A Case of a Solid Renal Mass Together

Asunción María Gonzalvez Gasch1, Carlos Mirete Ferrer3, Raquel Laveda Cano2,
Javier Satorres Rosas1
1Department of Internal Medicine and 2Department of Gastroenterology, San Jaime Hospital.
Torrevieja, Alicante, Spain. 3Department of Internal Medicine, San Vicente Hospital.
Alicante, Spain
Context Pancreatic cysts may be incidentally
detected in asymptomatic patients evaluated
for other clinical manifestations. Microcystic
adenomas are particularly rare among
pancreatic cyst neoplasms. They are benign
lesions and can present as solitary pancreatic
tumors or as a radiological manifestation
combined with other cystic and tumoral
lesions affecting different organs.
Case report A 50-year-old man presented
with hematuria. A computed tomography scan
of the abdomen showed a 9-centimeter renal
mass in the left kidney consistent with a
renal-cell carcinoma as well as a cystic lesion
the head of the pancreas. The
histopathological study of the cystic mass,
following a computed tomography guided
biopsy, showed a microcystic adenoma.
Therefore, further studies were performed so
as to assess the relationship between both
lesions and determine the final diagnosis.
Conclusions Microcystic adenomas are
exceedingly rare tumors among pancreatic
cysts. The combination of a solid renal mass
and a pancreatic cystic lesion should lead to a
broad differential diagnosis. Pancreatic
magnetic resonance imaging has been proven
to be particularly useful in evaluating cystic
masses. The presence of walls and internal
septations in the pancreatic mass with
gadolinium enhancement should raise the
possibility of an underlying Von Hippel-
Lindau syndrome.
Renal-cell carcinoma is responsible for most
solid renal lesions, hematuria being the most
common clinical presentation (50-60%) [1].
However, early-warning signs are often
missing, resulting in a delayed diagnosis and
advanced metastatic disease. Dynamic
intravenous contrast-enhanced computed
tomography (CT) is used in the evaluation of
every renal mass to define its characteristics
and determine the extent of the lesion [2].
Abdominal CT may depict a pancreatic lesion
which has remained asymptomatic until that
point. In fact, this condition has been reported
as the most usual way of presenting
pancreatic cystic lesions [3]. Although cystic
neoplasms represent less than 10% of the
pancreatic neoplasms [4], cystic pancreatic
lesions are increasingly being detected owing
to the generalized use of abdominal
ultrasonography, CT and magnetic resonance
imaging (MRI) for the evaluation of different
conditions. Microcystic adenoma is a rare
pancreatic cystic lesion [4, 5] and when

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JOP. Journal of the Pancreas – http://www.joplink.net – Vol. 6, No. 2 – March 2005. [ISSN 1590-8577]
linked with renal-cell carcinoma, the
possibility of a neoplasia syndrome may arise.
Von Hippel-Lindau disease (VHLD) results
from a mutation in the VHL gene leading to
the development of several benign and
malignant lesions in different organs,
characteristically, cystic and vascular lesions
[6]. We describe the case of a patient
presenting with hematuria due to a renal-cell
carcinoma in which a pancreatic lesion was
visualized on the imaging study of the renal
A 50-year-old man was admitted to the San
Jaime Hospital (Torrevieja, Alicante, Spain)
following a two-day history of hematuria
without back or abdominal pain. He also did
not show dysuria, tenesmus, fever, weight
loss or anorexia.
The patient had been well until three months
earlier when he presented with back pain and
hematuria in another institution. He was
successfully treated with antibiotics and
remained asymptomatic thereafter.
His past medical history revealed
hypercholesterolemia and a 50 g alcohol daily
intake. He had had a right renal cyst operated
on 12 years before.
On physical examination, he was a healthy-
appearing patient. He was afebrile with
normal blood pressure and abdomen
exploration was unremarkable.
