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348
S. Shrivastava
Ferrari etal., in 2014, described a phyllodes tumor of the prostate in a healthy
young man presenting with symptoms of urinary tract infection [5]. Bostwick and
colleagues presented a three-stage grading system for phyllodes prostate tumors by
classifying the histological ndings as low, intermediate, and high grades. They
reported 50% recurrence rates even in low-grade tumors. There is an elevated risk
of malignant occurrence with multiple recurrences of this tumor, leading to
decreased survival rates [6]. There is a remarkable resemblance between the phyllodes neoplasm of mammary glands and the prostate. Typically, leaf-like stromal
protrusions and cystically dilated ducts are seen in both neoplasms. They display
variable features such as stromal necrosis, mitotic activity, multicellularity, and
atypia [4]. Immunohistochemical staining with various markers like Ki-67, proliferative nuclear antigens, and protein 53 (p53) expression is used for assessment [7].
The verumontanum is a vital structure of the prostatic urethra due to the presence
of ejaculatory ducts, which play an essential role in reproduction. Polyps or cysts of
the verumontanum can present with the symptoms of urinary tract infection and
dysuria [8]. Phyllodes tumors typically occur in the breast tissue of females [9].
However, it is rare in verumontanum, and therefore this patient presenting with
atypical symptoms, was diagnosed with prostatic hyperplasia. Diagnostic investigations include per- rectal ultrasound, urodynamic ow studies, magnetic resonance
imaging, prostate- specic antigen (PSA) levels, and histopathological evaluation of
prostatic biopsy. Phyllodes tumors exhibit distinctive features of epithelial and stromal tissues, which are essential for diagnostic conrmation [9]. They are classied
on the basis of histological appearances such as inltration of lump margins,
necrotic debris, overgrowth, atypical proliferation, or count of mitotic cells per
high-power eld [9]. The ndings of multicellularity and mitotic activity signify
malignancy and metastatic possibility in the breast. In this case report, the tumor
was identied as low grade with the Ki-67 proliferation index of 12% and minimal
tumor proliferation. The prognosis and dependable outcomes of phyllodes tumors
are still ill-dened. The phyllodes tumors of seminal vesicles are usually regarded
as high-grade tumors due to high mitotic activity, stromal proliferation, and
pleomorphism.
For breast phyllodes tumors, wide excision with clear margins is indicated to
prevent recurrences and improve survival [10]. In this case, complete resection of
the tumor was performed by plasmakinetic intervention expecting better long-term
outcomes. The patient fared well with quick postoperative recovery.
Conclusion
Phyllodes tumor of verumontanum is hardly reported, and this is the only case
reported to date. This neoplasm is rare, known to occur in female breast tissue, and
behaves similarly to the histological pattern in the prostatic tumor. Patients with
phyllodes growth in the prostate present with prostatic hyperplasia symptoms and
are often misdiagnosed before surgery. Young patients presenting with bladder
47 Phyllodes Tumor Misdiagnosed asBenign Prostatic Hypertrophy andaCyst
349
outow obstruction and detected to have a posterior urethral mass should be suspected of a probable phyllodes tumor. Cystoscopy should always be considered to
conrm the location and nature of the tumor. Complete surgical excision with intact
margins is always advised. This patient behaved well postoperatively with excellent
recovery and improved urodynamics. However, long-term follow-up is mandatory
to look for recurrences and timely intervention. The nature of this neoplasm in terms
of management, survival, and neoplastic behavior is still debatable.
References
1. Tang J, He L, Long Z, Wei J.Phyllodes tumor of the verumontanum: a case report. Diagn
Pathol. 2015 Dec;10(1):1–4.
2. Razi A, Yahyazadeh SR, Saffar H.Phyllodes tumor of the prostate: Long-term follow up of a
rare condition. Urology Case Reports. 2020 Jan;1(28):101015.
3. Bostwick DG, Meiers I, Hossain D. Phyllodes tumor of the prostate: current concepts and
update on classication of prostatic soft tissue tumors. AJSP: Reviews & Reports. 2007 Jul
1;12(4):160–5.
4. Aydogdu O, Atesci YZ, Karakose A, Demirtas E.Benign phyllodes tumour of the prostate: an
extremely rare entity. Can Urol Assoc J. 2014 Jul;8(7–8):E548.
