Invasive Marjolin’s Ulcer Penetrating the Scalp to Involve Bone and Dura Mater: A Case Report
Article information
Abstract
Marjolin’s ulcers are rare aggressive conditions that arise from chronic skin lesions and diseases. Various cell types may be observed in these lesions; however, squamous cell carcinoma is the most common type. Squamous cell carcinoma due to Marjolin’s ulcer has a greater tendency to metastasize than squamous cell carcinoma due to other causes. This condition occurs primarily on the lower extremities, although a few cases have been reported of it occurring on the scalp. Moreover, cases of penetration of bone and dura mater are observed even more rarely. In this article, we present a rare case of a Marjolin’s ulcer that penetrated the scalp and invaded the bone and dura mater.
Introduction
Neoplastic changes occurring in the scar tissue of chronic ulcerative wounds are a well-known process. This condition mostly appears after post-burn scars but can also appear after various types of other scars. The term “Marjolin’s ulcer” is used to describe this type of rare and aggressive cutaneous malignancy that occurs in previously traumatized and chronically inflamed skin [1]. Marjolin’s ulcers occur in 0.8% to 2% of burn scars, most commonly in patients who have had deep partial or full thickness burns left to heal by secondary intention. Compared to sun exposure-associated skin malignancies, scar-associated carcinomas have a higher rate of regional metastases, worse prognosis, and greater mortality [2,3]. They are most commonly found on the lower limbs, and the scalp is a relatively uncommon site for scar-related carcinoma [1]. Additionally, there are few reports of Marjolin’s ulcers penetrating the bone and dura. In this article, we present a rare case of a Marjolin’s ulcer that penetrated the scalp and invaded the bone and dura mater. In presenting this case report, we strictly adhered to the ethical guidelines for research involving human subjects. Prior to the surgical procedures and participation in this report, informed consent was obtained from the patient.
Case
A 57-year-old female presented to the clinic with a fungating ulcer measuring approximately 5×7 cm over the mid-occipital area. This ulcer occurred 2 years prior, with active bleeding occurring frequently for the past year (Fig. 1). She had sustained burn injuries at the same site during childhood, which resulted in chronic scarring all over the scalp. Tissue biopsy specimens of the scalp lesion revealed a poorly differentiated squamous cell carcinoma, while computed tomography (CT) and brain magnetic resonance imaging (MRI) revealed extension into the bone and parietal dura mater. Fortunately, no regional lymph node metastases were detected by whole body positron emission tomography-CT. She underwent surgery with 3 cm clear margins down to the parietal bones. The locally invaded parietal bone and dura were also resected by a size of 10×10 cm. No metastatic tumor was noted on the brain parenchyma. After meticulous bleeding control, the operative field was irrigated and duroplasty was done. The bone defect was covered with an artificial skull plate (Medpor; Porex Surgical Inc.) to protect the underlying brain tissue from external trauma. Titanium plates and screws were used to fix the Medpor plate, and the adjacent fasciocutaneous scalp flap was elevated and rotated clockwise to cover the defect (Fig. 2). The patient healed well without any complications, and received radiotherapy for 3 months after the operation (Fig. 3). Follow-up surveillance was conducted to monitor patient progress. Brain MRI was performed every 6 months postoperatively. Imaging revealed no definite evidence of tumor recurrence or significant lymph node enlargement. Two years have passed since the surgery, and the patient has shown good progress with no recurrence or worsening (Fig. 4).
Preoperative images. Preoperative clinical photograph (A) and magnetic resonance imaging (B, C) of the tumor. A 57-year-old female presented to the clinic with a fungating ulcer measuring approximately 5×7 cm over the mid-occipital area. Tissue biopsy and brain magnetic resonance imaging revealed invasive squamous cell carcinoma of the scalp with extension into bone and parietal dura mater (arrows).
Intraoperative image. The 10×10 cm resection defect of metastatic parietal bone and dura was covered with Medpor (Porex Surgical Inc.) which was secured with titanium plates and screws. The adjacent fasciocutaneous scalp flap was rotated clockwise to cover the lesion.
