Desmoplastic Infantile Ganglioglioma, Childhood Tumor

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#PrayersForEllie administrator 1 Views • 2 years ago

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On December 21st, 2012 we welcomed our second beautiful daughter into this world. For the first three months, everything seemed fine, and our biggest issue was deciding what brand of diapers to buy. At four months old, we started to notice that her soft spot looked different, and was actually bulging from her head.

After talking to two different doctors, both who said they thought this was normal, we finally found a doctor who thought she deserved a second look. May 7th, 2013 was the day our life changed, after taking her to the family physician, he insisted that we take her to the emergency room for a CT scan. The hospital thought that she just had fluid on the brain, also known as, hydrocephalus. After a ultrasound, cat scan, and MRI; Ellie was diagnosed with a brain tumor that was 1/3rd the size of her brain. I can't describe the feelings you feel when you hear the words "your child has a tumor" its a rollercoaster of emotions, and all I remember is crying, and asking god "why?"

We were seated in a tiny room, as the neurosurgeon told us that our four month old daughter would need brain surgery right away, and that her chances of survival were slim. We were asked if we wanted to sign a DNR, and then we were told that if she did make it she would possibly have brain damage, and wouldn't be the "normal" little girl we knew. May 8th, 2013 Ellie underwent her first brain surgery to remove the tumor, this surgery took nine hours; I have never thrown up so much in my life.

On May 9th, 2013 our daughter was alive! May 12th, 2013 we found out that her ventricles weren't working, and that is when she was diagnosed with Hydrocephalus. She underwent her second brain surgery that day, to place a VP shunt. Later that week, we got her tumor biopsy back which told us her tumor was Desmoplastic infantile ganglioglioma/astrocytoma; there have only ever been 60 known cases, and very little information on it, and because of that our doctors weren't sure how to treat it.

We waited three months, and scanned her again; those scans showed tumor growth large enough to need another brain surgery and at eight months old our little girl unelliederwent her third 7 hour brain surgery. By now we knew what to expect and we knew that she would be just fine, especially since we decided to take her to a new hospital, one that had more experience in childhood tumors.

By now Ellie has surpassed every statistic, she has rolled over and started crawling; proving every doctor wrong! She is a miracle, by the grace of god, she is a walking and talking miracle. Three weeks after Ellie's third brain surgery scans again showed tumor growth, this is when we decided to start our little eight month old baby, on chemotherapy.

By now we have already been through hell and back, but I would walk through hell a million times for my girls. Chemotherapy has been a rough road, Ellie has developed neuropathy as a side effect of one of her chemo drugs, and she is in constant pain.

Childhood cancer treatment in Bangalore visit  https://medihealthcare.in/ for more details
Childhood cancer treatment in Bangalore visit https://medihealthcare.in/ for more details administrator 3 Views • 2 years ago

Childhood cancer
Cancer in children can occur anywhere in the body, including the blood and lymph node systems, brain and spinal cord (central nervous system, or CNS), kidneys, and other organs and tissues.
Most of the time, there is no known cause for childhood cancers. Childhood cancers may behave very differently from adult cancers, even when they start in the same part of the body.
Types of childhood cancer
"Childhood cancer," also called pediatric cancer, is a general term used to describe a range of cancer types found in children. Below are the most common types of cancer diagnosed in children under age 15:
• Leukemia (accounts for about 29% of childhood cancer cases), Acute lymphoblastic leukemia (ALL), Acute myeloid leukemia (AML), Brain and spinal cord tumors (26%), also called central nervous system (CNS) tumors, Glial tumors, Astrocytoma, Oligodendroglia, Ependymoma, Choroid plexus carcinoma, Oligoastrocytoma, Glioblastoma, Mixed glial neuronal tumors, Ganglioglioma, Desmoplastic infantile ganglioglioma, Pleomorphioc xanthoastrocytoma, Anaplastic ganglioglioma, Neural tumors, Gangliocytoma, Neurocytoma, Embryonal tumors, Medulloblastoma, Medulloepithelioma, Ependymoblastoma, Atypical Teratoid/Rhabdoid tumor, Pineal tumors, Pineocytoma, Neuroblastoma (6%), a tumor of immature nerve cells. The tumor often starts in the adrenal glands, which are located on top of the kidneys and are part of the body’s endocrine (hormonal) system, Wilms tumor (5%), a type of kidney tumor, Non-Hodgkin lymphoma (5%) and Hodgkin lymphoma (3%), cancers that begin in the lymph system, Rhabdomyosarcoma (3%), a type of tumor that most commonly begins in the striated skeletal muscles. Non-rhabdomyosarcoma soft tissue sarcomas can also occur in other parts of the body, Retinoblastoma (2%), an eye tumor, Osteosarcoma (2%) and Ewing sarcoma (1%), tumors that usually begin in or near the bone, Germ cell tumors, rare tumors that begin in the testicles of boys or ovaries of girls. Rarely, these tumors can begin in other places in the body, including the brain, Pleuropulmonary blastoma, a rare kind of lung cancer, Hepatoblastoma and hepatocellular carcinoma, types of liver tumors

