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Case report |
1 Department of Clinical Oncology, Velindre NHS Trust, Cardiff, 2 Department of Gastroenterology, University Hospital of Wales, Cardiff, 3 Department of Pathology, Llandough Hospital, Penarth and 4 Department of Thoracic Surgery, Llandough Hospital, Penarth, UK
Correspondence: Dr R Jacob, Department of Radiation Oncology, Fox Chase Cancer Center, 7701 Burholme Avenue, PA 19111, USA
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Abstract |
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Introduction |
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We report a case of squamous cell carcinoma arising within an oesophageal cyst affecting the upper third of the oesophagus. The malignant cyst was not amenable to primary surgical resection and hence was treated using chemo-radiotherapy. There was no evidence of malignancy on post-treatment biopsy and the patient is free of recurrence 4 years after treatment.
This report describes the unusual presentation, response to treatment and long-term morbidity of treatment. We observed that chemo-radiotherapy is an effective alternative treatment modality to achieve disease control and possible cure where squamous cell carcinoma complicates an oesophageal foregut cyst and primary surgical resection of the cyst is not possible.
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Case report |
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General examination was unremarkable except for signs of weight loss. Local examination showed fullness on the left side of the neck, though no discrete mass was palpable. Apart from mild normochromic anaemia, her full blood count and biochemistry were normal. Indirect laryngoscopy showed sluggish movement of the vocal cords bilaterally.
Plain radiographs of the neck showed a soft tissue shadow in the pre-vertebral area in keeping with a mass lesion. A barium swallow demonstrated a posterior mass in the region of the hypopharynx causing extrinsic compression of the oesophagus. A CT scan confirmed the presence of a cylindrical mass 4 cm in diameter extending from the post-cricoid region to the root of the great vessels. There was evidence of tracheal and oesophageal displacement with extrinsic compression of the latter. The mass demonstrated a pseudocapsule, which enhanced with contrast and had a homogeneous internal component. These findings were thought to be in keeping with an oesophageal duplication cyst, a pre-vertebral abscess or a submucosal oesophageal tumour (Figures 1a, b
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The patient underwent attempted excision of the mass and neck exploration under general anaesthesia. Per-operatively, a sausage-shaped mass was identified within the wall of the oesophagus, extending from the post-cricoid level to a point about 2 cm inside the chest. The lower margin of the mass was just palpable from the neck. Complete excision of the mass was not feasible and surgery was discontinued after obtaining multiple biopsies. Frozen section biopsy showed squamous cell carcinoma and the overall impression was that of carcinoma complicating a foregut cyst.
Microscopy of the biopsy material showed highly inflamed tissue with some multinucleate giant cells and foci of squamous cell carcinoma (Figure 2) with evidence of adjacent lymph node involvement.
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CT scans performed 8 weeks after completion of treatment showed a complete response of the mass to chemo-radiation. (Figures 1c, d
) there was no evidence of malignancy on endoscopy or in the biopsies taken from the site of opening of the foregut cyst. In view of the excellent response of the tumour to treatment and patient-preference, it was decided to withhold surgery.
4 months post chemo-radiotherapy the patient developed recurrent dysphagia and a repeat endoscopy was performed. This revealed a pinpoint stricture in the upper oesophagus (20 cm per orum) and findings were consistent with a radiation-induced, fibrotic stricture. There was no evidence of malignancy in the biopsies taken from the strictured area.
Stenting of the stricture was not possible due to its high position and oesophageal dilatation was considered the only way to relieve her dysphagia. This was initially required every 2 weeks but is currently performed every 8 weeks when dysphagia starts to return. Local steroid injection into the stricture was attempted but did not extend the time interval between dilatations. The patient remains under regular clinical and endoscopic surveillance and is free of recurrent disease 4 years after diagnosis and treatment. She remains dependent on repeated endoscopic dilatation but is otherwise well and maintains a normal diet.
