ganglioneuroma ( gn ) is a rare ( 0% to 6% of incidentalomas ) , differentiated , benign , slow - growing tumor that commonly arises from sympathetic ganglion cells and is composed of mature schwann cells , ganglion cells , and nerve fibres .
the family of tumors originating from ganglion cells includes gn which is a benign lesion , ganglioneuroblastoma which is of intermediate differentiation , and neuroblastoma which is a highly malignant lesion .
neuroblastoma and ganglioneuroblastoma most often occur in infants and children , whereas gn tends to occur in adolescents and young adults .
gns may arise anywhere along the paravertebral sympathetic plexus and occasionally from the adrenal medulla .
their localization is the retroperitoneal ( 32% to 52% ) , mediastinal ( 39% to 43% ) , or cervical region ( 8% to 9% ) .
when such tumors arise from the adrenal medulla , their assessment and management are similar to that of other adrenal tumors .
abdominal pain or the palpation of an abdominal mass , or both of these , are among the most common presenting features .
occasionally , symptoms like hypertension , diarrhea , and virilization may develop as a result of mixed hormonally active tumors secreting catecholamines , vasointestinal peptide ( vip ) , androgens , or all of these .
the standard treatment suggested for benign adrenal tumors is laparoscopic surgery . at present , relative contraindications to laparoscopic adrenalectomy are a definitive or presumed diagnosis of invasive adrenal cortical carcinoma or circumstances that make a minimally invasive approach technically difficult , such as large tumors .
we present herein a case of a large , incidentally diagnosed adrenal ganglioneuroma that was removed completely by the laparoscopic approach .
a 23-year - old woman was admitted to the endocrine ward of our hospital , for investigation and management of a 13-cm mass arising from the right adrenal .
this mass was found incidentally following ultrasonography ( us ) of the abdomen while the patient was being investigated for oligomenorrhea . on admission , she was in good health and asymptomatic .
blood pressure was within the normal range . on physical examination , a mild discomfort at deep palpation of the right quadrant
general endocrinology data synacthen test ( august 12 , 2004 ) overnight 1 mg dexamethasone ( dex ) test ( august 14 , 2004 ) computed tomography ( ct ) of the abdomen showed a 1278-cm homogenous mass of the right adrenal ( figure 1 ) .
magnetic resonance imaging ( mri ) showed a solid mass measuring 1377.5 cm arising from the right adrenal . on t2-weighted mr images ,
after intravenous injection of gadolinium , the mass showed a progressive , heterogeneous , and delayed enhancement ( figure 2 ) .
abdominal computed tomographic scan indicating the large benign tumor . magnetic resonance imaging . after intravenous injection of gadolinium
a laparoscopic approach was decided on , despite the large size of the tumor , because of the low risk of malignancy based on radiology findings .
we prefer the transperitoneal lateral decubitus approach , as the best for maximal exposure of the gland and major vessels . using the hasson technique ,
laparoscopic exploration of the abdominal cavity was normal , and the tumor was easily identified .
the right triangular ligament and the retroperitoneal liver attachments were cauterized and divided to allow liver retraction . after dividing the retroperitoneum ,
the inferior periadrenal fat was carefully dissected from the upper pole of the right kidney and the renal vein identified .
the adrenal vein was subsequently identified , dissected , double - clipped , and divided .
the specimen was extracted through an extension of the incision done for the hasson technique .
the surgical specimen was oval and elastic in consistency , measuring 139.56 cm and weighing 390 g. the patient was discharged on the second postoperative day with bowel function returned to normal and able to resume normal physical activity . by gross inspection ,
a large encapsulated tumor mass of 139.56 cm , weighed 390 g. on the surface of the tumor , small foci of flattened adrenal tissue were recognized . by sectioning ,
the tumor had a solid homogenous appearance and was grayish - white to yellowish on the cut surface .
mature ganglion cells scattered or arranged in small clusters were also noted ( figure 3 ) .
two years after the procedure , there is no evidence of recurrence on abdominal ct scan , and the cosmetic result is satisfactory .
top : low magnification shows ganglioneuroma ( right ) with adjacent normal adrenal cortex ( left ) ( h&e 10 ) .
we prefer the transperitoneal lateral decubitus approach , as the best for maximal exposure of the gland and major vessels . using the hasson technique ,
laparoscopic exploration of the abdominal cavity was normal , and the tumor was easily identified .
the right triangular ligament and the retroperitoneal liver attachments were cauterized and divided to allow liver retraction . after dividing the retroperitoneum ,
the inferior periadrenal fat was carefully dissected from the upper pole of the right kidney and the renal vein identified .
the adrenal vein was subsequently identified , dissected , double - clipped , and divided .
the specimen was extracted through an extension of the incision done for the hasson technique .
the surgical specimen was oval and elastic in consistency , measuring 139.56 cm and weighing 390 g. the patient was discharged on the second postoperative day with bowel function returned to normal and able to resume normal physical activity .
