•
Thoracic Manifestations of Renal Celf
Diagnostic Radiology
Carcinoma 1
Anne Latour, M.D., F.R.C.P.(C)2, and Harry S. Shulman, M.D., F.R.C.P.(C) Over 1fJ of patients with renal cell carcinoma will present with metastases with no symptoms referable to the kidney. Early metastatic disease is a result of the unique accessibility of the kidney to Iymphohematogenous pathways. The thorax is particularly vulnerable. Several of the more unusual manifestations involVing the thorax are illustrated. including 1 case with intra-alveolar metastases. Kidney neoplasms, metastases • (Renal cell carcinoma. 8[1].324). (Thoracic metastases, 6[8].330). Thorax, neoplasms
INDEX TERMS:
Radiology 121:43-48, October 1976
•
•
HE VARIETY of pathways by which renal cell carci-
Lymphogenous - - - _ r e g i o n a l nodes - - - - - _ • . Mediastinum
Tnoma can metastasize is responsible for the wide and
• Pulmonary lymphatics
confusing spectrum of clinical and radiographic presentations. Occasionally, the manifestations are sufficiently unusual that their malignant nature is not initially apparent. In fact, 30-45 % of patients with renal cell metastases have no symptoms referrable to the kidney (1, 5). This tumor has been reported involvlnq such unusual sites as larynx (6), gallbladder (12), meninges (22), breast (7), and ear (18), but the lung is the most common site (5). In Bennington's autopsy review of 523 cases (5), a 55% incidence of pulmonary metastases was found. Mediastinal involvement has been reported in 8-10 cases (3). Thus, over 60 % of metastatic renal cell carcinoma will involve the thorax at some time before death. From another point of view, however, less than 5 % of all metastatic disease to the lungs is renal in origin (5). Although the most common form of metastasis to the thorax is that of multiple pulmonary nodules, many other forms of thoracic involvement may occur including large, solitary pulmonary masses (20), pleural involvement with effusion or pleural masses (14), hilar or mediastinal adenopathy (13), bony thorax involvement with rib or vertebral destruction, and subpleural or paravertebral soft-tissue masses (20). Rarer thoracic presentations include direct extension of tumor from the renal vein to the right atrium, ventricle, and main pulmonary artery (21) [often associated with embolism of tumor tissue into the lung (26)], calcification in tumor thrombus in the pulmonary arteries (24), and pulsating anterior chest wall mass (19). Two extremely rare and puzzling features of hypernephroma are the occasional appearance of metastases up to 20 years or more after removal of the primary (11) and, conversely, the spontaneous disappearance of pulmonary metastases (2, 16).
Lymphohematogenous---. lymphatics \ . thoracic duet
shunyo
Hematogenous - Renal
veins~
\
.>
Right heart - - - . . lungs
lye
1,
Batson s
Plexus~.
Intercostal &_ _ • Lungs
. Bronchial veins
. Mediastinum • Pleura · Thorcci c cage
Diag. 1.
The routes of renal cell carcinoma spread to the thorax.
Lymphogenous; mainly to regional nodes, and only rarely to distant nodes, probably because of nodal obstruction. Lymphohematogenous; when a node becomes blocked with tumor, shunting of cells presumably occurs both antegrade and retrograde (9). Once completely filled with tumor, the surrounding connective tissue may be invaded, including small veins. In addition, cells reaching the lymphatics eventually enter the thoracic duct which empties into the innominate vein and thus reach the lung. As Coman has discussed (10), once tumor emboli have reached the lung, they may gain access to arteriovenous shunts in the lung, thus bypassing the capillary network to reach the systemic circulation. Hematogenous; by direct extension into the inferior cava where there is access: (a) Antegrade to the heart and lung (the most common route of spread and responsible for the "cannon ball" picture of renal cell metastases). (b) Retrograde in the vena cava or from the renal vein to the pelvis. (c) Shunting from the caval to the portal system (and ultimately back to the inferior vena cava via hepatic veins). (d) Batson's plexus; a network of valveless veins surrounding the vertebral column (and spinal cord), extending from the skull almost to the pelvis and via the vasa vaso-
ANATOMIC CONSIDERATIONS
The kidneys' relationships provide hypernephroma with a unique access to many metastatic routes (Diag. 1). Metastases may be: --
From the Department of Radiology, Sunnybrook Medical Centre, University of Toronto, Ontario, Canada. Presented in part at the Canadian Association of Radiologists Annual Meeting, May 1975. 2 Current address: Department of Radiology, Queen Mary Veteran's Hospital, 4565 Queen Mary Rd., Montreal, Quebec, Canada. elk 1
43
44
ANNE LATOUR AND HARRY S. SHULMAN
October 1976
Fig. 1. Both lungs are fairly symmetrically involved with a predominantly interstitial process, although air-space disease is present in the right upper lobe. The close-up of the right lower lung shows to better advantage the characteristic coarsely thickened interstitial septa of Iymphangitic carcinomatosis (arrows). Hilar adenopathy is partially obscured by the adjacent lung involvement.