5. Ferrari M, Capitanio U, Rizzo N, Freschi M, Montorsi F, Rigatti P. Onset of hydronephrosis
and lower urinary tract symptoms in a previously healthy young man: Phyllodes tumor of the
prostate as a potential diagnosis. Can Urol Assoc J. 2014 Jul;8(7–8):E561.
6. Bostwick DG, Hossain D, Qian J, Neumann RM, Yang P, Young RH, di Sant’agnese PA,
Jones EC.Phyllodes tumor of the prostate: long-term followup study of 23 cases. J Urol. 2004
Sep;172(3):894–9.
7. Shiraishi K, Mohri J, Eguchi S, Kamiryo Y, Sanefuji H.Phyllodes tumor of the prostate: recurrent obstructive symptom and stromal proliferative activity. Int J Urol. 2004 Sep;11(9):801–4.
8. Sadler TW.Langman’s medical embryology. 7th ed. Baltimore: Williams & Wilkins; 1995.
9. Xu LW, Wu HY, Yu YL, Zhang ZG, Li GH.Large phyllodes tumour of the seminal vesicle:
case report and literature review. J Int Med Res. 2010 Oct;38(5):1861–7.
10. Spitaleri G, Toesca A, Botteri E, Bottiglieri L, Rotmensz N, Boselli S, Sangalli C, Catania C,
Toffalorio F, Noberasco C, Delmonte A.Breast phyllodes tumor: a review of literature and a
single center retrospective series analysis. Crit Rev Oncol Hematol. 2013 Nov 1;88(2):427–36.
Chapter 48
Large Calcied Renal Artery Aneurysm
Misdiagnosed asIntrapelvic Calculus
ShashwatShrivastava
Learning Objectives
By the end of this presentation, the clinician will be able to:
1. Discuss and consult with the radiology department on the slightest of doubtful
images which are not in consensus with the patient’s clinical and physical
examination.
2. Express the importance of practicing a guided surgical approach which could
help prevent operative complications especially under adverse surgical ndings.
3. Evaluate elderly female patients presenting in the sixth decade of life with
hypertension and low-density lesions in the renal pelvis for a possibility of renal
artery aneurysm and deem further tests.
4. Infer patients having a lesion occupying the course of renal artery branches as an
underlying case of vascular pathology.
Introduction
The incidence of renal artery aneurysm (RAA) is remarkably low, approximating
to 0.09% in the general population. However, in the hypertensive subgroup, the
incidence rates can reach up to 2.5% [1]. Previously, most of the information on
RAA came from autopsy studies, and the incidence was claimed to be extremely
rare (<0.01%–0.09%) [2, 3]. The angiographic records improved the diagnosis
accuracy, but the RAA incidence rates barely managed to rise to 0.3%–0.7% [3].
RAA is shown to have a slow growth rate with no difference in the rates to changes
S. Shrivastava (*)
St. Martinus University Faculty of Medicine, Willemstad, Curacao
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
H. Tohid et al. (eds.), The Misdiagnosis Casebook in Clinical Medicine,
https://doi.org/10.1007/978-3-031-28296-6_48
351
352
S. Shrivastava
in aneurysm morphology or calcication [2]. We still have limited information on
their occurrence, natural history, and prognosis with or without treatment [4]. The
incidence rates are slowly on the rise due to frequent use of cross-sectional
imaging [4].
Below is a rare occurrence of RAA, which was incidentally found during the
operation [1]. The patient was mistakenly diagnosed with a large right renal calculus during ultrasound and was scheduled for percutaneous nephrolithotomy (PCNL)
as a mandatory therapeutic intervention. In this case, the patient had concomitant
calcications within the renal artery aneurysm, which skewed the diagnosis toward
a renal calculus upon imaging [1]. Laying emphasis on the images produced by the
computed tomography (CT) scan and ultrasound becomes vital to differentiate
between a calcied RAA and renal calculus. Important markers like acoustic
shadow and low-density regions must raise concern for an underlying vascularity
and facilitate radiologist consultation [1].
Also, paying close attention to these ndings can avoid mistreatment and invasive therapeutic procedures which may potentiate harm especially under an alternate diagnosis. PCNL intervention around RAA territory can elevate the risk of
aneurysmal rupture. This case was managed heroically by carefully abiding by the
basics of PCNL procedure and maneuvering the operation route to escape the risk
of aneurysmal rupture. More elaborate picture of this has been presented in the discussion section below.
