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OJHAS Vol. 9, Issue 4:
Oct-Dec, 2010) |
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| Ganglioneuroma Always A Histopathological Diagnosis |
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Prasad K Shetty,
Balaiah K,
Gnana Prakash S,
Prasanna K Shetty,
Department of Pathology
and Radiology, Bhagwan Mahaveer Jain Hospital, Bangalore, India
and Department of Pediatrics, Vaatsalya Hospital, Gokul Road,
Hubli, India. |
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Address For Correspondence |
Department
of Pathology,,
Bhagwan
Mahaveer Jain Hospital,
Millers
Road,
Bangalore-560052,
India.
E-mail:
dr.pkshetty@gmail.com |
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Shetty PK, Balaiah K, Gnana Prakash S, Shetty PK. Ganglioneuroma Always A Histopathological Diagnosis. Online J Health Allied Scs.
2010;9(4):19 |
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Submitted: Nov 4,
2010; Accepted: Dec 28, 2010; Published: Jan 20, 2011 |
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| Abstract: |
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Neuroblastoma,
ganglioneuroblastoma and ganglioneuroma arise from sympathetic tissue
in the neck, posterior mediastinum, adrenal gland, retroperitoneum and
pelvis Ganglioneuromas are commonly seen in childhood.
They are highly differentiated benign tumors and are compatible with
long-term disease free survival. Retroperitoneal localization is relatively
frequent for these tumors. Due to its rarity and lack of specific radiological
findings diagnosis is always postoperative. Here, we
present a case of Retroperitoneal ganglioneuroma which was undiagnosed
before surgery.
Key Words: Ganglioneuroma; Retroperitoneum; Computed Tomography
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A
8-year-old boy presented with a 6-month history of pain abdomen and
distension. He had a good appetite and unaltered bowel and bladder habits.
On physical examination his vital signs were normal. On per abdominal
examination, about 8x8 cm non tender, non fluctuating mass was palpated
at lower mid abdomen. No hepatosplenomegaly / Ascitis was noted. Bowel
sounds were existing and normal. Bilateral hernial orifices were normal.
Bilateral testicles were normal in the scrotum. All laboratory studies
were within normal ranges and included Complete Hemogram, Liver Function
Tests and Renal Function Tests. His chest X ray was normal. Contrast
enhancing computed tomography (CT) scan of the abdomen showed 7.7x5.5x4.8cms
mixed attenuation well defined mass with no significant contrast enhancement
in the retroperitoneum pushing the right kidney laterally and inferior
venacava anteriorly.
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Figure
1,2: Contrast enhancing CT scan showing retroperitoneal mass. |
Radiological
features were suggestive of a Neuroblastoma / Retroperitoneal Sacroma.
Based on radiological findings a laprotomy followed by total resection
of the tumor was performed. There was no evidence of Ascites/ Peritoneal
seedling.
Grossly
the tumor was a large encapsulated mass measuring 7x5cms outer surface
was gray white gleasining and cut surface was solid, homogenous, dull
gray white cut surface.
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Figure 3,4: Gross photograph of ganglioneuroma
outer and cut surface. |
Histologically,
tumor was composed of Schwann cells, fibrous tissue and embedded within
were large cells with abundant cytoplasm, large nuclei and prominent
nucleoli (ganglion cells). There was no evidence of atypia / mitosis
/ necrosis.
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Figure
5, 6: Hematoxylin and eosin of 10x and 40x respectively shows fibrocollagenous
tissue and Schwann cells with ganglion cells. |
Based on the histopathological features a diagnosis of retroperitoneal
ganglioneuroma was made. Post- operative period was uneventful, patient
was discharged on 8th postoperative day.
Neuroblastomas,
ganglioneuroblastomas and ganglioneuromas are tumors of the sympathetic
nervous system that arise from the neuroectodermal cells derived from
the neural crest cells. These tumors differ in their degree of cellular
and extracellular maturation; Neuroblastoma tend to be aggressive and
occur in younger patients (Average 2 years), whereas Ganglioneuroma
occur in older children (Average 7 years).1 Ganglioneuromas
may occur spontaneously or during the therapy for neuroblastomas with
either chemotherapy or radiation therapy.2,3 The reported
incidence of ganglioneuroma is one per million population. They are
mostly sporadic but there are a few reports of ganglioneuromas associated
with neurofibromatosis type II and multiple endocrinologic neoplasia
type II.4 Ganglioneuromas can be found in the central
nervous system or peripherally in the sympathetic system. The most common
localization is the posterior mediastinum followed by the retroperitoneal
space.5 Retroperitoneal ganglioneuromas are usually non-functioning
and asymptomatic until they reach large sizes in which case they cause
symptoms due to local expansion and pressure on adjacent structures.