A
B
Fig. 2. A. This radiograph was obtained 3 years prior to the last admission and shows the changes of a previous right thoracoplasty for T.B. B. The current radiograph shows an enlarged right hilus, proved at autopsy to be enlarged nodes secondary to metastatic renal cell carcinoma.
Vol. 121
THORACIC MANIFESTATIONS OF RENAL CELL CARCINOMA
45
Diagnostic Radiology
rum almost to the knees and elbows. Being valveless, changes in thoracic or intra-abdominal pressure (for example, with coughing or straining) can result in blood flowing caudally, as well as cranially. Anastomoses are extensive and include azygos, hemiazygos, lumbar, bronchial, intercostal, and other systemic veins. It is this plexus with its multiple systemic connections that is likely responsible for some of the more bizarre metastatic sites previously mentioned. Direct invasion; in addition to immediately surrounding tissues becoming involved, direct invasion may result in smallvascular structures being invadedand thus ultimately providing access to the systemic circulation. The following cases illustrate some of the unusual presentations of renal cell carcinoma in the thorax and include a metastatic manifestation in the lungs which, to our knowledge, has not been previously reported. CASE REPORTS CASE I (T.B.): This 49-year-old man presented with hip pain 7 wk. after a fall but no fracture was found. While in the hospital, he became progressively dyspneic. The chest radiograph revealed a predominantly interstitial process involving both lungs and probable bilateral hilar adenopathy (Fig. 1). Metastatic survey showed multiple radiolucent defects in the skull while a radionuclide image revealed that the liver was enlarged and contained multiple defects. A percutaneous trephine biopsy of the lung showed undifferentiated tumor. At autopsy, a left renal cell carcinoma had invaded the renal vein and metastases were found in lungs, cervical, mediastinal, and hilar nodes, heart. and pericardium. Microscopically, the pulmonary lymphatics and perilymphatic interstitium were diffusely involved, with scattered nodules of metastatic carcinoma within the lung parenchyma and on the pleural surface.
Fig. 3. The admission radiograph reveals an opaque right hemithorax with mediastinal shift to the contralateral side. No comment can be made regarding possible pulmonary parenchymal involvement on the right side. No abnormality is seen on the left side.
COMMENT: This man died of respiratory failure due to diffuse metastatic disease and yet had no symptoms referrable to his kidney. The possibility of metastatic pulmonary malignancy was raised, although the kidney was not considered a likely primary source. Growth into the renal vein is the most common form of spread and the tumor may extend up and down the vena cava, into the heart and pulmonary artery, and may even involve the ovarian and spermatic veins (especially on the left). Spread to the lungs in this case may have occurred along two pathways-direct embolization from the renal vein and via Iymphohematogenous spread. The Iymphangitic pattern illustrated in this case is most often associated with adenocarcinoma of lung, stomach, breast, and pancreas (17, 25). In a review of 275 cases of Iymphangitic carcinomatosis, Yang found only four renal primaries (1.5%) (25). II (R.D.): This 47-year-old, asymptomatic man was found to have a right hilar mass on a routine chest radiograph (Fig. 2). Evidence of a previous thoracoplasty for tuberculosis was present. No surgery was performed as the patient was medically unfit. Grand mal seizures subsequently developed and he deteriorated rapidly and died. Postmortem examination revealed renal cell carcinoma involving the right kidney and adrenal gland, brain, and right hilar nodes. Isolated small metastases were found in the right lung. CASE
COMMENT: When hilar involvement is present, it is usually associated with widespread pulmonary disease,
Fig. 4. Destruction of the third rib posteriorly (*) is accompanied by a mass with the characteristic configuration of an extrapulmonary lesion. Although metastatic carcinoma was the most likely diagnosis, the kidney was not considered a likely primary source.
