Bilateral Pulmonary Hilar Lymphadenopathy

Diagnostic Radiology

An Unusual Manifestation of Metastatic Renal Cell Carcinoma 1 Robert T. Reinke, M.D., Charles B. Higgins, M.D., Gen Niwayama, M.D., Ronald H. Harris, M.D., and Paul J. Friedman, M.D. Four patients with bilateral pulmonary hilar adenopathy secondary to Iymphangitic spread from renal cell carcinoma were examined. Two additional cases had adenopathy secondary to nasopharyngeal carcinoma. Patients may initially present with bilateral pulmonary lymphadenopathy or as late as 3 Y2 years after the discovery of the primary renal tumor. The mechanism of Iymphangitic spread probably is related to reflux of tumor emboli from the thoracic duct into the bronchomediastinal trunks because of incompetent lymphatic valves. In one case gallium imaging demonstrated bilateral hilar isotopic uptake as well as periaortic uptake. INDEX TERMS: Kidney neoplasms, metastases. Lung neoplasms, radionuclide diagnosis • Lymph nodes, enlargement. (Mediastinal metastasis, 6 [7].337) • (Renal cell carcinoma, 8 [1].324) Radiology 121:49-53, October 1976

• nephrotomography revealed a mass in the middle of the left kidney. Selective renal angiography with epinephrine demonstrated arterial encasement and neovascularity. A radical nephrectomy was performed. Pathological examination revealed a large papillary adenocarcinoma of the left kidney with extension outside the renal capsule, invasion of the left renal vein, and metastasis to the periaortic lymph nodes. Postoperatively, the patient had acute renal failure and required hemodialysis. A thoracic roentgenogram 15 months later showed bilateral hilar enlargement (Fig. 2,A). Bronchoscopic biopsy of the left bronchus revealed metastatic adenocarcinoma. Subsequently, a right-sided pleural effusion appeared along with pleural masses. Over a 2-week period during which prednisone (10 mg, o.d.) and Progesterone (80 mg, o.d.) were given, there was marked enlargement of the hilar nodes and pleural masses on the right side (Fig. 2,B). The patient died and no autopsy was obtained.

hilar adenopathy without significant symptoms is so characteristic of sarcoidosis that this diagnosis is frequently accepted without histologic confirmation. On the other hand, isolated hilar adenopathy due to metastases from an extrathoracic malignancy is considered to be very rare (1). A recent report proposed that hilar adenopathy in a patient with an extrathoracic tumor should not automatically be considered as metastatic but that hilar nodes should be biopsied to rule out a coincidental granulomatous process (1). To our knowledge, only one report of roentgenographic presentation with hilar metastases from renal carcinoma has appeared in the English literature (2). We recently examined 4 patients with bilateral hilar adenopathy secondary to renal carcinoma.

B

ILATERAL

PULMONARY

CASE III: A 29-year-old black man presented with gross hematuria and right flank pain. An excretory urogram revealed a right renal mass. A thoracic roentgenogram showed a right hilar mass while liver and bone scans were normal. Renal angiograms showed a renal mass with neovascularity. Mediastinoscopyand biopsy of a right peribronchial node were positive for adenocarcinoma. A right nephrectomy also revealed adenocarcinoma. A thoracic roentgenogram obtained 4 months later revealed extensive bilateral hilar adenopathy and right paratracheal adenopathy (Fig. 3,A and B). This patient was lost to follow-up.

CASE REPORTS CASE 1: A 19-year-old Chinese woman experienced abdominal swelling followed 8 months later by malaise, fatigue, weakness, and the appearance of a mass in her left groin. Another 8 months later she had one episode of hematuria and experienced edema of her left leg. Initial physical examination revealed a large mass below the left costal margin, extensive bilateral inguinal lymphadenopathy, and a pelvic mass which prompted the initial diagnosis of lymphoma. The thoracic roentgenogram demonstrated bilateral hilar adenopathy without parenchymal abnormalities (Fig. 1,A). Biopsy of the left inguinal node revealed papillary adenocarcinoma. Subsequently, excretory urography demonstrated a calcified left renal mass and ultrasonograms showed a normal spleen and a solid left pelvic mass. An 18FI bone scan was compatible with metastatic disease in the left humerus, left pubic symphysis, and the right femur. A scan with 7 6 Ga citrate (4 mCi) demonstrated both mediastinal and bilateral hilar uptake of the isotope (Fig. 1,B) and also uptake in the periaortic nodes. The patient underwent radiotherapy but was lost to follow-up.

CASE IV: A 66-year-old white man was first evaluated for painless hematuria in January of 1971 and underwent a left radical nephrectomy for a focal papillary renal carcinoma which invaded the left renal vein. Metastatic disease in the lymph nodes was treated with postoperative irradiation. In March 1973, the patient presented with enlargement of the left epididymis; biopsy revealed metastatic adenocarcinoma. He also complained of weight loss, shortness of breath, and one episode of hemoptysis. Skin tests were negative except for a positive histoplasmin test. Excretory urography revealed only absence of the left kidney. In June 1974, the patient was asymptomatic but the thoracic roentgenogram showed bilateral hilar adenopathy and parenchymal branching structures (Fig. 4, A and B). Serological tests for fungi were negative. Cell Bloc preparations of sputum were negative on 12 occasions. Currently, the patient is alive 1 year and 7 months after the appearance of hilar adenopathy.