This case report highlights the clinical markers which should raise suspicion for
the likelihood of renal artery aneurysm and details on how the clinical adversity was
rectied. It also signies the importance of deducing the image ndings accurately
and going for second consultation amid uncertainty. We hope to educate physicians
on a rare case of a patient having RAA being misdiagnosed as renal calculus and
reduce further rates of misdiagnosis through learnings from this case report.
Clinical Case Presentation
A 69-year-old woman was admitted to the hospital after nding a large right renal
calculus on ultrasound examination. She has a past medical history of renal calculi
and diabetes mellitus. The ultrasound revealed the presence of a large calculus in
the right renal pelvis, multiple calculi in the left kidney, and hydronephrosis in both
the kidneys. The shadows in the right renal pelvis were described as a “hyperechoic
focus with an acoustic shadow.” The ultrasound ndings were reinforced after
doing the kidney-ureter-bladder (KUB) radiograph. However, the core of the calculus was noticed to be radiolucent on plain X-ray. The patient was devoid of any
discomfort, and signs and symptoms were unremarkable. The computed tomography urography (CTU) also yielded similar outcomes. A large calculus and hydrops
in the upper calyx were seen on the right kidney, and the left kidney showed multiple left renal calculi with hydronephrosis. However, upon reassessing CTU
results, a low-density lesion was seen within the calculus on a
48 Large Calcied Renal Artery Aneurysm Misdiagnosed asIntrapelvic Calculus
non-contrast- enhanced CTU sequence. The density of the right renal calculus was
noted to be 508 HU with a size of 3 × 2.3cm. This low-density lesion was hypothesized as an underlying infection or presence of a foreign body. In the light of new
ndings, empirical antibiotics were initiated. Percutaneous nephrolithotomy
(PCNL) was decided on the right renal pelvis calculus. Posterior lower renal calyces were chosen as the puncture site to establish an operation route. No calculus
was witnessed in the renal pelvis during the operation; however, a calcied lesion
was felt with the forceps under the mucosa of the renal pelvis. The doctors decided
to remove the mucosa using a holmium laser until the calculus was visible. To their
surprise, while cutting the surface of the calculus, a uctuating unruptured aneurysm was observed. This led to a new diagnosis of an unruptured renal artery aneurysm (RAA), and the previous diagnosis was dismissed. The operation was ceased
immediately, and the patient was transferred to the endovascular unit. Computed
tomography (CT) angiography was performed after checking hemoglobin levels
post-PCNL. CT angiography exposed multiple aneurysms in both the kidneys. The
right RAA was the largest one, located on the rst bifurcation of the renal artery.
On a contrast-enhanced CT scan, a branch of the right renal artery exhibited connectivity with the aneurysm. The endovascular unit later performed an RAA embolization after the ninth day of PCNL [1].
353
Differential Diagnosis
1. Intrapelvic calculus: Hyperechoic lesion on the imaging test skewed the diagno-
sis toward an intrapelvic calculus.
2. Foreign body: Absence of typical signs of urolithiasis along with a high-density
lesion must raise suspicion for a foreign body.
3. Infectious etiology: Strong echo on ultrasound and radiolucency on X-ray could
have raised a possibility for air-uid levels which is a predominant marker for
underlying infection. Absence of fever pointed against an infectious source.
Discussion
A renal artery aneurysm is dened as the dilation of a segment of a renal artery that
is twice the diameter of a normal renal artery [4]. The American College of
Cardiology (ACC) and the American Heart Association (AHA) elucidate renal
artery aneurysm as size >1.5 times the diameter of the adjacent disease-free proximal arterial segment [5]. The general incidence of RAA in the general population is
estimated to be 1% [3]. RAA is typically witnessed in the sixth decade of life.
