Presentation on theme: "Staging and Therapeutic Regimen"— Presentation transcript:
1Staging and Therapeutic Regimen Wilm’s TumorNeuroblastomaHydronephrosisSacrococcygeal teratoma
2TNM Staging T ((0),1–4): size or direct extent of the primary tumor N (0–3): degree of spread to regional lymph nodesN0: tumor cells absent from regional lymph nodesN1: tumor cells spread to closest or small number of regional lymph nodesN2: tumor cells spread to an extent between N1 and N3.N3: tumor cells spread to most distant or numerous regional lymph nodesM (0/1): presence of metastasisM0: no distant metastasisM1: metastasis to distant organs (beyond regional lymph nodes)Just in case i-ask yung general staging method =)
9Resectable TumorsProcedure of choice: Radical nephrectomy and lymph node sampling via a transabdominal incisionPreoperative biopsy should not be performedMost important role of the surgeonto ensure complete tumor removal without rupture and perform an assessment of the extent of diseaseNational Wilms Tumor Study (NWTS) GroupUS National Cancer Institute.
10Surgery: Nephrectomy Radical Removal of the kidney, the ipsilateral adrenal gland, and all the fat contained within Gerota's fascia.If there is no evidence of adrenal involvement by the tumor on the CT scan, the adrenal gland can be sparedPartialControversial; not recommended except for bilateral tumorsNational Wilms Tumor Study (NWTS) GroupUS National Cancer Institute.
11Routine exploration of the contralateral kidney is not necessary if technically adequate imaging studies do not suggest a bilateral process. If the initial imaging studies are suggestive of regional and contralateral kidney involvement, the contralateral kidney should be formally explored to rule out bilateral involvement. This should be done prior to nephrectomy since the diagnosis of bilateral disease would dramatically alter the approach.
12Massive, Nonresectable Unilateral Tumors, Bilateral Tumors Candidates for preoperative chemotherapy because of the risk of initial surgical resection.National Wilms Tumor Study (NWTS) GroupUS National Cancer Institute.
13Major Treatment Conclusions of the National Wilms Tumor Studies (NWTS 1—5) Routine, postoperative radiation therapy of the flank is not necessary for children with stage I tumors or stage II tumors with favorable histology (FH) when postnephrectomy combination chemotherapy consisting of vincristine and dactinomycin is administered.The prognosis for patients with stage III FH is best when treatment includes: (a) dactinomycin, vincristine, doxorubicin, and 10.8 Gy of radiation therapy to the flank; or (b) dactinomycin, vincristine, and 20 Gy of radiation therapy to the flank.The addition of cyclophosphamide to the combination of vincristine, dactinomycin, and doxorubicin does not improve prognosis for patients with stage IV FH tumors.Just in case slide
14Major Treatment Conclusions of the National Wilms Tumor Studies (NWTS 1—5) A single-dose of dactinomycin per course (stages I–II FH, stage I anaplastic, stage III FH, stages III–IV, or stages I–IV clear cell sarcoma of the kidney) is equivalent to the divided-dose courses, and results in the same event-free survival, greater dose intensity, and is associated with less toxicity and expense.Eighteen weeks of therapy is adequate for patients with stage I FH whereas other patients can be treated with 6 months of therapy instead of 15 months.Tumor-specific loss of heterozygosity for combined 1p and 16q predicts recurrence of FH Wilms tumor and may be used to select patients for more aggressive treatment.Just in case slide
16“International Neuroblastoma Staging System" (INSS) established in 1986 and revised in 1988 stratifies neuroblastoma according to its anatomical presence at diagnosis:Stage1Localized tumor confined to the area of origin2AUnilateral tumor with incomplete gross resection; identifiable ipsilateral and contralateral lymph node negative for tumor2BUnilateral tumor with complete or incomplete gross resection; with ipsilateral lymph node positive for tumor; identifiable contralateral lymph node negative for tumor3Tumor infiltrating across midline with or without regional lymph node involvement; or unilateral tumor with contralateral lymph node involvement; or midline tumor with bilateral lymph node involvement4Dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S4SAge <1 year old with localized primary tumor as defined in Stage 1 or 2, with dissemination limited to liver, skin, or bone marrow (less than 10 percent of nucleated bone marrow cells are tumors)
17International Neuroblastoma Risk Group Staging System (INRGSS) StageL1Localized disease without image-defined risk factorsL2Localized disease with image-defined risk factorsMMetastatic diseaseMSMetastatic disease "special" where MS is equivalent to stage 4S
18Shimada Histopathologic Classification System Describes tumors as either favorable or unfavorable histology based on the degree of differentiation, mitosis-karyorrhexis index, schawannian stromaIn general, children of any age with localized neuroblastoma and infants <1yr with advanced disease and favorable disease characteristics have a high likelihood of disease free survivalBy contrast, older children with advanced stage disease have significantly decreased chance for cure despite intensive therapy
