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AJR 2005; 185:268-272
© American Roentgen Ray Society

Clinical Observations

Renal Medullary Carcinoma: CT and MRI Features

Netta M. Blitman1, Robert G. Berkenblit2, Alla M. Rozenblit2 and Terry L. Levin1

1 Department of Radiology, Albert Einstein College of Medicine, Children's Hospital at Montefiore, 3400 Bainbridge Ave., Bronx, NY 10467.
2 Department of Radiology, Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, NY 10467.

Received July 16, 2004; accepted after revision September 23, 2004.

 
Address correspondence to N. M. Blitman.


Abstract
Top
 Abstract
Introduction
 Materials and Methods
 Results
 Discussion
 References
 
OBJECTIVE. We review the cross-sectional imaging findings of six cases of pathologically proven renal medullary carcinoma in patients with sickle cell trait. MRI findings were available in three of the patients. To our knowledge, only one previous report has addressed MRI features of this rare disease.

CONCLUSION. In young patients with sickle cell trait, an infiltrative renal mass with associated retroperitoneal adenopathy and caliectasis are characteristic findings of renal medullary carcinoma on CT and MRI.


Introduction
Top
 Abstract
 Introduction
Materials and Methods
 Results
 Discussion
 References
 
Renal medullary carcinoma is a rare tumor first designated as a distinct pathologic entity in 1995 by Davis et al. [1] at the Armed Forces Institute of Pathology. A unique feature of this tumor is the strong association with sickle cell trait. Since the initial description of this entity, reports in the radiology literature have been limited [2-5]; the largest series by Davidson et al. [2] consisted of five patients.

We retrospectively evaluated the CT, MRI, and sonography features of renal medullary carcinoma in six patients to further characterize the imaging findings. We also compared the value of CT with that of MRI in those patients in whom both techniques were performed.


Materials and Methods
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 Abstract
 Introduction
 Materials and Methods
Results
 Discussion
 References
 
We retrospectively evaluated the imaging studies of patients with pathologically proven renal medullary carcinoma. Charts were compiled by discharge diagnosis using a computer database of the medical records. We identified six patients (age range, 15-27 years) who presented to our institution between 1996 and 2003. Three were males. CT scans for all six patients, MR images for four patients, and sonograms for one patient were available for review. One MRI examination, which was performed without gadolinium, was technically suboptimal and was excluded from the study; thus, three MRI studies were included in the study. This study was exempt from the approval process by our institutional review board; no additional patient consent was required.

Six CT examinations were performed on a helical single-detector scanner (HiSpeed Advantage, GE Healthcare) with a slice thickness of 5 mm (n = 3), 10 mm (n = 2), or 7 mm (n = 1) and pitch of 1 to 1.5. Two patients, both older than 21 years, had unenhanced CT scans and one of these patients had a delayed scan. All patients received IV iohexol 300 (Omnipaque 300, Sanofi) that was administered at a dose of 1 mL/lb (2 mL/kg) of body weight and administered at a weight-adjusted rate of 1 to 2 mL/sec. They also were given 600 to 800 mL of 3% meglumine diatrizoate (Gastrografin, Bracco Diagnostics) orally. In the four patients in whom only enhanced CT scans were available, calcifications were assessed using bone window settings.

The three MR examinations were performed on a 1.5-T magnet: two on one scanner (Signa, GE Healthcare) and one on another scanner (Gyroscan, Phillips Medical Systems). MRI studies were performed using T1-weighted spin-echo (TR range/TE range, 466-650/12-14) or gradient-echo (4.5-4.6/1-1.1; flip angle, 15°) sequences (or both) and T2-weighted fast spin-echo sequences (2,000-3,000/92-120) with and without fat suppression in the axial and coronal planes. Slice thickness ranged from 5 to 10 mm. Contrast-enhanced (0.1 mmol/kg of gadopentetate dimeglumine [Magnevist, Schering]) T1-weighted or gradient-echo sequences were performed in all three cases. Sonography was performed on a unit (model 128 XP, Acuson) using a 4-MHz vector transducer.

Imaging studies were evaluated by one pediatric radiologist with a certificate of added qualification and one experienced body imaging radiologist in consensus. Tumors were evaluated for their location, presence of necrosis or hemorrhage (or both), pelvocaliectasis, calcification, extrarenal extension, vascular invasion or encasement, and regional or distant metastases. Imaging findings were directly compared with intraoperative findings, when available, and surgical pathology. Five patients underwent nephrectomy; the tumor in the sixth patient was deemed unresectable at surgery, but tissue was obtained for diagnosis. Medical records were reviewed for the presence of sickle cell trait, ethnicity of the patient, presentation, and outcome.



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  		Fig. 1A —17-year-old boy with renal medullary carcinoma. CT image shows right upper pole renal medullary carcinoma. Mass (arrow) is infiltrative, and kidney retains its reniform shape.