Women being more prone to bromuscular dysplasia have higher incidence rates of
renal artery aneurysm [6]. A study done by Lumsden etal., on 28 reported RAA
cases, brought similar results, with 18 women and 10 men being aficted with
354
S. Shrivastava
RAA.The etiology of RAAs was essentially atherosclerosis (75%), bromuscular
disease (21%), and Ehlers-Danlos syndrome (4%) [7]. The majority of RAA patients
are asymptomatic with unremarkable signs [3]. A few patients present with hematuria, abdominal pain, and ank pain. Hypertension seems to be the most common
clinical nding in patients having RAA.Calcications are observed in 56% of the
cases [8], making it crucial to distinguish between renal calculi and the presenting
aneurysm. Computed tomography is the most prevalent contemporary diagnostic
modality, followed by magnetic resonance imaging (MRI), ultrasonography, and
catheter-based arteriography [8]. If the RAA has concomitant calcications, its propensity to get misdiagnosed as a renal calculus escalates dramatically, especially if
the calcied vessel traverses through the sinus [9]. The ultrasound and CT scan
showed a 3×2.3cm-sized calculus on the right renal pelvis in the presenting case.
The CT scan noted a low central density of 508 HU which should have given an
inclination toward RAA, especially when considering the patient’s gender and her
sixth decade of life. Ultrasound gave similar remarks of the presence of a hyperechoic lesion and plain lms revealed the core as radiolucent. The two differentials
to explain these ndings were a foreign body and an underlying infection. Calcied
RAA did not occur as a potential differential at the time, which mistakenly led to
PCNL surgery [1]. The paper goes on to say how following two principles of PCNL
surgery prevented the risk of complications. First is by following the direction of
renal papillae and creating an operation route instead of directly targeting the renal
calculus. Secondly, the surface was manipulated rst during lithotripsy instead of
focusing on the calculus’s core [1]. Currently accepted indications for RAA intervention include the size of >2 cm, female gender within childbearing age, and
symptoms like pain, hematuria, and medically refractory hypertension (HTN) [3].
Open repair and endovascular repair have been the two primary strategic interventions for RAA for a long time. Recent advancements in endovascular repair have
given the procedure an equal footing with open repair, especially considering its
minimally invasive attributes. Upon comparison, no differences are reported in mortality, perioperative morbidity, length of hospital stays, and freedom from re-intervention [3, 8]. However, Cochennec F etal. state that despite endovascular repair
being a good alternative, it remarkably raises the propensity of aneurysmal reperfusion. The authors treat endovascular repair as the primary choice of intervention in
elderly patients and patients with ruptured aneurysms [10]. For this patient, the
endovascular repair was chosen because the calcied wall was destroyed by the
PCNL procedure. Chen etal. thought of the possibility of the aneurysmal wall being
corroded by urine, which might increase the risk of rupture [1].
Conclusion
The primary reason for the unnecessary invasive procedure was the wrong diagnosis
that was made before the operation. Poor attention was paid to the anomalies noted
on imaging. The radiolucent core was given a differential of an underlying
48 Large Calcied Renal Artery Aneurysm Misdiagnosed asIntrapelvic Calculus
355
infection, which failed to respond to the antibiotic therapy. More awareness should
be maintained while deciphering KUB, CT scan, and ultrasound statuses, and physicians should be mindful of rare causes in the setting of clinical adversity. Ring-like
calcication in the kidney region and strong echo lesions around the renal sinus
rmly hint at a possibility of RAA. The patient’s age, female gender, history of
hypertension, and low-density core surrounded by calcications must have raised
suspicion of an underlying renal artery aneurysm. Chen etal. admit that the absence
of classical signs and symptoms of urolithiasis should have paved the way for an
alternate diagnosis.
References
1. Chen C, Wang X, Xin F, He L, Jiang K, Shao J, Xie L.Large calcied renal artery aneurysm
in the renal sinus misdiagnosed as an intrapelvic calculus followed by mistakenly performed
PCNL: a case report. BMC Nephrol. 2020 Dec;21(1):1–5.
2. Tham GU, Ekelund L, Herrlin K, Lindstedt EL, Olin T, Bergentz SE.Renal artery aneurysms.
Natural history and prognosis. Ann Surg. 1983 Mar;197(3):348.
3. Coleman DM, Stanley JC.Renal artery aneurysms. J Vasc Surg. 2015 Sep 1;62(3):779–85.
4. González J, Esteban M, Andrés G, Linares E, Martínez-Salamanca JI.Renal artery aneurysms.
Curr Urol Rep. 2014 Jan;15(1):1–9.
