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«National Program of Cancer Registries Education and Training Series How to Collect High Quality Cancer Surveillance Data Case 1: CNS History and ...»

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National Program of Cancer Registries Education and Training Series

How to Collect High Quality Cancer Surveillance Data

Case 1: CNS

History and Physical Examination

See Radiation Oncology Consult

Imaging

9/14 CT of the brain: 3.5 x 1.8 cm enhancing left hemisphere mass with significant surrounding edema.

9/14 CT of the chest, abdomen, and pelvis: no abnormalities detected.

9/14 PET scan of brain: no abnormalities detected.

9/17 MRI, brain and brain stem: There is a posterior left temporal ring-enhancing mass and significant surrounding edema involving much of the left temporal lobe and hemisphere. The size and configuration of the ventricular system is within normal limits. Right hemispheric cortical sulci as well as central subarachnoid cisterns are symmetric and unremarkable. No additional lesions are noted.

Impression: Left posterior temporal lobe lesion. Primary considerations would be for primary or metastatic brain tumor.

Procedures 9/21 Craniotomy with partial resection and Gliadel wafer implantation.

Pathology 9/21 Glioblastoma multiforme, giant cell type.

Radiation Oncology Consult 9/27 History of Present Illness: This pleasant 58-year-old male was in his usual state of good health until recently. He had been experiencing subtle difficulties with word-finding, misspelling words, concentration, and reading over the last month or so. He underwent initial evaluation at another facility.

Work-up included a CT scan of the brain, which showed a left hemisphere mass. He was taken to the operating room on 9/21 and underwent what sounds like a partial resection with Gliadel wafer implantation.

9/27 Physical Exam: This is a pleasant healthy-appearing male resting comfortably in no acute distress.

His head is shaved. He has a bandage in place over the left side of his scalp. His pupils were equal, round and reactive to light. Extraocular movements were intact. There was no scleral icterus. Cranial nerves II–XII were grossly intact bilaterally. Sensation was grossly intact in all four extremities. Negative Romberg. Finger-to-nose testing was normal. Rapid alternating movements were normal. He was able to spell “world” backwards without any difficulty. Short-term memory, however, was 0/3. He answered all questions appropriately and speech was fluent. He was able to give the names of multiple objects without difficulty.

Impression: 58-year-old male with a new diagnosis of glioblastoma multiforme. Karnofsky Performance Score is estimated at 90. He underwent a partial resection with placement of Gliadel wafers. We recommend postoperative external beam radiation therapy for a total of approximately six weeks.

Additionally, the patient has discussed chemotherapy with the medical oncologist and it sounds as if he was planning for concurrent Temozolomide followed by additional adjuvant Temozolomide Central Nervous System (CNS) Exercises

–  –  –

12/8 Overall, the patient tolerated his treatments well. The patient did complain of having some fatigue as well as headaches in the morning. The patient did develop seizures during the course of his treatment, however, his seizure medication was adjusted and he has not had any seizure since.

Date Treatment Started: 10/18 Date Treatment Ended: 12/8

–  –  –

History and Physical Examination 4/29 History of Present Illness: This 62-year-old female has visual problems and had a visual exam done last week. She notes that her vision has deteriorated over the last month and is worse in the right eye. She reports having an occasional headache but these respond to Ibuprofen. Her son has noted some personality changes over the last one to two months. Her husband notes decreased initiative in looking for employment and an increase in fatigue level. She denies any other neurological abnormalities.

4/29 Physical Exam: She is alert, oriented times three and in no acute distress. Her speech is clear. She has a flat effect. Vision in the right eye is 20/200. Vision in the left eye is 20/50. She has a temporal field deficit in the right eye to finger count confrontation. Otherwise cranial nerves II through XII are intact.

Muscle strength is strong. Sensation is intact. Deep tendon reflexes are 1–2 and symmetrical. Her gait is stable. All other systems are normal.

Imaging

4/27 MRI, brain: There is an extra-axial well-defined homogeneously enhancing mass, measuring 7.2 x

7.2 x 5.2 cm, attached to the anterior cranial fossa. The lesion is consistent with olfactory groove meningioma. It compresses the anterior horn of the lateral ventricles bilaterally and stretches the optic chiasm and optic nerves inferiorly. The pituitary stalk and lamina terminalis are also stretched posteriorly.

