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Solitary Pulmonary Metastasis Presenting 20 Years After Primary Resection of Wilms Tumor

2005; Elsevier BV; Volume: 80; Issue: 11 Linguagem: Inglês

10.4065/80.11.1514

1942-5546

Joseph G. Parambil, Gregory L. Aughenbaugh, Telma C. Pereira, Delmar J. Gillespie, Jay H. Ryu,

Urological Disorders and Treatments

Wilms tumor is the most common renal malignancy of childhood. Relapse occurs most often within 4 years of initial diagnosis, and the most common site of metastasis is the lung. We describe a 22-year-old man who presented with hemoptysis and a solitary pulmonary lesion 20 years after primary resection of Wilms tumor. Computed tomography of the chest showed an indeterminate pulmonary mass of heterogeneous attenuation with no other in-trathoracic abnormalities. Surgical resection revealed a solitary pulmonary metastasis from Wilms tumor. Further evaluation yielded no evidence of extrathoracic metastases. This case shows that late relapse in the form of a solitary pulmonary mass can occur in patients with Wilms tumor. Wilms tumor is the most common renal malignancy of childhood. Relapse occurs most often within 4 years of initial diagnosis, and the most common site of metastasis is the lung. We describe a 22-year-old man who presented with hemoptysis and a solitary pulmonary lesion 20 years after primary resection of Wilms tumor. Computed tomography of the chest showed an indeterminate pulmonary mass of heterogeneous attenuation with no other in-trathoracic abnormalities. Surgical resection revealed a solitary pulmonary metastasis from Wilms tumor. Further evaluation yielded no evidence of extrathoracic metastases. This case shows that late relapse in the form of a solitary pulmonary mass can occur in patients with Wilms tumor. Wilms tumor, or nephroblastoma, is the most common primary renal malignancy of childhood. With an annual incidence of 8 cases per 1 million children younger than 15 years, it represents 6.3% of cases of childhood cancers1Breslow N Olshan A Beckwith JB Green DM Epidemiology of Wilms tumor.Med Pediatr Oncol. 1993; 21: 172-181Crossref PubMed Scopus (381) Google Scholar but is rare in adults.2Babaian RJ Skinner DG Waisman J Wilms' tumor in the adult patient: diagnosis, management, and review of the world medical literature.Cancer. 1980; 45: 1713-1719Crossref PubMed Scopus (74) Google Scholar When Wilms tumor recurs, the most common site of distant metastasis is the lung.3Sutow WW Breslow NE Palmer NF D'Angio GJ Takashima J Prognosis in children with Wilms' tumor metastases prior to or following primary treatment: results from the first National Wilms' Tumor Study (NWTS-1).Am J Clin Oncol. 1982; 5: 339-347Crossref PubMed Scopus (40) Google Scholar Relapse, either local or with distant metastases, occurs most often within 4 years of initial presentation.4Firoozi F Kogan BA Follow-up and management of recurrent Wilms' tumor.Urol Clin North Am. 2003; 30: 869-879Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar We describe a 22-year-old man who presented with hemoptysis and a solitary pulmonary mass that proved to be an isolated metastasis from a Wilms tumor resected 20 years previously. On computed tomography of the chest, this pulmonary lesion had features of a heterogeneously enhancing pulmonary mass. A 22-year-old white man, a current smoker, presented to the Mayo Clinic in Rochester, Minn, for evaluation of 2 episodes of hemoptysis. The first episode had occurred 6 weeks before presentation with symptoms of cough associated with physical exertion and productive of a blood clot. The second episode occurred 1 week before presentation with similar symptoms and expectoration of a 6-cm blood clot. The tissue was evaluated histopathologically and was characterized as “necrotic debris.” The patient's medical history was remarkable for stage II Wilms tumor of the left kidney diagnosed at age 26 months, when he presented with a palpable abdominal mass and hematuria. Surgical treatment of the malignant neoplasm included en bloc resection of the left kidney, hilar structures, left adrenal gland, and proximal two thirds of the left ureter. On histopathologic examination, the tumor had penetrated the renal capsule and invaded the renal sinus vessels. However, the surgical margins were free of disease, and the tumor had a favorable histology (absence of anaplasia). Retroperitoneal lymphadenectomy yielded benign lymph nodes. Surgical resection was followed by 18 weeks of adjuvant chemotherapy that included a combination of vincristine and actinomycin D. Chest radiography performed periodically until the patient was 10 years old showed no evidence of