particularly of the Iymphangitic variety as in CASE I. The pulmonary parenchymal component, however, may not be radiographically apparent. Hilar adenopathy as the only radiological manifestation of metastatic disease is uncommon. In Arkless' series of 152 patients (3), 11 had
46
ANNE LATOUR AND HARRY S. SHULMAN
Fig. 5.
October 1976
Postero-anterior and lateral radiographs reveal a sharplydefinedsolitary mass.Onthe lateral film, the poor definitionanteriorly is the result of chest wall invasion. No other lesion could be identified radiographically.
mediastinal adenopathy involving carinal, hilar, or paratracheal areas. In fact, 5 of this group had teft-sided cervical or supraclavicular adenopathy. This pattern is typical of other infradiaphragmatic tumors that spread via lymphatics. CASE III (J.H.): This 67-year-old man was admitted complaining of right-sided chest pain, shortness of breath, and abdominal distension for 5 days. The chest radiograph revealed an opacifiedright hemithorax with slight shift of the mediastinum to the left (Fig. 3). Seven hundred milliliters of serosanguinous fluid was removed from the right pleural space. Ten days later, massive rectal bleeding developed due to right-sideddiverticulitis which required right hemicolectomy. Postoperatively, he became oliguric and died. At autopsy,the right lung was found to be completelyencased in pleurathat was extensively infiltrated with metastatic renalcell carcinoma. Metastatic involvement of the lung parenchyma was present, but not extensive.
COMMENT: As with CASES I and II, no symptoms referrable to the kidney were present prior to death. Although the lungs were involved with metastatic tumor at autopsy, this was obscured by the rather extensive and unusual pleural involvement. Pleural involvement is often secondary to the lung involvement, but in this case the far more extensive pleural disease could also be explained on the basis of communications between parietal pleural veins and Batson's plexus (4). CASE IV (G.F.): This 69-year-old man presented with fatigue, right flank pain, and hematuria. A right nephrectomy was performed for renal cell carcinoma. A chest radiographobtained 14 mo. postoperatively (Fig. 4) demonstrateddestruction of the right third rib associated with a mass.
COMMENT: "Disappearing bone" is one of the hallmarks of metastatic renal cell carcinoma, although metastatic thyroid carcinoma can produce a similar appearance. However, an associated mass such as was seen in this case is uncommon. Chest wall involvement might occur via systemic spread following passage of cells through the lungs, but is more likely a result of communications between the inferior vena cava, Batson's plexus, and intercostal veins. CASE V (J.R.): This59-year-old manhada nephrectomy 3 yearsprior to this admission; follow-up chest radiographs were negative for 18 mo. He was then lost to follow-up for the next 18 mo., after which he returned to his physician complaining of right-sided chest pain. A chest radiographwas obtained(Fig. 5) and the diagnosisconfirmed by open biopsy.
COMMENT: About 2 % of metastatic renal cell carcinomas present as a solitary pulmonary mass (8) and these generally have a fairly good prognosis. Chest wall invasion, however, is a poor prognostic sign and in most cases an indication of inoperability. CASE VI (J.P.): This 43-year-Old man presented with gradually increasing shortness of breathover 2 mo. Threeweeksbeforeadmission, he hadhematuria andflankpain,and 1 wk. laterhe notedblood-streaked sputum. On examination, both lung bases were dull, air entry was decreased, and rales were heard bilaterally. The admission chest radiograph revealed extensive air-space disease (Fig. 6) and a urogram showed a nonfunctioning right kidney. Diagnoses considered included renal vein thrombosis and Goodpastures' syndrome. A venogram showedtumor in the renal vein growing into the inferior vena cava and angiography revealed the presence of a huge right hypernephroma. The
Vol. 121
THORACIC MANIFESTATIONS OF RENAL CELL CARCINOMA
47
Diagnostic Radiology
Fig. 6. Although there is probably an interstitial component, it is masked by the extensive bilateral air-space disease (seen to better advantage on the close-up of the right lower lung). In view of the hematuria and hemoptysis, this was initially believed to represent pulmonary hemorrhage.