No hydrocephalus is noted.

Impression: Olfactory groove meningioma.

Procedures 5/20 Bifrontal craniotomy for resection of olfactory groove meningioma. As the frontal lobes were retracted superiorly and laterally, the tumor easily came into view. It was dissected free from the surrounding brain using bilateral electrocautery and patties. After several hours of meticulous dissection of the tumor sparing the capsule and performing several internal debulkings, the tumor was removed in its entirety. There were no areas of significant damage to the surrounding brain. The tumor was the size of a tangerine. To facilitate removal the ultrasonic aspirator had to be used.





Pathology 5/20 Gross: A. Multiple friable tan irregularly shaped soft tissue fragments measuring 1.8 x 1.4 x 0.4 cm in aggregate. B. Multiple irregularly shaped friable soft tan-red and brown tissue fragments measuring 8.5 x 7.8 x 2.5 cm in aggregate.

Microscopic: A, B. The tumor is composed of nests, vague lobules and fascicles of cells with mildly spindled plump nuclei with bland cytologic features. Cell borders are indistinct. The chromatin is finely dispersed. No mitoses are identified. The lesion is cellular, but no necrosis, increased NC ratio, prominent nucleoli, or sheet-like growth patterns are identified. No brain invasion is seen.

Diagnosis: A, B. Brain, bifrontal tumor, excision: meningioma.

–  –  –

History 4/4/XX This 21-year-old female had a midline pineal mixed germ cell tumor excised seven years ago. At that time, embryonal carcinomatous, seminomatous, and teratomatous components were identified. At that time, she also had associated hydrocephalus on CT scan, and a ventriculo-peritoneal shunt was inserted. She now has lesions of the right parieto-parasagittal area and left temporal area and undergoes stereotactic biopsy of the parieto-parasagittal area.

Procedures 4/4/XX Parieto-parasagittal stereotactic biopsy Pathology 4/4/XX Gross: The first specimen is designated “tumor-center” and consists of multiple fragments of brain tissue measuring 1.5 cm in aggregate diameter. They are submitted in toto as A. The second specimen is designated “tumor at edge”. The third specimen is designated as “tumor at edge” with no further distinguishing designations from the previous. The fourth specimen is designated “tumor at edge” with no distinguishing designation from the previous specimens. These three specimens are submitted to the Laboratory for Millipore filtration.

Microscopic: Sections reveal portions of blood and portions of brain parenchyma with increased numbers of glial cells. These astrocytic cells have a protoplasmic appearance in most of the biopsy;

however there are focal fibrillary features. There is no evidence of anaplasia or necrosis.

Immunoperoxidase staining is positive for vimentin, glial fibrillary acidic protein, and S-100 protein, and negative for cytokeratin, alpha fetoprotein, beta human chorionic gonadotropin, and placental alkaline phosphatase.

Final Diagnosis: Brain, right parieto-parasagittal region, stereotactic biopsy; low-grade astrocytoma.

Autopsy Date: 04/17/XX Clinical History The patient was a 21-year-old white female who was admitted on April 14, XX in a coma. This young woman was found to have a mixed germ cell tumor located in the pineal region at age 14. The neoplasm was successfully treated with partial resection, irradiation, and chemotherapy. At age 15 she was found to have right lung metastases which were treated with irradiation, chemotherapy, and resection. She then did until late March XX when she developed aphasia. She returned to the hospital for evaluation and was found to have right parasagittal and a left temporal frontal mass. Stereotactic biopsy was performed on April 4, XX, which was diagnosed as a low-grade astrocytoma. The patient was scheduled for left craniotomy with tumor biopsy, debulking, and partial lobectomy on April 18. However the patient became increasingly more lethargic over the day of admission. When she was found to be unarousable by her parents, she was brought to the hospital where she was admitted directly to the Intensive Care Unit.

Physical examination on admission was remarkable for unresponsiveness except to pain and posturing in response to pain. Vital signs were stable at normal levels. The left pupil was fixed and dilated while the right was less dilated and reactive. There were no purposeful movements or localization.