5. Hirsch AT, Haskal ZJ, Hertzer NR, Bakal CW, Creager MA, Halperin JL, Hiratzka LF, Murphy
WR, Olin JW, Puschett JB, Roseneld KA. ACC/AHA guidelines for the management of
patients with peripheral arterial disease (lower extremity, renal, mesenteric, and abdominal
aortic). J Vasc Interv Radiol. 2006 Sep 1;17(9):1383–98.
6. Stanley JC, Rhodes EL, Gewertz BL, Chang CY, Walter JF, Fry WJ.Renal artery aneurysms:
signicance of macroaneurysms exclusive of dissections and brodysplastic mural dilations.
Arch Surg. 1975 Nov 1;110(11):1327–33.
7. Lumsden AB, Salam TA, Walton KG.Renal artery aneurysm: a report of 28 cases. Cardiovasc
Surg. 1996 Apr 1;4(2):185–9.
8. Klausner JQ, Lawrence PF, Harlander-Locke MP, Coleman DM, Stanley JC, Fujimura N, Itoga
NK, Mell MW, Duncan AA, Oderich GS, Rizvi AZ.The contemporary management of renal
artery aneurysms. J Vasc Surg. 2015 Apr 1;61(4):978–84.
9. Chen S, Meng H, Cao M, Shen B. Renal artery aneurysm mimicking renal calculus with
hydronephrosis. Am J Kidney Dis. 2013 Jun 1;61(6):1036–40.
10. Cochennec F, Riga CV, Allaire E, Cheshire NJ, Hamady M, Jenkins MP, Kobeiter H, Wolfe
JN, Becquemin JP, Gibbs RG. Contemporary management of splanchnic and renal artery
aneurysms: results of endovascular compared with open surgery from two European vascular
centers. Eur J Vasc Endovasc Surg. 2011 Sep 1;42(3):340–6.
Chapter 49
Anastomosing Hemangioma Misdiagnosed
asRenal Cell Cancer
Rupanshu
Learning Objectives
By the end of this presentation, the clinician will be able to:
1. Differentiate the ndings from imaging techniques of anastomosing hemangioma from that of renal cell carcinoma.
2. Compare and contrast the relationship between end-stage renal disease (ESRD)
and renal cell carcinoma.
3. Discuss the differential diagnosis of anastomosing hemangioma.
4. Analyze anastomosing hemangioma and why it can be misdiagnosed as renal
cell carcinoma.
5. Discuss the ndings from imaging techniques of anastomosing hemangioma.
Introduction
Renal adenocarcinoma, or renal cell carcinoma or hypernephroma, is one of the
most common subtypes of cancer seen in patients with end-stage kidney disease [1].
On the other hand, anastomosing hemangioma (AH) is a rare and benign vascular
neoplastic vascular lesion. It is mainly composed of irregularly anastomosing
sinusoidal- like spaces lined by endothelial cells. They are also more frequently seen
in end-stage renal disease (ESRD) [2]. It is primarily asymptomatic, often discovered incidentally, as was found in this case by imaging studies, owing to previously
existing benign or malignant tumors. The ndings of anastomosing hemangioma
Rupanshu (*)
St. Martinus University Faculty of Medicine, Willemstad, Curacao
e-mail: rupanshu.rupanshu@martinus.edu
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
H. Tohid et al. (eds.), The Misdiagnosis Casebook in Clinical Medicine,
https://doi.org/10.1007/978-3-031-28296-6_49
357
358
Rupanshu
from radiology are similar to those of renal cell carcinoma [3, 4]. Previous studies
have also reported the incidental detection of anastomosing hemangiomas in the
kidneys or the adrenal glands [3].
Ever since its original description in the genitourinary system, an increasing
number of anastomosing hemangiomas have been reported, deep in the body in
various organs, with the same histologic features. In 2009, Montgomery and
Epstein described a series of vascular tumors—named “anastomosing hemangioma”—with a complex architecture characterized by anastomosing vessels, rare
hobnailing, and benign behavior [5]. Anastomosing hemangiomas have been
reported to occur most commonly in the kidney. However, they can occur in other
sites as well, such as the testes, thigh, abdominal wall, ovaries, adrenal gland,
liver, and even gastrointestinal tract. Patients suffering from ESRD with an anastomosing hemangioma can be listed for living-donor kidney or deceased-donor
kidney transplantation. The difculty comes in because the imaging ndings for
anastomosing hemangiomas are similar to those for renal cell carcinomas [6]. The
mean age of presentation of an anastomosing hemangioma is 50 years (range,
15–83years), and the male sex accounted for 68.8% of the cases reported in the
literature. The patients were mostly asymptomatic, with 62% of the cases documented as incidental ndings [7]. Microscopically, the typical pathological image
is sinusoidal anastomosing capillary- sized vessels with biological features of
inltration. Herein, a case is described in which an anastomosing hemangioma
was misdiagnosed as a renal cell carcinoma before kidney transplantation
took place.
