Author Information
Gwendolyn Fernandes*, SV Parulekar**
(* Associate Professor, Department of Pathology, **
Professor and Head, Department of Obstetrics & Gynecology, Seth GS Medical
College & KEM Hospital, Parel, Mumbai, India.)
Abstract
A paraganglioma of the urinary bladder is a rare tumor that
develops from the chromaffin tissue of the sympathetic nervous system situated
outside the adrenal medulla. It is even more rare in pregnancy. We present a
rare case that became symptomatic only in successive pregnancies.
Introduction
A paraganglioma, also known as extra-adrenal
pheochromocytoma, is an extra-adrenal tumor. It develops from the chromaffin
tissue of the sympathetic nervous system
outside the adrenal medulla. It can cause severe hypertension and its
deleterious effects onthe mother and fetus both. Diagnosis and management of a
paraganglioma of the urinary bladder may be more difficult in pregnancy.
Case report
A 33 year old woman,
married for 3 years, gravida 2 para 1 living 0, with 3 months of amenorrhea,
presented with frank hematuria and occasional micturition syncope during the
last 6 days. There was no history of any pain in abdomen or from loin to groin,
and no dysuria. She gave a history of similar occurrence during her last
pregnancy a year ago, which had been complicated by severe hypertension,
convulsions, and had ended in a stillbirth. The symptoms had resolved after the
childbirth, and had not recurred until 6 days ago. Her general condition was
fair, pulse rate 68/min, respiratory rate 18/min, and blood pressure 120/70 mm
Hg. Systemic examination revealed no abnormality. The uterus was of 12-14
weeks’ size. Speculum examination showed no abnormality. Vaginal examination
showed a closed cervix, and a 3x1.5x1.5 cm transverse, nontender mass in the
area of the urinary bladder, behind the pubes. A diagnosis of a bladder mass
was made. Abdominal and pelvic ultrasonography (USG) showed a 6.5x5.5 cm sized
mass in the inferior wall of the bladder, a 10.8x5.6 cm hematoma within the
bladder and an intrauterine gestation of 12 weeks. Magentic resonance imaging
(MRI) of the abdomen and pelvis showed a 5.3x4.8x5.4 cm mass in the left and
inferior to the urinary bladder, a blood clot in the bladder, and an intrauterine
gestation of 12 weeks. There was no mass in the adrenal glands. A self
retaining urinary catheter was passed into the bladder. Plasma metanephrine
level was found to be 126 picogram/ml. Hemogram, fasting and postprandial blood
sugar levels, liver and renal function test results were normal, except that
her hemoglobin level was 9 g/dL.
Her blood pressure
showed episodic elevation to 149/90 mm Hg over a number of days. She was
administered amlodipine 5 mg qd, prazosin 2.5 mg qd by the endocrinologist as a
preventive measure, along with adequate hydration and salt intake. 17 alpha
hydroxyprogesterone caproate 250 mg deep IM was administered to prevent an
abortion triggered by operation on the bladder. Two units of packed red cells
were transfused. Transurethral biopsy of the tumor was done. Its histopathology
showed it to be a paraganglioma of the bladder. MRI brain showed no metastases.
Partial cystectomy along with excision of the paraganglioma was done. After
insertion of DJ stents into the ureters and placing a suprapubic drain, the
bladder was reconstructed. The abdomen was closed after placing two
intraperitoneal drains. The patient’s blood pressure rose to 190/110 mm Hg
during surgery, but came down to 90/60 mm Hg after surgery. She had to be administered
a dopamine infusion to maintain her blood pressure for 12 hours. She made an
uneventful recovery thereafter.
The tumor was
unencapsulated, measuring 8x6x4 cm with a dark brown color. Smooth muscle
fibres of the urinary bladder could be identified at the periphery. The
formalin fixative used to fix the specimen had a light brown color (figure 1).
Histology showed the tumor to be composed of large sheets of basophilic
polygonal neoplastic cells situated amidst the muscularis propria of the
urinary bladder. The sheets had a focal Zell Ballen pattern i.e. nest of cells
surrounded by a thin fibrovascular stroma. Individual cells had moderately
pleomorphic round nuclei with prominent nucleoli and granular basophilic
cytoplasm. The diagnosis was a
paraganglioma of the urinary bladder (figures 2, 3, and 4).
Figure 1.
Paraganglioma of the urinary bladder. Characteristic dark brown colored tumor measuring 8 x 6 x 4 cm. Lighter
colored smooth muscle fibers of the urinary bladder are seen in the lower
portion.
Figure 2. Large
sheets of basophilic chromaffin tumor cells surrounded by muscle fibers. (H
& E X 100)
Figure 3. Another
microphotograph showing nests of basophilic tumor cells splaying the muscularis
propria of the urinary bladder. (H & E X 100)
Figure 4. High power
view of the chromaffin cells showing characteristic Zell Ballen pattern. i.e.
small nest of cells showing alveolar arrangement. (H & E X 400)
The patient received
adequate antenatal care. She had some incontinence of urine up to term, which
was explained to be due to reduced capacity of the bladder and urge
incontinence. She underwent a cesarean section at 38 weeks of gestation for a
breech presentation and uteroplacental insufficiency, delivering a baby
weighing 2.8 kg. Postoperatively she was put on darifenacin 7.5 mg qd. She was
advised regular follow up examinations to detect any recurrence.
Discussion
A paraganglioma, also known as extra-adrenal
pheochromocytoma, is an extra-adrenal tumor that develops from the chromaffin
tissue of the sympathetic nervous system situated outside the adrenal medulla.[1]
It accounts for 6% of all paragangliomas and less than 0.05% of all bladder
tumors.[2] It is more common in women than in men ( 3:1), mainly in
the age group 30-50.[3] About 17% of them are hormonally inactive.[4]
Its clinical features include headache, palpitations,
sweating, and fainting (especially with micturition), paroxysmal hypertension
and hematuria. USG detects it in 8% cases, showing a submucosal homogeneous,
well delineated, vascular mass, sometimes with
necrosis at its center. MRI in diagnostic in almost 100% cases, also
defining its extension accurately.[5,6] Cystoscopy both shows the
mass and permits transurethral resection. A biopsy is not recommended, as it
can cause serious hemorrhage, hypertensive crisis, and its diagnostic rate is
low. The histological features of the tumor are as described in this case.
About 5-15% cases are malignant. Histology cannot distinguish between benign
and malignant tumors, and only local invasion or distant metastases are
diagnostic of malignancy.[7,8] Transurethral resection is adequate
if the tumor is less than 2-3 cm in size and does not infiltrate deep.[9]
In all other cases open surgery is preferred. Pregnancy does not contraindicate
the diagnostic and therapeutic approaches discussed. It is a unique situation
in that the hypertension caused by the tumor may be mistaken for toxemia of
pregnancy.[10] Our case was unique in that she had hematuria
suggestive of the presence of a bladder mass even in her past pregnancy
associated with hypertension, presumably due to the paraganglioma, which did
not cause any symptoms in between pregnancies. This then would be the first
case of a paraganglioma of the bladder that became manifest during successive
pregnancies only. This patient did not have any family history of such a
disorder. However she was counseled to have the family members screened inview
of about 10% cases being familial or genetic in nature.[11] She was
also advised to have at least one follow-up every year, since recurrences as
late as 10 years are known to occur.[12]
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Citation
Fernandes G, Parulekar SV. Paraganglioma of Bladder in
Pregnancy. JPGO 2014. Volume 1 Number 10. Available
from: http://www.jpgo.org/2014/10/paraganglioma-of-bladder-in-pregnancy.html