowenstein tumor) involving a Pilonidal sinus: a case report and review of the literature. J Surg Oncol 22:47–50 3. Chintapatla N, Safarani S, Kumar N, Haboubi (2003) Sacrococcygeal pilonidal sinus: historical review, pathological insight and surgical options. Tech Coloproctology 7:3–8 4. Terr JL, Gaisford JC, Hanna DC (1961) Pilonidal sinus carcinoma. Am J Surg 102:465–469 5. Bree E, Zoetmulder FA, Christodoulakis M, Aleman BM, Tsiftsis DD (2001) Treatment of malignancy arising in pilonidal disease. Ann Surg Oncol 8:60–64 6. Pekmezci S, Hiz M, Saribeyoglu K, Akbilen D, Kapan M, Nasirov C, Tasci H (2001) Malignant degeneration: an unusual complication of pilonidal sinus disease. Eur J Surg 167:475– 477 7. Abboud B, Ingea H (1999) Recurrent squamous-cell carcinoma arising in sacrococcygeal pilonidal sinus tract: report of a case and review of the literature. Dis Colon Rectum 42:525–528 8. Pilipshen SJ, Gray G, Goldsmith E, Dineen P (1981) Carcinoma arising in pilonidal sinuses. Ann Surg 193:506–512 9. Cilingir M, Eroglu S, Karacaoglan N, Uysal A (2002) Squamous carcinoma arising from chronic pilonidal disease. Plast Reconstr Surg 15(110):1196–1198 10. Davis KA, Mock CN, Versaci A, Lentrichia P (1994) Malignant degeneration of pilonidal cysts. Am Surg 60:200–204 11. Jeddy TA, Vowles RH, Southam JA (1994) Squamous cell carcinoma in chronic pilonidal disease. Br J Clin Pract 48: 160–161 12. Mashiah A, Kogan I, Nissim F (1984) Primary squamous cell carcinoma of the pilonidal area. Cutis 33:582–583 13. Melo CR, Melo IS (1989) Carcinoma cuniculatum of the buttock. An unusual case with an inverted growth. Dermatologica 179: 38–41 14. Pilipsen SJ, Gray G, Goldsmith E, Dineen P (1981) Carcinoma arising in pilonidal sinuses. Ann Surg 193:507–512 15. Schwartz RA (1995) Verrucous carcinoma of the skin and mucosa. J Am Acad Dermatol 32:1–21 16. Crowther ME, Shepherd JH, Fisher C (1988) Verrucous carcinoma of the vulva containing human papillomavirus—11. Case report. Br J Obstet Gynaecol 95:414–418
114 17. Gur E, Neligan PC, Shafir R, Reznick R, Cohen M, Shpitzer T (1997) Squamous cell carcinoma in perineal inflammatory disease. AnnPlast Surg 38:653–657 18. Kaplan RP (1987) Cancer complicating chronic ulcerative and scarifying mucocutaneous disorders. Adv Dermatol 2:19–46 19. Davis KA, Mock CN, Versaci A, Lentrichia P (1994) malignat degeneration of pilonidal cysts. Am Surg 60:200–204 20. Schwartz RA (1995) Verrucous carcinoma of the skin and mucosa. J Am Acad Dermatol 32:1–21
Langenbecks Arch Surg (2008) 393:111–114 21. Kulaylat MN, Gong M, Doerr RJ (1996) Multimodality treatment of squamous cell carcinoma complicating pilonidal disease. Am Surg 62:922–929 22. Bartelink H, Roelofsen F, Eschwege F et al (1997) Concomitant radiotherapy and chemotherapy is superior to radiotherapy in the treatment of locally advanced anal cancer: results of a phase III randomized trial of the European Organization for Research and Treatment of Cancer Radiotherapy and Gastrointestinal Cooperative groups. J Clin Oncol 5:203–210
