SANS Tumor Flashcards
Seorang pasien berusia 65 thn dgn KPS dari 70 mengalami reseksi subtotal dari 7 cm Low Grade Glioma frontal kiri dgn pemetaan fungsional intraoperatif. Menurut percobaan EORTC, apakah manajemen yg plg tepat setelah reseksi bedah? A. Terapi radiasi 59 Gy B. Terapi radiasi 45 Gy C. Kemoterapi saja D. Serial post-op imaging
B. Terapi radiasi 45 Gy According to the EORTC, the lesion’s size and the patient’s age and KPS portend a poor prognosis, indicating that postoperative radiation therapy to a dose of 45 Gy would be the most appropriate postoperative management. Traditionally, low grade gliomas are often surgically resected and followed with serial imaging with reservation of radiation therapy until signs of tumor progression. According to the European Organization for Research and Treatment of Cancer (EORTC) trials, this patient has a high-risk unfavorable prognostic score for tumor progression. According to this grading system, 1-point was added for each of the following negative prognostic factors: age 40 or greater, tumor diameter 6 cm or greater, tumor crossing the midline, and presence of neurologic symptoms. Therefore, in subgroups at high risk for tumor progression, early radiation therapy after surgical resection is initiated as it can increase PFS and seizure control without any effect on overall survival. Studies have shown that there is no difference in OS or PFS between those receiving 45 Gy and 59.4 Gy of radiation. Therefore, the risk of greater radiation complications at higher doses has not shown to be more beneficial at stopping tumor progression.
Apakah karakteristik histologis meningioma yang digambarkan di daerah dilingkari dari slide?
A. Mitotic
B. Verocay bodies
C. Proliferasi endotel
D. Vacuolation
E. S-100 reactivity
D. Vacuolation
Vacuolation or intranuclear clear spaces are characteristically seen with meningiomas and are attributed to glycogen invagination into cellular nuclei. Endothelial proliferation is characteristically seen with malignant gliomas. Mitotic figures are a non-specific indicator or cellular proliferation. S-100 reactivity is generally seen with schwanommas.
Seorang pria 24 thn datang ke ruang emergensi dengan keluhan kejang parsial kompleks onset baru. Dia scr neurologis normal. CT menunjukkan massa berbatas tegas, dengan kalsifikasi samar di lobus temporal kanan. MRI menunjukkan massa berbatas tegas, kista seperti massa yg hipointense pada T1 dan hiperintense pada T2-weighted imaging. Reseksi bedah dilakukan dan diagnosis patologis adalah ganglioglioma. Manakah dari pernyataan berikut ini BENAR ?
A. Komponen glial dari tumor ini cenderung menjadi oligodendroglial
B. Tumor ini hampir selalu meningkatkan kontras pada pencitraan CT dan MRI
C. Stain sel ganglion untuk synaptophysin, yang membantu menegakan diagnosis pada kasus sulit
D. Kebiasaan biologis diprediksi oleh histologi tumor
E. Terapi radiasi diindikasikan untuk seluruh gangliogliomas yang telah direseksi subtotal
C. Stain sel ganglion untuk synaptophysin, yang membantu menegakan diagnosis pada kasus sulit
Gangliogliomas are rare, slow-growing tumors of the central nervous system (CNS) that are seen primarily in young adults and children, with a peak incidence at 11 years of age. These lesions may occur in any location within the CNS, but the majority are supratentorial. The temporal lobe is the most common site, with the next most common being the frontal and parietal lobes. Temporal lobe lesions typically present with seizures. Patients presenting with seizures tend to have a better prognosis because they usually are diagnosed and treated earlier.
Imaging characteristics are variable, and enhancement can vary from none to striking. These lesions most commonly appear as well-delineated masses that are hypointense on T1-weighted images and hyperintense on T2-weighted images. Cysts and calcifications can be seen.
Microscopic analysis typically reveals well-differentiated but neoplastic ganglion cells mixed with glial stroma, usually containing astrocytes. Infrequently, anaplastic degeneration occurs and involves the astrocytic component. Biological behavior is not predicted by histology, because many histologically anaplastic gangliogliomas do not demonstrate clinically aggressive behavior. Identification of ganglion cells can be facilitated with synaptophysin staining. Synaptophysin is a membrane glycoprotein of synaptic vesicles that is ubiquitously expressed in all neurons and in many endocrine cells. It is currently the most widely used marker for nerve terminals.
Treatment of these lesions is surgical, and long-term survival is the rule, even with incompletely resected lesions. The value of radiation therapy is unknown and is not recommended after gross total resection. Radiation is usually reserved for progressive growth observed on follow-up imaging.