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Discussion |
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Oesophageal cysts often present with dysphagia (70%), epigastric pain (20%) or retro-sternal pain (10%). Respiratory symptoms or tracheal compression are more common with upper oesophageal cysts and manifest in early childhood. Other localized presenting symptoms include vascular compression, or mediastinitis secondary to cyst rupture. Malignant transformation within these cysts is an extremely rare event, which may occur several years after primary diagnosis of the condition.
Foregut cysts may be evaluated by endoscopy and using imaging techniques such as endoscopic ultrasound, plain radiograph, barium studies and CT scanning [4, 5, 7]. On non-enhanced CT images there may be high attenuation for cyst contents due to the presence of cholesterol or calcium, or low attenuation if fat is present [5]. The role of MRI and PET is being evaluated [8].
Precise distinction between the different types of oesophageal cysts requires its complete excision and histological assessment. Inclusion and retention cysts are lined by squamous or columnar epithelium. Benign developmental cysts are composed of an outer, fibrous capsule, multiple layers of smooth muscle cells and an inner lining of ciliated epithelium, which is most commonly columnar. It has not been possible to categorize the type of oesophageal cyst in our patient, as a complete surgical excision was not performed.
When malignant transformation occurs within the cysts, biopsy findings may range from dysplasia or in situ changes to frank infiltrating carcinoma [8, 9]. The most common histology is an adenocarcinoma, though squamous carcinoma has also been reported to occur within oesophageal cysts [10].
The management of foregut cysts varies depending on the site and nature of the lesion and the symptoms it produces [2, 4, 7]. Options include a watch and wait policy, repeated aspiration either endoscopically or under radiological guidance, injection of sclerosing agents or complete surgical excision.
Chemo-radiation is being increasingly used in the radical management of oesophageal cancers with improvement in control rates over conventional radiotherapy [12]. A dose of 50 Gy is commonly used when chemo-radiation is used alone, and 45 Gy when it is combined with surgery [13, 14]. This patient was treated using chemo-radiotherapy based on our experience in using this modality to treat oesophageal cancers.
The radiation dose in our patient was limited to 45 Gy as surgery was initially planned, and also because the spinal cord was very close throughout the Planned Target Volume (PTV). Whilst this radiation dose may be criticised as being inadequate, the negative biopsy and disease free status at 4 years are encouraging. Despite the negative biopsy, it is difficult to accurately predict long term control rates following chemo-radiation using 45 Gy.
This is the first report of a successful response of a squamous cell carcinoma arising in an oesophageal foregut cyst to standard chemo-radiotherapy, though this modality has been tried previously in the treatment of foregut cyst cancers. Lee et al reported the case of a 37-year-old patient treated using chemo-radiotherapy and oesophagectomy, although long-term outcome was not recorded [9]. McGregor et al treated a 65-year-old man with unresectable squamous cell carcinoma arising from an intramural oesophageal squamous epithelial cyst, using split course radiotherapy of 3000 rads each, and 10 courses of injection Bleomycin, at a dose of 300 mg [11]. The treatment gave a temporary improvement in dysphagia but the patient died 7 weeks after completion of treatment.
The treatment in our case has been complicated by the development of a radiation-induced oesophageal stricture, which was too high to be managed by oesophageal stenting. Also, there was little response to local steroid injection. Repeated endoscopic dilatation remains the best option for this patient, despite the associated discomfort and potential risk of perforation.
As with primary oesophageal malignancy, surgical excision remains the treatment of choice for a malignant foregut cyst. If this is not possible, chemo-radiotherapy can offer excellent disease control and the possibility of cure. A dose of 45 Gy or more is required, though with higher doses the risk of unacceptable morbidity increases. Optimal follow-up for these tumours follows the same lines as that for primary oesophageal cancer.
Received for publication June 11, 2002. Revision received December 2, 2002. Accepted for publication February 13, 2003.
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