Hodgkin lymphoma (15%), Thyroid cancer (11%), Central nervous system (CNS) tumors (10%), Acute lymphoblastic leukemia (ALL) (8%), Non-Hodgkin lymphoma (8%), Germ cell tumors, including testicular cancer (8%) and ovarian cancer (2%), Soft tissue sarcoma (7%), Bone tumors (7%) including osteosarcoma and Ewing sarcoma, Melanoma (6%), acute myeloid leukemia (AML) (4%)

Symptoms and Signs


Continued, unexplained weight loss, Headaches, often with early morning vomiting, Increased swelling or persistent pain in the bones, joints, back, or legs, Lump or mass, especially in the abdomen, neck, chest, pelvis, or armpits,Development of excessive bruising, bleeding, or rash, Constant, frequent, or persistent infections, A whitish color behind the pupil, Nausea that persists or vomiting without nausea, Constant tiredness or noticeable paleness, Eye or vision changes that occur suddenly and persist, Recurring or persistent fevers of unknown origin.

Make an appointment with your doctor if you have any persistent signs or symptoms related to childhood cancer treatment that worry you

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Gangliogliomas
Gangliogliomas administrator 2 Views • 2 years ago

Gangliogliomas are a group of slowly growing neoplasms composed of both neural and glial elements. Most gangliogliomas are supratentorial in location, and the temporal lobes are the predominant site. However, they can also arise in the cerebellum, brainstem, suprasellar region, and spinal cord.

Desmoplastic infantile ganglioglioma, also known as a DIG tumor, is a form of ganglioglioma occurring in young patients, typically less than 2 years of age. The majority of patients with this tumor present around 5-6 months of age. There is no gender predilection. DIG tumors are typically large, supratentorial, heterogeneously-appearing mass lesions with both solid and cystic components, located predominantly in the temporal lobes.

The predominant histopathologic feature is striking desmoplasia, characterized by a dense stroma incorporating glial, neuronal, and fibroblastic elements. This tumor may fill in the subarachnoid space and can extend along perivascular spaces. The DIG tumor is identified with intensely positive GFAP immunohistochemistry as well as positive trichrome stain due to the abundant collagen. Although the bulk of the DIG is superficial and seemingly well demarcated, some lesions include densely cellular focally infiltrative regions resembling a primitive neuroectodermal tumor. Nevertheless, the overall prognosis is favorable, and most patients have prolonged survivals after surgical excision of the DIG tumor.

Radiologic Overview:

The characteristic lesion of desmoplastic infantile ganglioglioma is a solid or partially cystic supratentorial, heterogeneous enhancing mass lesion, usually located in the temporal lobe of a young child or early adolescent.

On CT examination, this lesion may appear hypodense with both cortical and white matter involvement. There may be sulcal effacement and midline shift, depending on the size of the lesion. There usually is heterogeneous contrast enhancement of this lesion.

On MRI, the cystic component of the lesion will be dark on T1, bright on T2, and low signal on FLAIR images. There usually is adjacent vasogenic edema involving the cortical gray and white matter, which is bright signal on the FLAIR sequence. Again, the DIG tumor is characteristically located in the frontotemporal lobes.

Key points:

Rare pediatric brain tumor accounting for less than 1% of all cerebral neoplasms.
Massive, partially cystic tumor occurring predominantly in the frontotemporal lobes in a child less than 1-2 years of age.
Favorable prognosis after surgical resection

References:

Atlas, Scott W., ed. Magnetic Resonance Imaging of the Brain and Spine. 2nd Edition: New York, NY: Lippincott-Raven Publishers. 1996.
Barkovich, A. James. Pediatric Neuroimaging. 3rd Edition: Philadelphia, PA: Lippincott Williams & Wilkins. 2000.
Vandenberg, SR. "Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy." Brain Pathology. 1993;3:275-281.

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