Onco Flashcards
CT MRI findings of medulloblastoma
-HOMER WRIGHT ROSETTES (high yield)
-Monomorphic sheet of undifferentiated cells classically noted as small blue round cells
- Solid homogeneous contrast medium enhancing mass in posterior fossa causing fourth ventricular obstruction and hydrocephalus
Immuno positivity for this marker is useful in confirming diagnosis of Choroid plexus tumors
transyrethin (prealbumin
Account for 2 to 3% of traveled brain tumors within the first decade of life.
Histological is similar to medulloblastoma and I composed of undifferentiated or poorly differentiated Neuro epithelial cells
Supratentorial primitive neuroectodermal tumors (SPNETs)
It’s a common brain tumor of childhood accounting for 7 to 10%. They often present with endocrine abnormality such as growth failure and delayed sexual Maturation.
MRI findings solid tumors at cystic structures contain fluid of intermediate densities CT scan show calcifications
Craniopharyngioma
Treatment for craniopharyngioma
surgery
radiation therapy controversial.
Chemotherapy no role
Analysis of these 2 protein markers maybe useful in establishing diagnosis and monitoring germ cell tumors
alpha-fetoprotein
Beta human chorionic gonadotropin
This tumor is a very aggressive embryonal malignancy that occurs in children less than five years old and Can occur at any location in the neuroaxis.
Histology: heterogeneous pattern of cells with Rhabdoid cells that express epithelial membrane antigen and Neuro filament antigen
GEnetics: Partial or complete deletion of chromosome 22Q11.2 Invitation in the INI1 gene
A typical teratoid/rhabdoid tumor
Test imaging modality in brain tumors
Cranial CT scan in critical/urgent cases (unstable, decreased sensorium, etc). However some mass may be poorly defined)
Cranial MRI with and without contrast in stable patients
Class of brainstem to mass/tumors with very poor outcome independent of histologic findings
Diffuse intrinsic tumors
What are the posterior fossa tumors of childhood (5)
Medulloblastoma
cerebellar astrocytoma
Brainstem glioma
Ependymoma
Atypical teratoid/Rhabdoid
Most common extracranial solid tumor in children in the most commonly diagnosed malignancy in infants
Neuroblastoma
Syndromes associated with neuroblastoma name six
- Pepper syndrome-Massive involvement of the liver with metastatic disease with or without respiratory distress
- Horner syndrome- Unilateral ptosis meiosis and anhidrosis associated with traffic or cervical primary tumor and symptoms does not resolve with tumor section
- Hutchinson syndrome- Limping and irritability and young child associated with bone and bone marrow metastasis
- Opsoclonus- Myoclonus Ataxia syndrome- myoclonic jerk and random eye-movement with or without cerebellar ataxia
- KERNER – MORRISON syndrome- Intractable secretory diarrhea Jetta to my resection of vasointestinal peptides
- Neurocristopathy syndrome - Neuroblastoma with other neural stress disorders like hirschsprung
Diagnostics and tumor markers In neuroblastoma
- Urine homovanillic acid (HMA)
- Urine Vanillylmandelic acid (VMA)
- Iodine – 123 meta-iodobenzylGUANIDINE (123-MIBG)
- Hemorrhage and Notifications on x-ray and CT scan
International neuroblastoma staging system (INSS) Stage wherein tumors extend beyond the structure of origin but not across the midline
Stage II
IIa- no Ipsilateral lymph node involvement
IIb - With Ipsilateral lymph node involvement
International neuroblastoma staging system (INSS) Stage wherein Tumors are confined to the organ or structure of origin and are completely rejected
INSS stage I
International neuroblastoma staging system (INSS) Stage wherein Tumors extend beyond the midline with or without bilateral lymph node involvement
INSS stage three
International neuroblastoma staging system (INSS) Stage wherein Tumors are disseminated with metastasis to bones, bone marrow liver distant lymph nodes and other organs
INSS stage four
International neuroblastoma staging system (INSS) Stage wherein Neuroblastoma in children less than one year old with dissemination to liver, Skin, and/or bone marrow without bone involvement and with a primary tumor that would otherwise Be staged as I NSS stage one or two
The kalokang I NSS stage 4S
Treatment for neuroblastoma stage one and two
Surgery and observation
Treatment for I NSS stage 4S neuroblastoma
OBSERVATION
Stage 4s has a very good prognosis because many regress spontaneously
Treatment for intermediate Risk Neuroblastoma
Surgery, chemotherapy, and sometimes with radiation therapy
Most common primary malignant Renal tumor of childhood. It is the second most common malignant abdominal tumor in childhood
Wilms tumor
The top 1 and 2 Malignant bone tumors
Number one osteosarcoma
Number two Ewing’s sarcoma
Conditions that are risk factor for developing osteosarcoma
Retino blastoma
Li-Fraumeni
Paget Disease
Radiotherapy
Symptoms in osteosarcoma in and ewing’s sarcoma Includes local bone pain and swelling. What are the differences between their symptoms
OsteoSA : local pain and swelling often with history of injury
Ewings SA : Local pain and swelling usually associated systemic symptoms such as FEVER and WEIGHT LOSS
Radiographic findings of Sunburst pattern with Sclerotic destruction
Osteosarcoma
Radiographic finding off primary lytic multilaminar Periosteal reaction (Onion skinning) (Moth eaten)
Ewing’s SARCOMA
Treatment of osteosarcoma and ewings sarcoma
Osteosarcoma: chemotherapy, ablative surgery of primary tumor
Ewings SARCOMA: Radiotherapy and/or surgery of primary tumor
Primitive neuroectodermal tumor that arise in the chest wall. It belongs to the Ewing sarcoma family of tumors
Askin Tumors
Most common benign tumors of infancy of course and 5% of term infants, Mostly located in the head and neck region
Hemangiomas
Primary site of visceral involvement in hemangioma
Liver
Syndrome characterized by a rapidly enlarging Lesion, Thrombocytopenia, micro angiopathic hemolytic anemia, and coagulopathy as a result of platelet and red blood cell trapping an activation of the clotting system Within the vasculature of the hemangioma
Kassabach -meritt syndrome