recurrence. The patient had no history of bleeding diathesis, recent trauma, respiratory infections, or other lung diseases. On physical examination, he appeared healthy and in no acute distress. He was afebrile, and his vital signs were normal. There were no palpable lymph nodes, and his lungs sounded clear. Abdominal examination revealed a healed surgical scar and no palpable masses or tenderness. Laboratory studies yielded normal results on complete blood cell count, coagulation parameters, and screening serum chemistries. Chest radiography showed a solitary pulmonary mass measuring 4.4 × 4.1 cm in the right lower lobe. Computed tomography of the chest performed with intravenous contrast revealed a sharply marginated lesion with heterogeneous enhancement (Figure 1). No associated mediastinal or hilar adenopathy was noted. Computed tomography of the abdomen and pelvis showed postoperative changes from the previous left nephrectomy and retroperitoneal lymphadenectomy but otherwise normal findings. Bronchoscopy revealed no endobronchial lesions, and no biopsy specimens were obtained. Surgical resection of the indeterminate pulmonary mass was advised because of the recurrent hemoptysis, the relatively large size of the lesion, and the patient's history of cancer. A right thoracotomy with right lower lobectomy and mediastinal lymphadenectomy was performed. Histopathologic examination revealed a malignant monophasic tumor composed of small round to oval cells with scant cytoplasm and overlapping nuclei with evenly distributed chromatin and small nucleoli, characteristic of the blastema pattern of Wilms tumor (Figure 2). Mitotic figures were abundant. Although triphasic patterns are most characteristic in Wilms tumors, biphasic and mono-phasic lesions are often observed. Immunohistochemical stains showed the tumor cells to be negative for WT1 (a marker that is present in most but not all Wilms tumors). However, the neoplasm was strongly positive for CD56 (a marker present in 100% of Wilms tumors), thereby establishing the diagnosis of metastatic Wilms tumor.5Muir TE Cheville JC Lager DJ Metanephric adenoma, nephrogenic rests, and Wilms' tumor: a histologic and immunophenotypic comparison.Am J Surg Pathol. 2001; 25: 1290-1296Crossref PubMed Scopus (139) Google Scholar Bilateral whole-lung irradiation and adjuvant chemotherapy with etoposide, carboplatin, and cyclophosphamide were initiated. This case shows that late relapse with a solitary pulmonary metastasis can occur in patients with Wilms tumor, the most common renal malignancy of childhood. Most patients with Wilms tumor come to medical attention before the age of 5 years.6Merguerian PA Chang B Pediatric genitourinary tumors.Curr Opin Oncol. 2002; 14: 273-279Crossref PubMed Scopus (12) Google Scholar Surgical resection is the primary means of achieving local control. Operative staging and histological grading are used to guide subsequent management, which may include chemotherapy and radiotherapy. The overall cure rate for Wilms tumor currently exceeds 85%.6Merguerian PA Chang B Pediatric genitourinary tumors.Curr Opin Oncol. 2002; 14: 273-279Crossref PubMed Scopus (12) Google Scholar The lung is the most common site of metastasis from Wilms tumor.3Sutow WW Breslow NE Palmer NF D'Angio GJ Takashima J Prognosis in children with Wilms' tumor metastases prior to or following primary treatment: results from the first National Wilms' Tumor Study (NWTS-1).Am J Clin Oncol. 1982; 5: 339-347Crossref PubMed Scopus (40) Google Scholar About 10% of patients have detectable pulmonary metastases at the time of diagnosis,7D'Angio GJ Rosenberg H Sharples K Kelalis P Breslow N Green DM Position paper: imaging methods for primary renal tumors of childhood: costs versus benefits [published correction appears in Med Pediatr Oncol. 1993;21:695].Med Pediatr Oncol. 1993; 21: 205-212Crossref PubMed Scopus (52) Google Scholar and 47% have lung metastases at relapse.8Green DM Fernbach DJ Norkool P Kollia G D'Angio GJ The treatment of Wilms' tumor patients with pulmonary metastases detected only with computed tomography: a report from the National Wilms' Tumor Study.J Clin Oncol. 1991; 9: 1776-1781PubMed Google Scholar Nearly all relapses from this tumor are diagnosed within 4 years of initial presentation.4Firoozi F Kogan BA Follow-up and management of recurrent Wilms' tumor.Urol Clin North Am. 2003; 30: 869-879Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar In contrast to the course in our patient, pulmonary metastases from Wilms tumor generally are not associated with respiratory symptoms and are identified by imaging studies.9D'Angio GJ Evans AE Breslow N et al.The treatment of Wilms' tumor: results of the National Wilms' Tumor Study.Cancer. 