Seorang wanita berusia 34 thn dgn tingkat prolaktin 48 ng/ml (normal, kurang dari 25 ng / mL). Dia memiliki amenore tetapi tidak galaktore. Penjelasan yg tepat untuk kombinasi temuan ini adalah :
A. Nilai lab false negatif (hook effect)
B. Nilai lab false positif
C. Macroadenoma dengan stalk effect
D. Empty sella
E. Prolactinoma
C. Macroadenoma dengan stalk effect
Prolactinomas generally show levels of prolactin > 100 ng/mL. The presence of amenorrhea without galactorrhea means little. This level (48 ng/ml) is most consistent with stalk effect. Stalk effect is the result of compression of the pituitary stalk by a mass lesion (often a non-secretory macroadenoma), resulting in interruption of delivery of the inhibitory releasing factor, dopamine, from the hypothalamus to the pituitary gland. In this circumstance, prolactin levels of 50 to 100 ng/ml are common. Empty sella syndrome is not generally associated with elevated prolactin levels. Laboratory error, based on the hook effect, should be considered. In this circumstance, extremely high levels of prolactin ( > 10,000 ng/ml) may overwhelm some forms of bioassay, resulting in an abnormal level orders of magnitude lower than the true measurement. However, this rare laboratory error is largely obviated by most modern assay systems.
Seorang pria berusia 50 thn dengan massa enhancing tunggal di daerah periventicular frontal kanan. Biopsi menunjukkan primary CNS lymphoma. Apakah manajemen yg plg tepat?
A. Stereotactic radiosurgery
B. Reseksi bedah dan kemoterapi
C. Hanya reseksi bedah
D. Hanya kemoterapi
E. Hanya radioterapi
D. Hanya kemoterapi
The treatment for CNS lymphoma consists of chemotherapy alone. Primary CNS lymphoma (PCNSL) is a form of non-Hodgkin’s lymphoma that arises from the spinal cord, brain, eyes, or leptomeninges. Treatment with methotrexate has been shown to improve both disease control and survival in patients with this disease. The addition of procarbazine and vincristine (MPV) has also been shown to improve disease control.
Pria 58 thn right handed datang dgn keluhan diplopia dan sakit kepala bifrontal. Pada pemeriksaan didapatkan oftalmoplegia kiri parsial. Hasil MRI tertera di gambar. Biopsi menunjukkan tumor ini merupakan chordoma. reseksi skull-based subtotal ekstensif dilakukan. Dalam kasus ini apakah pilihan terapi terbaik selanjutnya?
A. Radioterapi fractionated konvensional
B. Procarbazine, CCNU dan vincristine kemoterapi
C. Radioterapi sinar proton
D. Gamma knife atau LINAC-based radiosurgery
C. Radioterapi sinar proton
Chordomas account for only 0.15% of intracranial tumors and are most likely to be located in the clivus or sacrum although they can be located anywhere along the spinal axis. The tumors tend to be lobulated, gelatinous masses surrounded by a pseudocapsule. Microscopically, they consist of physaliphorous, stellate and transitional cells. Although the tumors are typically histologically benign they usually have a malignant clinical course with frequent recurrence and local mass effect related symptomatology relating to cranial nerve and brainstem compression. While surgical resection is a mainstay of therapy, these tumors are difficult to cure surgically and adjuvant therapy is often used. A variety of anterior midline surgical approaches are used, including transphenoidal, transoral, and midfacial approaches. Reconstruction of the skull base dura is often a technical challenge and can lead to significant morbidity. Although there are sporadic reports of tumor regression with chemotherapy, there is still no widely accepted role for this treatment modality. Similarly, these tumors are mostly resistant to conventional fractionated radiotherapy. But they do appear to be sensitive to higher doses of radiation than can be safely delivered via conventional fractionation. Two methods that are used to more safely deliver higher dose radiation to these tumors are radiosurgery and proton beam radiotherapy. In this case, the tumor is too large for single fraction radiosurgical treatment. Proton beam radiotherapy relies upon the Bragg peak effect to produce relative dose concentration within the tumor and sparing of surrounding tissues.
Seorang wanita berusia 59 thn dgn riwayat kanker paru non-small cell datang dgn metatasis otak soliter dan tidak ada bukti dari penyakit ekstrakranial aktif. Dia mengeluhkan sakit kepala tetapi sebaliknya secara fungsional independen. Apakah recursive partitioning analysis (RPA) class pasien ini?
A. 3
B. 2
C. 1
D. 4
E. 5
C. 1
A patient is RPA 1 if their age is less than 65, KPS >70 (or functionally independent), control of primary disease, and no extracranial metastatic spread. RPA 2 are patients older than 65 and KPS>70, with or without primary control and/or extracranial metastatic disease. RPA 3 is assigned for patients with a KPS<70. RPA classes 4-5 do not exist for classifying metastatic disease.
