Annals of Surgical Oncology 8:60-64 (2001)
© 2001 Society of Surgical Oncology
Treatment of Malignancy Arising in Pilonidal Disease
Eelco de Bree, MD,
Frans A. N. Zoetmulder, MD, PhD,
Manousos Christodoulakis, MD,
Berthe M. P. Aleman, MD and
Dimitris D. Tsiftsis, MD, PhD
From the Departments of Surgical Oncology (EdB, FANZ) and Radiotherapy (BMPA), The Netherlands Cancer Institute/Antoni van Leeuwenhoek Huis, Amsterdam, The Netherlands, and Department of Surgical Oncology (MC, DDT), University Hospital, Herakleion, Greece.
Correspondence: Address correspondence to E. de Bree MD, Department of Surgical Oncology, University Hospital, P.O. Box 1352, 71110 Herakleion, Greece; Fax: 0030-81-542059.
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ABSTRACT
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BACKGROUND: Malignant degeneration is a rare complication of pilonidal disease and is associated with a high recurrence rate and poor prognosis compared with regular nonmelanoma skin cancer. Treatment in our departments and in the international literature was evaluated.
METHODS: We analyzed the data from three patients with malignant degeneration who were treated in our departments and an additional 56 patients who were found after an extensive literature search.
RESULTS: A total of 47 males and 12 females, with a mean age of 52 years, were most frequently primarily treated with surgery. After a mean follow-up time of 28 months, 20% of all patients died with evidence of disease and an additional 10% died of unrelated causes. The overall recurrence rate was 39%, with a median time to recurrence of only 9 months. The local recurrence rate was lower when radiotherapy was added to surgical treatment alone (30% vs. 44%). Re-excision of local recurrence resulted in some long-term survivals.
CONCLUSIONS: Early diagnosis and treatment may lead to improvement of the relative poor prognosis. Surgical treatment should be tailored according to the locoregional extent. The high recurrence rate after surgical treatment can be reduced by the addition of radiotherapy. Although repeat surgery for recurrent disease may involve extensive resection and morbidity, this may result in prolonged survival.
Key Words: Pilonidal disease— • Malignant degeneration— • Squamous cell carcinoma— • Surgical treatment— • Radiotherapy.
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INTRODUCTION
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Pilonidal disease is a very common disease, especially in men, and often complicated by infection. It is usually located in the sacrococcygeal area, but is also found at other sites such as in the umbilicus, the axilla, on the sole of the foot, the penis, the clitoris, and in the anal canal.1 Controversy continues about whether pilonidal cysts are congenital or acquired. Asymptomatic pilonidal sinus is usually treated conservatively, whereas pilonidal sinus abscesses are drained and symptomatic pilonidal sinuses are excised. However, there is a high recurrence rate after surgical excision regardless of the technique used. Malignant degeneration is a rare complication observed mainly in chronic, recurrent, and neglected primary pilonidal disease and is associated with poor prognosis compared with regular nonmelanoma skin cancer. Its recurrence rate after surgical treatment is high. Until now, only 56 such cases have been reported in the international literature.2–12 Herein, we report three new cases treated in the Departments of Surgical Oncology and Radiotherapy at the University Hospital in Herakleion, Greece, and at The Netherlands Cancer Institute in Amsterdam during the last 5 years. In a collective review, the data of these 59 cases were analyzed, especially regarding treatment and outcome, to form treatment recommendations with the aim of reducing the recurrence rate and improving survival.
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PATIENTS AND METHODS
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Case Reports
Case 1
A 48-year-old male with a 25-year history of recurrent infected pilonidal sinus presented with a sacrococcygeal tumor to the Department of Surgical Oncology at the University Hospital in Herakleion, Greece. Diagnostic biopsy demonstrated hyperplastic papillomatosis and chronic inflammation, without signs of malignancy. Local excision with secondary wound healing followed. Histological examination revealed well-differentiated squamous cell carcinoma, which had been completely removed. Five months later, a local recurrence of 7 cm in diameter was excised. The surgical margins were again microscopically free of tumor. However, 1 year later he presented with perineal recurrence with infiltration of the anal sphincter on endoscopic ultrasonography. Again, there were no clinical or radiological signs of lymph node or distant metastases. After a preoperative course of chemoradiotherapy (5 x 5Gy in combination with 5-fluorouracil and leucovorin in a 1-week period) an abdominoperineal resection was performed. Histological examination demonstrated malignant infiltration of the anorectal canal and resected lymph nodes and surgical margins were free of tumor. Three years after the last treatment, the patient was in good condition and free of disease.