Fig. 7. A. Magnification (10X) shows extensive filling of alveoli with tumor cells. No normal lung tissue can be seen. B. Magnification (30X) reveals the characteristic clear cell which was identical in appearance to the histology found in the kidney. Although both capillaries and lymphatics were also found filled with tumor, very little tumor was found in the alveolar septae or supporting connective tissues of the lung.
patient deteriorated rapidly, disseminated intravascular coagulopathy developed, and he died 10 days after admission.
COMMENT: This case shows features which, to our knowledge, have not been previously reported. Tumor cells of the same clear cell type found diffusely infiltrating the kidney were demonstrated in the alveoli (Fig. 7) and extending proximally as far as the terminal bronchioles. No tumor was present above this level in the tracheobronchial tree. Tumor was found within capillaries and lymphatics, but alveolar septal involvement was almost nonexistent. No endobronchial metastases were found. Wood (23) has shown that large cancer cells can pass through capillaries by changing shape to conform to the size of the capillary. Pump (15) has shown the presence of lymphatics in human pulmonary alveoli and their communication with subpleural lymphatics. It is postulated that the tumor cells metastasized hematogenously and/or Iymphohematogenously, migrating from capillaries and/or
lymphatics through alveolar septae into the air spaces where they were able to grow until available nutrition was exhausted. This was likely a continuing process and not a single episode as some of the lung sections showed alveoli filled with necrotic tumor, presumably representing older metastatic disease, while other areas contained the viable tumor cells illustrated in Figure 7.
SUMMARY
Carcinoma of the kidney, because of its unique access to multiple metastatic routes, can give rise to remote metastases before there is evidence of renal disease clinically. The thorax, for reasons discussed, is particularly accessible. As 30-45 % of patients with renal metastases have no symptoms referrable to the primary lesion, a knowledge of the varied presentations is helpful in directing attention to the primary.
48
ANNE LATOUR AND HARRY S. SHULMAN
REFERENCES 1. Adolfsson G: Hypernephroma metastatic to the lung with no demonstrable primary. J Urol 97:221-224, Feb 1967 2. Andrews JT: Spontaneous disappearance of pulmonary metastases in carcinoma of the kidney. Med J Aust 2:241-242, Aug 1965 3. Arkless R: Renal carcinoma: how it metastasizes. Radiology 84:496-501, Mar 1965 4. Batson OV: The function of the vertebral veins and their role in the spread of metastases. Ann Surg 112:138-149, Jul 1940 5. Bennington JL, Kradjian RM: Renal carcinoma. Philadelphia, Saunders, 1967, Chpt. VI, pp 156-169 6. Bergstedt,M, Herberts G: Radical extirpation before diagnosis of primary tumor. Acta Otolaryngol (Stockholm) 54:95-98, Feb 1962 7. Charache H: Metastatic tumors in the breast; with a report of ten cases. Surgery 33:385-390, Mar 1953 8. Chute R, Ireland EF Jr, Houghton JD: Solitary distant metastases from unsuspected renal carcinomas. J Urol 80:420-424, Dec 1958 9. Cole WH: The mechanismsof spreadof cancer. Surg Gynecoi Obstet 137(5):853-871, Nov 1973 10. Coman DR: Mechanismsresponsible for origin and distribution of blood-borne tumor metastases; review. Cancer Res 13:397-404, Jun 1953 11. Gerard FP, Sabety AM, Madaras JS: Latent lung metastases of renal tumour. Ann Thorac Surg 7:27-29, Jan 1969 12. Graham AP: Malignancy of the kidney, survey of 195 cases. J Urol 58: 10-21, Jul 1947 13. Markewitz M, Taylor DA, Veenema RJ: Spontaneous regression of pulmonary metastases following palliative nephrectomy. Casereport.Cancer20:1147-1154,JuI1967 . 14:. M.oody DL, Edlich RF, Gegaudas E: The roentgenologic Identification of pulmonary metastases: evaluation of an operatively proved.series. Dis Chest 51:306-310, Mar 1967
October 1976
