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management of pinealoblastom
by Singla, Jun 14, 2008 11:44AM
My mother is USHA SINGLA,48 Yrs, R/O Delhi,
India.
She suffers from pineal region tumour. Her
history is as follows:
PRESENT COMPLAINTS:
Diplopia on levoelevation and
dextroelevation for 17 yrs.
Headache for last 1 month.
Detailed history:
Pt. was apparently well 17 yrs back (1991),
when she developed headache and diplopia.
On investigations, i.e. brain imaging in the
form of MRI and CT- scans with contrast enhancement, a growth was reported
in the region of Pineal gland invading into the third ventricle and
hydrocephalus. Further scanning did not reveal any metastasis of the
tumourous growth. At that time tumor markers for germinoma in CSF were
negative. The patient was operated for the raised ICT, and
Ventriculo-peritoneal shunt was placed. But following the surgery, she
developed severe life-threatening infection for which she received
antibiotics and the shunt was removed. Surgeons took decision to give a
trial of therapeutic Radiotherapy, for tumor, without any histopathological
diagnosis. Tumor responded to radiotherapy dramatically and follow-up MRI
showed no anatomical trace of tumor, though hydrocephalus persisted.
Symptoms of raised ICT decreased after radiotherapy.
Patient stayed well for 7 years when she
developed episodic sharp shooting type of pain in upper part of right side
of her face (in the distribution of first and second division of trigeminal
nerve). She was then diagnosed as a case of Trigeminal Neuralgia and was
started on carbamazepine. MRI head revealed no abnormal vessel compressing
upon trigeminal root.
She was symptom free till 2004(MRI scan in
year 2000,2002 revealed no tumourous lesion, but residual hydrocephalus
without periventricular ooze was present), when she again developed features
of raised ICT in the form of headache, double vision and urinary
incontinence and dementia. The brain imaging at that revealed massive
ventriculomegaly with periventricular ooze but no growth was present on MRI.
Tumor markers for germinoma were negative in CSF. She was taken to G.B. Pant
Hospital and shown to neurosurgeon. He performed third ventriculostomy and
during the procedure found a fibrotic membrane obstructing the outflow of
third ventricle. He ruptured the membrane and relieved the obstruction. No
growth was found during the ventriculostomy.
She was again symptom free for next two
years. In 2006, her Trigeminal Neuralgia got aggravated and symptoms were
unresponsive to maximal medical management. At that time, to control her
symptoms a trigeminal nerve block was given to her at AIIMS.
She is pain free for approximately one and a
half years.
Presently she is complaining of headache in
right side of her head, persistent and that increases on laughing, neck
movements.
Her latest scans show a massive growth in
the pineal region of size of 3 X 3 cm. MRI spine is normal. Her tumor
markers are again negative (AFP, CEA, betaHCG), done in serum.
she underwent endoscopic biopsy of her tumor.
Histopathological report of same is:
Section shows a cellular tumor comprising of cells displaying round
to oval hyperchromatic nuclei. These cells are arranged in sheets and at
places also display perivascular pseudorosetting. Focal atypical mitosis and
focal areas of necrosis are also seen.
Immunohistochemistry: Strongly reactive for Synaptophysin. Negative
for PLAP, PAS and CD99.
Impression: High Grade tumor possibly 1) Pinealoblastoma 2) Pineal
parenchymal tumor with intermediate differentiation
Please guide me regarding further management of my mother's illness.
India.
She suffers from pineal region tumour. Her
history is as follows:
PRESENT COMPLAINTS:
Diplopia on levoelevation and
dextroelevation for 17 yrs.
Headache for last 1 month.
Detailed history:
Pt. was apparently well 17 yrs back (1991),
when she developed headache and diplopia.