The initial laboratory findings revealed no
abnormalities except for creatinine 1.3 mg/dL
(115 mmol/L; reference range: 44.25-110
mmol/L), total cholesterol 239 mg/dL (6.1
mmol/L; reference range: 3.7-5.18 mmol/L),
LDL cholesterol 162 mg/dL (4.1 mmol/L;
reference range: 0-3.79 mmol/L) and uric acid
7.7 mg/dL (0.45 mmol/L; reference range:
0.14-0.41 mmol/L). Urinalysis was positive
for blood, and a urine culture was negative.
An abdominal ultrasonography was
performed in the Emergency Room showed a
solid 8x8 cm mass in the left kidney and a
simple cyst in the right kidney.
The admitting diagnosis of the treating
physician was hematuria due to a renal cell
carcinoma. Abdominal CT for extension
study identified a 9 cm renal mass in the left
kidney with areas of necrosis (Figure 1) and a
heterogeneous mass in the head of the
pancreas measuring 5 cm in diameter. Thus, a
new medical approach was needed.
Cancer antigen (CA) 19.9 level was normal.
A fine needle aspiration of the pancreatic
mass was performed; however, the sample
was not suitable for pathological diagnosis.
We therefore asked for a CT-guided biopsy of
the pancreatic mass. In the meantime between
both pathological studies, a pancreatic MRI
was performed, revealing clusters of simple
cysts all over the pancreas (head, corpus and
tale) as well as a 5 cm lobulated mass at the
head of the pancreas containing multiple cysts
with thin walls and internal septations,
Figure 1. An abdominal computed tomography scan
shows a large solid renal mass in the left kidney (white
Figure 2. Abdominal magnetic resonance imaging
reveals a lobulated mass in the head of the pancreas
(white arrow) containing cysts and clusters of simple
cysts over the pancreas (black arrow).

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showing high signal on T2-weighted images
and with gadolinium enhancement of
septations (Figure 2). These findings
suggested a microcystic adenoma which was
later confirmed in the pathological specimen
(Figures 3 and 4). Signs for VHLD were then
actively searched for.
No retinal angiomas were seen on dilated eye
examination. A cerebellar hemangioblastoma
measuring 1 cm in diameter was easily
assessed by contrast-enhanced T1-weighted
craniospinal MRI (Figure 5). A second well-
defined lesion, also with 1 cm in diameter was
found in the spinal cord (Figure 6). Without
typical contrast-enhancement in the MRI, the
most probable radiological diagnosis is a cyst.
The final diagnosis was, therefore, VHLD.
Renal-cell carcinoma accounts for 80-85% of
malignant kidney tumors [1]. All potentially
malignant localized lesions require surgery
[7], allowing pathological diagnosis of the
tissue obtained at surgery. Although other
histologic types of renal cancer could present
as a 9 cm heterogeneous renal mass [1], they
are treated in the same way as renal-cell
carcinoma. Therefore, since renal-cell
carcinoma is responsible for most malignant
renal lesions, the differential diagnosis was
focused on the pancreatic mass whose
etiology was far more doubtful.
As in many other cases, the patient showed no
particular signs or symptoms related to the
Figure 3.
Pancreatic microcystic adenoma.
Hematoxylin-eosin (H&E) staining.
Figure 5. Craniospinal magnetic resonance imaging
demonstrates a cerebellar haemangioblastoma (white
Figure 6. Craniospinal magnetic resonance imaging
demonstrates a spinal cord cyst (white arrow).
Figure 4.
Pancreatic microcystic adenoma.
Immunohistochemical study.

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JOP. Journal of the Pancreas – http://www.joplink.net – Vol. 6, No. 2 – March 2005. [ISSN 1590-8577]
cystic pancreatic lesion which had been
detected by the abdominal CT performed for
the evaluation of the renal mass [3]. Despite
the fact that his past medical history showed
no recurrent attacks of upper abdominal pain
and he had never been admitted for acute
pancreatitis, the possibility of a pancreatic
pseudocyst or chronic pancreatitis had to be
considered in the differential diagnosis of the
cystic pancreatic mass owing to the alcohol
abuse. Similarities in the radiological
presentation have previously been described
with pancreatic cysts, pancreatic pseudocysts
[8] and chronic pancreatitis [9].