15. Pump KK: Lymphatics of the human pulmonary alveoli: preliminary report. Chest 58:140-146, Aug 1970 16. Schapera HE,Oppenheimer GO: Spontaneous disappearance of pulmonary metastases in hypernephroma. J Mt Sinai Hosp NY 34: 11-16,1967 17. Schwarz MI, Whitcomb ME, Goldman AL: The spectrum of diffuse pulmonary infiltration in malignant disease. Chest 64:88-93, Jul 1973 18. Sellstrom LG: Hypernephroma metastases in the ear and nose region. Acta Otolaryngol (Stockholm) 55:545-552, Nov-Dec 1962 19. Tamisiea DF, Shields JB: A pulsating anterior chest mass. A radiographic exercise. Mo Med 68:103-105, Feb 1971 20. Weigensberg IJ: Metastatic renal carcinoma: unusual and deceptive presenting features. South Med J 65:611-616, May 1972 21. Willis RA: Pathology of tumours. London, Appleton, Century, Crofts, 1967, 4th ed, pp 456-470 22. Wong TW, Bennington JL: Metastasis of a mammary carcinoma to an acoustic neuroma. Report of a case. J Neurosurg 19: 1088-1093, Dec 1962 23. Wood S Jr: Pathogenesis of metastasis formation observed in vivo in the rabbit ear chamber. Arch Pathol 66:550, Oct 1958 24. Woo-Ming M, Murray SM, Batison EM: Calcified tumourthrombusof the left pulmonary artery due to metastatic renal carcinoma. Br J Radiol 39:267-271, Apr 1966 25. Yang SP, Lin CC: Lymphangitic carcinomatosis of the lungs. The clinical significance of its roentgenologic classification. Chest 62:179-187, Aug 1972 26. Yates HB: Suddenoperative death due to tumour embolism. Br Med J 2:202, 16 Jul 1955 Dr. H. S. Shulman Department of Radiology Sunnybrook Medical Centre 2075 Bayview Ave. Toronto, Ontario M4N 3M5, Canada
Thoracic Manifestations of Renal Celf
Diagnostic Radiology
Carcinoma 1
Anne Latour, M.D., F.R.C.P.(C)2, and Harry S. Shulman, M.D., F.R.C.P.(C) Over 1fJ of patients with renal cell carcinoma will present with metastases with no symptoms referable to the kidney. Early metastatic disease is a result of the unique accessibility of the kidney to Iymphohematogenous pathways. The thorax is particularly vulnerable. Several of the more unusual manifestations involVing the thorax are illustrated. including 1 case with intra-alveolar metastases. Kidney neoplasms, metastases • (Renal cell carcinoma. 8[1].324). (Thoracic metastases, 6[8].330). Thorax, neoplasms
INDEX TERMS:
Radiology 121:43-48, October 1976
•
•
HE VARIETY of pathways by which renal cell carci-
Lymphogenous - - - _ r e g i o n a l nodes - - - - - _ • . Mediastinum
Tnoma can metastasize is responsible for the wide and
• Pulmonary lymphatics
confusing spectrum of clinical and radiographic presentations. Occasionally, the manifestations are sufficiently unusual that their malignant nature is not initially apparent. In fact, 30-45 % of patients with renal cell metastases have no symptoms referrable to the kidney (1, 5). This tumor has been reported involvlnq such unusual sites as larynx (6), gallbladder (12), meninges (22), breast (7), and ear (18), but the lung is the most common site (5). In Bennington's autopsy review of 523 cases (5), a 55% incidence of pulmonary metastases was found. Mediastinal involvement has been reported in 8-10 cases (3). Thus, over 60 % of metastatic renal cell carcinoma will involve the thorax at some time before death. From another point of view, however, less than 5 % of all metastatic disease to the lungs is renal in origin (5). Although the most common form of metastasis to the thorax is that of multiple pulmonary nodules, many other forms of thoracic involvement may occur including large, solitary pulmonary masses (20), pleural involvement with effusion or pleural masses (14), hilar or mediastinal adenopathy (13), bony thorax involvement with rib or vertebral destruction, and subpleural or paravertebral soft-tissue masses (20). Rarer thoracic presentations include direct extension of tumor from the renal vein to the right atrium, ventricle, and main pulmonary artery (21) [often associated with embolism of tumor tissue into the lung (26)], calcification in tumor thrombus in the pulmonary arteries (24), and pulsating anterior chest wall mass (19). Two extremely rare and puzzling features of hypernephroma are the occasional appearance of metastases up to 20 years or more after removal of the primary (11) and, conversely, the spontaneous disappearance of pulmonary metastases (2, 16).