Postoperative magnetic resonance imaging. (A) Axial and (B) sagittal views. Arrows indicate the extent of skull bone removal and reconstruction with Medpor (Porex Surgical Inc.).
Photograph taken 14 months after surgery. No dehiscence, infectious signs, or discharge was observed. The wound healed well and there were no signs of recurrence.
Discussion
Carcinoma arising from chronic ulcers was first described by Marjolin in 1928 and later described in more detail by Emsen [4]. Although the term “Marjolin’s ulcer” is often thought of as being limited to malignant transformation of burn scars, it can be applied to scars resulting from any type of injury. This occurs primarily on the lower extremities, although some cases have been reported on the scalp. Cases of penetration of bone and dura mater are observed even more rarely. Maillard and Landolt were the first to report resection of skin cancer that had invaded the brain [5].
There are still various hypotheses regarding the mechanisms of malignant transformation in chronic scar tissue. Currently, the most widely accepted theory is that it results from a series of repetitive ulcers or trauma and healing due to the biological activity of chronic abnormal keratinocytes. A report by Treves and Pack [6] evaluated more than 2,000 skin cancer patients and found that 2% of all squamous cell carcinomas and 0.3% of basal cell carcinomas resulted from burn scar conversion. Histologically, squamous cell carcinoma was found most often, followed by basal cell carcinoma [6,7].
There are two main types of Marjolin’s ulcer according to the time span between ulceration and malignant transformation. The first is a very rare acute type that occurs within a year, and the second is the more common chronic type that occurs after 12 months. However, in most cases, the development of malignancy in Marjolin’s ulcers tends to be slow, with the average time to malignant change being 35 years [8]. This case is especially rare in that Marjolin’s ulcer rapidly invaded the dura within 2 years of the occurrence of ulceration.
The three most important prognostic factors of Marjolin’s ulcer of the scalp are pathologic type and grade of the lesion and presence of lymph node metastasis at the time of the diagnosis. Three years after diagnosis, the overall survival rate for patients with Marjolin’s ulcer is 65%–75%, decreasing to 35%–50% if metastases are present from the start [9]. Therefore, it is critical to detect early signs that indicate the possibility of malignant degeneration in chronic wounds to perform early biopsies in order to establish the diagnosis as quickly as possible. Although it is uncommon for Marjolin’s ulcers to develop within several months after a primary burn, if the wound persists chronically for more than 3 months and shows signs suspicious of malignancy (bleeding, ulceration, or severe fungating granulation tissue), clinicians should not hesitate and delay biopsy from the lesion.
If squamous cell carcinoma is confirmed, treatment should be initiated as soon as possible. Because of its high aggressiveness, radical resection with wide margins is necessary. A margin of at least 2–5 cm is recommended, and all bone and dura mater suspected of invasion should be completely removed. After excision, split-thickness skin grafting is preferred to cover the defect if possible as this helps in early detection of recurrence. However, if bone exposure is present it may need to be covered with a local or free flap. After surgical treatment, a close examination of distant sites should be performed, such as defined lymph node dissection or sentinel lymph node biopsy. Adjuvant chemotherapy and radiotherapy may also be performed if necessary [10].
In conclusion, squamous cell carcinoma caused by Marjolin’s ulcer has a much higher tendency to spread than skin carcinomas caused by other causes; Marjolin’s ulcers of the scalp can also penetrate the bone and dura mater. As early evaluation and aggressive wide excision are critical for a complete cure, Marjolin’s ulcers should be approached and treated more aggressively than ordinary squamous cell carcinomas. If the progression of wounds lasts for more than 3 months, it should be considered a warning sign and closely monitored; biopsy of suspicious lesions should not be hesitated even if it has not been very long since the initial injury occurred. In addition to whole body imaging studies, extensive surgical removal should be performed with lymph node dissection or sentinel lymph node biopsy if needed. After resection and reconstruction surgery, all patients should be closely monitored for possible recurrence or metastasis.
Notes
This work was supported by the research fund of Hanyang University (HY-202000000000508). Jae-A Jung is an editorial board member of the journal but was not involved in the peer reviewer selection, evaluation, or decision process of this article. No other potential conflict of interest relevant to this article was reported.