| Japanese Journal of Clinical Oncology | Pages |
Introduction
Case Report
Discussion
References
Prolonged Survival in a Nasopharyngeal Carcinoma Patient with Multiple Metastases: A Case Report and Review of the Literature
INTRODUCTION
Nasopharyngeal carcinoma (NPC) has an incidence of 5.36/100 000 persons/year and ranks as the 6th and 11th leading cause of cancer death in males and females respectively in Taiwan (1 ). Most cases of early-stage disease can be cured by local radiotherapy, with long-term survival (2 ,3 ). A survey reported in 1982/83 in Taiwan showed that the actuarial and relapse-free survivals of 966 patients at all stages were 82% and 49% at one year, 43% and 33% at 5 years and 36% and 22% at 10 years respectively, supporting Ho's classification of 5 stages for survival analysis (4 ,5 ). Actuarial survival for stages I, II, III, IV according to Ho's classification (6 ) has been reported to be 80.8%, 71.5%, 40.7% and 17.7% respectively at 5 years and 48.4%, 33.4%, 30% and 9.5% respectively at 10 years in Hong Kong, although stage V (any distant metastases) data were not reported (7 ).
In nasopharyngeal carcinoma patients diagnosed as having metastatic disease ab initio, the prognosis is poor, possibly reflecting the intrinsically aggressive behavior of the tumor (8 ). When metastatic disease develops after curative radiotherapy, bone is the most frequently involved site, followed by the lungs and liver. More than half of all patients have more than one metastatic site (9 ). Although the median survival of all patients with metastatic disease has been reported to be 8 months (9 ), [lung only, 11.8-12.5 months (5 ,9 ), bone only 6.5-8 months (5 ,9 ), liver, 3.8-5.4 months (5 ,9 ), skin and lymph node involvement below the clavicle, 20 months (5 )], long-term survival is occasionally observed (5 ,8 ,9 ,10 ).
CASE REPORT
A 36-year-old male was diagnosed as having nasopharyngeal carcinoma in January 1985. The histology report was poorly differentiated epidermoid cell carcinoma (Fig. 1 ), clinical staging T4N0M0, stage IV according to Ho's classification (6 ). CT scan of the nasopharynx and skull base showed a large right-sided abnormal soft tissue density in the nasopharynx, involving the right maxillary, ethmoid and sphenoid sinuses, with bony destruction of the right pterygoid plate, roof of the nasal cavity and floor of the sphenoid sinus. Curative radiotherapy of 70 Gy was given and control of the primary lesion was good (11 ).
DISCUSSION
Our patient survived 82 months from the time metastatic disease was first diagnosed and almost 10 years from the first presentation. This may have reflected the biologic behavior of his disease, having attained a 3.5-year disease-free interval after curative radiotherapy for T4 disease. Even after metastasis had occurred, the tumor was still highly responsive to treatment by many different chemotherapeutic agents, especially cisplatin/5-fluorouracil combination chemotherapy as well as single-agent ifosfamide.
Reports of long-term survival of patients with nasopharyngeal carcinoma after development of metastatic disease have been limited. In the largest serial report of 17 long-term survivors who survived more than two years after initial diagnosis of metastatic disease, analysis concluded that patients with only intrathoracic metastases, showing complete response to chemotherapy, and one patient with a completely resectable thyroid metastasis, were able to survive prolonged periods of more than 5 years (8 ). Comparable survival data have also been reported for three other patients with a similar scenario of lung-only metastases (5 ). Shorter survival periods were attained by patients with bone-only, or bone and intrathoracic metastases (8 ). The longest survivor was a 25-year-old man in whom multiple foci of pulmonary metastases were reduced to a single focus after chemotherapy (cisplatin-based). Radiotherapy was then given to the residual pulmonary disease and the patient remained clinically disease-free 117 months later (8 ,9 ). Other reports have included a patient who received an anti-lymphoma regimen consisting of cyclophosphamide, vincristine, procarbazine and prednisolone (20 ), and survived at least 12 years; the histology of the tumor was reviewed and confirmed to be anaplastic carcinoma (11 ).
Nasopharyngeal carcinoma is a very chemosensitive tumor. Excellent response has been attained with cisplatin-based chemotherapy in patients with recurrent or previously untreated advanced nasopharyngeal carcinoma. The response rates for combinations of cisplatin/5 fluorouracil/leucovorin, belomycin/epirubicin/cisplatin and ifosfamide/cisplatin for metastatic undifferentiated carcinoma of the nasopharynx have been around 60-90%, with complete response rates of 20-40% (11 ,13 -16 ,18 -19 ) and from the subset of complete responders there appears to be a subgroup of potentially long-term survivors. The typical profile of a long-term survivor with metastatic disease would be: initial diagnosis before the age of 40 years (7 ,8 ), long disease-free survival after initial radiotherapy prior to relapse, metastatic disease limited to the lung (with or without mediastinal lymph node involvement), without local recurrence, and complete response to aggressive multimodal therapy (8 ), the chemotherapy regimen being platinum based (8 ,11 ,18 ) and ifosfamide perhaps playing a potentially significant role.
Another unusual feature noted in our patient was the fact that metastatic disease remained stable and unchanged without any treatment for one year. Our patient underwent bronchoscopic biopsy at that time and the pathology was carcinoma, poorly differentiated type. Even after four previous chemotherapy combinations, the patient still attained near-complete remission with ifosfamide lasting 11 months. To date, there has been little information on single agent ifosfamide in NPC, although preliminary data have been reported (17 ). Nasopharyngeal carcinoma patients with mainly thoracic metastases may still enjoy prolonged disease-free control if the tumor is chemosensitive.
References
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Last modification: 19 May 1998
Copyright© Japanese Journal of Clinical Oncology, 1997.
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