Background: 131I Meta‐iodobenzylguanidine (131I MIBG) scintigraphy can detect chromaffin tumours with a high specificity but its sensitivity remains limited. In this study, the influence of clinical ...features and tumour pathology on the results of 131I MIBG of patients with phaeochromocytomas and paragangliomas was examined.
Methods: The records of 104 patients operated on for chromaffin tumours who had pre‐operative 131I MIBG were reviewed. Demographic data, clinical features, biochemical results and pathology of tumours were analysed. The size of the tumour was assessed by the three measured diameters of the specimen and its calculated volume. Univariate relationship between tumours’ characteristics and the results of 131I MIBG were examined.
Results: Out of 119 tumours, 104 (87%) were detected by 131I MIBG. Mean ± SD largest diameter and volume of the tumours were 6.0 ± 2.4 cm (range 0.8–11.5 cm) and 68 ± 74 cm3 (range 0.2–421 cm3). Results of 131I MIBG were significantly correlated with both the largest diameter of the tumour (P < 0.01) and by its volume (P < 0.001). 131I MIBG was negative in 35.5% of tumours < 20 cm3 and in 2.6% of those < 20 cm3. No other criteria were correlated with positive 131I MIBG, including aetiology, pathology or catecholamine secretion pattern.
Conclusions: The result of 131I MIBG scanning in patients with chromaffin tumours is significantly correlated with the size of the tumour.
Background Ectopic hormone-secreting pheochromocytomas
are rare; only case reports exist in the literature. This
condition has been linked with increased malignancy,
familial syndromes, and ACTH ...secretion. We wanted to
test these hypotheses and shed light on the nature of ectopic
hormone-secreting pheochromocytomas.
Methods This is a multicenter (francophone) observational
study. Inclusion was based upon abnormal preoperative
hormone tests in patients with pheochromocytoma
that normalized after removal of the tumor. Where
possible, immunohistochemistry was performed to confirm
that ectopic secretion came from the tumor.
Results Sixteen cases were found: nine female and seven
male patients. Median age was 50.5 (range 31–89) years.
Most presented with hypertension, diabetes, or cushingoid
features. Ten patients had specific symptoms from the
ectopic hormone secretion. Two had a familial syndrome.
Of eight patients with excess cortisol secretion, three died
as a result of the tumor resection: two had pheochromocytomas
15 cm and their associated cortisol hypersecretion
complicated their postoperative course. The other died
from a torn subhepatic vein. The 13 survivors did not
develop any evidence of malignancy during follow-up
(median 50 months). Symptoms from the ectopic secretion
resolved after removal of the tumor. Immunohistochemistry
was performed and was positive in eight tumors: five
ACTH, three calcitonins, and one VIP.
Conclusions Most pheochromocytomas with ectopic
secretion are neither malignant nor familial. Most ectopic
hormone-secreting pheochromocytoma cause hypercortisolemia.
Patients with a pheochromocytoma should be
worked up for ectopic hormones, because removal of the
pheochromocytoma resolves those symptoms. Associated
cortisol secretion needs careful attention.
The classic morphological techniques for the localization of insulinomas and gastrinomas are of limited value. Endoscopic ultrasonography and somatostatin receptor scintigraphy have shown high ...sensitivity for the detection of gastroenteropancreatic endocrine tumors. The aim of the study was to evaluate the sensitivity of endoscopic ultrasonography and that of somatostatin receptor scintigraphy in the localization of insulinomas and gastrinomas.
This retrospective study concerned 54 patients with insulinoma (n=29) or gastrinoma (n=26) operated on between March 1991 and March 2000 and who had at least one among the two tested examinations. Forty-two patients had scintigraphy (17 with insulinoma, 25 with gastrinoma), 47 had endoscopic ultrasonography (28 with insulinoma, 17 with gastrinoma). One of the ten patients with MEN 1 had both tumors. All diagnosis were confirmed by histologic examination.