2 Although symptoms of autonomic dysfunctions are usually seen
in patients with hormone secreting ganglioneuromas, such symptoms may
also be seen in patients with paravertebral ganglioneuromas compressing
the autonomic fibers of the lumbosacral plexus.6 Also, there are functional ganglioneuromas that were found
to release peptides such as vasoactive intestinal peptides (VIP), somatostatins
and Neuropeptide Y (NPY) in the literature. These tumors may cause some
symptoms like diarrhea, sweating and hypertension related to those peptides.
Diarrhea in this patient can be caused by this kind of intestinal peptides.
Since ganglioneuromas may release catecholaminergic peptides, surgeons
should be aware of the possibility of hypertensive crisis during the
surgery.7 Radiological examination also has no diagnostic
value in most cases. Because of the rarity of retroperitoneal ganglioneuromas
and absence of any characteristic radiologic features, imaging of these
tumors is not reliable and diagnostic.8 Preoperative diagnosis
of retroperitoneal ganglioneuroma is often difficult and the diagnosis
is usually based on histopathological findings after surgical excision
of the tumor.8Although in some cases aspiration cytology
with fine needle has been reported to be useful in the preoperative
diagnosis of adrenal ganglioneuroma, since the tumoral tissue can contain
fractions of less well differentiated areas, surgical exploration is
required to achieve a definitive diagnosis and risk assessment.9 Grossly, they are large, encapsulated masses of firm consistency
with an homogenous, solid, grayish white cut surface. Areas with different
color or consistency should be sampled for microscopic examination with
the suspicion of less differentiated foci. They can be multiple and
or associated with other independent types of neurogenous neoplasms
such as neuroblastoma and pheocromocytoma.8
Microscopically, it consists of a spindle cell tumor resembling a neurofibroma
but shows numerous ganglion cells. Microscopically ganglioneuromas have
consists of a spindle cell tumor composed of neuritic processes, Schwann
cells and perineural cells and show numerous ganglion cells.8,10
Ganglioneuromas
are typically slowly growing, benign tumors and have a tendency
to remain clinically silent for a considerable time. Most patients have
prolonged survival without any evidence of progression. According to
many authors, surgical excision is sufficient for the treatment.9
Preoperative or postoperative chemotherapy or radiotherapy have no value
in the treatment except it was associated with ganglioneuroblastoma
changes when there might be some role of chemotherapy. As it is a slow
growing tumor, gross total surgical removal with preservation of organ
functions is a feasible surgical option.2,3
In
conclusion the ganglioneuroma arises from sympathetic ganglion which
is a very rare disease and affects children more often than adults.1
It is a benign, slow growing tumor and since it is difficult to distinguish
from other tumors due to lack of image findings diagnosis is always
made histologically.2
- Lonergan GJ, Schwab CM, Suarez ES, Carlson CL. Neuroblastoma, ganglioneuroblastoma,
and ganglioneuroma: radiologic-pathologic correlation. Radiographics
2002;22:911-934.
Zugor V, Amann K, Schrott KM, Schott GE. Retroperitoneal
ganglioneuroma. Aktuelle Urol 2005;36(4):349-352. Moriwaki Y, Miyaka M, Yamamoto T et al. Retroperitoneal ganglioneuroma:
a case report and review of the Japanese literature. Intern Med 1992;31:82-85. Jain M, Shubha BS, Sethi S, Banga V, Bagga D. Retroperitoneal ganglioneuroma:
report of a case diagnosed
by fine-needle aspiration cytology, with review of the literature. Diagn
Cytopathol 1999;21:194-196. Chang CY, Hsieh YL, Hung GY, Pan CC, Hwang B.
Ganglioneuroma presenting as an asymptomatic huge
posterior mediastinal and retroperitoneal tumor.
J Chin Med Assoc 2003;66:370-374. Hayes FA, Green AA, Rao BN. Clinical manifestations of ganglioneuroma.
Cancer 1989;63(6):1211-1214. Gentile S, Rainero I, Luda E, Pinessi L. Autonomic dysfunction associated
with multiple pelvic ganglioneuromas. Acta Neurol Scand 2001;104:54-56.
Bjellerup P, Theodorsson E, Kogner P. Somatostatin and vasoactive intestinal
peptide in neuroblastoma and ganglioneuroma: chromatographic characterization
and release during surgery. Eur J Cancer 1995;31A:481-485. Rosai J. Chap 16: Adrenal gland and other paraganglia. In: Ackermans
surgical pathology, vol 1, 8th edn. Mosby, St. Louis; 1996. pp1015-58.
Radin R, David CL, Goldfarb H, Francis IR. Adrenal and extraadrenal
retroperitoneal ganglioneuroma: imaging findings in 13 adults. Radiology 1997;202:703-707. Kleihues P, Cavenee WK. Classification of tumors, pathology and genetics
of tumors of the nervous system. World Health Organization, Lyon 2000. pp
153-161.
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