is an extremely rare type of benign tumour that
affects neural crest cells. Neural crest
tumours are typically metabolically inactive and
common sites are the retroperitoneum and thoracic
cavities [2,3]. They are composed of sympathetic
ganglion cells, Schwannian stroma, fibrous tissue,
and nerve fibres [4,5]. Reports of ganglioneuromas
developing from the kidney are scant. Here we
report a case of renal ganglioneuroma with nodal
metastasis managed by surgical resection.
A 38 years old
female presented with a history of anorexia and
early satiety. On examination she had a palpable
mass per abdomen in right lower quadrant, firm to
hard and fixed. Margins were not palpable. A
screening ultrasound revealed a retroperitoneal
mass in close association with right kidney. Her
renal function tests were normal. The CECT scan
[Fig 1] revealed a large heterogenous mass,
predominantly cystic, containing hypoenhancing
areas and calcific foci, in retroperitoneum in
right suprarenal region pushing the IVC medially.
Dimensions of the mass were - solid component 7.8
x 2.8 x 8.4 cm and cystic component 17.8 x 12.1 x
18.6cm. Enlarged retrocrural lymph nodes 13 x 20mm
were seen. The left kidney and adrenal gland were
normal along with the liver, spleen, gallbladder,
pancreas, bowel and bones. The differential
diagnosis included araganglioma, malignant non
functioning pheochromocytoma, adrenal carcinoma
and retroperitoneal sarcoma. Functional evaluation
of urine metanephrines, cortisol and DHEA were
1: CT images showing a large right
explorative laparotomy through a Makuchi incision.
There was no evidence of peritoneal or liver
metastases. A large retroperitoneal cystic mass
was palpable. Retroperitoneum was entered by
reflecting the caecum and ascending colon along
the white line of Toldt. Superior and lateral
margin planes were maintained and dissected out.
Medially it was seen having a solid component
having retrocaval extension. Inferormedially it
was seen adherent to the renal capsule. Meticulous
dissection was done to dissect out the retrocaval
extension of the mass. A right nephrectomy was
done as the mass was not separable from the right
kidney. A few enlarged lymph nodes were present
densely adherent around the IVC. They were
meticulously dissected. RP mass and nodal specimen
were sent for histopathology separately [Fig 2].
Post operative period was uneventful. Abdominal
drain was removed on the third post operative day.
Patient was discharged on fifth post operative
2: A) Right kidney with ganglioneuroma
mass B) Retrocrural lymph nodes
confirmed the tumor to be a ganglioneuroma.
Grossly, the mass was seen arising from the upper
pole of the kidney with the renal capsule
contiguous with the pseudocapsule of the tumor. It
had myxoid areas with haemorrhage and multiple
cystic areas. Both the nodal masses also had
myxoid areas. Histological examination showed
ganglioneuroma tissue, including mature ganglion
cells, Schwann cells and fibrous tissue. Myxoid
change noted within the tumor cells as well.
Immunohistochemistry was done and it confirmed the
diagnosis of ganglioneuroma and ruled out
Given the benign nature of the tumor, no further
adjuvant treatment was required. The patient has
now been followed closely in our outpatient clinic
and is doing well at 6 months post operative.
masses often present late or with vague symptoms
regardless of their histology. Primary tumors of
the retroperitoneal space are rare. They are
malignant in the majority of cases – about 80%
. Sarcomas constitute the bulk of malignant
retroperitoneal tumors. The most common include
liposarcomas, malignant fibrous histiocytoma, and
leiomyosarcoma. Most common benign tumors are
Neural crest tumors (schwannoma, neurofibroma),
paragangliomas, fibromas, angiomyolipomas of renal
origin, and lipomas.
neuroblastomas and ganglioneuroblastomas are
referred collectively as neuroblastic tumors
originating from the sympathetic nervous system
and neural crest cells [4,5]. Ganglioneuromas are
the most differentiated, least severe tumors and
often occur in older patients with evidence
showing more occurrences in females over men. This
kind of tumor was first described by Loretz in
neuroblastomas and ganglioneuroblastomas,
ganglioneuromas usually show little metabolic
activity, with only 37% of ganglioneuromas causing
increased catecholamine and urine levels (vs.
90-95% of neuroblastomas and
ganglioneuroblastomas). Our patient did not have
increased urine metanephrines. CT and magnetic
resonance imaging are also unable to differentiate
between these tumors; the only method for
diagnosis of ganglioneuroma is through
histopathological analysis. Due to possible
microscopic foci of malignant neuroblastoma cells
within an otherwise benign tumor, complete
surgical excision is usually required .
ganglioneuromas are found arising from
retroperitoneal and posterior mediastinal tissue
(37.5% and 41.5% respectively), but can also occur
in any sympathetic tissue [1-5]. Retroperitoneal
ganglioneuromas are rare and only make up
0.72-1.6% of primary retroperitoneal tumors .
Primary renal ganglioneuromas are even rarer.
Cooper SS et al  reported a case of renal
ganglioneuroma presenting as an apparent renal
artery aneurysm which was managed by
angioembolisation and left hand-assisted
extensions of ganglioneuromas can also occur, but
are rare and it is debated whether they develop
naturally or are previous metastases of
neuroblastomas that spontaneously matured. These
metastases most often occur in lymph nodes
adjacent or in close proximity to the central
ganglioneuroma, but have been reported to extend
into bone [3,7]. Our patient was found to have
diagnosis of retroperitoneal GN is difficult,
which is due to variable histological structure of
the tumor. In the presented case there was a
suprarenal solid cystic mass. According to the
available literature, ganglioneuromas is most
often diagnosed on the basis of histopathological
examination following tumor excision .
Treatment of GN
consists of complete surgical resection of the
tumor, when possible . Preoperative or
postoperative chemotherapy or radiotherapy has no
value, unless the GN was associated with
ganglioneuroblastoma changes. In this case, there
might be some role for chemotherapy . Some
investigators have indicated that conservative
management in cases that are not amenable to
complete resection still results in a good
prognosis [2,10]. However, surgery should be
performed for the following conditions: symptoms
resulting from the tumor, encroachment on
vertebral foramina, marked growth in size, and
increased secretory activity of catecholamine. It
should be kept in mind that GNs have a metastatic
potential. For example, one study reported that
removal of an abdominal GN with the surrounding
lymph nodes revealed another GN in one of the
lymph nodes .
The possibility of
slow progression and late recurrence of GNs has
been reported; therefore, long-term periodic
radiologic surveillance postoperatively is
necessary to assess the malignant potential of
these tumors [9,11]. GNs are compatible with
long-term, disease-free survival even with
unsatisfactory surgical treatment. The prognosis
is good. In the case of GN recurrence, surgery
should be considered .
Ganglioneuroma is a rare, benign retroperitoneal
tumor, which may be difficult to differentiate
from other lesions occurring in this localization.
Few cases have been described in the literature.
There are no diagnostic methods to identify
ganglioneuromas. Surgical excision is the most
effective treatment with few cases of recurrence.
Routine physical and radiological follow-up is
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