Case 2
In a peripheral hospital, a 59-year-old male underwent a microscopically incomplete excision of a well-differentiated squamous cell carcinoma of chronic infected pilonidal sinus, existing and draining for approximately 30 years. Nine months later, after complementary excisions, he developed a local recurrence with infiltration in the anal sphincter causing fecal incontinence. A diverting colostomy was made and biopsy of the anorectal junction was positive for tumor infiltration. Subsequently, he was referred to the Department of Surgical Oncology at the University Hospital in Herakleion, Greece, for further treatment. A 10-cm large perianal tumor was seen. There were no clinical or radiological signs of lymph node or distant metastases. After a preoperative course of chemoradiotherapy (5 x 5Gy in combination with 5-fluorouracil and leucovorin in a 1-week period) an abdominoperineal resection including distal sacrum and coccyx was performed. The defect was closed by an omentoplasty and gluteal rotation flaps. Histologically, the tumor infiltrated the anorectal junction and the surgical margins were locally small. One and a half years later he presented with a ‘cysto-perineal’ fistula. Biopsy of the fistula tract was positive for recurrent squamous cell carcinoma. Cystoprostatectomy with excision of recurrent tumor in the pelvis and ileal urinary diversion was performed. The resection was histologically irradical and palliative chemoradiotherapy was given. However, there was rapid tumor progression in the pelvis and the patient died 44 months after the initial diagnosis of pilonidal sinus carcinoma.
Case 3
A 67-year-old man with a 5-year history of a recurrently infected pilonidal sinus presented with a large, ulcerative, smelling, and centrally necrotic tumor of 26 cm in diameter with multiple infected fistula in the sacrococcygeal area ( Fig. 1) to the Department of Surgery of the Netherlands Cancer Institute. Biopsy revealed squamous cell carcinoma. The anus and rectum appeared free of tumor involvement. Fine-needle aspiration of bilateral enlarged inguinal lymph nodes revealed no malignant cells. Computed tomography did not demonstrate suspicion on sacrococcygeal ossa involvement and locoregional lymph node, lung, or liver metastases. Six weeks after preoperative radiotherapy (50 Gy in 25 fractions in 5 weeks) with minimal clinical response he underwent surgical treatment. With the patient in supine position, a right rectus abdominis myocutaneous flap based on the inferior epigastric vessels was harvested and the omentum was mobilized. After bilateral inguinal lymph node dissection for suspected nodal metastases, the rectus abdominis flap and the omentoplasty were placed in the presacral area and the abdominal wall was closed in the regular way. In prone position, orientating biopsies were taken for frozen section examination to determine the extension of the tumor. The huge tumor mass with distal sacrum and coccyx was excised, sparing the anorectal canal. The rectus abdominis myocutaneous flap and the omentoplasty filled the large defect ( Fig. 2) and the latter was covered by a split skin graft. Histological examination demonstrated a large, ulcerative, badly differentiated squamous cell carcinoma with focal infiltration of resection margins and extended bilateral inguinal lymph node involvement. The postoperative course showed intractable psychosis, which resulted in refusal of food and fluid intake and finally, supportive treatment also. He developed heart ischemia and failure, pneumonia, and deep venous thrombosis in the left leg with suspicion of pulmonary embolism. However, wound healing was satisfactory. The patient died 4 months after initiation of the treatment. The patient’s family did not permit postmortem examination.
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FIG. 2. Patient 3 in prone position with the rectus abdominis myocutaneous flap (RA) and omentoplasty (O) filling the large defect after surgical excision of the tumor.  = anal canal, RL = right leg, LL = left leg.
