Rare Cause of Stricture Esophagus—Sarcoma: A Case Report and Review of the literature

Adenocarcinoma and squamous cell carcinoma account for the vast majority of oesophageal malignancies. Other malignancies known to occur in the oesophagus include melanoma, sarcoma, and lymphoma. Among the sarcomas, carcinosarcoma is the commonest with both carcinomatous and sarcomatous elements followed by leiomyosarcoma of mesenchymal origin. Other sarcomas reported in the literature are liposarcoma, synovial sarcoma, myxofibrosarcoma, Ewing's sarcoma, granulocytic sarcoma, histiocytic sarcoma, schwannoma rhabdomyosarcoma, and epithelioid sarcoma. We report a case of malignant spindle cell tumour of oesophagus. Sarcomas of esophagus present as a polypoid exophytic soft tissue mass. Our patient presented with a stricture which is a rare presentation. Locally aggressive treatment with surgery is beneficial, and local palliative treatment including radiotherapy is worthwhile.


Case Report
Sixty-three-year-old man who was a dentist by profession from West Bengal presented in March 2005 with complaints of dysphagia to solids for 12 yrs aggravated for 1 month. He had no loss of appetite but had loss of weight. His physical examination was unremarkable. Upper gastroesophagoscopy revealed a smooth, benign appearing stricture at 36 cm beyond which the mucosa was normal. There was a 1.5 cm diameter smooth mucosal bulge noted in the fundus of the stomach just beyond the gastroesophageal (GE) junction. Biopsy form of the bulge was taken and was reported as mild chronic gastritis with Helicobacter pylori infestation. He was treated with proton pump inhibitors for six weeks.
The dysphagia worsened within a span of ten months, and repeat endoscopy revealed a stricture at the same place. Biopsy was not suggestive of malignancy. Barium swallow revealed dilated thoracic esophagus with smooth tapered narrowing ( Figure 1). CT scan was done which revealed a lower esophageal wall thickening of 11 mm from T9 to GE junction ( Figure 2) with periesophageal soft tissue mass and periesophageal, lesser omental, and peripancreatic nodes along with left pleural effusion and adjacent lung atelectasis. Pleural fluid cytology and pleural biopsy did not show any evidence of malignancy. He was planned for stricture resection and exploratory laparotomy. Peroperatively, lower esophageal thickening was noted and a large, friable, periesophageal tumour mass extending along the crus of the diaphragm involving the coeliac nodes was found. There were multiple enlarged friable fleshy nodes. Multiple biopsies were taken from the perioesophageal tissue and nodal mass, and feeding jejunostomy was done. Histopathologically, fibroadipose tissue with a cellular tumour composed of fascicles of spindle shaped   cells with plump oval to elongated, moderately pleomorphic, mitotically active nuclei (4-5 per 10 high power fields) was found ( Figure 3). Many haemosiderophages, foci of recent haemorrhage, hyalinization, and congestion were present; the tumor was focally positive for Vimentin and was negative for SMA, CD 34, CAM 5.2, cytokeratin, desmin, S100, and CD 117. Hence, a diagnosis of malignant spindle cell tumor with sarcoma being a possibility was made.
After literature search, the decision was made to treat as sarcoma esophagus with palliative intent as no formal protocols were available. He received radiation therapy of 46 Gy in 23 fractions by AP-PA portals using Telecobalt machine. CT scan done 6 weeks after radiotherapy showed stable disease. He received 4 cycles of chemotherapy with Doxorubicin (50 mg/m 2 , Day1) and Ifosfamide (5000 mg/m 2 , Day 1) given at 3 weekly intervals. Third cycle was given at a delayed date with reduced dose due to earlier neutropenic sepsis and herpes zoster.
Repeat CT scan after 4 cycles of chemotherapy showed stable disease. In view of hematological toxicities he had, further chemotherapy was not given. The patient was explained regarding further options-continuing chemotherapy with increased risk of side effects and lesser response rate versus best supportive care, and he opted for best supportive care. Endoscopic stenting was done as palliation. He expired after six months at hometown due to stent blockage or gastrointestinal obstruction beyond the stent. He has had a survival of 20 months from the time of diagnosis with a good quality of life without dysphagia.