by gross inspection , a large encapsulated tumor mass of 139.56 cm , weighed 390 g. on the surface of the tumor , small foci of flattened adrenal tissue were recognized . by sectioning ,
the tumor had a solid homogenous appearance and was grayish - white to yellowish on the cut surface .
mature ganglion cells scattered or arranged in small clusters were also noted ( figure 3 ) .
two years after the procedure , there is no evidence of recurrence on abdominal ct scan , and the cosmetic result is satisfactory .
top : low magnification shows ganglioneuroma ( right ) with adjacent normal adrenal cortex ( left ) ( h&e 10 ) .
ganglioneuromas are rare tumors , and there is a relative paucity of information in the literature concerning their exact incidence .
this is clinically relevant as the incidence of all adrenal tumors is increasing due to the availability of sophisticated imaging techniques and expertise ; approximately , 1% to 10% of abdominal ct scans report incidentally an adrenal tumor .
it is encountered rather more frequently in the literature when comparing its incidence among other large tumors of greater than 6 cm in maximum diameter .
approximately 100 cases of adrenal gn and 112 cases of extra adrenal gn have been reported in the japanese literature .
the imaging based differential diagnosis of an asymptomatic , nonfunctioning giant adrenal mass without evidence of systematic disease is broad and includes besides gn , neuroblastoma , ganglioneuroblastoma , myelolipoma and angiomyolipoma . in the present case , the differential diagnoses suggested on mri findings were benign tumor of the cns ( gn ) and myelolipoma .
both ct scan and mri are superior to us in detecting and characterizing an adrenal mass . following the availability of studies comparing radiologically found and histologically proven dimensions of adrenal lesions
, it was shown that ct scanning might underestimate the tumor size by 16% to 47% , whereas mri does so by 20% .
retroperitoneal gns appear as well - defined masses that are oval , crescentic , or lobulated .
they tend to surround major blood vessels ; the result is absent or mild compromise of the lumen .
intratumoral calcifications are present in 2.4% to 40% of cases ; however , no calcifications were observed in our case .
areas of low attenuation that do not enhance are common , although such an enhancement is usually of low intensity .
an interesting pattern consists of delayed heterogeneous uptake of contrast media , which was observed on the mri of the present case .
adrenal myelolipoma is a rare benign tumor composed of mature fat tissue associated with proliferating hematopoietic cells .
ct features are characteristic if lipid content is demonstrated , which was not observed in our case .
hemorrhagic adrenal adenoma , adrenocortical carcinoma , metastatic tumors to the adrenal gland , and angiomyolipoma of the upper renal pole may also simulate gn or myelolipoma .
the other tumors of the cns ( neuroblastoma and ganglioneuroblastoma ) develop at the same sites as the gn , but they occur in much younger patients and particularly in children . such tumors are usually quite aggressive , having developed metastases in bone , liver , and lung in approximately 50% of cases .
it has recently been demonstrated that scintigraphy and positron emission tomography ( pet ) can complement ct and mri in diagnosing gns .
laparoscopic procedures are associated with less postoperative discomfort , shorter hospital stay , less postoperative disability , and a lower rate of complications .
there are few absolute contraindications for laparoscopic adrenalectomy , and most of them are not specific to adrenal surgery .
large but well encapsulated adrenal masses without evidence of local invasion can be removed laparoscopically .
the lateral transabdominal approach offers the best visualization of major vessels adjacent to the adrenals .
the possibility of malignancy should be appreciated preoperatively ( using ct , mri , or both ) as well as intraoperatively .
invasion of periadrenal tissues or organs is not well suited to laparoscopic techniques and needs conversion to an open procedure .
several authors limit the laparoscopic adrenalectomy to lesions less than 6 cm in size , whereas others have performed laparoscopic adrenalectomy on tumors up to 13 cm in diameter without any significant morbidity .
extensive experience in advanced laparoscopic techniques and open adrenal surgery are mandatory to manipulate and laparoscopically excise large tumors .
in summary , adrenal ganglioneuroma is a rare benign adrenal tumor that needs careful evaluation and surgical treatment .
 we report on a 23-year - old woman with a right adrenal tumor 13 cm in diameter who was treated by laparoscopy . 
 the patient was asymptomatic , and the tumor was incidentally diagnosed on abdominal ultrasonography . a subsequent computed tomography ( ct ) of the abdomen confirmed a 1278-cm homogenous mass of the right adrenal . 
 magnetic resonance imaging ( mri ) showed a solid mass measuring 1377.5 cm arising from the right adrenal . 
 laparoscopic complete excision of the mass was accomplished through a transabdominal lateral approach . 
 the postoperative period was uneventful , and the patient was discharged on the second postoperative day . 
 histology was consistent with an adrenal ganglioneuroma . 
 two years later , there is no evidence of recurrence on abdominal ct scan . 
INTRODUCTION
CASE REPORT
Technical Aspects of the Procedure
Histology Report
DISCUSSION
CONCLUSION