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Malignant paraganglioma of the urinary bladder:
Immunohistochemical study of prognostic indicators.Endocr
Pathol. 2005 Winter;16(4):363-9.
Using various
immunohistochemical markers, the objective of our study was to
assess whether correlation exists between growth potential of
paraganglioma (pheochromocytoma) cells and formation of metastasis.
The patient was a 28-yr-old man who presented with intermittent
episodes of gross hematuria due to a mass in the urinary bladder. He
had no constitutional symptoms to suggest paraganglioma. Histologic,
immunohistochemical, and electron microscopic investigation of the
surgically removed tissue proved that the tumor was a malignant
paraganglioma with metastases in the regional lymph nodes. The
immunohistochemical tests were not supportive of high cell
proliferation index, indicating that metastases can develop in the
absence of rapid multiplication of the tumor cells. Abnormalities in
vascular architecture and marked expression of VEGF in the tumor
cells may be regarded as prognostic signs to predict the formation
of metastases.
Paraganglioma of
the urinary bladder: a lesion that may be misdiagnosed as urothelial
carcinoma in transurethral resection specimens.Am
J Surg Pathol. 2004
Jan;28(1):94-100.
Paraganglioma
of the urinary bladder is a rare tumor with characteristic
histologic and immunohistochemical features. However, in our
experience, it may be misdiagnosed as urothelial cancer because of
1) its frequent involvement of the muscularis propria; 2) morphology
that may suggest urothelial cancer in transurethral resection
specimens, particularly if there are artifactual changes induced by
that procedure; 3) failure of pathologists to include it in their
differential diagnosis when evaluating a bladder tumor; and 4) only
a minority of the cases are associated with symptoms that might
prompt consideration of the diagnosis. Distinction between
paraganglioma and urothelial cancer is important because of likely
different therapeutic options. In this report, we describe our
experience with the histopathology of paragangliomas of the urinary
bladder with emphasis on the histologic features that have led to
their being misdiagnosed as conventional urothelial cancer and, most
importantly, those that will help pathologists recognize this rare
tumor of the bladder. Fifteen cases of paraganglioma of the urinary
bladder were studied, 11 of them consult cases. They affected
patients (8 male, 7 female) with a mean age of 49.5 years; only two
had symptoms suggestive of the diagnosis, including hypertension
during cystoscopy and episodic headache. Three consult cases were
submitted with a diagnosis of "transitional cell carcinoma" and 4
with a diagnosis only of "bladder tumor." Histologically, "zellballen"
and diffuse patterns were present in 12 (80%) and 3 (20%) of the
cases. A delicate fibrovascular stroma was obvious in 14 (93%)
cases. Other patterns included irregular nests and pseudorosette
formation. Tumor necrosis, significant cautery artifact, and
muscularis propria invasion were present in 1 (7%), 3 (20%) cases,
and 10 (67%) cases, respectively. All 15 tumors were composed of
large polygonal cells with abundant granular cytoplasm. Focal clear
cells were present in 3 (20%). The nuclei were mostly uniform,
although occasional pleomorphic nuclei were seen in 6 (40%) cases,
and 2 (13%) had frequent pleomorphic nuclei. Mitoses were rare
overall, and no abnormal mitotic figures were found. The major
histologic features that led to misdiagnosis included a diffuse
growth pattern, focal clear cells, necrosis, and muscularis propria
invasion, with significant cautery artifact compounding the
diagnostic problems. Immunohistochemically, 2 of 2 tumors were
positive for neuron-specific enolase, 9 of 10 tumors for
chromogranin, and 2 of 3 tumors for synaptophysin; 3 of 3 tumors
were negative for cytokeratin and 1 of 1 tumor negative for HMB-45.
Paraganglioma of the urinary bladder may be misdiagnosed as
urothelial cancer, but a careful search for the characteristic
histologic features and, if necessary, supportive
immunohistochemical studies, should lead to a correct diagnosis.
Extra-adrenal pheochromocytoma (paraganglioma) of the urinary
bladder: a case report.
Hinyokika Kiyo.