 



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  		Fig. 1B —17-year-old boy with renal medullary carcinoma. Photograph shows gross pathologic specimen. Ruler shows measurement in cm.

 

Results
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 Abstract
 Introduction
 Materials and Methods
 Results
Discussion
 References
 
All patients had sickle cell trait as evaluated by either hemoglobin electrophoresis (n = 5) or sickled erythrocytes at electron microscopy (n = 1). Four were black, and two were Hispanic. The presenting features included gross hematuria alone in three patients, gross hematuria and back pain in one patient, gross hematuria and palpable right upper quadrant mass in one patient, and palpable right upper quadrant mass alone in one patient. None of the patients had a history of urinary tract infection or calculi.

All tumors were located centrally within the right kidney, were hypovascular, and showed an infiltrative pattern with ill-defined margins (Figs. 1A, and 1B). Caliectasis was present in all cases. Retroperitoneal adenopathy was present in five patients. It was heterogeneous and ranging in volume from small (n = 1) or moderate (n = 2) to extensive (n = 2). Tumor necrosis was present in four patients. Intratumoral hemorrhage and subcapsular hemorrhage (Fig. 2A) were present in four patients and one patient, respectively. None of the tumors showed calcification. The right renal vein was thrombosed in two patients; pathology confirmed the presence of tumor thrombi in both of these cases. The vascular pedicle was encased without thrombosis in three patients. Two patients had liver metastases, three patients had pulmonary metastases, one had both liver and pulmonary metastases, and one patient had no metastases at the time of diagnosis. The CT appearance of pulmonary metastases included small nodules (n = 1), cannonball lesions (n = 1), or a thick pleura-based rind (n = 1).



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  		Fig. 2A —20-year-old man with renal medullary carcinoma. CT image obtained with contrast material shows infiltrative mass (black arrow) and subcapsular hematoma (white arrow) indenting substance of kidney. Regional adenopathy (arrowhead) is present.

 
In those patients in whom both CT and MRI were performed, multiplanar gadolinium-enhanced MRI was found to be similar to CT in delineating the borders of the mass (Figs. 3B and 3C), assessing regional adenopathy (Figs. 2A and 2B), and defining the renal pedicle (Figs. 2A and 2B). The multiplanar capability of MRI made it easier to evaluate the global extent of disease (Fig. 2C). MRI was superior in detecting intratumoral hemorrhage (Figs. 4A, and 4B) and increased the conspicuity of the liver metastases (Figs. 4A, and 4B). In one case, surgically documented renal vein invasion was not visualized on the CT scan.



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  		Fig. 3B —15-year-old girl with renal medullary carcinoma. CT scan obtained 2 days after A shows distinct infiltrative mass (lower arrow), and caliectasis (upper arrow). Right paraaortic adenopathy (arrowheads) displaces the renal artery.

 


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  		Fig. 3C —15-year-old girl with renal medullary carcinoma. Delayed contrast-enhanced T1-weighted spin-echo MR image (TR/TE, 506/12) at slightly higher level than B shows similar features. Arrow denotes tumor.

 


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  		Fig. 2B —20-year-old man with renal medullary carcinoma. Axial 3D fat-suppressed gradient-echo MR image (TR/TE, 4.5/1.1) obtained with contrast material at same level as A shows renal artery and vein separated by adenopathy (arrowhead). Right renal artery is encased.

 


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  		Fig. 2C —20-year-old man with renal medullary carcinoma. Coronal 3D fat-suppressed gradient-echo MR image (4.5/1.1) obtained with contrast material shows extent of adenopathy (arrow) is better assessed on MRI than CT (A).

 


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  		Fig. 4A —27-year-old man with renal medullary carcinoma. Axial CT scan shows multilocular right renal lesion without visible hemorrhage. Note subtle metastatic liver lesion (arrow).

 


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  		Fig. 4B —27-year-old man with renal medullary carcinoma. T2-weighted fast spin-echo fat-saturation MR image (TR/TE, 2,500/96) comparable to A shows hypointense hemosiderin rim and central hypointense septations, which is consistent with chronic hemorrhage (arrowheads). Note also conspicuity of liver metastasis (arrow) is increased on MRI as compared with CT (A).

 
The single sonogram failed to depict a distinct renal mass—even in retrospect—and showed an enlarged right kidney with areas of increased medullary echogenicity (Fig. 3A). This finding was incorrectly ascribed to papillary necrosis in the original interpretation. Two days after sonography, a renal mass was detected on CT.



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  		Fig. 3A —15-year-old girl with renal medullary carcinoma. Sonogram shows increased medullary echogenicity. No discrete mass is identified.