19Shimada Histopathologic Classification System Favorable histology groupPatients of any age with stroma-rich tumors without a nodular patternPatients younger than 18 months with stroma-poor tumors, an MKI of less than 200/5000 (200 karyorrhectic cells per 5000 cells scanned), and differentiated or undifferentiated neuroblastsPatients younger than 60 months with stroma-poor tumors, an MKI of less than 100/5000, and well-differentiated tumor cells
20Shimada Histopathologic Classification System Unfavorable histology groupPatients of any age with stroma-rich tumors and a nodular patternPatients of any age with stroma-poor tumors, undifferentiated or differentiated neuroblasts, and an MKI more than 200/5000Patients older than 18 months with stroma-poor tumors, undifferentiated neuroblasts, and an MKI more than 100/5000Patients older than 18 months with stroma-poor tumors, differentiated neuroblasts, and an MKI of /5000Patients older than 60 months stroma-poor, differentiated neuroblasts, and an MKI less than 100
22Stage of the cancerChild's agePrognostic markers (hyperdiploid tumor DNA, N-myc, H-ras)ChemotherapySurgeryRadiation therapyRetinod therapy
23Intermediate Risk Group Low Risk GroupIntermediate Risk GroupHigh Risk GroupChildren at low risk often require surgery as their only treatmentChemotherapy is typically given after surgery if less than half the tumor can be removed (carboplatin, cyclophosphamide, doxorubicinetoposide)Infants with 4S disease and no symptoms can often be watched carefully with no treatment, because these cancers often mature or go away on their ownSurgery with chemotherapy before or after the procedure, or radiation therapy if chemotherapy is not effective“Second look surgery"More aggressive treatmentCombination of chemotherapy, surgery, and radiationHigh-dose chemotherapy followed by a stem cell transplant Retinoid drug 13- cis-retinoic acid (isotretinoin) is often given for 6 months after other treatments are completed.
24Recurrent Neuroblastoma For low- and intermediate-risk neuroblastomas that recur in the same area where they started, surgery with or without chemotherapy may be appropriateFor higher-risk cancers or those that recur in distant parts of the body, treatment is usually more intense, and may include a combination of chemotherapy, surgery, and radiation therapyintensive treatment with high-dose chemotherapy/radiation therapy, followed by a donor stem cell transplant
25Chemotherapy Anti-neoplastic agents Side effects cyclophosphamide or ifosfamidecisplatin or carboplatinvincristinedoxorubicin (Adriamycin)etoposideteniposidetopotecanhair lossmouth soresloss of appetitenausea and vomitingincreased chance of infections (due to low white blood cell counts)easy bruising or bleeding (due to low blood platelet counts)fatigue
26Surgery Diagnosis and treatment Complete resection Use of adjuvant therapies
27Radiation TherapyUses high-energy rays or particles to kill cancer cellsExternal beam radiation therapyMIBG radiotherapy
28External beam radiation therapy uses radiation focused on the cancer from a source outside the bodyto destroy neuroblastoma cells that remain behind after surgery and chemotherapyto try to shrink tumors before surgery, making them easier to remove at the time of surgeryto treat larger tumors that are causing serious problems (such as trouble breathing) and do not respond quickly to chemotherapyas part of the treatment regimen (along with high-dose chemotherapy) before a stem cell transplant in children with high-risk neuroblastomato help relieve pain caused by advanced neuroblastomamild skin reactions, nausea, diarrhea, or fatigue
29MIBG radiotherapy chemical similar to norepinephrine Once injected into the bloodstream, the MIBG goes to the sites of tumors anywhere in the body, where it delivers its radiationchild will need to stay in a special room for a few daysnausea and vomiting, lower blood cell counts
30Retinoid Therapy chemically related to vitamin A “differentiating agents”13-cis-retinoic acid (isotretinoin) reduces the risk of recurrence after high-dose chemotherapy and stem cell transplant6 month therapydrying and cracking of the lips, dry skin or eyes, nosebleeds and changes in the nails
32Hydronephrosis (Grading) Society of Fetal Urology (SFU)Grading SystemMild hydronephrosis (Grade I or II)Moderate hydronephrosis (Grade III)Severe hydronephrosis ( Grade IV)GradeNo splitting of the central renal echo complexISlight splitting of the central renal echo complexIIDilated renal pelvis and some fluid in calycesIIIPelvis dilated beyond sinus, calyces uniformly dilatedIVPelvis and calyces dilated, parenchyma thinWala daw staging accdg to dinoFernbach SK, Maizels M, Conway JJ. Ultrasound grading of hydronephrosis: introduction to the system used by the Society for Fetal Urology. Pediatr Radiol. 1993;23:478-80
34Treatment (Medical)The role of medical treatment is limited to pain control and treatment or prevention of infectionMost conditions require either minimally invasive or open surgical treatment.Two notable exceptions are(1) oral alkalinization therapy for uric acid stones and(2) steroid therapy for retroperitoneal fibrosisVourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
35Treatment (Medical)Neonates with hydronephrosis are at high risk for pyelonephrosisTherefore, all neonates diagnosed with unilateral or bilateral hydronephrosis should be started on antibiotic prophylaxisExample: Amoxicillin 10 mg/kg per 24 hSchwartz’s Manual of Surgery 8th Ed.