Lymphohematogenous---. lymphatics \ . thoracic duet
shunyo
Hematogenous - Renal
veins~
\
.>
Right heart - - - . . lungs
lye
1,
Batson s
Plexus~.
Intercostal &_ _ • Lungs
. Bronchial veins
. Mediastinum • Pleura · Thorcci c cage
Diag. 1.
The routes of renal cell carcinoma spread to the thorax.
Lymphogenous; mainly to regional nodes, and only rarely to distant nodes, probably because of nodal obstruction. Lymphohematogenous; when a node becomes blocked with tumor, shunting of cells presumably occurs both antegrade and retrograde (9). Once completely filled with tumor, the surrounding connective tissue may be invaded, including small veins. In addition, cells reaching the lymphatics eventually enter the thoracic duct which empties into the innominate vein and thus reach the lung. As Coman has discussed (10), once tumor emboli have reached the lung, they may gain access to arteriovenous shunts in the lung, thus bypassing the capillary network to reach the systemic circulation. Hematogenous; by direct extension into the inferior cava where there is access: (a) Antegrade to the heart and lung (the most common route of spread and responsible for the "cannon ball" picture of renal cell metastases). (b) Retrograde in the vena cava or from the renal vein to the pelvis. (c) Shunting from the caval to the portal system (and ultimately back to the inferior vena cava via hepatic veins). (d) Batson's plexus; a network of valveless veins surrounding the vertebral column (and spinal cord), extending from the skull almost to the pelvis and via the vasa vaso-
ANATOMIC CONSIDERATIONS
The kidneys' relationships provide hypernephroma with a unique access to many metastatic routes (Diag. 1). Metastases may be: --
From the Department of Radiology, Sunnybrook Medical Centre, University of Toronto, Ontario, Canada. Presented in part at the Canadian Association of Radiologists Annual Meeting, May 1975. 2 Current address: Department of Radiology, Queen Mary Veteran's Hospital, 4565 Queen Mary Rd., Montreal, Quebec, Canada. elk 1
43
44
ANNE LATOUR AND HARRY S. SHULMAN
October 1976
Fig. 1. Both lungs are fairly symmetrically involved with a predominantly interstitial process, although air-space disease is present in the right upper lobe. The close-up of the right lower lung shows to better advantage the characteristic coarsely thickened interstitial septa of Iymphangitic carcinomatosis (arrows). Hilar adenopathy is partially obscured by the adjacent lung involvement.
A
B
Fig. 2. A. This radiograph was obtained 3 years prior to the last admission and shows the changes of a previous right thoracoplasty for T.B. B. The current radiograph shows an enlarged right hilus, proved at autopsy to be enlarged nodes secondary to metastatic renal cell carcinoma.
Vol. 121
THORACIC MANIFESTATIONS OF RENAL CELL CARCINOMA
45
Diagnostic Radiology
rum almost to the knees and elbows. Being valveless, changes in thoracic or intra-abdominal pressure (for example, with coughing or straining) can result in blood flowing caudally, as well as cranially. Anastomoses are extensive and include azygos, hemiazygos, lumbar, bronchial, intercostal, and other systemic veins. It is this plexus with its multiple systemic connections that is likely responsible for some of the more bizarre metastatic sites previously mentioned. Direct invasion; in addition to immediately surrounding tissues becoming involved, direct invasion may result in smallvascular structures being invadedand thus ultimately providing access to the systemic circulation. The following cases illustrate some of the unusual presentations of renal cell carcinoma in the thorax and include a metastatic manifestation in the lungs which, to our knowledge, has not been previously reported. CASE REPORTS CASE I (T.B.): This 49-year-old man presented with hip pain 7 wk. after a fall but no fracture was found. While in the hospital, he became progressively dyspneic. The chest radiograph revealed a predominantly interstitial process involving both lungs and probable bilateral hilar adenopathy (Fig. 1). Metastatic survey showed multiple radiolucent defects in the skull while a radionuclide image revealed that the liver was enlarged and contained multiple defects. A percutaneous trephine biopsy of the lung showed undifferentiated tumor. At autopsy, a left renal cell carcinoma had invaded the renal vein and metastases were found in lungs, cervical, mediastinal, and hilar nodes, heart. and pericardium. Microscopically, the pulmonary lymphatics and perilymphatic interstitium were diffusely involved, with scattered nodules of metastatic carcinoma within the lung parenchyma and on the pleural surface.