The recursive portioning analysis (RPA) was developed to predict survival for patients who present with intracranial metastatic disease. Gaspar et al. developed his scoring system using the Radiation Therapy Oncology Group (RTOG) data, and showed that survival was affected by the following prognostic variables: pre-operative Karnofsky performance status (KPS), status of primary disease, age, and metastatic spread.
Seorang pria berusia 62 thn datang dgn riwayat sakit kepala, ataksia dan nistagmus. MRI dilakukan. Manakah diagnosis yg plg mungkin ?
A. Subependymoma
B. Diffuse intrinsic pontine glioma
C. Chordoid glioma
D. Central neurocytoma
E. Choroid plexus papillloma
A. Subependymoma
This is a fourth ventricular tumor with minimal peripheral enhancement in an adult. It was found to be a subependymoma. Subependymomas are generally characterized by slow growth and an indolent clinical course (and, in fact, are often discovered incidentally). They tend to occur in adults and most often arise in the region of the lower medulla and project into the fourth ventricle. They may also arise in the frontal horn of the lateral ventricle where they may attach to the septum pellucidum or the lateral ventricular wall. Contrast enhancement may occur although is usually not striking. They can cause obstructive hydrocephalus, but not in this case. Complete resection can be curative and routine postoperative radiation is not generally employed. Choroid plexus papillomas generally demonstrate intense, homogeneous contrast enhancement. Chordoid gliomas are rare tumors of the third ventricle occurring predominantly in middle-aged females. Central neurocytomas primarily involve the third and lateral ventricles, fourth ventricular occurences have not been reported. Diffuse intrinsic pontine glioma appears on MR imaging as global enlargement and signal change in the brainstem, centered in the pons, rather than as a 4th ventricular mass.
Seorang pria 62 thn datang dgn penglihatan ganda onset subakut, ketidakseimbangan dan sakit kepala. Pemeriksaan neurologis signifikan untuk disfungsi cerebellar dan diplopia pada pandangan horizontal ke kanan. Pada MRI ditemukan massa d = 4.3 cm di cerebellum kanan dengan minimal enhancement atau edema. Apakah diagnosis plg mgkn?
A. Hemangioblastoma
B. Metastatic carcinoma
C. Lhermitte-Duclos
D. Medulloblastoma
E. Pilocytic astrocytoma
C. Lhermitte-Duclos
Lhermitte-Duclos Disease (LDD), or dysplastic gangliocytoma of the cerebellum, is a relatively rare disease. It has been reported in all age groups (from birth to 74 years of age) with a mean age at presentation of 34 years. It consists of a focal or diffuse engorgement of cerebellar folia behaving as a space-occupying lesion. LDD shows progressive growth. Clinical manifestations are related to a posterior fossa mass effect and secondary obstructive hydrocephalus. Surgical excision is the only effective treatment to definitely relieve the symptoms. MRI clearly shows the typical striated, laminated or ��tiger striped�� appearance (arrow) of the involved cerebellar tissue. Thus, the diagnosis can often be achieved preoperatively. In some cases these lesions were seen in the context of Cowden Disease, which is a genetic condition characterized by the presence of cutaneous and non-cutaneous hamartomas as well as breast, thyroid, gastro-intestinal and genito-urinary neoplasias. This disorder is caused by the gene at chromosome 10q22-23.
Apakah neoplasma suprasellar yg menunjukkan karakteristik epitel skuamosa dan pertumbuhan yang solid pada orang dewasa tua?
A. Metastatic adenocarcinoma
B. Germinoma
C. Pituitary adenoma
D. Papillary craniopharyngioma
D. Papillary craniopharyngioma
The only suprasellar neoplasm in adults that displays a squamous epithelial component is craniopharyngioma. Papillary craniopharyngiomas, in contrast to the more common classic adamantinomatous form, occur in older patients (adults, peak at 40-50 years) and most commonly form solid sheets of squamous epithelium with pseudopapillary formations. Papillary craniopharyngiomas lack the wet keratin, lack palisading peripheral cells of adamantinous craniopharyngioma, and frequently lack calcification.
Berdasarkan pedoman American Academy of Neurology, bagaimanakah seharusnya pasien dgn metastasis otak tanpa riwayat kejang diperlakukan berkaitan dgn profilaksis kejang?
A. Tidak ada indikasi untuk profilaksis kejang
B. Obati dgn levatiracetam (Keppra)
C. Obati dgn phenytoin (Dilantin)
D. Obati dgn gabapentin (Neurontin)
A. Tidak ada indikasi untuk profilaksis kejang
Based on level 3 evidence, patients with solid brain metastases but without a history of seizures do not require seizure prophylaxis therapy.
These recommendations are based on a single underpowered randomized controlled trial (RCT) which did not detect a difference in seizure occurrence. There has otherwise been a lack of properly designed studies to provide higher level evidence or rationale for the optimal management of such patients with respect to seizure prophylaxis.