The sensitivity of scintigraphy for the localization of insulinomas was 47%. There was one false positive. Sensitivity of endoscopic ultrasonography for insulinomas was 85%. The sensitivity of scintigraphy in the detection of gastrinomas was 65% for the tumors in the duodenopancreatic area, 20% for the tumors in the pancreatic tail and 71% for metastasis. The sensitivity of endoscopic ultrasonography was 46% for duodenal tumors, 75% for pancreatic tumors and 57% for lymph node metastasis. The combination of both localization studies increased sensitivity to 94%.
Endoscopic ultrasonography and somatostatin receptor scintigraphy are the gold standard for localization of gastrinomas. Association of both examinations increases the sensitivity. Scintigraphy for the detection of insulinomas should be performed when endoscopic ultrasonography is negative.
Background Ectopic abnormal parathyroid glands are relatively
common in the superior mediastinum but are rarely
situated in the aortopulmonary window (APW). The embryological
origin of these abnormal ...parathyroid glands is controversial.
The purpose of this investigation was to investigate
the embryological origin and the surgical management of
abnormal parathyroid glands situated in the APW.
Methods The databases of patients operated on for primary,
secondary, and tertiary hyperparathyroidism at eight
European medical centers with a special interest in endocrine
surgery were reviewed to identify those with APW adenomas.
Demographic features, localization procedures, and
perioperative and pathology findings were documented. The
embryological origin was determined based on the number
and position of identified parathyroid glands.
Results Nineteen (0.24%) APW parathyroid tumors were
identified in 7,869 patients who underwent an operation for
hyperparathyroidism (HPT) and 181 patients (2.3%) with
mediastinal abnormal parathyroid glands. Ten patients had
primary, eight had secondary, and one had tertiary HPT.
Sixteen patients had undergone previous unsuccessful cervical
exploration. In three patients, an APW adenoma was
suspected by preoperative localization studies and was cured
at the initial operation. Sixteen patients had persistent HPTof
whom 15 were reoperated, resulting in 6 failures. Evaluation
of 17 patients who had bilateral neck exploration allowed us
to determine the most probable origin of the APW parathyroid
tumors: 12 were supernumerary, 4 appeared to originate
from a superior, and 1 from an inferior gland.
Conclusions Abnormal parathyroid glands situated in the
APW are rare and usually identified after an unsuccessful
cervical exploration. Preoperative imaging of the mediastinum
and neck are essential. The origin of these ectopically
situated tumors is probably, as suggested by our data, from a
supernumerary fifth parathyroid gland or from abnormal
migration of a superior parathyroid gland during the
embryologic development.
Background
:
131
I Meta‐iodobenzylguanidine (
131
I MIBG) scintigraphy can detect chromaffin tumours with a high specificity but its sensitivity remains limited. In this study, the influence of ...clinical features and tumour pathology on the results of
131
I MIBG of patients with phaeochromocytomas and paragangliomas was examined.
Methods
: The records of 104 patients operated on for chromaffin tumours who had pre‐operative
131
I MIBG were reviewed. Demographic data, clinical features, biochemical results and pathology of tumours were analysed. The size of the tumour was assessed by the three measured diameters of the specimen and its calculated volume. Univariate relationship between tumours’ characteristics and the results of
131
I MIBG were examined.
Results
: Out of 119 tumours, 104 (87%) were detected by
131
I MIBG. Mean ± SD largest diameter and volume of the tumours were 6.0 ± 2.4 cm (range 0.8–11.5 cm) and 68 ± 74 cm
3
(range 0.2–421 cm
3
). Results of
131
I MIBG were significantly correlated with both the largest diameter of the tumour (
P
< 0.01) and by its volume (
P
< 0.001).
131
I MIBG was negative in 35.5% of tumours < 20 cm
3
and in 2.6% of those < 20 cm
3
. No other criteria were correlated with positive
131
I MIBG, including aetiology, pathology or catecholamine secretion pattern.
Conclusions
: The result of
131
I MIBG scanning in patients with chromaffin tumours is significantly correlated with the size of the tumour.