Squamous cell carcinoma related to long standing pilonidal-disease

ARTICLE

Auteur(s) : Iosif Chatzis¹, George Noussios², Anastasios Katsourakis¹, Efthimios Chatzitheoklitos¹

¹Surgical Department, Agios Dimitrios General Hospital, Thessaloniki, Greece
²Laboratory of Anatomy in Department of Physical Education and Sports Sciences (Serres), Aristotle University, Thessaloniki, Greece

Squamous-cell carcinoma arising from a pilonidal sinus tract is an unusual complication of a common condition. It occurs in about 0.1% of patients with recurrent pilonidal disease and is more common in men. Approximately 59 cases of carcinoma related to the disease have been reported in the literature [1]. All occurred in the setting of long-standing pilonidal disease, with a mean duration of 23 years [2]. We present a case of a squamous-cell carcinoma which developed on a long-standing pilonidal disease.

A 50-year-old male presented to Outpatient Clinics complaining of a foul-smelling discharge from the sacro-coccygeal region. He had a history of untreated long-standing (15 years) pilonidal disease with sinus tracts. On inspection, there were two huge fungating tumours on both buttocks which extended deeply and were centrally necrotic with a purulent discharge (figure 1A). A complete clinical and radiological evaluation with the help of abdominal (upper, lower, retroperitoneal) and chest computed tomography revealed metastases only in pelvic bones.

The patient was operated, the tumor and the presacral fascia were excised with adequate margins. The histopathological examination showed “well differentiated squamous-cell carcinoma of verrucous type” (figure 1B). Due to positive margins the patient was re-operated and negative margins were achieved. Subsequently, the wound defect was covered with split-thickness skin grafts.

After complete healing (figure 1C), the patient refused further treatment or follow-up. Two years later the patient presented again, this time with a painful ulcerous lesion on the left groin which was biopsied and proved to be metastatic disease. The patient again refused further investigation or treatment and was referred to the pain clinic. He died 30 days later.

Pilonidal sinuses arise in the sacrococcygeal region superior to the anus. Common complications include cellulitis, abscess formation, and recurrent sinus development. Less commonly, sacral osteomyelitis and meningitis can occur. Malignant degeneration occurs in approximately 0.1% of pilonidal sinuses. Males are most often affected (80%), with a mean age at diagnosis of 50 years [3]. The average duration of antecedent pilonidal disease is 23 years. The mechanism by which malignant degeneration arises in a pilonidal sinus is believed to be the same as for other chronically inflamed wounds, such as scars, skin ulcers and fistulas.

Pilonidal carcinoma has a rather distinctive appearance, with the diagnosis frequently suspected by inspection, based on the presence of a long-standing and persistent pilonidal sinus with drainage, sudden rapid growth, overgrowth above the skin level, friability, ulceration, hemorrhage in the tissues, external bleeding, and most commonly, bleeding in sinus that has been present for many years [4].

Treatment of choice remains en bloc resection, including the presacral fascia, because surgery appears to provide the only means for cure. In our case surgery was performed for local control. Wide excision with tumor-free margins should be performed, including skin, subcutaneous tissue, muscle, and, if indicated, portions of the sacrum and coccyx. Surgical treatment has yielded five-year disease-free states in 55% percent of patients. Closure of the ensuing defect may be accomplished with mesh grafts, split-thickness skin grafts, or vascularized flaps, including gluteal rotation flaps and gluteal advancement flaps.

Some authors propose consideration of adjuvant chemotherapy and radiation to decrease the local recurrence rate. When radiotherapy is added to surgery alone, recurrence rates decrease from 44% to 30%. Re-excision of local recurrence resulted in some long-term survivals [2, 5].

Carcinoma arising from pilonidal disease is a rare complication occurring in the setting of long standing inflammation. Local recurrence is common and tends to occur early. Repeat surgery for recurrent disease may involve extensive resection. Treatment with adjuvant chemotherapy and radiation therapy is proposed by some authors.

Acknowledgements

References

2 Abboud B, Ingea H. Recurrent Squamous-Cell Carcinoma arising in sacrococcygeal pilonidal sinus tract. Dis Colon Rectum 1999; 42: 525-8.

3 Williamson JD, Silverman JF, Tafra L. Fine-Needle aspiration cytology of metastatic squamous-cell carcinoma arising in a pilonidal sinus, with literature review. Diagn Cytopathol 1999; 20: 367-70.

4 Sagi A, Rosenberg L, Greiff M, Mahler D. Squamous-cell carcinoma arising in a pilonidal sinus: a case report and review of the literature. J Dermatol Surg Oncol 1984; 10: 210-2.

5 Fasching MC, Meland NB, Woods JE, Wolff BG. Recurrent squamous-cell carcinoma arising in pilonidal sinus tract-multiple flap reconstructions: report of a case. Dis Colon Rectum 1989; 32: 153-8.