Discussion
Our case presented and continues to present significant challenges to
the attending physicians.
Firstly, the diagnosis was challenging. Clinical signs and symptoms were
nonspecific and were all associated with local compression, with no
symptoms due to catecholamine excess. Preoperative MRI can be
diagnostic, however is frequently confounded, as it was in our case,
with schwannoma, meningioma or ependymoma. The initial histopathological
diagnosis was also incorrect, having the review of the specimen changed
the initial diagnosis.
Secondly, as is described in the literature[,9, 10,11,12,]., spinal
PG disease recurrence can occur decades after surgical resection, which
highlights the need for long follow up periods with regular
imagiological surveillance.
Thirdly, the optimal therapeutic strategies after primary resection
remain unknow. Adjuvant radiation was performed after the first
resection, however solid evidence in the literature for this approach is
lacking. A second surgery with complete resection was performed after
the first recurrence. There is some evidence of superiority of this
approach versus radiation therapy.
Lastly the very small number of reported cases of PG where
leptomeningeal dissemination has occurred means that treatment must be
determined on an individual basis. Our review of the literature
identified 6 described cases [6,7]. [Taken together these cases
seem to suggest an indolent, insidious nature of the disease, even in
the setting of dissemination.
Therapeutic options are limited as somatostatin analogs do not cross the
blood-brain barrier. Radiotherapy and alkylating agents that penetrate
the CNS can produce disease stability and their role in the management
of leptomingeal dissemination warrants further investigation [5,8,
9,13]
Documentation of rare cases such as this one is important for enhancing
clinical awareness, stimulation of novel research into biomarkers of
malignant potential and development of treatment protocols.