Dr.Dafalla Ahmed Babiker Jazan University

Transcription

1 Dr.Dafalla Ahmed Babiker Jazan University

2

3 Brain tumors are the second commonest malignancy in children Infratentorial tumors are more common As a general rule they do not metastasize out of the CNS, but it may occurs along CSF pathways

4

5 pathogenesis There is >100 histologic categories The following categories constitute 80% of pediatric brain tumors, they are astrocytoma medulolblastoma ependymomas craniopharyngioma

6 CNS tumors are of varying degree of malignancy, note that a benign tumor which can not be removed because of its location is more serious than a malignant tumor that can be removed Diagnosis may be delayed because of non specific symptoms at the beginning

7 Clinical manifestations depends on the tumor location, tumor type and age of the child Infratentorial: increased ICP e.g headache, vomiting, irritability, papilledema. ataxia,cranial nerves palsies, UMNL e.g hemiparesis, hyperreflexia

8 Supratentorial motor weakness, sensory changes, worsening hand writing, speech disorders, seizures, subtle change in personality, tumors of suprasellar region may present with neuroendocrine deficit

9 Investigations brain MRI Treatment cerebellar astrocytoma: surgery medulloblastoma: surgery and radiotherapy ependymoma : surgery and radiotherapy

10 Nephroblastoma

11 Also known as Wilms tumor embryonal neoplasm of the kidney Most cases of Wilms tumor are sporadic, although 1-2%" of patients have afamily hisrory. Familial predisposition to Wilms tumor is inherited in an autosomal dominant manner One Wilms tumor gene, WT1 located at chromosome 11 20% Familial type gene chrom. 19

12 occurs in children between 2-5 yr of age accounts for 6% of pediatric cancer It may arise in one or both kidneys bilateral Wilms tumor ocurrs in 7% of cases

13 Several syndromes are reported in patients with Wilms tumor e.g - WAGR syndrome - Denys-Drash syndrome - Beckwith-Wiedemann syndrome - Neurofibromatosis(von Recklinghause disease) - Von Willebrand disease - The genirourinary anomalies most commonly associated with Wilms tumor are hypoplasia, duplications of the collecting systems, hypospadias, and cryptorchidism.

14 Two broad categories - favorable form is the more common form and usually carries a good prognosis - unfavorable form there is high rate of tumor relapse and death

15 abdominal mass noticed during dressing or bathing the child These renal masses vary in size,usually are smooth and firm May cross the midline Some patients may present with abdominal pain and vomiting hematuria is seen in12-25% of patients. Hypertension also due to renal ischemia

16 any abdominal mass in a child must be considered malignant till reaching diagnosis CBC Renal function and liver function tests tumor markers secreted by the suspected tumor Abd u/s CT abd confirm of the intrarenal origin of the mass, MRI &CT for mets. biopsy

17

18 Differential diagnosis -Neuroblastoma -Non-Hodgkin lymphoma -Rhabdomyosarcoma -Germ cell/teratoma -Hepatoblastoma -Hepatoma

19 Stage I Wilms tumor is confined to the kidney and, by definition, is excised completely with the capsular surface intact. Stage II Wilms tumor also is confined to the kidney, although the capsule is penetrated or tumor is present in the perirenal soft tissue. Stage III wilms tumor has postsurgical residual nonhematogenous extension. Spread is confined to the abdomen and may involve the perirenal bed, draining lymph nodes, or the surrounding tissue and organs. Stage IV characterized by hematogenous metastases The metastases usually involve the lungs and occasionally the liver Stage V bilateral renal involvemenr

20 Surgical excision Chemotherapy Prognosis -tumor size, stage, and histology -The prognosis is worse in patients with a larger tumor (>500g), advanced stage (III and IV), and unfavorable histologic subtype -more than 60% of patients with all stages generally survive -Stages I through III have a cure rate varying from 88 98%

21 Neuroblastoma

22 cancer of the peripheral sympathetic nervous system the third most common pediatric cancer, accounting for about 8% of childhood malignancies most frequently diagnosed neoplasm in infants, accounting for 28 39% of neonatal malignancies 90% of cases are diagnosed before 5 yr of age More common in boys

23 NB includes a spectrum of tumors with variable degrees of neural differentiation, ranging from undifferentiated small round cells (neuroblastoma) to tumors containing mature ganglion cells (ganglioneuroblastoma or ganglioneuroma) Familial NB is found in 1 2% of cases

24 may develop at any site of sympathetic nervous system tissue signs and symptoms of NB reflect the tumor site and extent of disease Most cases of NB arise in the abdomen, either in the adrenal gland or in retroperitoneal sympathetic ganglia Usually a firm, nodular mass is palpable in the flank or midline that is causing abdominal discomfort

25 NB originates from cervical, thoracic, or pelvic ganglia in 30% of cases Metastatic disease can be associated with fever, irritability, failure to thrive, bone pain, bluish subcutaneous nodules, orbital proptosis, and periorbital ecchymoses The most common sites of metastasis are the long bones and skull, bone marrow, liver, lymph nodes, and skin Lung metastases are rare, occurring in less than 3% of cases

26 Less commonly, children present first with neurologic signs and symptoms include Horner syndrome, opsomyoclonus(dancing eyes and dancing feet)(autoimmune) Some tumors produce catecholamines that can cause increased sweating and hypertension, and some release vasoactive intestinal peptide, causing a secretory diarrhea.

27 Children younger than 1 yr of age can also present with a unique stage, 4S, that often includes subcutaneous tumor nodules, massive liver involvement, and a small primary tumor without bone involvement.

28 Proptosis & ecchymosis

29 plain radiographs, CT, or MRI : mass often contains calcification and hemorrhage Tumor markers including elevated homovanillic acid (HVA) and vanillylmandelic acid (VMA) in urine, are elevated in 95% of cases bone marrow :rosette is characteristic bone scan

30 Thoracic NB

31 Rosette in NB

32 International Neuroblastoma Staging System (INSS) stage 1 includes tumors confined to the organ or structure of origin. Stage 2 tumors extend beyond the structure of origin but not across the midline, with (stage 2B) or without (stage 2A) ipsilateral lymph node involvement. Stage 3 tumors extend beyond the midline, with or without bilateral lymph node involvement. Stage 4 tumors are disseminated to distant sites

33 Stage 4S refers to children younger than 1 yr of age with dissemination to liver, skin, or bone marrow without bone involvement and with a primary tumor that would otherwise be stage 1 or 2.

34 Determined by age at diagnosis, stage, MYCN status(genetic factor) surgery for stages 1 cure >90% surgery for stages 2 ± chemorherapy or radiotherapy cure >90% Stage 3 & 4 surgery + chemorherapy or radiotherapy Stage 4S also has a favorable prognosis, with nearly 100% survival with supportive care only, because the tumor regresses spontaneously - Infants younger than 2 months we need low dose cyclophosphamide

35 Treatment of high-risk NB chemothrapy and resection of the primary tumor followed by focal radiation then stem cell transplantation

36