Clinical Case Presentation
A 35-year-old woman suffering from lupus nephritis was admitted to the emergency
department for the suspected uremic symptoms of nausea and general malaise. She
had received hemodialysis due to ESRD, and living-donor kidney transplantation
from her father was planned. On preoperative contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI), a 1.7cm renal cell carcinoma
was found in the right kidney. After staining was done that took place after the radical nephrectomy, irregularly shaped vascular spaces of different sizes were observed,
with these spaces having an anastomosing pattern. With these ndings of the anastomosing hemangioma similar to those of a renal cell carcinoma on imaging, histology examination was important to conrm the diagnosis of anastomosing
hemangioma and prevent misdiagnosis and delay in listing for kidney
transplantation.
During the pretransplantation medical workup, an enhanced CT of the abdomen
was done which revealed a heterogeneous mass of 1.7cm in diameter, located in the
upper pole of the right kidney. On MRI, it was revealed that this mass was presented
with high signal intensity on T2-weighted images, with heterogeneous enhancement done in the right kidney. Based on these ndings, a diagnosis of renal cell
carcinoma, stage T1aN0, was made. Because the right renal mass was small, with
49 Anastomosing Hemangioma Misdiagnosed asRenal Cell Cancer
no associated symptoms, a simultaneous right radical nephrectomy and kidney
transplant were planned by the surgeon and urologist.
Open radical nephrectomy was performed through an incision in the subcostal
area; the patient was then positioned for the kidney transplant. Hematoxylin and
eosin staining was performed after nephrectomy revealed irregularly shaped vascular spaces of various sizes with an anastomosing pattern. After immunostaining, the
sample was positive for cluster of differentiation (CD)34 and CD31 and negative for
podoplanin, human herpesvirus-8, and glucose transporter-1. Based on these ndings, a denitive diagnosis of the anastomosing hemangioma was made.
Her laboratory values indicated levels of blood urea nitrogen and serum creatinine had increased manyfold, with her serum inorganic phosphate level also higher
than normal.
The treatment was planned with emergent hemodialysis and living-donor kidney
transplantation from her father. After the kidney transplantation, good renal function was achieved, with no tumor recurrence [8].
359
Differential Diagnosis
1. Angiosarcoma: Hemangiomas are rare and benign as they don’t metastasize to
other tissues. Angiosarcoma has diffusely inltrative growth patterns also seen
in anastomosing hemangioma with multilayering of endothelial cells and mitotic
activity.
2. Kaposi sarcoma: Kaposi sarcoma generally affects immunocompromised
populations.
Discussion
When Montgomery and Epstein rst described an anastomosing hemangioma of the
genitourinary tract, it was concluded that such types of hemangiomas were rare and
benign in contrast to an angiosarcoma which is cancer that aficts the inner lining
of blood vessels and lymph [5]. Hence, nephrectomy was not required clinically for
this benign vascular neoplasm. Moreover, patients having ESRD who have an anastomosing hemangioma can be listed for living-donor kidney transplantation or registered for deceased-donor kidney transplantation. The patient was scheduled for a
liver transplant; therefore the quick surgical removal of the heterogeneously enhancing kidney tumor took place immediately to prevent a delay in the transplant. The
difculty comes in that the imaging ndings for anastomosing hemangiomas are
similar to those for renal cell carcinomas, including heterogeneous enhancement of
lesions on a CT scan and hyperintensity on T2-weighted MRI images [6]. As subcutaneous biopsy of vascular lesions does pose a considerable challenge because of
the risk of profound bleeding [3], anastomosing hemangiomas have been diagnosed
by nephrectomy in the majority of reported cases.