CASE II: A 61-year-old white man had one episode of hematuria 2 months prior to admission. Two weeks prior to admission, a nontender varicocele developed on the left side. Excretory urography and

1From the Departments of Radiology (R T. R, C. B. H., R H. H., P. J. F.) and Pathology (G.N.), University and VA Hospitals, University of California, elk San Diego, School of Medicine, La Jolla, Calif. Accepted for publication in May 1976.

49

50

ROBERT

T.

October 1976

REINKE AND OTHERS

, ti'

't

1

,"v.., \'. 1

'li

Fig. 1. CASE 1: A. Note on this close-up view that there is bilateral hilar adenopathy and the left upper lobe is entirely normal. B. An anterior scan of the chest and upper abdomen using 4 mCi 67Ga citrate demonstrates obvious bilateral hilar uptake of the isotope in addition to mediastinal isotopic uptake. There is localization of the isotope in the left upper lobe (small black arrow) which is normal on the chest radiograph. Furthermore, periaortic nodes take up the isotope (open arrow) and there is also isotopic uptake in the left renal tumor (large black arrow). Normal liver (L) uptake is present on the right side.

Fig. 2. CASE II: A. A chest radiograph on 10/16/73,15 months after surgical removal of renal cell carcinoma, shows bilateral hilar adenopathy, more marked on the left. In addition, the ductus node is minimally enlarged. B. A chest radiograph dated 4/12/74 shows perihilar enlargement on the right side along with extensive pleural metastases. These increased markedly over the previous 3 weeks during treatment with prednisone and Progesterone.

DISCUSSION

Renal cell carcinoma commonly mimics other disorders.

Nonurologic findings are more common than the classic triad of gross hematuria, flank pain, and mass, which occurs in only about 10-15 % of patients (3, 4). There are no

Vol. 121

BILATERAL PULMONARY HILAR LYMPHADENOPATHY

51

Diagnostic Radiology

'8

Fig. 3. CASE III: A and B demonstrate massive bilateral hilar adenopathy, slightly more marked on the right. In addition, there is a large right paratracheal node and marked enlargement the ductus node. This patient had positive mediastinoscopy for renal cell carcinoma.

Fig. 4. CASE IV: A. A chest roentgenogram demonstrates bilateral hilar adenopathy, more marked on the right, in a patient with previous renal cell carcinoma and previous metastases. There are at least three parenchymal lesions present (one in the right upper lobe, one in the right lower lobe, and one in the left midlung). B. A whole lung tomogram demonstrates the right upper lobe and right lower lobe lesions well. The bilateral hilar adenopathy is well shown, particularly on the left (just below left mainstem bronchus). The right lower lobe lesion has a branching appearance which is most commonly seen in mucoid impaction. In view of the patient's history, this most probably represents tumor embolism (unproved). The patient is alive 1 year and 7 months later.

presenting symptoms directly related to the primary tumor in 30-45 % of the patients (5, 6). Solitary pulmonary metastasis, as the initial presenting feature is seen with renal cancer and occurs in about 2 % of all cases (7, 8). These metastases may precede the detection of the primary tumor by a period of years. While it has been previously noted that metastatic renal carcinoma may cause pul-

monary hilar adenopathy (2, 9, 10), this has not been adequately stressed in the radiological literature. Metastatic dissemination of renal carcinoma occurs along three routes; the most important is direct invasion of the renal vein and its tributaries, with tumor embolization to the lungs. The second route for dissemination is via the retroperitoneal lymphatics and thoracic duct into the su-

ROBERT T. REINKE AND OTHERS

52

Table I:

October 1976

Clinical Features of Patients with Bilateral Hilar Adenopathy Secondary to Metastatic Renal Cell Carcinoma

Case

Presenting Symptoms

II

Left upper quadrant abdominal swelling, left inguinal mass, hematuria Hematuria

III IV

Hematu ria Hematuria

Subsequent Symptoms*

Time Interval t

Parenchymal Abnormalities

Pleural Disease

At admission

Asymptomatic Hemoptysisasymptomatic later

15 mos. postoperative At admission 3 1/2 yrs. postoperative

Easily Identifiable Extrathoracic Disease

Histologic Confirmation of Cause of Pulmonary Adenopathy (Hilar Biopsy)

+

Late

Late

+ +

Late

* At

second presentation because of positive chest ro~ntgenograms. t Time interval between discovery of primary tumor and onset of bilateral hilar adenopathy.