Pancreatic metastasis from renal-cell
carcinoma is a rare presentation. However,
this condition has already been reported [10,
11]; in fact, the most common primary tumor
origin of solitary pancreatic metastasis is
renal-cell carcinoma. A pathological study is
required to differentiate primary and
secondary pancreatic tumors.
Although dynamic intravenous contrast-
enhanced CT is an accurate technique for
detecting and characterizing a pancreatic mass
[2], an MRI with intravenous gadolinium is
superior to CT in evaluating some findings.
MRI may depict additional septa or
enhancement [12, 13]. The evaluation of the
pancreatic mass with MRI in this particular
patient identified the cystic lesion and was
particularly useful in determining the
presence of thin walls and internal septations
with gadolinium enhancement, which is
typical in microcystic adenomas [14].
Pancreatic cysts occur in approximately 40%
of patients with VHLD while microcystic
adenomas are less common, with a reported
incidence of 4% [5]. Interestingly, a recent
review article of pancreatic cystic neoplasms
[4] adds a lot of information regarding the
pathology, diagnosis and management of
these tumors. Chromosomal alterations of the
gene for VHLD have been found in DNA
extracted from many microcystic adenomas in
several series [15]. They are benign lesions
[16] and, although management has not been
fully established, if the likelihood of these
lesions becoming symptomatic is very low,
non-invasive treatment with continued
radiological observation is acceptable [4, 17].
Hemangioblastomas of the central nervous
system are the most common tumors in
VHLD, affecting 60-80% of all patients [18].
They are normally placed in the cerebellum or
the spinal cord, and associated cysts occur in
30-80% of hemangioblastomas. A cerebellar
hemangioblastoma was found in our patient
together with a well-defined spinal lesion
consistent with a cyst.
The combination of renal-cell carcinoma,
pancreatic microcystic adenoma, pancreatic
cysts and cerebellar hemangioblastoma
provides the diagnosis of VHLD. The patient
had no past familiar history of VHLD,
suggesting a type 1 VHLD. It is an autosomal
dominant neoplasia syndrome resulting from
a mutation in the VHL gene on chromosome
3 [19]. This mutation leads to the
development of benign and malignant lesions
in many different organs [6], including: the
central nervous system (brainstem,
endolymphatic sac, adrenal glands, pancreas,
kidneys, epididymis (male) and broad
ligament (female). In 1996, there were more
than 137 distinct mutations reported in
affected families, including missense, non-
sense, microdeletion, insertion, deletion and
splice site [20]. Any of these mutations can be
detected in nearly 100% of the affected
families [21]; however, genetic testing is a
challenge in de novo cases. Only 20% of such
patients test positive in genetic studies [22].
Figure 7. Renal-cell carcinoma. Hematoxylin-eosin
(H&E) staining.

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Although genetic studies are not needed for
the diagnosis of VHLD, the patient was found
to have a deletion of exon 3 of the VHL gene.
His relatives are now being tested for VHLD;
he has no children to be studied.
Since the renal-cell carcinoma was 9 cm in
diameter and perinephric fat was involved, the
kidney could not be preserved and a total
nephrectomy (including perirenal fat) was
performed as recommended [23]. The
diagnosis was confirmed by the pathological
sample (Figure 7). The patient is now
symptom-free and is being followed in
outpatient consultation. Because VHLD is a
chronic problem with a high frequency of new
lesions arising with time and the recurrence of
those already treated [17], patients need to be
included in a routine scheduled follow-up for
screening and early diagnosis which has been
proven to increase median survival [24]. The
recommended tests [17] in VHLD type 1 are:
ophtalmoscopy, plasma or 24 h urinary
catecholamines, MRI of craniospinal axis and
CT of abdomen (both radiological
explorations before and after contrast
infusion) and audiological function tests only
if clinically indicated. All the screening tests
are performed yearly.
