British Journal of Radiology (2004) 77, 1050-1052
© 2004 British Institute of Radiology
doi: 10.1259/bjr/26921127
Primary melanoma of the oesophagus well palliated by radiotherapy
G B Fogarty, MBBS, FRANZCR1,
C J Tartaglia, MBBS2 and
L J Peters, MD1
1 Department of Radiation Oncology, Peter MacCallum Cancer Centre, A'Beckett Street, Melbourne, Victoria and 2 Northern Hospital, Epping, Victoria, Australia
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Abstract
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A case of a 16 cm primary melanoma of the mid oesophagus in a Caucasian male is reported. Radiological investigations at presentation revealed asymptomatic mediastinal and lower oesophageal metastases. The patient was treated with hypofractionated radiotherapy and achieved durable local disease control and excellent palliation of his dysphagia and chest pain until his death from widespread metastatic disease 5 months after treatment. The role of external beam radiotherapy in the treatment of primary oesophageal melanoma is reviewed.
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Case report
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A 77-year-old Caucasian man presented with a 2 month history of increasing dysphagia to solids. Associated symptoms included retrosternal chest pain and gradual weight loss over the past year of approximately 5 kg. Physical examination revealed a relatively fit patient; cancer specific examination was unremarkable.
Oesophagoscopy revealed a long, haemorrhagic, friable, polypoid tumour from 24 cm to 36 cm from the incisors. A further nodule was noted inferiorly at the cardio-oesophageal junction. Histology and immunohistochemistry demonstrated malignant melanoma. Surrounding squamous epithelium showed junctional activity favouring a primary lesion above a metastasis.
CT of the chest and abdomen demonstrated an extensive oesophageal tumour extending from just below the level of the upper margin of the sternum to 4–5 cm below the carina (Figure 1
). Total length of tumour was approximately 16 cm. The axial dimensions of the tumour were maximal around the level of the carina where it measured approximately 4 cm x 5 cm. A solitary mediastinal lymph node measuring just over 1 cm in maximal diameter was noted in the pretracheal space. Fluorodeoxyglucose positron emission tomography (FDG-PET) of the neck, thorax and abdomen demonstrated increased radiotracer uptake in the posterior mediastinum. A focal abnormality was also noted at the level of the diaphragm (Figure 2). These areas coincided with abnormalities on oesophagoscopy and CT. Separate focal abnormalities were also noted in the posterior aspect of the superior mediastinum and the right paratracheal region of the thoracic inlet reflecting probable further lymph node metastasis. Repeat physical examination was unable to identify any other possible primary site, either cutaneous, ocular or another mucosal lesion.
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Figure 2. Sagittal FDG-PET showing a lesion involving the upper and mid thoracic oesophagus and a secondary lesion at level of diaphragm.
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The patient was assessed as being incurable and declined palliative bypass surgery. Radiotherapy for palliation of dysphagia was offered. The patient received 36 Gy in six fractions over 3 weeks, at two fractions per week, using a conformal technique. Spinal cord dose was 11 Gy over the whole course of radiotherapy. Apart from an episode of melaena at 18 Gy for which the patient declined gastroscopy and which did not recur, the treatment was uneventful. In particular, there were no other acute toxicities of treatment.
The patient's dysphagia resolved shortly after treatment. At 1 month and 4 months following completion of radiotherapy, barium swallows showed no evidence of a mass lesion or abnormal oesophageal motility. CT at 4 months likewise showed no residual mass or thickening in the oesophagus. Unfortunately, new multiple liver metastases were demonstrated together with metastases to lymph nodes of the lesser sac, the right adrenal gland, and the right upper lobe of the lung, all outside the treated radiation volume. The patient died of metastatic disease 5 months post treatment with a patent oesophagus.
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Discussion
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Primary malignant melanoma of the oesophagus is rare. It accounts for 0.5% of all non-cutaneous melanomas and 0.1% of all malignant oesophageal tumours [1, 2]. The demography and findings on presentation were reviewed by Sabanathan et al [3]. Most cases present in the 6th to 7th decades and the male to female ratio is 2:1. Like other oesophageal malignancies, primary melanomas usually present with symptoms of dysphagia, epigastric or retrosternal pain and weight loss. Symptoms are typically present for only a few months before diagnosis. Melaena is an uncommon presentation but may occur or may complicate treatment, as in our case.
At the time of investigation primary malignant melanoma usually manifests as a bulky tumour located in the mid to lower oesophagus. This makes the mass readily apparent on barium swallow. Endoscopy typically demonstrates a friable irregularly pigmented polypoid mass, though amelanotic lesions have been described [4]. Satellite lesions or oesophageal melanosis may also be seen [6, 7]. Diagnosis requires tissue sampling. However, submucosal growth means endoscopic biopsy may not be diagnostic [3]. The high propensity of oesophageal melanomas to metastasize makes accurate staging an essential pre-requisite for attempted curative resection.
