(juvenile) pilocytic astrocytoma - surgically confirmed

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Clinical Presentation: 7 y.o. boy

History

Patient is a 7 year-old male who presented with intractable vomiting of 24 hours duration. According to his mother, he was in his normal state of health until one day prior to coming to the hospital. On that day, he awoke in the morning and began to vomit. Mom kept him home from school and he continued to vomit throughout the day. He was seen in the ED at NNMC and bolused with 1L NS and started on maintenance IVF. Patient was also given 4mg Zofran, but was still unable to tolerate even small sips of clear fluids.

The patient denies sore throat, abdominal pain, diarrhea, dysuria, rash, visual changes or neck pain. He has been afebrile. Several of his friends at school have experienced similar symptoms. Baseline labs were drawn and the patient admitted with a presumed diagnosis of viral gastroenteritis.

On HD#1, patient began to complain of diffuse headaches and he continued to have emesis/dry heaves throughout the day. He was still unable to tolerate po liquids or Tylenol, and he was given a Tylenol suppository that evening for his headache with minimal relief. Patient remained afebrile.

Emesis and unchanged headaches continued on HD#2. An acute abdominal series was WNL, but showed large amounts of stool. Because of this constellation of symptoms, a head CT and MRI were also obtained at this time.

Physical Examination and Laboratory

No abnormal finding on physical exam.
Labs: CBC 7.4>15.3/44.3<420
Chem 7: 142/4.3 104/26 10/0.4 96

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CT Scan

MRI

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Image Findings

CT:
1) Large cystic and solid enhancing mass in the right cerebellum with associated hydrocephalus.
2) Linear enhancement pattern over the anterior surface of the pons in a pattern most likely representing venous engorgement rather than CSF spread of tumor. No evidence of drop mets in the spine.
MRI:
1) Large cystic and solid enhancing mass in the right cerebellum. There is associated obstructive hydrocephalus.

2) Linear enhancement pattern over the anterior surface of the pons in a pattern most likely representing venous engorgement rather than CSF spread of tumor. No evidence of drop mets in the spine.

Differential Diagnosis

1) Juvenile pilocytic astrocytoma (JPA)
2) Medulloblastoma
3) Ependymoma

Diagnosis

(juvenile) pilocytic astrocytoma - surgically confirmed :: Diagnosis Topic Read Topic

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Treatment and Followup

Tx and Followup:
This patient underwent surgical excision of the tumor plus adjuvant radiotherapy. Corticosteroids can be given to reduce tumor edema.

Topic Discussion

Discussion Author(s): ENS Lisa Inverso

The most common cerebellar tumors in children are JPA (juvenile pilocytic astrocytoma) and medulloblastoma. Patients most often present with headache due to increased ICP and/or ataxia. Nausea, vomiting, cranial nerve palsies and hydrocephalus are also common findings. Cerebellar astrocytomas usually occur between the ages of 2 and 20 years. They are most often histologically benign and cystic in appearance; cystic tumors have a more favorable outcome. Treatment usually consists of surgical excision and radiation therapy. Five-year survival is close to 90% if complete surgical excision is possible.

Medulloblastomas, in contrast to astrocytomas, tend to be highly malignant. They can spread to the subarachnoid space and the ventricle and may metastasize outside the nervous system. Without treatment, medulloblastoma can cause death in just a few months after presentation. Treatment includes surgical excision plus adjuvant radiotherapy and chemotherapy. Chemotherapy may delay the need for radiotherapy, which reduces the incidence of treatment-related neurotoxicity; chemotherapeutic drugs include cyclophosphamide, vincristine, cisplatin and etoposide. Five-year survival is less than that for JPA, and ranges from 44 to 55 percent with treatment. However, recent advances in combined Chemo and Rt can improve five year survival to over 80% in selected series.

CT and MRI scans are the procedures of choice for diagnosing and localizing tumors and intracranial masses and are truly revolutionizing the field of neurodiagnosis. MRI has been especially useful in diagnosing posterior fossa and spinal cord tumors. CT can be less effective because the surrounding bone may limit resolution.

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Contributor Credits

Topic Author(s): ENS Lisa Inverso
Submitted by: MS-4 USU Teaching File -
Case/Image Editor: James G. Smirniotopoulos, M.D. -

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-2 :: Case - 5934 :: - (juvenile) pilocytic astrocytoma - surgically confirmedMedical Image Database, Atlas, and Teaching File All Cases are Peer Reviewed by an Editoral Panel

Clinical Presentation :: History and Physical Exam - Close/Open :: | Print -

Clinical Presentation: 7 y.o. boy

- First Image :: Image Inventory - (May Show Answer ...)

Continuing Education: CME and CNE ::

-2 :: Case - 5934 :: - (juvenile) pilocytic astrocytoma - surgically confirmed
Medical Image Database, Atlas, and Teaching File
All Cases are Peer Reviewed by an Editoral Panel