Tumors Flashcards
Appearance of hemangioblastoma in the spine? What is the secreted factor and associated secondary disease?
Intramedullary cyst with enhancing mural nodule. Sometimes secretes erythropoietin causing secondary polycythemia Vera. Can lead to strokes
They are rich in blood vessels and clear cells (from lipid content)
Which immunohistochemical markers is most accurate in diagnosing a central neurocytoma?
Strong staining for the neuronal marker synaptophysin, as well as for neuron-specific enolase, is characteristic of central neurocytoma. Glial tumors are usually vimentin and GFAP positive. Meningiomas, on the other hand, are usually positive for vimentin and EMA, but negative for GFAP. S100 stains for neural crest cell derived tumors, including schwannomas, melanocytes, chondrocytes, etc.
In children, the most common intramedullary spinal cord neoplasm is?
Astrocytomas are the most common intramedullary tumor in the pediatric population accounting for 60% of these tumors. This is followed by ependymomas at 30% and developmental tumors at approximately 4%
Which primary tumors has the greatest propensity to metastasize to the brain?
Melanoma is the 6th most common cancer in the U.S. among adults and has the highest propensity to metastasize to the brain. Up to 75% of patients who die from melanoma have brain metastases and in 50% of cases the cause of death is brain metastases.
Lung, breast and colon cancer are common brain metastases encountered in practice due to the high incidence of these cancer types.
Prostate cancer often metastasizes to bone so skull and spine mets are much more common compared to brain metastases.
The prognosis associated with melanoma including patients with brain metastases has improved due to treatment options with BRAF inhibitors and immunotherapy. 50-60% of patients demonstrate response of intracranial brain mets to treatment with immunotherapy
What are the criteria for NF1 diagnosis?
involves mutations/deletion of the neurofibromin gene on 17q
autosomal dominant, though almost 50% of cases arise sporadically
diagnostic criteria for NF1 are met if a person has two or more of the following:
1) Six or more cafe-au-lait macules that have a maximum diameter of greater than 5 mm in prepubertal patients and greater than 15 mm in post pubertal patients
2) Two or more neurofibromas of any type, or one plexiform neurofibroma
3) Freckling in the axillary or inguinal region
4) Optic glioma
5) Two or more Lisch nodules
6) A characteristic osseous lesion, such as sphenoid wing dysplasia or thinning of long bone cortex
7) A first-degree relative with NF1 by the above criteria
Extraneural metastases from a medulloblastoma most commonly occur in which anatomic locations?
Medulloblastoma is the most common brain tumor in children and 7-10% of patients will develop extraneural metastases. Extraneural metastases can involve bone (85% of cases), bone marrow (27%), lymph nodes (15%), lung (6%) and liver (6%).
Which radiosurgical doses is most likely to provide tumor control and hearing maintenance in a patient with a 1-cm vestibular schwannoma?
At a dose of 12-13 Gy, the facial nerve function is reportedly very well preserved.
Studies evaluating higher doses have found 8% facial dysfunction at a mean marginal dose of 13.4 Gy (Rowe et.al.), 16% at 14 Gy (Matthieu et.al.), 33% at 17 Gy (Rowe et.al.) and 50% at 15-16 Gy (Mallory et al. only evaluating 78% of all patients).
Doses lower than 12-13 Gy have been associated with less effective tumor control.
A 25-year-old man is evaluated because of a one-month history of headache and unsteady gait. An MR image and a CT scan are shown. This patient’s abnormality is most likely in which of the following vascular distributions?
A choroid plexus papilloma is seen in the left lateral ventricle. The choroid of the lateral ventricles is supplied by the posterior choroidal artery arising from the posterior cerebral artery.
The medial posterior choroidal artery supplies the choroid plexus of the third ventricle. The lateral posterior choroidal artery supplies the choroid plexus of the lateral ventricles.
Which study is best to differentiate tumor from abscess?
MR spectroscopy
Contents, locations, and types of dermoid cysts
most common congenital lesion of the scalp and calvarium
result from failure of disjunction of the neuroectoderm
contain epithelium, hair follicles, sebaceous glands, and other cutaneous elements.
Nasal and midline sub-occipital (inion) cysts are more likely to be associated with a small overlying pit or tract and have a higher risk of intracranial and intradural extension (generally between the leaves of the falx cerebri or falx cerebelli, respectively).
Extracranial cysts present as palpable or enlarging lumps or with local infection. Cysts with intracranial extension may also present due to recurring meningitis or, rarely, with intracranial mass effect. The treatment in all cases is surgical excision.
Most common location for and appearance on MRI of DNET?
Dysembryoplastic neuroepithelial tumor (DNET):
WHO grade I lesion that has glioneuronal elements
associated with cortical dysplasias.
most common in the temporal lobe (50-80%) followed by the frontal lobe.
DNETs can account for 20% of medically refractory epilepsy.
usually well circumscribed on imaging with a “bubbly” appearance
hyperintense on T2 with patchy enhancement on T1 post contrast imaging.
A 47-year-old woman has a focal motor seizure seven months after undergoing stereotactic radiosurgery for metastatic breast cancer lesions. Today, an MR image of her head shows loss of enhancement within the confines of the treated right frontal lobe lesion. Findings include a ring of contrast enhancement in the right frontal lobe surrounding the treated tumor, and hyperintensity in the right frontal lobe on T2-weighted MR image and FLAIR, which are consistent with edema. A PET scan with FDG demonstrates hypometabolism within the right frontal lobe lesion. The lesion is most likely which of the following?