However, the optimal screening test for
abdominal manifestations of patients with
VHLD remains unclear though. Abdominal
ultrasound has poor sensitivity and a negative
predictive value especially for adrenal and
pancreatic lesions. CT abdominal examination
remains an excellent test for screening and is
fully accepted by most physicians [17], who
use MRI only in patients with renal
impairment or contrast allergy. However,
many authors believe that MRI provides high
definition abdominal imaging without
radiation risk [14], which is especially
important in young patients with VHLD who
need yearly screening radiological tests.
Furthermore, MRI allows one to perform
abdominal and craniospinal imaging in the
same visit with only one injection of contrast,
making it easier for the patient to cope with
this chronic problem. It has also recently been
reported [13] that MRI can provide more
accurate information regarding renal cysts
than CT but additional studies are needed for
the appropriate interpretation of MRI imaging
of renal cysts.
With approximately 1 per 36,000 people
affected [25], VHLD is a rare syndrome
commonly affecting young patients with a
mean age onset of symptoms and signs at 31
years of age [24]. Although in the present
case the patient was a 50-year-old and he was
admitted because of hematuria due to a renal-
cell carcinoma (that is know to be the main
cause of death [24]), he is still alive.
In conclusion, our case illustrates two rare
conditions. First, the delayed presentation of
the symptoms and second, the presence of a
pancreatic microcystic adenoma, which has
only been reported in 4% of patients as noted
Received December 15th, 2004 - Accepted
January 10th, 2005
Keywords Carcinoma, Renal Cell; Pancreas;
Pancreatic Neoplasms
Abbreviations CA: cancer antigen; VHLD:
Von Hippel Lindau disease
Carlos Mirete Ferrer
Avenida Oscar Esplá 11, 1º, pta. 1.
03130 Santa Pola, Alicante
Phone: +34-965.413.031
Fax: +34-965.677.072
E-mail: mirete_car@gva.es
1. Kosary CL, McLaughlin JK. Kidney and renal
pelvis. In: Miller BA, Ries LAG, Hankey BF, eds:
SEER cancer statistics review, 1973-1990. Bethesda,
MD, USA: National Cancer Institute, 1993. (NIH
publication no. 93-2789, XI.1-XI.22).
2. Warshauer DM, McCarthy SM, Street L,
Bookbinder MJ, Glickman MG, Richter J, et al.
Detection of renal masses: sensitivities and specificities
of excretory urography/linear tomography, US and CT.
Radiology 1988; 169:363-5. [PMID 3051112]

Page 6
JOP. J Pancreas (Online) 2005; 6(2):172-177.
JOP. Journal of the Pancreas – http://www.joplink.net – Vol. 6, No. 2 – March 2005. [ISSN 1590-8577]
3. Bassi C, Salvia R, Molinari E, Biasutti C, Faleoni
M, Pederzoli P. Management of 100 consecutive cases
of pancreatic serous cystadenoma: wait for symptoms
and see at imaging or viceversa? World J Surg 2003;
27:319-23. [PMID 12607059]
4. Brugge WR, Lauwers GY, Sahani D, Fernandez-
del Castillo C, Warshaw AL. Cystic neoplasms of the
pancreas. N Engl J Med 2004; 351:1218-26. [PMID
5. Hough DM, Stephens DH, Johnson CD, Binkowitz
LA. Pancreatic lesions in von Hippel-Lindau disease:
prevalence, clinical significance and CT findings. AJR
Am J Roentgenol 1994; 162:1091-4. [PMID 8165988]
6. Friedrich CA. Von Hippel-Lindau syndrome: a
pleomorphic condition. Cancer 1999; 86(11
suppl):2478-82. [PMID 10630173]
7. Robson CJ, Churchill BM, Anderson W. The
results of radical nephrectomy for renal cell carcinoma.