2004 Nov;50(11):787-90.
The case of a
49-year-old male patient with paraganglioma of the urinary bladder
is presented here. The patient's only complaint was of gross
hematuria: sustained hypertension and post-micturitional
hypertension were not presented. Transurethral resection was
performed to diagnose the bladder tumor. Pathological examination
resulted in the diagnosis that the resected tissue was a
paraganglioma. Computed tomography, magnetic resonance imaging and
iodine-131-labeled metaiodbenzylguanidine scintigraphy revealed that
the tumor was a primary paraganglioma in the urinary bladder. Plasma
concentrations of the catecholamines were virtually within the
normal limits. Hypertensive crisis was not revealed during the
transurethral resection. The tumor was non-functional. Partial
cystectomy was performed. The patient has remained disease-free for
five months after surgery.
Paraganglioma of the urinary bladder: can biologic potential be
predicted?
Cancer.
2000 Feb 15;88(4):844-52
BACKGROUND:
Paraganglioma of the urinary bladder is rarely encountered and its
biologic behavior is uncertain. The authors sought to determine the
prognostic factors that would predict patient outcome. METHODS: The
Mayo Clinic experience over 53 years with paraganglioma of the
bladder was reviewed. All histologic slides from 16 patients were
reviewed by the authors. Eight cases were examined
immunohistochemically with cytokeratin (AE1/3, cytokeratin 7, and
cytokeratin 20), vimentin, S-100 protein, neuroendocrine markers (chromogranin,
synaptophysin, and neuron specific enolase), p53 protein, and MIB-1.
DNA ploidy was determined by digital image analysis in formalin
fixed, paraffin embedded tissue. The mean follow-up was 6.3 years
(range, 0.4-16.4 years). RESULTS: Paraganglioma usually occurred in
young adult women (mean age, 45 years; range, 16-74 years). The
male-to-female ratio was 1 to 3. The common symptoms and signs were
hypertension and hematuria. The tumors were usually located
intramurally in the lateral and posterior wall of the bladder and
were multifocal in 3 cases (18%). Seven patients were treated by
transurethral resection, eight by partial cystectomy, and one by
radical cystectomy. T classification was T1 (1 patient), T2 (9
patients), T3 (2 patients), and T4b (4 patients). At the time of
diagnosis, one patient had distant metastasis and one had regional
lymph node metastasis. One patient developed metastasis 1 year after
diagnosis and died of the disease 1.5 years later. None of the
patients with T1 or T2 tumors had recurrence or tumor progression.
All tumors were aneuploid. The mean MIB-1 labeling index was 1.5%
(range, 0.03-7.0%). The tumor cells displayed immunoreactivity for
S-100 protein and neuroendocrine markers and were negative for p53
(except 1 case) and cytokeratin. CONCLUSIONS: Paraganglioma of the
urinary bladder occurs mostly in young adult women. Patients with
tumor of advanced classification (>/=T3) are at risk of recurrence,
metastasis, and dying of the disease, whereas patients in this study
with T1 or T2 disease had favorable outcomes after complete tumor
resection.
Paraganglioma
of the urinary bladder: an immunohistochemical study and report of
an unusual association with intestinal carcinoid.
Aust
N Z J Surg. 1993 Sep;63(9):740-5.
Two cases of
paraganglioma of the urinary bladder are reported. Their
immunohistochemical profiles and the clinical features are compared
with other cases in the literature. The three pan-endocrine markers
(neuron-specific enolase, synaptophysin and chromogrannin) were
positive in both cases. Positivity to other neuropeptides (including
the present two cases and those in literature) includes
adrenocorticotropic hormone (three out of five cases), calcitonin
(two out of nine cases), gastrin (two out of six cases), glial
fibrillary acidic protein (one out of five cases), glucagon (two out
of six cases), serotonin (five out of nine cases), and somatostatin
(four out of eight cases). A previously unmentioned association
between paraganglioma of the urinary bladder and carcinoid in the
gastrointestinal tract is noted in one of the present cases. This
peculiar association highlights the importance of multiplicity of
tumours of the neuroendocrine system other than the classical
multiple endocrine neoplasia syndromes.
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