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Literature Review
Recently, Davis et al.2 reported on their incomplete review on a total of 44 cases of malignant degeneration of pilonidal cysts. After an extensive literature survey, an additional 12 cases could be added.3–12
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RESULTS
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Patients
The data from the three patients treated in our departments and the additional 56 patients were evaluated ( Table 1). They were 47 males (80%) and 12 females whose ages varied from 18 to 86 years, with a mean age of 52 years. The duration of symptomatic pilonidal disease before the development of carcinoma was known in 51 cases and has been reported from 1 month to 62 years, with a mean of 22 years. Seventy-five per cent of the patients had symptoms for more than 10 years, while in only 6% the presentation was an acute septic process with duration of less than a year. Most frequently these malignancies were observed in patients with recurrent disease and less often in patients with untreated neglected symptomatic pilonidal disease. Histological examination demonstrated squamous cell carcinoma in 54 of 59 cases (92%). In the remaining cases basal cell carcinoma (n = 3) and adenocarcinoma (n = 1) were found or the tumor had not been classified (n = 1). Besides our third case, seven other cases have been reported with inguinal lymph node metastases at time of diagnosis.8
Treatment
The primary treatment for pilonidal sinus malignancy was surgery only (n = 42), surgery and radiotherapy (n = 8), surgery, radiotherapy, and chemotherapy (n = 3), surgery and chemotherapy (n = 1), radiotherapy and chemotherapy (n = 1), and chemotherapy only (n = 1). Three patients refused any kind of treatment.
Survival
Duration of follow-up was mentioned in 44 cases. Mean follow-up time was 28 months and varied from 1 month to 25 years. Outcome was reported for 56 patients, of which 54 had been treated ( Table 2). Two of the 3 patients who refused treatment died after 8 months and 2 years, respectively, from metastatic disease, while the outcome of the third patient is unknown. In total 20% of the patients died with evidence of disease after a mean follow-up of 30 months, while an additional 10% died of unrelated causes. All 11 patients who died of this disease were male. Of the 11 patients who died with disease, 6 patients had surgery only as primary treatment, 2 had surgery and radiotherapy, 1 had radiotherapy and chemotherapy, and 2 had refused any treatment. Regional lymph node metastasis proved to be a very bad prognostic sign. Seven of these eight patients died after a median and mean period after diagnosis of 7 and 9 months, respectively.
Recurrence
The overall recurrence rate for the 54 treated patients with follow-up data was 39%. Local recurrence, lymph node, and distant recurrence rates were 31%, 4%, and 15% respectively. The median time to recurrence was only 9 months. Local recurrence after surgery alone (there was one case that also had chemotherapy) as primary treatment was seen in 44% of patients versus local recurrence in 30% in cases treated by combination of surgery and radiotherapy initially, with or without systemic chemotherapy. There was no significant relation between duration of symptoms of pilonidal disease and survival or recurrence rate. Male gender seemed to be an unfavorable factor not only for survival, but also for recurrence. Of the treated males, 43% developed recurrent disease, whereas only 18% of the females developed recurrent disease.
Treatment of Recurrence
Thirteen patients were treated with re-excision of local recurrent tumor, while 5 also received adjuvant radiotherapy. In 5 of the 13 patients, abdominoperineal resection was necessary. Follow-up data were available on 10 of these patients undergoing potentially curative re-excision. Mean follow-up time was 4.8 years. Four patients died of their disease 15 months, 18 months, 3 years, and 7 years after re-excision. One patient died 3 months after re-excision from a myocardial infarction. Three of the 5 patients who were free of disease were living longer than 10 years after re-excision.
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DISCUSSION
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Malignant degeneration occurs in approximately 0.1% of patients with chronic untreated or recurrent pilonidal disease.4,7 Similar to other chronic ulcerative and scarifying cutaneous disorders, it is believed that this process is caused by the release of free oxygen radicals by activated inflammatory cells, inducing genetic damage and neoplastic transformation. Additionally, the normal repair DNA mechanism is impaired in chronic inflammation and predisposes to malignancy.6,13 The first case was reported by Wolff in 190014 and an additional 58 such cases have been reported in the world literature, including our own three cases.2–12,15
Diagnosis may be made on clinical examination and biopsy of a chronic ulcer with friable and necrotic margins, or it may occur on an incidental finding on histological examination of excised pilonidal sinus. Lesions are usually large tumors and frequently more than 5 cm in diameter.4 The malignant lesions are deeply invasive into the subcutaneous tissue in the great majority of cases. Underlying bone is involved in 8% of cases.4 Rarely, the diagnosis is established after fine-needle aspiration of a metastatic inguinal lymph node.8 Endoscopy may be necessary to rule out extension in the rectum. Computerized tomography 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, and to complement physical examination of the inguinal lymph nodes.2,9 Not withstanding extensive local and regional disease, distant metastases are rare at presentation.