The clinical impression at the time of admission was that the patient suffered left uncal herniation secondary to the known left temporal frontal mass. She was intubated and treated with hyperventilation, Mannitol, and Decadron. By the following morning the patient had awakened, responded to her family,

–  –  –

and was able to take liquids. However, her level of consciousness subsequently diminished. No further aggressive measures were undertaken and death occurred on April 17, XX at 12:45 PM.

Summary and Interpretation The autopsy confirmed the presence of residual mixed germ cell tumor in the pineal region with fibrosis and differentiation to ganglioglioma. Malignant gliomatosis was also present with diffuse infiltration of the cerebral hemispheres consistent with gliomatosis cerebri. This malignant gliomatosis developed after several symptom free years following irradiation and chemotherapy for the mixed germ cell tumor of the pineal region. The cause of death in this case is attributed to gliomatosis cerebri which caused bilateral uncal herniation, cerebellar tonsillar herniation, herniation of the left cingulate gyrus and right hippocampal gyrus, and compression of the mid brain and anterior lobe of the cerebellum.

Neuropathology Report

Gross Examination: The cerebral hemispheres are diffusely swollen, more so on the left hemisphere than the right. The gyri over the convex surfaces of the hemispheres are diffusely flattened. The sulci are in general obliterated. The right superior frontal gyrus, anteriorly, the gyri over the left temporo-parietooccipital region, and the left superior temporal gyrus throughout its extent are particularly broadened or swollen. There is a small defect at the anterior part of the right superior frontal gyrus, which represents the site of previous biopsy. As the base of the brain, the left uncus shows marked herniation, while the right uncus shows mild herniation. The posterior portion of the right hippocampal gyrus also shows mild herniation into the incisura of the tentorium; the herniated portion has compressed and the medial surface of the right anterior lobe of the cerebellum. The tegmentum of the midbrain on that side is also markedly compressed. The arteries at the base of the brain are unremarkable. The optic nerves appear to be somewhat swollen. Coronal sections through the frontal gyrus. It measures approximately 2 cm anteroposteriorly, 2 cm transversely and 3–4 cm dorsoventrally. It involves the cortex of the superior frontal gyrus extending to the central white matter and has reached the white matter of the cingulate gyrus and corpus callosum. The right cerebral hemisphere is generally compressed due to a marked expansion of the left cerebral hemisphere. The left cingulate gyrus is herniated beneath the cerebral falx and the midline structures are shifted to the right as a result of marked swelling of the left hemisphere. In the left cerebral hemisphere, there is a massive greenish-gray, almost semi-translucent, soft lesion which has occupied most of the posterior temporal and parietal white matter extending to the occipital white matter. The lesion extends medially to involve the entire lateral wall of the atrium, then central white matter of the temporal lobe up to the plane of the subthalamic nuclei. Anterior to this plane, the temporal white matter over the roof of the temporal horn, mesial temporal region and the cortex and white matter of the superior temporal gyrus are mainly affected. The lesion extends almost to the temporal pole. The left frontal white matter is diffusely swollen as compared with the right despite that the white matter texture appears to be normal. The corpus callosum is somewhat more voluminous than usual. The splenium of the corpus callosum is completely degenerated. A part of the degenerated splenium is replaced by a lobulated grayish-white nodule which measures approximately 1.5 x 1.0 cm involving mainly the right half of the genu; it protrudes to the atrium. Near the nodule, the fornix is somewhat degenerated more so on the right than the left. The internal veins are widely patent but surrounded by some rusty brown-colored tissue, attached to this gray-white mass is another small nodule which is pedunculated and appears to be the pineal gland. It measures approximately 0.8 x 0.5 x 0.5 cm. It is situated between the internal cerebral veins and the tectum of the midbrain where the pineal gland sits. The ventricles are patent, although somewhat narrowed. Transverse sections through the brain stem disclose marked compression of the midbrain from side to side, particularly at the tegmentum and tectum on the right. There is focal necrosis in the tectum. The cerebellum again shows severe compression of the dorsal surface with scaphoid compression and relative elevation of the superior vermis. The cerebellar tonsils are mildly herniated.

Transverse sections through the cerebellum and brain stem disclose no significant abnormalities. The spinal cord shows no significant abnormalities.

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