Staging methods for local, regional and distant disease have evolved significantly over the time period encompassed in the literature reviews. This precludes direct comparison of outcomes in patients having different staging procedures. Endoscopic ultrasound, contrast oesophogram and CT are currently the standard techniques used. The radiological features of primary oesophageal melanoma have been described in two small series by Golleb et al [8] and Yoo et al [9] and are similar to those found in our patient.
The value of FDG PET scanning as a staging tool has not been established for this entity. However, by extrapolation from experience with cutaneous melanoma, PET may be the preferred imaging technique for metastatic disease [10–13]. Damian et al [12] reviewed 100 patients with stage II to IV cutaneous melanoma who underwent PET imaging. A total of 415 radiologically, clinically or histopathologically confirmed metastatic lesions were evaluated, 388 (93%) of which were detected by PET. In 20 patients, metastases were clearly seen with PET 6 weeks to 6 months prior to detection with conventional imaging. Holder et al [13] prospectively evaluated 103 FDG-PET scans in 76 patients with stage II to IV cutaneous melanoma and compared the results with CT. They demonstrated that PET scanning had a sensitivity of 94% and specificity of 83% compared with 55% and 84%, respectively, for CT scanning. Four false negative scans (4%) were thought to be due to smaller (<0.5 cm) lesions or diffuse areas of melanoma without mass effect. When comparing sites of the metastatic lesions PET and CT were equivalent in detection of pulmonary metastases. However, PET scanning was superior to CT scanning in detection of regional and mediastinal lymph nodes, liver and soft tissue metastases. In addition to confirming known sites of disease, PET scanning in our patient revealed two additional sites of probable metastatic involvement not evident on CT.
Due to its rarity, randomized controlled trials of treatment options for primary oesophageal melanoma are not feasible. Retrospective review of case studies is the best level of evidence to date. Most authorities state that the only potentially curative treatment is surgical resection, subtotal en bloc oesophagectomy being the preferred procedure. Local resection is generally inadequate because of submucosal growth of tumour and in situ melanocytic dysplasia at sites distant from the primary tumour [3, 14].
Sabanathan et al [3] in a review of 139 cases up to 1988 analysed survival by treatment modality. They reported a median survival of between 7 months and 12 months in 67 patients who underwent radical resection and a 5 year survival of 4.2%. All other treatment options and combinations were associated with poorer median survival. Radiotherapy alone was used in only nine patients, in whom a median survival of 2.6 months was reported. However, two of these nine patients each survived 51 months. Volpin et al [15] updated the Sabanathan series with their own patient and a further 98 patients found on literature search. Volpin et al [15] calculated the actuarial survival to be 37% at 5 years by reviewing 25 cases studies treated between 1989 and 2000 with radical resection. Neither this review nor a smaller, overlapping review by Yoshidome et al [16] addresses the quality of palliation provided by surgery versus radiotherapy.
Most reviewers conclude that surgery is the preferred treatment for primary melanoma of the oesophagus based on the generally poorer survival of patients treated with radiation than in those undergoing resection [3, 13, 15, 16]. However, it is difficult to estimate the extent of selection bias in such conclusions, which are based on retrospective analysis. Another reason cited for not recommending radiotherapy is that melanoma is considered to be radioresistant [18]. This assertion is not supported by radiobiological studies [19] or the case study we report here in which there was a complete radiological response with a relatively low total dose of hypofractionated radiation – 36 Gy in six fractions given twice weekly. This schedule fitted in well with the palliative intent of this treatment and was associated with minimal treatment-related toxicity. Other hypofractionated regimens have had success in treating gross disease. The Princess Margaret experience as reported by Johanson et al [20] details the 0-7-21 regimen where 8 Gy fractions are given on days 0, 7 and 21 to a total dose of 24 Gy in 3 fractions. In nine patients treated with gross residual nodular cutaneous melanoma post lymphadenectomy, six had stable disease or a complete response in the treated volume at 9 months, another two had stable disease until death, which occurred before 9 months.
Regardless of treatment modality, life expectancy for patients with primary malignant melanoma of the oesophagus is short. Approximately 50% of patients have metastatic disease at presentation and even in those considered suitable for radical resection, long term survival is rare [3]. Better selection of patients by PET staging may increase the yield of curative resections but the fact remains that for the great majority of patients the aim of treatment should be improved quality of life by relief or prevention of oesophageal obstruction. The correct end point to compare different treatments is therefore quality of life and symptom palliation, not necessarily increased survival. Our case demonstrates that external beam radiotherapy can usefully palliate patients with even very large tumours. Radiotherapy enabled this patient to avoid a major (futile) surgical procedure with all its associated morbidities, including the valuable limited remaining life expended in recovery from oesophagectomy. Radiotherapy should be considered before other palliative procedures such as oesophageal stenting, laser ablation, chemotherapy and palliative surgery [21].
Received for publication October 7, 2003.
Revision received May 5, 2004.
Accepted for publication July 1, 2004.
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References
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Abstract
Case report
