Ann Thorac Surg 2007;83:1893-1894
© 2007 The Society of Thoracic Surgeons
Case Reports
Prolonged Survival in a Patient With Recurrent Pulmonary Metastases Secondary to Mucinous Cystadenocarcinoma of the Appendix With Pseudomyxomatous Peritonei
Aftab A. Khan, MD, MRCSa,*,
Jeymi Tambiah, MS, FRCSa,
Paul Cane, FRCPathb,
Loic Lang-Lazdunski, MD, PhDa
a Department of Thoracic Surgery, Guys Hospital, Kings College London, London, United Kingdom
b Department of Histopathology, Guys Hospital, Kings College London, London, United Kingdom
Accepted for publication December 4, 2006.
* Address correspondence to Dr Khan, Department of Thoracic Surgery, Guys Hospital, London SE1 9RT UK (Email: iftee{at}hotmail.com).
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Abstract
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We report a 65-year-old man presenting with recurrent pulmonary metastases 20 years after an appendectomy for mucinous cystadenocarcinoma with pseudomyxomatous peritonei. He underwent bilateral staged metastatectomies for metastases 7 years after the diagnosis and further metastasectomy after a recent recurrence. This is a rare case of recurrent pulmonary metastatic mucinous cystadenocarcinoma, and despite poor prognosis and nondefinitive initial treatment, this patient remains alive and well 20 years later.
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Introduction
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The long-term prognosis of appendiceal mucinous cystadenocarcinoma (MCA) with pseudomyxomatous peritonei (PMP) is poor in the absence of radical therapy. Rarely, presentation with pulmonary metastasis is seen.
A 65-year-old man initially presented with an acute abdomen and underwent laparotomy in 1985. He was found to have a ruptured appendix mucocele and PMP, and an appendicectomy was performed. Histopathology revealed the appendix mass to be MCA. Radiotherapy was subsequently given to his abdomen. He recovered uneventfully, but was lost to follow-up.
Seven years later in 1992, a chest roentgenogram taken to investigate persistent cough revealed bilateral pulmonary lesions. Computed tomography (CT) showed a large mass in his right upper lobe and two lesions in his left lung. No recurrence of disease was seen in the abdomen.
Staged resections were performed through a bilateral thoracotomy. The first was a right-sided operation that revealed a 10-cm lesion containing gelatinous material distending the upper lobe bronchus, with lung consolidation and abscess formation. A right upper lobectomy with sleeve resection was performed.
A left thoracotomy 3 months later revealed a 6-cm tumor within the lingula and a 3-cm tumor located in the lower lobe. A lingulectomy removing the upper lobe mass and metastasectomy of the lower lobe mass were performed. Pathology confirmed these lesions were metastatic MCA appendix deposits. Recovery was uneventful, but again he was lost to follow-up.
Thereafter in 2005, he was investigated for breathlessness and chest roentgenogram showed a new right-sided lesion. Chest CT and positron emission tomography confirmed a large, lower lobe apical segmental tumor (Fig 1). The forced expiratory volume in 1 second (FEV1) was measured at 1.1 liters. Metastasectomy was subsequently performed through a redo thoracotomy. Pathology revealed the lesion to be MCA (Fig 2). Immunostaining was positive for CK-20 and negative for CK-7, and thyroid transcription factor-1 antibodies confirmed its abdominal origin rather than being primary lung MCA (Fig 3
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Fig 1. Preoperative computed tomography scan of the chest shows a large, loculated soft tissue mass with curvilinear mineralization in the apical segment of right lower lobe.
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Fig 2. Hematoxylin and eosin staining shows the tumor consists of lakes of mucin lined by goblet cells and set within a fibrous stroma (x200 original magnification).
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Fig 3. Immunohistochemical section from recent lung metastasis specimen stained positive for cytokeratin-20 antibody, confirming the abdominal origin of the mucinous cystadenocarcinoma (x200 original magnification).
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The patient was reviewed 9 months after this operation. The chest roentgenogram was satisfactory, he has good exercise tolerance, and his FEV1 remains at 1.1 liters.
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Comment
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PMP results from rupture of an appendix mucocele. Appendiceal mucoceles in turn can result from benign (cyst, mucosal hyperplasia, cystadenoma) or malignant (cystadenocarcinoma) processes. The incidence of malignant appendiceal tumors is as high as 0.5% of appendicectomy specimens. Of these, MCAs represent about 8% [1]. They are a cystic variant of adenocarcinoma and produce copious mucus. The prognosis of MCA presenting with PMP is poor [2].
Although intraabdominal and pelvic spread is common, extraabdominal spread is uncommon. Sporadic reports of metastasis from MCA appendix to other organs exist [3]. Pulmonary metastasis is extremely rare, with only a handful of cases reported [4, 5].
Surgical treatment of MCA appendix presenting with PMP usually consists of right hemicolectomy if the primary tumor (mucocele) appears malignant and appendicectomy if it appears benign. This could be a difficult decision to make.
More recent protocols consist of aggressive cytoreductive surgery, including total omentectomy, and often splenectomy, rectosigmoid colectomy, and resection of other involved organs, followed by heated intraperitoneal chemotherapy. This treatment has been pioneered and developed by Sugarbaker and colleagues [2], who have just reported a 63% survival at 5 years from a series of 385 patients treated with this approach. The natural history of this disease is such that they state "in the absence of this [cytoreductive surgery and intraperitoneal chemotherapy] approach no long term survivors have ever been reported in the medical literature" [3].
Mortman and colleagues [4] have also reported 3 patients with metastatic pulmonary MCA appendix. All underwent initial cytoreductive surgery with heated intraperitoneal chemotherapy. On discovering their lung metastases on follow-up, 2 patients underwent further lobectomy with lymphadenectomy and 1 was treated with wedge resection. All were reported as being disease-free at 2 to 8 years of follow-up.
The case presented here reports recurrent pulmonary metastatic MCA appendix and is one of a handful of cases of pulmonary metastases reported in the literature to date. Also, despite receiving suboptimal treatment at presentation compared with recent opinion, this patient has defied all known expectations and is currently well 20 years after initial diagnosis. Finally, serial metastasectomy was well tolerated, with no deterioration in lung function. This observation was probably due to excision of nonfunctional lung tissue, allowing remaining viable lung to expand.
In conclusion, pulmonary metastasis of appendiceal MCA with PMP is very rare. Moreover, recurrence can occur many years after initial presentation, suggesting that such patients should have lifelong follow-up with at least an annual chest roentgenogram. Serial lung metastasectomy is well tolerated but cannot be regarded as curative. Also, this case of prolonged survival challenges currently accepted views of the natural history of this disease.
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References
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- Deans GT, Spence RA. Neoplastic lesions of the appendix Br J Surg 1995;82:299-306.[Medline]
- Sugarbaker PH, Chang D. Results of treatment of 385 patients with peritoneal surface spread of appendiceal malignancy Ann Surg Oncol 1999;6:727-731.[Medline]
- Dhage-Ivatury S, Sugarbaker PH. Update on the surgical approach to mucocele of the appendix J Am Coll Surg 2006;202:680-684.[Medline]
- Mortman KD, Sugarbaker PA, Shmookler BM, DeGuzman VC, Soberman MS. Pulmonary metastases in pseudomyxoma peritonei syndrome Ann Thorac Surg 1997;64:1434-1436.[Abstract/Free Full Text]
- Lee BY, Kim HS, Lee SH, et al. Pseudomyxoma peritonei: extraperitoneal spread to the pleural cavity and lung J Thorac Imaging 2004;19:123-126.[Medline]
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Abstract
Introduction