36Treatment (Surgical)The specific treatment of a patient with hydronephrosis depends on the etiology of the processAny signs of infection within the obstructed system warrant urgent intervention because infection with hydronephrosis may progress rapidly to sepsisThe potential for loss of renal function also adds to the urgencyVourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
37Treatment (Surgical)Urethral catheterization to help rule out a lower tract cause for hydronephrosis and hydroureterDifficulty in placing a Foley catheter may suggest urethral stricture or bladder neck contractureUreteral stent placement in cases of intrinsic and extrinsic causes of hydronephrosis.Stents can bypass an obstruction and dilate the ureter for subsequent endoscopic treatmentVourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
38Treatment (Surgical) Nephrostomy tube Using the Seldinger technique, a tube ranging from 8-12F can be placedPlaced when a retrograde stent cannot be passed because of anatomic changes in the bladder or high-grade obstruction in the ureterSeldinger technique:Vourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
39Treatment (Surgical)Advances in endoscopic and percutaneous instrumentation have decreased the role of open or laparoscopic surgery for hydronephrosis.However, extrinsic causes of hydropnephrosis (retroperitoneal fibrosis, retroperitoneal tumors, and aortic aneurysms) still require treatment with open surgery.Some stones that cannot be treated endoscopically or with extracorporeal shockwave lithotripsy require open removalVourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
41Sacroccocygeal Teratoma SCTs are classified morphologically according to their relative extent outside and inside the body:Altman type I — entirely outside, sometimes attached to the body only by a narrow stalkAltman type II — mostly outsideAltman type III — mostly insideAltman type IV — entirely inside; this is also known as a presacral teratoma or retrorectal teratoma
42AAPSS Staging Classification of Sacrococcygeal Teratomas TypeDescriptionICompletely external, no presacral componentIIExternal component and internal pelvic componentIIIExternal component and internal component extending into abdomenIVCompletely internal and no external componentFetology: diagnosis & management of the fetal patient By Diana W. Bianchi, Timothy M. Crombleholme, Mary E. D'Alton
44Treatment Chemotherapy Cisplastin Bleomycin If serum AFP levels remain elevated, chemotherapy containing cisplastin for four cycles is recommendedCarboplatinEtoposideAvery's diseases of the newborn By H. William Taeusch, Roberta A. Ballard, Christine A. Gleason, Mary Ellen Avery
45Treatment JEB Regimen Etoposide 120 mg/m2i.v. over 1 hour Carboplatin i.v. over one hour on day 2 in a dose calculated as 600 mg/m2Bleomycin 15 mg/m2 i.v. over 15 minutesS.N. Huddart Æ J.R. Mann Æ K. Robinson Æ F. Raafat J. Imeson Æ P. Gornall Æ M. Sokal Æ E. GrayP. McKeever Æ A. OakhillSacrococcygeal teratomas: the UK Children’s Cancer Study Group’s experience. I. Neonatal
46TreatmentSurgerycomplete surgical excision through a chevron-shaped buttock incisionmost tumors can be completely removed using a sacral approachIf preoperative imaging demonstrates significant intra-abdominal extension of the tumorcombined abdominal-sacral approachTownsend: Sabiston Textbook of Surgery, 18th ed.
47Care must be taken to individually ligate the vessels supplying the tumor, including the middle sacral artery and branches of the hypogastric arteriesAfter the tumor is excised, the levator muscle complex is secured to the presacral fascia, and the remaining wound is closed in layersTownsend: Sabiston Textbook of Surgery, 18th ed.Mature sacrococcygeal teratoma: case reportJN Legbo, WEk Opara, and JF Legbo Department of Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria