Journal of Clinical & Experimental OncologyISSN: 2324-9110

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Case Report, J Clin Exp Oncol Vol: 3 Issue: 3

Small Intestinal Obstruction due to Metastasis from Marjolins Ulcer: Report of a Case and Review of the Literature

Foroughi F* and Hosseini H
Department of Pathology, Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Corresponding author : Dr. Forough Foroughi
Department of Pathology, Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Tel: +98(21)23031248; Fax: +98(21)22432581
E-mail: [email protected]
Received: March 6, 2014 Accepted: June 3, 2014 Published: June 5, 2014
Citation: Foroughi F and Hosseini H (2014) Small Intestinal Obstruction due to Metastasis from Marjolin’s Ulcer: Report of a Case and Review of the Literature. J Clin Exp Oncol 3:3. doi:10.4172/2324-9110.1000126

Abstract

Marjolin's ulcer is an aggressive malignancy occurring years after chronic skin scar. The majority of these malignancies are squamous cell carcinomas. The rate of lymph node and distant metastasis is varied between 14 to 54% in different reports. Distant metastasis to the lung, brain, liver, and bone has also been reported. However, small bowel metastases are extremely rare. We present here a case of small bowel obstruction caused by metastatic squamous cell carcinoma from Marjolin’s ulcer.

Keywords: Marjolin's ulcer; Metastatic squamous cell carcinoma; Small bowel.

Keywords

Marjolin's ulcer; Metastatic squamous cell carcinoma; Small bowel.

Introduction

Developing malignancy in scars mainly resulting from burns or chronic wounds, so called Marjolin’s ulcer, is a well-documented phenomenon. Most of malignancies are reported to be aggressive squamous cell carcinomas [1].
Marjolin’s ulcer has a higher tendency for local recurrence and distant metastases through lymphatics. Rates of metastasis range from 20% to 35% in patients treated with amputation [2]. However, metastasis to small bowel is extremely rare [3].
We describe herein a case of Marjolin's ulcer metastasizing to the small bowel and peritoneal cavity. A review of relevant literature is performed in Pub Med and Scopus to find similar cases of small bowel metastasis from skin squamous cell carcinoma or Marjolin’s ulcer. To the best of our knowledge, this can be the second report in the literature.

Case Report

A 30-year-old woman came to the emergency ward with abdominal pain and distension, inability to defecate and pass gas, and fever from two days before admission. She also suffered from passing stool through vagina and right inguinal area for the previous two weeks.
In her past history, she had an old burn scar at the age of 3 which ended up in trans-metatarsal amputation because of delay in pursuing medical care. About one and half years ago, a non-healing ulcer was gradually appeared on the right foot and she developed right inguinal lymphadenopathy. The biopsy of both ulcer and inguinal lymph node showed squamous cell carcinoma. At that time, she underwent wide local excision and inguinal lymph node dissection. As the specimens from local excision had negative margins and no perineural involvement, adjuvant radiation therapy was done just for the inguinal area with a total dose of 5000 cG. She also received a course of adjuvant chemotherapy with cisplatin and 5-fluorouracil.
Physical examination of her present admission revealed abdominal tenderness and distention as well as an enterocutaneous fistula in the right inguinal area in addition to edema of the right leg.
In spiral computed tomography (CT) scan, pelvic structures could not be distinguished from each other and the fatty tissue was infiltrated by carcinomatous tissues (so called “frozen pelvis”). There was also a large fistula in the right inguinal region. A few lymph nodes with maximum diameter of 14mm in the right iliac cavity were identified.
The patient underwent an emergency exploratory laparotomy with the clinical diagnosis of bowel obstruction. At laparotomy, there were multiple tumor deposits involving the peritoneum, small bowel and omentum, with two small primary perforations in the small bowel. No evidence of fecal peritonitis was reported. There were also several masses in the pelvic cavity and the organs were adhered to each other. No liver metastasis was evident. The ileal segment between two perforated sites was resected and an ileostomy was put in place.
The histopathological examination showed moderatelydifferentiated squamous cell carcinoma, involving the omentum as well as outer layers of the small intestine (i.e. serosa and muscular layers) without mucosal invasion. This picture was consistent with metastasis from Marjolin’s ulcer.
After receiving post-operative care, she was advised to refer for oncology consultation and further follow-up and palliative care; but, she chose not to proceed with medical measures and she returned home in accordance to her family wishes.
Figure 1: Pelvic CT scan shows that pelvic structures cannot be distinguished from each other (“frozen pelvis”).
Figure 2: [A,B]: Microscopic section showed squamous cell carcinoma involving muscular and sub-mucosal layers of the small bowel [H & E, x100 (A), x400 (B)].