Epidemiology and Incidence.
Esophageal sarcomas are a rare entity [7]. Morphological variants of esophageal sarcomas as reported in the literature are summarized in Table 1.

Diagnosis.
Endoscopically, these are characterized by polypoid and exophytic masses [21] and rarely as ulcerating tumour [22]. Barium studies may show large intramural mass with ulceration/tracking, expansile intraluminal masses, or areas of luminal narrowing [23]. Stricture esophagus is a rare presentation. CT/MRI imaging may show inhomogenously enhancing intramural mass [23]. One of the indications for endoscopic ultrasound and its guided biopsy or fine needle aspiration cytology is submucosal esophageal tumors which otherwise may need open biopsy for diagnosis. This in turn may reduce the time of delay in diagnosis [24].

Pathology.
Considering histological appearance and immunohistochemistry of sarcomas of esophagus, carcinosarcomas has both carcinomatous and sarcomatous component. The sarcomatous component of carcinosarcoma is composed of dense interlacing bundles of spindle-shaped cells in the submucosa [25]. Pseudosarcoma shows squamous cell carcinoma and mesenchymal components without a transitional zone [19].
Synovial sarcoma has undifferentiated spindle cells similar in appearance to synovial sarcoma in other areas but overt mesenchymal differentiation showing smooth muscle, cartilage, or bone formation. X; 18 translocation is a sensitive marker and is demonstrated in 70 to 90% of synovial sarcomas. The specific t(X; 18) (p11.2; q11.2) results in the fusion gene product SYT-SSX [14].
Leiomyosarcoma has elongated cells forming interlaced bundles. Leiomyosarcoma constitutes about 0.5% of the malignant neoplasms of the oesophagus [12]. Oesophageal leiomyosarcoma possibly arises from the muscularis mucosa [3].
Liposarcoma has the lipogenic component containing giant multinucleated lipoblasts with multiple cytoplasmic lipid vacuoles giving grape-like appearance. The nonlipogenic component can resemble pleomorphic and/or myxoid MFH expansion of Malignant Fibrous Histiocytoma, a round cell sarcoma, a spindle liposarcoma, and/or an epithelioid carcinoma [13].
Histiocytic sarcoma has medium-sized tumor cells, indistinguishable from reactive histiocytes with moderate pleomorphism.
The tumor in our case was unique in that it did not have carcinomatous component and it was focally positive for Vimentin not making it any of the above-mentioned sarcomas of esophagus.
3.5. Treatment. Surgery, wherever possible, remains to be the mainstay of treatment [23]. Oesophagectomy/oesophagogastrectomy is the surgery of choice. Even if metastases are present, a palliative resection can still be performed [26]. Endoscopic resection is another surgical option available [27]. The role of adjuvant radiotherapy and chemotherapy is controversial [23]. Palliative procedures like stenting to relieve dysphagia improve quality of life [28].

Prognostic Factors.
Factors affecting survival included completeness of resection, growth pattern, postsurgical stage, tumour grade, and tumour location [18]. A rare case of spontaneous regression of oesophageal leiomyosarcoma is reported [29]. The more favourable prognosis associated with carcinosarcoma versus other oesophageal neoplasms has been attributed to early onset of symptoms, resulting in prompt diagnosis and a lower propensity for tumour invasion [30]. As in typical squamous cell carcinoma, early detection and treatment by surgical resection are needed to produce significant long-term survival [31].

The Present Case in the Context of the Literature.
Sarcoma is a rare entity among all esophageal malignancies. It presents as an exophytic mass, and in this case, it has presented as a stricture esophagus. Most of these tumors present in locally advanced and disseminated condition, one of the reasons being difficulty and hence delay in diagnosis. Inspite of best efforts, a group among them remains to be histologically uncharacterized. Here, we report a case of malignant spindle cell tumor of esophagus, a cause for a stricture esophagus. A definitive histopathological diagnosis could not be achieved.
Regarding treatment, there is a role of palliative resection even in case of inoperable disease. In view of locoregional failure, the role of aggressive local treatment should be emphasized.