perior vena cava. This route allows malignant cells to traverse the thoracic duct and superior vena cava and directly reach the pulmonary capillary bed (11). Another manifestation of this route includes reflux of tumor emboli into the bronchomediastinal trunks, paratracheal lymphatics, bronchopulmonary lymphatics, and interlobular lymphatics (12). This could result from incompetence of the lymphatic valves (12, 13) and would explain hilar adenopathy as a manifestation of metastatic renal cell carcinoma. Reflux into mediastinal nodes during lymphangiography normally occurs in 5-14 % of patients (11). Since incompetence of the lymphatic valves would be required, this might account for the rarity of this particular manifestation. The third route for dissemination is via Batson's paravertebral venous plexus to the head and neck (14, 15). These three metastatic routes determine roentgenologic manifestations peculiar to renal cell carcinoma. The most common sites of metastasis are the lungs, lymph nodes, liver, and bone (16, 17). The lungs are involved in about 50 % of patients with metastatic renal disease (6). Pulmonary metastases are common, usually consisting of multiple nodules of varying sizes in both lung fields (6). Less common forms of pulmonary metastases include solitary pulmonary nodules, endobronchial lesions, lymphangitis carcinomatosa, and tumor embolism (15, 18). Arkless reported that 11 of 152 patients with renal carcinoma had mediastinal (carinal, hilar, or paratracheal) adenopathy and that, in all 11 of these, concomitant lung seeding occurred, though this was not usually visible roentgenographically (9). This is comparable to the absence of demonstrable parenchymal disease in 3 out of 4 of our patients (TABLE I). This was also the experience of Braman (10), who reported a case of hilar adenopathy with endobronchial metastases without parenchymal abnormalities. SUMMARY

Renal cell carcinoma may manifest itself as bilateral

pulmonary hilar adenopathy. This probably occurs secondary to reflux of tumor emboli through incompetent lymphatic valves into the bronchomediastinal trunks. This may occur as late as 3% years after the discovery of the primary tumor. The gallium scan (positive in one patient) might be a useful procedure for evaluating the extent of the disease. In addition, we have seen two cases of nasopharyngeal carcinoma with a similar presentation. Knowledge of this uncommon picture may prevent clinical confusion.

REFERENCES 1. Winterbauer RH, Belic N, Moores KD: Clinical interpretation of bilateral hilar adenopathy. Ann Intern Med 78:65-71, Jan 1973 2. Khan A, Khan FA: Hypernephroma: A rare cause of bilateral adenopathy, and an example of the importance of tissue diagnosis in suspected cases of sarcoidosis. Chest 66:722-723, Dec 1974 3. Melicow MM, Uson AC: Nomurologic symptoms in patients with renal cancer. JAMA 172: 146-151, Jan 1960 4. Murphy GP, Schirmer HK: The diagnosis and treatment of hypernephroma. Geriatrics 18:354-360, May 1963 5. Holland JM: Natural history and staging of renal cell carcinoma. CA 25:121-133, May/Jun 1975 6. Holland JM: Proceedings: Cancer of the kidney-Natural history and staging. Cancer 32: 1030-1041, Nov 1973 7. Mostofi FK: Pathology and spread of renal cell carcinoma. An International symposium. Boston, Little & Brown, 1967, pp

41-86 8. Watson RC, Fleming RJ, Evans JA: Arteriography in the diagnosis of renal carcinoma: Review of 100 cases. Radiology 91: 888-897, Nov 1968 9. Arkless R: Renal carcinoma: How it metastasizes. Radiology 84:496-501, Mar 1965 10. Braman SS, Whitcomb ME: Endobronchial metastasis. Arch Intern Med 135:543-547, Apr 1975 11. Rosenberger A, Adler 0, Abrams HL: The thoracic duct: Structural, functional and radiologic aspects. CRC Crit Rev Radiol Sci 3:523-541, Dec 1972 12. Weidner WA, Steiner RM: Roentgenographic demonstration of intrapulmonary and pleural lymphatics during lymphangiography. Radiology 100:533-539, Sep 1971 13. Grant T, Levin B: Lymphangiographic visualization of pleural and pulmonary lymphatics in a patient without chylothorax. Radiology

113:49-50, Oct 1974 14. Weigensberg IJ: The many faces of metastatic renal carcinoma. Radiology 98:353-358, Feb 1971

Vol. 121

BILATERAL PULMONARY HILAR LYMPHADENOPATHY

15. Middleton RG: Surgery for metastatic renal cell carcinoma. J Urol 97:973-977, Jun 1967 16. Angervall L, Carlstrom E, Wahlqvist L, et al: Effects of clinical and morphological variables on spread of renal carcinoma in an operative series. Scan J Urol NephroI3:134-140, 1969 17. Robson CJ. Churchill BM, Anderson W: The results of radical nephrectomy for renal cell carcinoma. J Urol 101:297-301, Mar 1969

53

Diagnostic Radiology

18. Caplan H: Solitary endobronchial metastasesfrom carcinoma of kidney. Br J Surg 46:624-625, May 1959

Veterans Administration Hospital 3350 La Jolla Village Dr. San Diego, Calif. 92161

Bilateral pulmonary hilar lymphadenopathy. An unusual manifestation of metastatic renal cell carcinoma.

• Bilateral Pulmonary Hilar Lymphadenopathy Diagnostic Radiology An Unusual Manifestation of Metastatic Renal Cell Carcinoma 1 Robert T. Reinke, M...
402KB Sizes 0 Downloads 0 Views