Treatment of choice remains en-bloc resection, which may present enormous difficulties, because the tumor is able to extend along fistula tracts in a wide area of the sacrococcygeal and perineal regions. This means resection has to include at least the presacral fascia, but often also portions of the sacrum and coccyx and rectum. Closure of the ensuing defect may be accomplished with split thickness skin grafts or vascularized flaps, including gluteal and gracilis rotation flaps, as in patient 2.2,4,15 In patient 3, the use of local flaps was impossible due to the extension of the disease. To fill up the enormous defect, a rectus abdominis myocutaneous flap with a pedicle containing the epigastric vessels and an omentoplasty were used (Fig. 2), followed by a split skin graft to cover the latter. Transposition of the rectus abdominis myocutaneous flap based on the inferior epigastric vascular pedicle is a very useful, but rarely used, reconstruction technique for closure of large defects in the inguinal, perineal, and sacrococcygeal area after extended oncological resections.15–17 In one reported case, reconstruction with free latissimus dorsi myocutaneous flap was attempted after partial necrosis of local flaps, but failed due to thrombosis through alteration of the vessel walls caused by the local long lasting inflammation.3
Local recurrence after surgery alone as primary treatment was common (44%) and recurrent disease tended to occur early. This was not surprising in view of the ill-defined extension of the tumor along fistula tracts in this area. Initial combination of radical surgery with radiotherapy appeared to reduce local recurrence (30%). Therefore, radiotherapy should be considered part of the treatment in these tumors to reduce the local recurrence rate and also to improve the survival rate; radiotherapy should be given with a generous margin because of the ill-defined extension of the tumor. Chemoradiotherapy may be even more effective, analogous to anal squamous cell carcinoma. Concomitant radiotherapy and chemotherapy is proven to be superior to radiotherapy alone in locally advanced anal cancer.18 No data exist on the preferable sequence of surgery and radiotherapy. We tend to prefer preoperative radiotherapy, because it is expected to reduce the viability of tumor cells that may seed in the operation field and it might reduce the tumor’s size. Additionally, the fear of radiation damage to transposition flaps is avoided.
Inguinal lymph node metastasis is a very bad prognostic sign and is associated with a median survival time of only 7 months. Regional lymphadenopathy has been treated by lymph node dissection.4,7,8 Currently however, chemoradiotherapy or radiotherapy alone might be a treatment option for lymph node metastases to consider, analogous to the treatment of squamous cell anal cancer with inguinal lymphadenopathy.19 However, although chemoradiotherapy is the primary treatment in locally advanced anal cancer,18 the application of radiotherapy in pilonidal sinus malignancy has been mainly as adjuvant treatment and radiotherapy alone has not been curative in reported cases.4,7 Because the patient must be operated on anyhow, it seems convenient to perform lymphadenectomy for synchronous lymph node metastases instead of (chemo)radiotherapy only. In case of metachronous inguinal lymph node involvement, this treatment modality might be preferred over surgical treatment, especially when the poor prognosis is considered and no radiotherapy to this area preceded treatment of these lymph node metastases. In general, the role of (chemo)radiotherapy only as the primary treatment remains undefined, because there is only very limited experience with this treatment for this rare disease. Prophylactic regional lymphadenectomy has not been recommended.4,7 In view of the often large extent of these tumors, lymphatic mapping with sentinel node biopsy to identify early lymphatic metastases does not seem practical. The role of chemotherapy is unclear in the treatment of these tumors.
Biological behavior of sinus pilonidal carcinoma is much more aggressive compared to squamous cell carcinoma at other sites. Therefore, awareness of the possibility of malignant degeneration in pilonidal disease is essential for early recognition and treatment and subsequent improvement of the relative poor prognosis. Adequate information about the locoregional extent of the disease should lead to optimal planning and individual tailoring of the surgical procedure. Radiotherapy should be widely used in conjunction with radical local excision of primary and recurrent lesions to reduce the local recurrence rate and also to improve survival. Again, recurrence after primary treatment is commonly confined to the locoregional area. Therefore, repeat surgery for recurrent disease, which may involve extensive resection, seems worthwhile and may result in prolonged survival.
Received for publication April 14, 2000.
Accepted for publication August 18, 2000.
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ABSTRACT
INTRODUCTION