1976; 38: 633-646Crossref PubMed Scopus (360) Google Scholar10Breslow NE Churchill G Nesmith B et al.Clinicopathologic features and prognosis for Wilms' tumor patients with metastases at diagnosis.Cancer. 1986; 58: 2501-2511Crossref PubMed Scopus (71) Google Scholar Wilms tumor usually appears as a large spherical intrarenal mass on computed tomography, often with a well-defined rim of compressed renal parenchyma or pseudocapsule surrounding the tumor.11Maudgil DD McHugh K The role of computed tomography in modern paediatric uroradiology.Eur J Radiol. 2002; 43: 129-138Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar Pulmonary me-tastases from Wilms tumor have been described as homogeneous nodules or masses varying in number from 1 to 40 and ranging in size from 4 to 65 mm.8Green DM Fernbach DJ Norkool P Kollia G D'Angio GJ The treatment of Wilms' tumor patients with pulmonary metastases detected only with computed tomography: a report from the National Wilms' Tumor Study.J Clin Oncol. 1991; 9: 1776-1781PubMed Google Scholar12Bond JV Martin EC Pulmonary metastases in Wilms' tumour.Clin Radiol. 1976; 27: 191-195Abstract Full Text PDF PubMed Scopus (11) Google Scholar, 13Robertson PL Boldt DW De Campo JF Paediatric pulmonary nodules: a comparison of computed tomography, thoracotomy findings and histology.Clin Radiol. 1988; 39: 607-610Abstract Full Text PDF PubMed Scopus (40) Google Scholar, 14Wilimas JA Douglass EC Magill HL Fitch S Hustu HO Significance of pulmonary computed tomography at diagnosis in Wilms' tumor.J Clin Oncol. 1988; 6: 1144-1146Crossref PubMed Scopus (47) Google Scholar, 15Meisel JA Guthrie KA Breslow NE Donaldson SS Green DM Significance and management of computed tomography detected pulmonary nodules: a report from the National Wilms Tumor Study Group.Int J Radiat Oncol Biol Phys. 1999; 44: 579-585Abstract Full Text Full Text PDF PubMed Scopus (66) Google Scholar Solitary pulmonary nodules can be a diagnostic challenge. Approximately one half of uncalcified solitary pulmonary nodules are malignant.16Tan BB Flaherty KR Kazerooni EA Iannettoni MD The solitary pulmonary nodule.Chest. 2003; 123: 89S-96SCrossref PubMed Scopus (273) Google Scholar Although most of these malignant lesions are primary lung cancers, solitary metastases have accounted for up to 23% of resected solitary pulmonary nodules, with the breast and the colon representing the most common primary sites of origin.17Swanson SJ Jaklitsch MT Mentzer SJ Bueno R Lukanich JM Sugarbaker DJ Management of the solitary pulmonary nodule: role of thoracoscopy in diagnosis and therapy.Chest. 1999; 116: 523S-524SCrossref PubMed Google Scholar The likelihood of malignancy increases with the size of the pulmonary nodule. Uncalcified solitary pulmonary nodules larger than 3 cm (usually referred to as a “mass”) as seen in our patient are associated with a high likelihood of malignancy, but the young age of our patient made bronchogenic carcinoma an unlikely diagnosis.16Tan BB Flaherty KR Kazerooni EA Iannettoni MD The solitary pulmonary nodule.Chest. 2003; 123: 89S-96SCrossref PubMed Scopus (273) Google Scholar Carcinoid tumor, lymphomas, and sarcomas can present as primary pulmonary malignancies in young adults.16Tan BB Flaherty KR Kazerooni EA Iannettoni MD The solitary pulmonary nodule.Chest. 2003; 123: 89S-96SCrossref PubMed Scopus (273) Google Scholar17Swanson SJ Jaklitsch MT Mentzer SJ Bueno R Lukanich JM Sugarbaker DJ Management of the solitary pulmonary nodule: role of thoracoscopy in diagnosis and therapy.Chest. 1999; 116: 523S-524SCrossref PubMed Google Scholar Surgical resection has been performed for management of pulmonary metastases from a variety of solid tumors, particularly those that are relatively insensitive to chemo-therapy.18Rusch VW Pulmonary metastasectomy: current indications.Chest. 1995; 107: 322S-331SCrossref PubMed Google Scholar Complete resection of metastatic disease, the absence of extrathoracic disease, and a long disease-free interval between initial diagnosis and relapse are factors that define cases that are most likely to benefit from pulmonary metastasectomy.18Rusch VW Pulmonary metastasectomy: current indications.Chest. 1995; 107: 322S-331SCrossref PubMed Google Scholar To our knowledge, the interval between the primary resection and the diagnosis of pulmonary metastasis in the current case is the longest ever reported in patients with Wilms tumor and broadens the spectrum of behavior that can be seen with this tumor. In evaluating patients with a solitary pulmonary nodule and a distant history of previous cancer, the possibility of late metastasis should be considered.