Fig. 3. The admission radiograph reveals an opaque right hemithorax with mediastinal shift to the contralateral side. No comment can be made regarding possible pulmonary parenchymal involvement on the right side. No abnormality is seen on the left side.
COMMENT: This man died of respiratory failure due to diffuse metastatic disease and yet had no symptoms referrable to his kidney. The possibility of metastatic pulmonary malignancy was raised, although the kidney was not considered a likely primary source. Growth into the renal vein is the most common form of spread and the tumor may extend up and down the vena cava, into the heart and pulmonary artery, and may even involve the ovarian and spermatic veins (especially on the left). Spread to the lungs in this case may have occurred along two pathways-direct embolization from the renal vein and via Iymphohematogenous spread. The Iymphangitic pattern illustrated in this case is most often associated with adenocarcinoma of lung, stomach, breast, and pancreas (17, 25). In a review of 275 cases of Iymphangitic carcinomatosis, Yang found only four renal primaries (1.5%) (25). II (R.D.): This 47-year-old, asymptomatic man was found to have a right hilar mass on a routine chest radiograph (Fig. 2). Evidence of a previous thoracoplasty for tuberculosis was present. No surgery was performed as the patient was medically unfit. Grand mal seizures subsequently developed and he deteriorated rapidly and died. Postmortem examination revealed renal cell carcinoma involving the right kidney and adrenal gland, brain, and right hilar nodes. Isolated small metastases were found in the right lung. CASE
COMMENT: When hilar involvement is present, it is usually associated with widespread pulmonary disease,
Fig. 4. Destruction of the third rib posteriorly (*) is accompanied by a mass with the characteristic configuration of an extrapulmonary lesion. Although metastatic carcinoma was the most likely diagnosis, the kidney was not considered a likely primary source.
particularly of the Iymphangitic variety as in CASE I. The pulmonary parenchymal component, however, may not be radiographically apparent. Hilar adenopathy as the only radiological manifestation of metastatic disease is uncommon. In Arkless' series of 152 patients (3), 11 had
46
ANNE LATOUR AND HARRY S. SHULMAN
Fig. 5.
October 1976
Postero-anterior and lateral radiographs reveal a sharplydefinedsolitary mass.Onthe lateral film, the poor definitionanteriorly is the result of chest wall invasion. No other lesion could be identified radiographically.
mediastinal adenopathy involving carinal, hilar, or paratracheal areas. In fact, 5 of this group had teft-sided cervical or supraclavicular adenopathy. This pattern is typical of other infradiaphragmatic tumors that spread via lymphatics. CASE III (J.H.): This 67-year-old man was admitted complaining of right-sided chest pain, shortness of breath, and abdominal distension for 5 days. The chest radiograph revealed an opacifiedright hemithorax with slight shift of the mediastinum to the left (Fig. 3). Seven hundred milliliters of serosanguinous fluid was removed from the right pleural space. Ten days later, massive rectal bleeding developed due to right-sideddiverticulitis which required right hemicolectomy. Postoperatively, he became oliguric and died. At autopsy,the right lung was found to be completelyencased in pleurathat was extensively infiltrated with metastatic renalcell carcinoma. Metastatic involvement of the lung parenchyma was present, but not extensive.
COMMENT: As with CASES I and II, no symptoms referrable to the kidney were present prior to death. Although the lungs were involved with metastatic tumor at autopsy, this was obscured by the rather extensive and unusual pleural involvement. Pleural involvement is often secondary to the lung involvement, but in this case the far more extensive pleural disease could also be explained on the basis of communications between parietal pleural veins and Batson's plexus (4). CASE IV (G.F.): This 69-year-old man presented with fatigue, right flank pain, and hematuria. A right nephrectomy was performed for renal cell carcinoma. A chest radiographobtained 14 mo. postoperatively (Fig. 4) demonstrateddestruction of the right third rib associated with a mass.