 
Patient outcome has been uniformly abysmal. Three patients died at less than 8 months, two patients died within 13 months, and one patient is alive at 10 months after diagnosis with increasing metastatic burden.


Discussion
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
References
 
The first description of renal medullary carcinoma was by Davis et al. [1] in 1995. They designated a group of tumors occurring almost exclusively in patients with sickle cell trait (one had hemoglobin sickle cell disease) that shared similar morphologic and histologic features. Grossly, the tumor was poorly circumscribed with calyceal or renal pelvic extension. It was located in the renal medulla and satellite lesions were frequently present in the renal cortex or adjacent peripelvic soft tissue. Most tumors contained hemorrhagic areas and extensive necrosis. Microscopically, the tumor showed a reticular pattern of growth in which tumor cell aggregates formed spaces of various sizes. Prominent inflammatory elements and extensive stromal desmoplasia were noted. All tumors contained lymphatic or vascular invasion (or both). They were highly aggressive, often with metastases at the time of diagnosis.

In their original report of the imaging findings of renal medullary carcinoma, Davidson et al. [2] described the tumor as infiltrative and characterized by renal enlargement with preservation of an overall reniform shape. Tumor necrosis was a constant finding. Extrarenal spread patterns included direct local invasion (regional lymph nodes, liver, renal vein) and retroperitoneal soft-tissue invasion. The locations of distant metastases included the liver, lung, and omentum.

The tumors in our series had a remarkably similar appearance to one another. All were on the right and had an infiltrative pattern and caliectasis. Most had regional adenopathy, hemorrhage, and necrosis. Conclusions regarding the value of CT versus MRI are limited by the small number of cases. However, we agree with Khan et al. [5] that the multiplanar capability of MRI is useful in revealing the extent of parenchymal and nodal invasion. The MRI studies in our series (n = 3) were superior in detecting intratumoral hemorrhage and improved the conspicuity of liver metastases. Five of six patients had distant metastases at diagnosis. Interestingly, the pulmonary metastases had a different appearance in each patient: small nodules, cannonball lesions, and a thick pleura-based rind.

In our study, all patients had associated sickle cell trait. In the United States, sickle cell trait is most commonly seen in those of African American descent with an incidence of 7.5% [6]. However, two of the six patients in our series were of Hispanic origin. In one of these patients, the presence of sickle cell trait was unknown before tumor detection. This has been reported by other investigators [1, 7]. It should be noted that the sickle cell gene has also been found in the Mediterranean, southern Arabia, and central India and should be considered in patients of these origins as well [6].

Before its recognition as a separate entity, renal medullary carcinoma was often misclassified as a collecting duct carcinoma. Both tumors arise from the medullary portion of the kidney, have an infiltrative pattern, and are biologically aggressive [1, 8]. Both tumors are thought to arise from proliferating cells of the collecting duct epithelium. Features common to both renal medullary carcinoma and collecting duct carcinoma include a dense stromal desmoplasia, inflammatory reaction, and positive results on mucicarmine staining. Pathologically distinguishing features of collecting duct carcinoma include a cystic or papillary appearance on gross examination and a tubular or papillary growth pattern [8]. Collecting duct carcinoma is most often seen in adults and is not associated with a hemoglobinopathy. Although some pathologists believe that renal medullary carcinoma is an aggressive form of collecting duct carcinoma [8], the exact relationship between the tumors of the renal medulla remains in flux [9].

In addition to collecting duct carcinoma, other neoplastic and nonneoplastic conditions may mimic the central infiltrative pattern of renal medullary carcinoma. Renal lymphoma may be diffusely or focally infiltrative [10]. In children, it is most often bilateral and multifocal [11]. Renal involvement occurs most often with non-Hodgkin's lymphoma and is evident in only 5% of patients at initial staging [10]. Concomitant bulky perinephric disease and widespread lymphadenopathy should suggest the diagnosis. Rhabdoid tumor of the kidney is a rare but aggressive infiltrative tumor with a peritumoral fluid collection in 75% of the cases [12]. Mesoblastic nephroma, a benign tumor of spindle cells, may also be infiltrative [13]. Both tumors occur in much younger patients than those with renal medullary carcinoma (mean age, 11 months and 3 months, respectively). Wilms' tumor, the most common childhood renal neoplasm, is a cortical tumor that tends to grow by expansion and produces well-defined round masses [10]. Other infiltrative renal masses that usually occur in older patients include transitional cell carcinoma, sarcomatoid variants of renal cell carcinoma, and some renal metastases [10, 13]. Lastly, infectious entities such as acute bacterial nephritis [10, 13] may mimic the infiltrative nature of renal medullary carcinoma on imaging, but its clinical and laboratory features are remarkably different.

