Radiotherapy treatment of large esophageal leiomyosarcoma: A case report

Abstract

Abstract. Leiomyosarcoma of the esophagus is a rare type of tumor, characterized by a malignant phenotype and smooth muscle histology. Previously, barium studies have been used to identify areas of luminal narrowing, expansile intraluminal masses or large intramural masses with ulceration or tracking. Furthermore, endoscopic biopsies appear to be associated with a high false negative rate, particularly in cases where the mucosa is intact. The optimal treatment strategy is surgical resection, while the role of adjuvant radiotherapy and chemotherapy is controversial. In addition, the prognosis of patients with leiomyosarcoma of the esophagus is improved compared with patients suffering from squamous esophageal cancer. The present study described the case of a 48-year-old woman who presented with dysphagia and was diagnosed with a large leiomyosarcoma of the esophagus. The patient was successfully treated with radiotherapy and remains disease-free two years after the completion of treatment. In addition, the present study conducted a review of the relevant literature, reporting previous cases of esophageal leiomyosarcoma and potential strategies for the management of this disease. IntroductionSquamous cell and adenocarcinoma are the two most common types of esophageal malignancies, whereas esophageal leiomyosarcoma is a rare type of tumor, accounting for <1% of all malignant esophageal tumors (1,2). Since the first case of esophageal leiomyosarcoma was reported in 1902 (3), >165 cases of esophageal leiomyosarcoma have been described in the literature (4). Leiomyosarcomas are characterized by slow growth and late metastases and thus, exhibit a better prognosis than squamous cell carcinoma of the esophagus (5). The one-, three-and five-year survival rates of esophageal leiomyosarcoma in the Chinese population are 60.3, 42.8 and 32.1%, respectively (5). The most common symptom observed at diagnosis is progressive dysphagia which occurs in 64.7-90.0% of esophageal leiomyosarcoma patients. Other symptoms include retrosternal/back pain, weight loss and emesis (6,7). The development of definite treatment strategies is difficult due to the rarity of these tumors. Currently, surgical resection is the first-line treatment (6); however, surgical resection is not suitable for all patients. Although leiomyosarcoma is not sensitive to radiation, radiotherapy can be used to control the tumor effectively by appropriately increasing the radiation dose (8-12). The present study describes a case of leiomyosarcoma of the esophagus that was treated with radiation, rather than surgery. In addition, the current study conducted a review the relevant literature. Written informed consent was obtained from the patient. Case reportIn May 2009, a 48-year-old woman presented with 6 kg weight loss in two months and a six-month history of dysphagia to solids, which had been aggravated for two months, at the Shandong Cancer Hospital and Institute (Jinan, China). Computerized tomography (CT) scanning of the chest revealed the presence of a round so...

Histopathological landscape of rare oesophageal neoplasms

“…Clinical features and diagnostic approach: Dysphagia is the most common symptom in patients with OLMS, but other symptoms are retrosternal backpain, weight loss and/or emesis[ 138 ]. OLMS is reported to arise from the muscular layer of the oesophagus.…”

“…OLMS is reported to arise from the muscular layer of the oesophagus. EGDS, EUS, CT and PET/CT are useful, but the final diagnosis is usually made after histological examination of an oesophageal biopsy[ 138 ].…”

Rare cancer of oesophagus; Leiomyosarcoma

“…Preoperative misdiagnosis rates are as high as 82% in some case series (of the reported literature). Most of the cases were ultimately diagnosed by an oesophageal open biopsy and immunohistochemical analysis [4].…”

“…Recently endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB), using 19 G franseen tip needle and immunohistochemistry looking for smooth muscle actin (SMA), desmin is an accurate method for diagnosing leiomyosarcoma from other oesophageal tumours pre-operatively and may be used to guide treatment precisely [4]. The current treatment of choice for leiomyosarcoma of the oesophagus is radical oesophagectomy [5].…”

“…The place for adjuvant radiotherapy and chemotherapy is controversial [5]. Even though leiomyosarcoma has poor sensitivity to radiation, the tumour can be effectively controlled by increasing the radiation dose appropriately, which provide acceptable survival rates in patients who are unfit for surgery and metastatic disease [4].…”