Ann Thorac Surg 2007;83:314-315
© 2007 The Society of Thoracic Surgeons
Case Reports
Prolonged Survival Due to Spontaneous Regression and Surgical Excision of Malignant Mesothelioma
John E. Pilling, MRCSa,
Andrew G. Nicholson, FRCPathb,
Clive Harmer, FRCPc,
Peter Goldstraw, FRCSa,*
a Department of Thoracic Surgery, London, United Kingdom
b Department of Pathology, Royal Brompton Hospital, London, United Kingdom
c Department of Oncology, Royal Marsden Hospital, London, United Kingdom
Accepted for publication May 15, 2006.
* Address correspondence to Prof Goldstraw, Department of Thoracic Surgery, Royal Brompton Hospital, Sydney St, London, SW36NP United Kingdom (Email: p.goldstraw{at}rbh.nthames.nhs.uk).
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Abstract
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We report a case of malignant pleural mesothelioma with histologically proven spontaneous regression of pleural disease. During a 12-year follow-up there was a single recurrence, which was a lesion in the chest wall at 6 years that was surgically excised. A prominent host response to tumor was seen in both the primary tumor and the recurrence.
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Introduction
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Malignant mesothelioma is an exceptionally aggressive and almost universally fatal neoplasm arising from mesothelial cells that form the serosal lining of the pleural, peritoneal, and pericardial cavities. We report the case of a long-term survivor with malignant pleural mesothelioma (MPM) in which spontaneous regression of the disease has been histologically proven and whose recurrence was controlled with limited surgical excision.
A 58-year-old man presented in 1994 with a 3-month history of chest pain and dyspnea. He was a nonsmoker who had significant occupational exposure to asbestos 16 years previously. Left video-assisted thoracoscopic surgery at another hospital demonstrated a blood stained pleural effusion and tumor nodules infiltrating the posterior parietal pleura. The operative report clearly identified the area of macroscopic disease from which the biopsy was taken. Pleural biopsies showed fibrous thickening with infiltration by a malignant, predominantly epithelioid neoplasm. There were occasional sarcomatoid areas and a prominent inflammatory cell infiltrate. Immunohistochemistry showed a mesothelial phenotype with subsequent diagnosis of MPM. The patient consulted an oncologist, opting for radiological surveillance but no further active treatment at that time.
Five years later he presented with an enlarging, painless chest wall mass 5 cm in diameter overlying the anterior ends of the third to fifth ribs, distant from the previous video-assisted thoracoscopic surgery port sites (Fig 1). A needle biopsy confirmed malignancy and the patient proceeded to a left thoracotomy, multiple pleural biopsies, and chest wall resection with insertion of prosthesis. The chest wall mass was shown to be a completely excised epithelioid MPM in which there was a moderate host inflammatory response (Fig 2). There was a moderately high proliferation index. The mass was arising from the chest wall and was not in continuity with the parietal pleura. It was believed to be a hematogenous metastasis from the original MPM. Both the video-assisted thoracoscopic surgery biopsies and the chest wall lesion were subject to international pathologic review, which confirmed the diagnosis of MPM. Extensive parietal pleurectomy from the region of the previous VATS biopsy in the left paravertebral gutter showed mild fibrosis with no evidence of malignancy. Recovery from the operation was uneventful.
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Fig 2. Hematoxylin and eosin stained light micrograph of the excised chest wall metastasis. An epithelioid mesothelioma shows a moderate host inflammatory response with small round lymphocytes easily visible around the tumor cells.
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At follow-up 7 years after chest wall resection and 12 years after initial presentation, the patient remains asymptomatic with no radiologic evidence of recurrence. During this time, no adjuvant therapy has been given.
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Comment
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Survival after diagnosis of MPM is generally poor. Even in those fit for surgical palliation the median survival after surgery is 10 months [1]. Tri-modality therapy (ie, combined radical surgery, radiotherapy and chemotherapy) has had limited success outside of specific subgroups in which patients with epithelioid MPM, negative surgical resection margins, and unaffected extrapleural lymph nodes had a 5-year survival of 46% [2]. Although patients with prolonged survival after very limited treatment have been reported [3], it is extremely rare.
A number of studies have attempted to determine factors associated with prolonged survival after a diagnosis of MPM. Mark and Shin [3] described microscopic features, such as innocuous nuclei, sparsely distributed glands and tubules, micropapillae, and myoid stroma, which in their series of 6 patients were related to prolonged survival of at least 1 and as many as 5 years after diagnosis [3]. The German mesothelioma register showed that epithelioid subtype, age of less than 60 years at diagnosis, and female gender were associated with a better prognosis [4], but not to the timescale reported herein.
In laboratory studies, Comin and colleagues [5] showed a group of 7 patients who had survived more than 3 years had a significantly lower MIB-1 proliferation index (5.2%) compared with 12 controls with a median survival of 8 months whose MIB-1 proliferation index was 27.8% [5].
Spontaneous regression of MPM has not been previously documented histologically, although it was observed radiologically by Robinson and colleagues [6]. In their report, the patient had two areas of disease, one of which regressed and one that progressed to cause the patient’s demise. Our patient’s lesion, in the paravertebral gutter, which was initially biopsied, was proven to have regressed at his subsequent thoracotomy. Our patient’s tumor, like the anterior lesion in the report of Robinson and colleagues [6] had a prominent inflammatory cell infiltrate. Lymphohistiocytoid mesothelioma is a rare form of mesothelioma associated with a marked lymphocytic and plasmacytic infiltrate. In the small number of reports of this subtype of MPM there have been survivors for a maximum of 6 years after diagnosis [7]. These data suggest that spontaneous regression may be an immune-mediated phenomenon.
Our patient underwent successful local excision of his second lesion, which was located in the anterior chest wall. Allen and colleagues [8] presented a series of localized malignant mesothelioma that had a good prognosis after surgical excision. Half of their series of 23 patients were alive with follow-up ranging from 18 months to 11 years. In the rare case of a localized tumor in a fit patient, an aggressive surgical approach seems justified.
We report a case of MPM with spontaneous regression of pleural disease during a 12-year follow-up and only a single localized recurrence in the chest wall at 6 years. There is some evidence that a prominent host response to tumor may be responsible. Such phenomenon must be borne in mind when interpreting reports of nonrandomized treatment.
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References
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- Sugarbaker D, Flores R, Jaklitsch M, et al. Resection margins, extrapleural nodal status and cell type determine postoperative long term survival in trimodality therapy of malignant pleural mesothelioma: results in 183 patients J Thorac Cardiovasc Surg 1999;117:54-65.[Abstract/Free Full Text]
- Mark EJ, Shin DH. Diffuse malignant mesothelioma of the pleura: a clinicopathological study of six patients with a prolonged symptom free interval or extended survival after biopsy and a review of the literature of long term survival Virchows Arch A Pathol Anat Histopathol 1993;422:445-451.[Medline]
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- Comin CE, Anichini C, Boddi V, Novelli L, Dini S. MIB-1 proliferation index correlates with survival in pleural mesothelioma Histopathology 2000;36:26-31.[Medline]
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- Khalidi HS, Medeiros LJ, Battifora H. Lymphohistiocytoid mesothelioma: an often misdiagnosed variant of sarcomatoid malignant mesothelioma Am J Clin Pathol 2000;113:649-654.[Medline]
- Allen TC, Cagle PT, Churg AM, et al. Localized malignant mesothelioma Am J Surg Pathol 2005;29:866-873.[Medline]
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Abstract
Introduction