COMMENT: "Disappearing bone" is one of the hallmarks of metastatic renal cell carcinoma, although metastatic thyroid carcinoma can produce a similar appearance. However, an associated mass such as was seen in this case is uncommon. Chest wall involvement might occur via systemic spread following passage of cells through the lungs, but is more likely a result of communications between the inferior vena cava, Batson's plexus, and intercostal veins. CASE V (J.R.): This59-year-old manhada nephrectomy 3 yearsprior to this admission; follow-up chest radiographs were negative for 18 mo. He was then lost to follow-up for the next 18 mo., after which he returned to his physician complaining of right-sided chest pain. A chest radiographwas obtained(Fig. 5) and the diagnosisconfirmed by open biopsy.
COMMENT: About 2 % of metastatic renal cell carcinomas present as a solitary pulmonary mass (8) and these generally have a fairly good prognosis. Chest wall invasion, however, is a poor prognostic sign and in most cases an indication of inoperability. CASE VI (J.P.): This 43-year-Old man presented with gradually increasing shortness of breathover 2 mo. Threeweeksbeforeadmission, he hadhematuria andflankpain,and 1 wk. laterhe notedblood-streaked sputum. On examination, both lung bases were dull, air entry was decreased, and rales were heard bilaterally. The admission chest radiograph revealed extensive air-space disease (Fig. 6) and a urogram showed a nonfunctioning right kidney. Diagnoses considered included renal vein thrombosis and Goodpastures' syndrome. A venogram showedtumor in the renal vein growing into the inferior vena cava and angiography revealed the presence of a huge right hypernephroma. The
Vol. 121
THORACIC MANIFESTATIONS OF RENAL CELL CARCINOMA
47
Diagnostic Radiology
Fig. 6. Although there is probably an interstitial component, it is masked by the extensive bilateral air-space disease (seen to better advantage on the close-up of the right lower lung). In view of the hematuria and hemoptysis, this was initially believed to represent pulmonary hemorrhage.
Fig. 7. A. Magnification (10X) shows extensive filling of alveoli with tumor cells. No normal lung tissue can be seen. B. Magnification (30X) reveals the characteristic clear cell which was identical in appearance to the histology found in the kidney. Although both capillaries and lymphatics were also found filled with tumor, very little tumor was found in the alveolar septae or supporting connective tissues of the lung.
patient deteriorated rapidly, disseminated intravascular coagulopathy developed, and he died 10 days after admission.
COMMENT: This case shows features which, to our knowledge, have not been previously reported. Tumor cells of the same clear cell type found diffusely infiltrating the kidney were demonstrated in the alveoli (Fig. 7) and extending proximally as far as the terminal bronchioles. No tumor was present above this level in the tracheobronchial tree. Tumor was found within capillaries and lymphatics, but alveolar septal involvement was almost nonexistent. No endobronchial metastases were found. Wood (23) has shown that large cancer cells can pass through capillaries by changing shape to conform to the size of the capillary. Pump (15) has shown the presence of lymphatics in human pulmonary alveoli and their communication with subpleural lymphatics. It is postulated that the tumor cells metastasized hematogenously and/or Iymphohematogenously, migrating from capillaries and/or
lymphatics through alveolar septae into the air spaces where they were able to grow until available nutrition was exhausted. This was likely a continuing process and not a single episode as some of the lung sections showed alveoli filled with necrotic tumor, presumably representing older metastatic disease, while other areas contained the viable tumor cells illustrated in Figure 7.
SUMMARY
Carcinoma of the kidney, because of its unique access to multiple metastatic routes, can give rise to remote metastases before there is evidence of renal disease clinically. The thorax, for reasons discussed, is particularly accessible. As 30-45 % of patients with renal metastases have no symptoms referrable to the primary lesion, a knowledge of the varied presentations is helpful in directing attention to the primary.
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ANNE LATOUR AND HARRY S. SHULMAN
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