J Urol 1969; 101:297-301. [PMID 5765875]
8. Warshaw AL, Rutledge PL. Cystic tumors
mistaken for pancreatic pseudocysts. Ann Surg 1987;
205:393-8. [PMID 3566376]
9. Luetmer PH, Stephens DH, Ward EM. Chronic
pancreatitis: reassessment with current CT. Radiology
1989; 171:353-7. [PMID 2704799]
10. Yachida S, Fukushima N, Kanai Y, Nimura S,
Shimada K, Yamamoto J, Sakamoto M. Pancreatic
metastasis from renal cell carcinoma extending in the
main pancreatic duct: a case report. Jpn J Clin Oncol
2002; 32:315-7. [PMID 12411571]
11. Robbins EG 2nd, Franceschi D, Barkin JS.
Solitary metastasic tumors to the pancreas: a case
report and review of the literature. Am J Gastroenterol
1997; 91:2414-7. [PMID 8931428]
12. Semelka RC, Shoenut JP, Magro CM, Kroeker
MA, MacMahon R, Greenberg HM. Renal cancer
staging: comparison of contrast-enhanced CT and
gadolinium–enhancing fat-supressed spin-echo and
gradient-echo MR imaging. J Magn Reson Imaging
1993; 3:597-602. [PMID 8347952]
13. Israel GM, Hindman N, Bosniak M. Evaluation of
cystic renal masses: comparison of CT and MRI by
using the Bosniak Classification System. Radiology
2004; 231:365-71. [PMID 15128983]
14. Tattersall DJ, Moore NR. Von Hipple-Lindau
Disease: MRI of abdominal manifestations. Clin Radiol
2002; 57:85-92. [PMID 11977939]
15. Mohr VH, Vortmeyer AO, Zhuang Z, Libutti SK,
Walther MM, Choyke PL, et al. Histopathology and
molecular genetics of multiple cysts and microcysts
(serous) adenomas of the pancreas in von Hippel-
Lindau patients. Am J Pathol 2000; 157:1615-21.
[PMID 11073821]
16. Hamilton SR, Aaltonen LA. Pathology and
genetics of tumours of the digestive system. World
Health Organization Cassification of Tumours. Vol 2.
Lyon, France: IARC Press, 2000.
17. Lonser RR, Glenn GM, Walther M, Chew EY,
Libutti SK, Linehan WM, et al. Von Hippel-Lindau
disease. Lancet 2003; 361:2059-67. [PMID 12814730]
18. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH.
The natural history of central nervous system
hemangioblastomas in patients with von Hipple-Lindau
disease. J Neurosurg 2003; 98:82-94. [PMID
19. Linehan WM, Lerman MI, Zbar B. Identification
of the von Hippel-Lindau (VHL) gene. Its role in renal
cancer. JAMA 1995; 273:564-70. [PMID 7837390]
20. Zbar B, Kishida T, Chen F, Schmidt L, Maher ER,
Richards FM, et al. Germline mutation in the von
Hippel-Lindau diease (VHL) gene in families from
North America, Europe and Japan. Hum Mutat 1996;
8:348-57. [PMID 8956040]
21. Stolle C, Glenn G, Zbar B, Humphrey JS, Choyke
P, Walther M, et al. Improved detection of germline
mutations in the von Hipple-Lindau disease tumour
supppressor gene. Hum Mutat 1998; 12:417-23. [PMID
22. Sgambati MT, Stolle C, Choyke PL, Walther MM,
Zbar B, Linehan WM, et al. Mosaicism in von Hippel-
Lindau disease: lessons from kindreds with germline
mutations identified in offspring with mosaic parents.
Am J Human Genet 2000; 66:84-91. [PMID 10631138]
23. Motzer RJ, Bander NH, Nanus DM. Renal-cell
carcinoma. N Engl J Med 1996; 335:865-75. [PMID
24. Maher ER, Yates JR, Harries R, Benjamin C,
Harris R, Moore AT, et al. Clinical features and natural
history of von Hippel-Lindau disease. Q J Med 1990;
77:1151-63. [PMID 2274658]
25. Neumann HP, Wiestler OD. Clustering of features
of von Hippel-Lindau syndrome: evidence for complex
genetic locus. Lancet 1991; 337:1052-54. [PMID

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