Given the premise that brain metastases are probably less likely than primary brain tumors to cause seizures, it is noteworthy that previously published guidelines on the role of anticonvulsants in patients with brain tumors (either primary or secondary) have recommended against their prophylactic use.
Sebuah astrositoma dengan atypia sitologi kurang mitosis, nekrosis atau perubahan proliferasi vascular akan diklasifikasikan grade WHO :
A. IV
B. II
C. III
D. I
B. II
Fibrillary astrocytoma World Health Organization (WHO) grade II lacks mitotic activity, vascular proliferation, and tumor necrosis. Astrocytoma WHO grade I include pilocytic astrocytoma, dysembryoplastic neuroepithelial tumors, and subependymal giant cell astrocytoma. WHO grade III (or anaplastic) astrocytoma demonstrate mitoses and cellular pleomorphism but no necrosis. WHO grade IV astrocytoma (or glioblastoma) show high cellularity, nuclear and cellular pleomorphism, mitoses, endothelial proliferation and necrosis.
Selama reseksi schwannoma vestibular besar melalui sub-oksipital, pendekatan retrosigmoid, Anda tiba-tiba mengalami pembengkakan cerebellar parah dan herniasi, langkah terbaik berikutnya adalah :
A. Sebagai ahli anestesi memberikan manitol dan memulai hiperventilasi
B. Reseksi area yg dapat diakses pada cerebellum lateral
C. Keluarkan retractor dan tunggu jika bengkak berkurang
D. Buat burr hole parietoccipital dan letakkan catheter ventriculostomy posterior untuk drainase CSF
D. Buat burr hole parietoccipital dan letakkan catheter ventriculostomy posterior untuk drainase CSF
The patient is likely experiencing acute hydrocephalus related to brain edema and CSF obstruction. The only option that would rapidly change this process would be immediate CSF diversion; this would be performed the quickest by ventriculostomy placement via a parietoccipital approach. Mannitol and hyperventilation may also help but would be unlikely to reverse this problem.
Selama diseksi dan debulking tumor di aspek medial daerah pontomedullary, saraf wajah yg plg sering ditemui adalah di dekat :
A. Aspek Ventral-Central dari tumor
B. Aspek Ventral-Inferior dari tumor
C. Aspek Ventral-Superior
D. Aspek superficial (dorsal) dari tumor
C. Aspek Ventral-Superior
The most common location to find the facial nerve during dissection of vestibular schannomas is at the ventral-superior aspect of the tumor. With larger tumors, the variability of the course of the facial nerve (FN) increases, contributing to the increased risk of FN injury during these cases.
Untuk low grade gliomas, apakah kisaran diameter maksimal (cm) yg telah teridentifikasi sbg ambang batas untuk prognosis negatif ?
A. 6-8
B. 4-6
C. 2-4
D. 8-10
E. Ukuran bukan penentu faktor prognosis
B. 4-6
Low grade gliomas with a maximal diameter greater than 4-6 cm (measured on FLAIR MRI) are associated with a worse prognosis. The 4 cm threshold was recently reported by the UCSF group as part of a comprehensive low grade glioma scoring system whereas the 6 cm threshold was reported by EORTC.
In addition to the prognostic implications of size, the preoperative grading system for low grade infiltrating gliomas that originated from UCSF identified three other factors: age greater than 50, KPS (Karnofsky Performance Score) of 80 or less, and location in eloquent brain. For each parameter that is present, 1 point is assigned and added for a total score. The total score can then be used to predict 5-year survival and 5-year PFS as shown in the table below.
Sum 5-year survival 5-year progression free survival
0-1 97% 76%
2 81% 49%
3-4 56% 18%
Manakah dari kelainan genetik berikut yg paling sering dikaitkan dengan tumor teridentifikasi dalam fotomikrograf ini :
A. Hilangnya heterozygosity (LOH) 1p dan 19q
B. Mutasi gen NF1
C. Hilangnya heterozygosity (LOH) kromosom 17
D. Mutasi gen TP53
E. Amplifikasi EGFR
A. Hilangnya heterozygosity (LOH) 1p dan 19q
The diagnosis of the tumor is oligodendroglioma. Somatic deletions of chromosomes 1p and 19q are some of the most common events in the tumorigenesis of oligodendrogliomas, occuring in about 40-70% of tumors. The presence of 1p and 19q deletions is highly associated with the oligodendroglial phenotype. Mutations of the TP53 gene in oligodendrogliomas are infrequent and less common than in astrocytic tumors. LOH of chromosome 17 is found infrequently in low grade oligodendrogliomas. EGFR amplification is most commonly associated with glioblastoma. NF1 gene mutation is most commonly seen in neurofibromas and not in oligodendrogliomas.