Radiation induced neoplasm.
The criteria for a radiation-induced tumor include: 1) a latency interval between delivery of the radiation and tumor development, 2) the tumor must arise in the irradiated region, 3) the neoplasm must be histologically distinct from the original irradiated tumor, and 4) an absence of a genetic predisposition for neoplastic transformation.
Most common type of brain tumor? Most common primary? Sex predisposition?
Metastasis are most common.
most common primary is glioma (males), second is meningioma (females)
Causes of brain tumors:
Radiation: Associated with meningiomas, fibrosarcomas, and gliomas.
Immunosuppression: Associated with lymphomas.
Viruses: EBV is associated with Burkitt lymphoma and nasopharyngeal carcinoma, and the human papillomavirus is associated with cervical carcinoma.
Chemotherapy: nitrosoureas.
Genetics: As seen in phakomatoses and Turcot syndrome (APC gene mutation on chromosome 5q with familial polyposis, colorectal cancer, and primary brain tumors).
Immunohistochemical stains and their associated tumors:
AFP: embryonal carcinoma, endodermal sinus tumor.
CEA: carcinoembryonic antigen.
Chromogranin: pituitary adenoma.
Common leukocyte antigen: lymphoma, germinoma.
Cytokeratin: carcinoma, craniopharyngioma, chordoma.
Desmin: rhabdosarcoma, teratoma.
Epithelial membrane antigen (EMA): carcinoma, meningioma, epithelial cysts.
GFAP: astrocytomas, other glial tumors.
Human melanoma black (HMB): melanoma.
Beta human chorionic gonadotrophin (HCG): choriocarcinoma and the syncytiotrophoblastic variant of germinomas.
Immunoglobulins kappa and lambda chains: lymphomas.
Neurofilament and synaptophysin: ganglioglioma, PNET.
Pituitary hormones: pituitary adenoma.
Prostate specific antigen: prostate carcinoma.
S100: schwannoma, neurofibroma, glioma, PNET, chordoma, melanoma, renal cell carcinoma.
Synaptophysin: tumors with neurons (ganglioglioma, central neurocytoma, etc.).
Transthyretin: choroid plexus tumors.
Vimentin: meningioma.
Paraneoplastic syndromes: Stiff man syndrome:
Involuntary muscle spasms and rigidity; 60% have antibodies to glutamic acid decarboxylase.
Paraneoplastic syndromes: Anti-Yo antibodies
These cause cerebellar degeneration and are associated with ovarian and breast cancer.
Paraneoplastic syndromes: Anti-Ri antibodies
These cause opsoclonus and are associated with breast cancer.
Paraneoplastic syndromes: Anti-Hu antibodies
These cause sensory neuropathy, encephalitis, and cerebellar degeneration. They are associated with oat cell pulmonary carcinoma or lymphoma.
Paraneoplastic syndromes: Lambert–Eaton syndrome
Antibodies to the presynaptic voltage-gated Ca2+ channels; associated with oat cell (small cell) lung carcinoma.
Paraneoplastic syndromes: Limbic encephalitis
subacute encephalitis. Gross examination appears normal.
perivascular mononuclear infiltrates, no viral inclusions.
medial temporal lobes, cingulate gyrus, and insula are predominantly affected
usually bilateral hyperintense lesions on T2-weighted MRI
most common in men in their mid-60s and manifests as memory impairment and altered mental status.
may be associated with testicular or lung cancer and anti-Ma protein antibodies. Limbic encephalitis should be differentiated from herpes encephalitis.
JPA: histopathology, survival, appearance on MRI
Is the outcome similar in adults?
second most common pediatric brain tumor
most frequently located in the cerebellum, brainstem, optic pathway, and infundibulum
biphasic pattern of loose cells and microcysts and also dense elongated hair-like astrocytes with Rosenthal fibers and eosinophilic granular bodies.
They tend to be noncystic in the medulla and optic pathway.
Leptomeningeal invasion, nuclear atypia, multinucleated cells, and vascular proliferation are frequently noted but are not adverse prognostic indicators. Approximately 10% contain calcium
Survival rate is 86–100% at 5 years, 83% at 10 years, and 70% at 20 years
In adults, it is not well circumscribed and has a worse outcome
common location and symptom associated with DNET and gangioglioma? How can the two be distinguised?
can both be in temporal lobe and cause seizures
differentiate with path: DNET has large neurons floating in mucin, chicken wire vascular pattern, with small oligodendrocytic cells (image)
ganglioglioma has perivascular lymphocytes, large multinucleated ganglion cells, may have calficiations, frequently cystic and positive for both GFAP and neurofilament
Calcified psammoma bodies and whorls are characteristic of which tumor?
Meningioma
Large stag horn blood vessels are characteristic of which tumor?
Hemangiopericytomas
positive for reticulin and vimentin, negative for EMA
Histopathology of chordoma:
characterized by physaliphorous round cells and a mucinous background
appearance of eosinophilic granuloma on histopathology?
Bilobed nuclei of eosinophils (black arrow-head), foamy macrophages (white arrow), and large grooved nuclei (black arrow) are seen in eosinophilic granuloma (Langerhans cell histiocytosis)
What are the histopathologic features of schwannoma?
compact areas called Antoni A (A) and loose areas called Antoni B (blasted apart; B)
Verocay bodies (V) have nuclear palisading around anuclear areas
Some nuclei are arranged like a school of fish (S).
They can occur in NF2
classic feature in seen in histology for yolk sac tumors?
schiller duval bodies