On investigations, i.e. brain imaging in the
form of MRI and CT- scans with contrast enhancement, a growth was reported
in the region of Pineal gland invading into the third ventricle and
hydrocephalus. Further scanning did not reveal any metastasis of the
tumourous growth. At that time tumor markers for germinoma in CSF were
negative. The patient was operated for the raised ICT, and
Ventriculo-peritoneal shunt was placed. But following the surgery, she
developed severe life-threatening infection for which she received
antibiotics and the shunt was removed. Surgeons took decision to give a
trial of therapeutic Radiotherapy, for tumor, without any histopathological
diagnosis. Tumor responded to radiotherapy dramatically and follow-up MRI
showed no anatomical trace of tumor, though hydrocephalus persisted.
Symptoms of raised ICT decreased after radiotherapy.
Patient stayed well for 7 years when she
developed episodic sharp shooting type of pain in upper part of right side
of her face (in the distribution of first and second division of trigeminal
nerve). She was then diagnosed as a case of Trigeminal Neuralgia and was
started on carbamazepine. MRI head revealed no abnormal vessel compressing
upon trigeminal root.
She was symptom free till 2004(MRI scan in
year 2000,2002 revealed no tumourous lesion, but residual hydrocephalus
without periventricular ooze was present), when she again developed features
of raised ICT in the form of headache, double vision and urinary
incontinence and dementia. The brain imaging at that revealed massive
ventriculomegaly with periventricular ooze but no growth was present on MRI.
Tumor markers for germinoma were negative in CSF. She was taken to G.B. Pant
Hospital and shown to neurosurgeon. He performed third ventriculostomy and
during the procedure found a fibrotic membrane obstructing the outflow of
third ventricle. He ruptured the membrane and relieved the obstruction. No
growth was found during the ventriculostomy.
She was again symptom free for next two
years. In 2006, her Trigeminal Neuralgia got aggravated and symptoms were
unresponsive to maximal medical management. At that time, to control her
symptoms a trigeminal nerve block was given to her at AIIMS.
She is pain free for approximately one and a
half years.
Presently she is complaining of headache in
right side of her head, persistent and that increases on laughing, neck
movements.
Her latest scans show a massive growth in
the pineal region of size of 3 X 3 cm. MRI spine is normal. Her tumor
markers are again negative (AFP, CEA, betaHCG), done in serum.
she underwent endoscopic biopsy of her tumor.
Histopathological report of same is:
Section shows a cellular tumor comprising of cells displaying round
to oval hyperchromatic nuclei. These cells are arranged in sheets and at
places also display perivascular pseudorosetting. Focal atypical mitosis and
focal areas of necrosis are also seen.
Immunohistochemistry: Strongly reactive for Synaptophysin. Negative
for PLAP, PAS and CD99.
Impression: High Grade tumor possibly 1) Pinealoblastoma 2) Pineal
parenchymal tumor with intermediate differentiation
Please guide me regarding further management of my mother's illness.
Post Comment
Pineal region tumors are derived from cells located in and around the pineal gland. The principle cell of the pineal gland is the pineal parenchymal cell or pinocyte. This cell is a specialized neuron related to retinal rods and cones.
Most tumors are a result of displaced embryonic tissue, malignant transformation of pineal parenchymal cells, or transformation of surrounding astroglia.
The symptoms of pineal region tumors can be as varied as their diverse histology. Therefore, a rigorous and uniform preoperative workup is a requisite for all patients thought to harbor a pineal region tumor.
Any endocrine abnormalities should be investigated prior to surgery. Patients presenting with signs and symptoms of raised intracranial pressure must receive a head CT scan or an MRI to assess the need for emergent management.
The workup of a patient with a pineal region tumor can be divided into radiologic and laboratory studies.
Initial management of patients with pineal region tumors should be directed at treating hydrocephalus and establishing a diagnosis. Preoperative evaluation should include (1) high-resolution MRI of the head with gadolinium; (2) measurement of serum and CSF markers, if available; (3) cytologic examination of CSF, if available; (4) evaluation of pituitary function if endocrine abnormalities are suspected; and (5) visual field examination if suprasellar extension of the tumor is noted on MRI.
The ultimate management goal should be to refine adjuvant therapy based on tumor pathology.Surgery, Chemotheraoy, Radiotherapy are all options.
You can access further information at the following sitehttp://www.emedicine.com/Med/topic2911.htm
Treatment options are best discussed with the attending doctor.