The most common presenting symptom of renal medullary carcinoma is gross painless hematuria, as seen in our series and the original series by Davis et al. [1]. There are often no accompanying symptoms. Gross hematuria is also the most common symptom in patients with sickle cell trait [14]. It is usually painless and self-limited and occurs in patients of the same age range (11-39 years) as reported by Davis et al. for renal medullary carcinoma [1, 14]. Sonography is often the initial imaging study in the workup of hematuria and is performed to exclude neoplasm, infection, or renal papillary necrosis. The one sonography examination performed in our series failed to show the tumor—even in retrospect. Wesche et al. [9] reported normal sonography findings in a patient whose tumor was revealed on CT 2 months after sonography. This raises the question of whether sonography alone is sufficient to evaluate hematuria in patients with sickle cell trait.

One of the most intriguing features of this tumor is its predilection for the right side. Of the 49 tumors reported up to 1998, 70% were on the right [5]. Nonetheless, in patients with sickle cell trait, gross hematuria has a clear left-side predominance as documented by Mostofi et al. [15]. The cause of hematuria is believed to be secondary to bleeding immediately beneath the renal pelvic epithelium in or near the renal papillae [15]. Davis et al. [1] suggested that renal medullary carcinoma arises in the same location. The association between these two entities, if any, is not yet clear. None of the normal portions of our five nephrectomy specimens showed evidence of renal papillary necrosis. More likely, the overwhelming link between sickle cell trait and renal medullary carcinoma suggests a genetic association [7].

In conclusion, an infiltrative, right-sided renal tumor with necrosis, caliectasis, and regional adenopathy in a young patient with sickle cell trait suggests the diagnosis of renal medullary carcinoma.


Acknowledgments
 
We thank Daniel Alterman for providing a case and Paula Ammirato for her assistance with the figures.


References
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 

  1. Davis CJ, Mostofi FK, Sesterhenn IA. Renal medullary carcinoma: the seventh sickle cell nephropathy. Am J Surg Pathol1995; 19:1 -11[Medline]
  2. Davidson AJ, Choyke PL, Hartman DS, Davis CJ Jr. Renal medullary carcinoma associated with sickle cell trait: radiologic findings. Radiology1995; 195:83 -85[Abstract/Free Full Text]
  3. Kalyanpur A, Schwartz DS, Fields JM, Rayes-Mugica M, Keller MS, Gosche J. Renal medulla carcinoma in a white adolescent. AJR 1997;169:1037 -1038[Free Full Text]
  4. Pickhardt PJ. Renal medullary carcinoma: an aggressive neoplasm in patients with sickle cell trait. Abdom Imaging1998; 23:531 -532[CrossRef][Medline]
  5. Khan A, Thomas N, Costello B, et al. Renal medullary carcinoma: sonographic, computed tomography, magnetic resonance and angiographic findings. Eur J Radiol2000; 35:1 -7[CrossRef][Medline]
  6. Hoffman GC. The sickling disorders. Lab Med1990; 21:797 -807
  7. Adsay NV, deRoux SJ, Sakr W, Grignon D. Cancer as a marker of genetic medical disease: an unusual case of medullary carcinoma of the kidney. Am J Surg Pathol1998; 22:260 -264[CrossRef][Medline]
  8. Srigley JR, Eble JN. Collecting duct carcinoma of kidney. Semin Diagn Pathol1998; 5:54 -67
  9. Wesche WA, Wilimas J, Khare V, Parham DM. Renal medullary carcinoma: a potential sickle cell nephropathy of children and adolescents. Pediatr Pathol Lab Med1998; 18:97 -113[CrossRef][Medline]
  10. Pickhardt PJ, Lonergan GJ, Davis CJ Jr, Kashitani N, Wagner BJ. Infiltrative renal lesions: radiologic-pathologic correlation. RadioGraphics2000; 20:215 -243[Abstract/Free Full Text]
  11. Chepuri NB, Strouse PJ, Yanik GA. CT of renal lymphoma in children. AJR 2003;180:429 -431[Abstract/Free Full Text]
  12. Agrons GA, Kingsman KD, Wagner BJ, Sotelo-Avila C. Rhabdoid tumor of the kidney in children: a comparative study of 21 cases. AJR 1997;168:447 -451[Abstract/Free Full Text]
  13. Hartman DS, Davidson AJ, Davis CJ Jr, Goldman SM. Infiltrative renal lesions: CT-sonographic-pathologic correlation. AJR 1988;150:1061 -1064[Abstract/Free Full Text]
  14. Crone RI, Jefferson SC, Pileggi VJ, Lowry EC. Gross hematuria in sickle cell trait. Arch Intern Med1957; 100:597 -603[Abstract/Free Full Text]
  15. Mostofi FK, Vorder Bruegge CF, Diggs LW. Lesions in kidneys removed for unilateral hematuria in sickle cell disease. Arch Pathol 1957;63:336 -351

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