Discussion

Marjolin's ulcer is an aggressive cancer occurring years after chronic skin scar. The most important risk factor is burn scar but other types of wounds including venous stasis ulcers, chronically traumatized wounds, decubitus ulcers, and osteomyelitis are also considered as underlying causes [4]. It was initially described by Jean- Nicholas Marjolin in 1828 [5]; however, the term Marjolin’s ulcer was first used in 1903 by Da Costa [6].
The prevalence of cancer occurrence is between 0.2% and 1.7% [7] with the average latency period of 35 years [5]. Usually, the diagnosis is made in the fifth decade of life, but has a wide range from 15 to 84 years [7,8]. It is 3 times more common in males than females [9]. Although, one series of 81 patients with lesions in the extremities revealed male to female ratio of approximately 1:2 [10].
Our patient was a woman in her third decade of life when she developed the malignancy, 21 years after the burn scar; this meant that she was rather young with a rather short latency period for cancer occurrence. Comparably, by reviewing 31 cases of Marjolin’s ulcer, Copcu et al. found a latency period of 19 years [1].
The majority of these malignancies are squamous cell carcinomas (SCC) [1]; but, rarely other types of malignancies such as basal cell carcinoma, malignant melanoma, liposarcoma, osteosarcoma, adenocarcinoma, and fibrosarcoma have also been reported [11]. Over 60% of Marjolin’s ulcers occur in the extremities [1,7].
Lymph node metastases occur in 15% to 32% of patients at the time of diagnosis [9,12]. In a review of 449 cases, Ochenduszkiewicz confirmed the highly metastatic potential of Marjolin’s ulcers. He found that approximately 30% of the cases had enlarged lymph nodes with possible metastases from which, 19% showed lymph node invasion and 13% showed distant metastases [13]. Rate of metastasis has been previously reported to be up to 53.8% from lower extremity lesions [14]; however more recent studies refer to it as uncommon [1,15].
Distant metastases to the lung, brain, liver, and bone have been detected [16,17]. However, small bowel and peritoneal metastases are extremely rare. We found only one case of metastatic SCC involving the omentum and small bowel in the literature which was recently reported by Alamoodi. The patient was a 34-year-old male with the history of Marjolin’s ulcer arising on burn scar; he presented with abdominal pain, distension and vomiting secondary to small bowel obstruction [3].
Abraham et al. also reported ileal obstruction as a consequence of skin SCC metastases to the small intestine; but in their case, the patient was a renal transplant recipient who was in prolonged exposure to immunosuppressive therapy [18].
Furthermore, small intestine is rarely involved with metastatic tumors from extra-abdominal sources, mostly from the adjacent structures like uterus, uterine cervix and colon [19-21]. There are also few case reports regarding distant sources contributing to intestinal metastatic SCC including lung [22], esophagus [23] and the head and neck [24,25].
After exclusion of possible primary origins, as evidenced by extensive clinical examination and chest computed tomography scan, we concluded that the SCC deposits in our patient had arisen from the previous Marjolin’s ulcer; specially, because the tumor had invaded from serosa to submucosa and the mucosa was normal which was a contradictory finding to primary SCC.
Prognosis seems to depend on the presence of metastasis, high histologic grade, and tumor size [10]. The overall 3 year survival for patients with Marjolin’s ulcer ranges from 65% to 75% and this falls to 35% to 50% with metastasis [26].
Tiftikcioglu, et al. in one series of 61 patients with lesion in the extremities found that the mean survival time was 4.5 years after at least 5 years follow up. The 5-year survival rate was 42.9% in upper extremity and 58.3% in lower extremity groups. Tiftikcioglu suggested that tumor size was an important prognostic factor in addition to histologic grade [10].
In another study by Saglik, of 13 patients who were followed for an average of 56 months, two died of the disease [12].
Prognosis is better for grade 1 (well-differentiated) SCC than for other less-differentiated histological types [2,27]. In one series, six out of seven amputee patients did not show local recurrence or metastatic dissemination over a period of five years. The authors believed that the good prognosis was due to the pathological type of the tumors, the efficient and definitive treatment, and the absence of lymph nodes and distant metastasis [27].

Conclusion

Small bowel metastasis from Marjolin’s ulcer is extremely rare and the treatment options remain debatable. However, physicians should have a high index of suspicion in patients with previous history of the disease presenting with abdominal symptoms.
Early recognition by detailed clinical and radiologic evaluations and close follow-up is needed to plan the most appropriate treatment to improve patients’ outcomes.
Acknowledgement: The authors would like to thank Dr. Alireza Ahmadvand for editing the manuscript and thank Mr. Sassan Panahi, computer graphic designer, for quality-editing the photos.

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