TNM Staging T ((0),1–4): size or direct extent of the primary tumor N (0–3): degree of spread to regional lymph nodes – N0: tumor cells absent from regional lymph nodes – N1: tumor cells spread to closest or small number of regional lymph nodes – N2: tumor cells spread to an extent between N1 and N3. – N3: tumor cells spread to most distant or numerous regional lymph nodes M (0/1): presence of metastasis – M0: no distant metastasis – M1: metastasis to distant organs (beyond regional lymph nodes)
Resectable Tumors Procedure of choice: Radical nephrectomy and lymph node sampling via a transabdominal incision Preoperative biopsy should not be performed Most important role of the surgeon – to ensure complete tumor removal without rupture and perform an assessment of the extent of disease National Wilms Tumor Study (NWTS) Group US National Cancer Institute.
Surgery: 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 spared Partial Controversial; not recommended except for bilateral tumors National Wilms Tumor Study (NWTS) Group US National Cancer Institute.
Routine 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.
Massive, Nonresectable Unilateral Tumors, Bilateral Tumors Candidates for preoperative chemotherapy because of the risk of initial surgical resection. National Wilms Tumor Study (NWTS) Group US National Cancer Institute.
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. Major 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.
“International Neuroblastoma Staging System" (INSS) established in 1986 and revised in 1988 stratifies neuroblastoma according to its anatomical presence at diagnosis: Stage 1Localized tumor confined to the area of origin 2AUnilateral tumor with incomplete gross resection; identifiable ipsilateral and contralateral lymph node negative for tumor 2BUnilateral tumor with complete or incomplete gross resection; with ipsilateral lymph node positive for tumor; identifiable contralateral lymph node negative for tumor 3Tumor 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 involvement 4Dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S 4SAge <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)
International Neuroblastoma Risk Group Staging System (INRGSS) Stage L1Localized disease without image-defined risk factors L2Localized disease with image-defined risk factors MMetastatic disease MSMetastatic disease "special" where MS is equivalent to stage 4S
Shimada Histopathologic Classification System Describes tumors as either favorable or unfavorable histology based on the degree of differentiation, mitosis-karyorrhexis index, schawannian stroma In 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 survival By contrast, older children with advanced stage disease have significantly decreased chance for cure despite intensive therapy
Shimada Histopathologic Classification System Favorable histology group – Patients of any age with stroma-rich tumors without a nodular pattern – Patients 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 neuroblasts – Patients younger than 60 months with stroma- poor tumors, an MKI of less than 100/5000, and well-differentiated tumor cells
Shimada Histopathologic Classification System Unfavorable histology group – Patients of any age with stroma-rich tumors and a nodular pattern – Patients of any age with stroma-poor tumors, undifferentiated or differentiated neuroblasts, and an MKI more than 200/5000 – Patients older than 18 months with stroma-poor tumors, undifferentiated neuroblasts, and an MKI more than 100/5000 – Patients older than 18 months with stroma-poor tumors, differentiated neuroblasts, and an MKI of /5000 – Patients older than 60 months stroma-poor, differentiated neuroblasts, and an MKI less than 100
Neuroblastoma: Therapeutic Regimen
Stage of the cancer Child's age Prognostic markers (hyperdiploid tumor DNA, N-myc, H-ras) Chemotherapy Surgery Radiation therapy Retinod therapy
Low Risk GroupIntermediate Risk Group High Risk Group Children at low risk often require surgery as their only treatment Chemotherapy 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 own Surgery with chemotherapy before or after the procedure, or radiation therapy if chemotherapy is not effective “Second look surgery" More aggressive treatment Combination of chemotherapy, surgery, and radiation High-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.
Recurrent Neuroblastoma For low- and intermediate-risk neuroblastomas that recur in the same area where they started, surgery with or without chemotherapy may be appropriate For 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 therapy intensive treatment with high-dose chemotherapy/radiation therapy, followed by a donor stem cell transplant
Chemotherapy Anti-neoplastic agents cyclophosphamide or ifosfamide cisplatin or carboplatin vincristine doxorubicin (Adriamycin) etoposide teniposide topotecan Side effects hair loss mouth sores loss of appetite nausea and vomiting increased chance of infections (due to low white blood cell counts) easy bruising or bleeding (due to low blood platelet counts) fatigue
Surgery Diagnosis and treatment Complete resection Use of adjuvant therapies
Radiation Therapy Uses high-energy rays or particles to kill cancer cells – External beam radiation therapy – MIBG radiotherapy
External beam radiation therapy uses radiation focused on the cancer from a source outside the body to destroy neuroblastoma cells that remain behind after surgery and chemotherapy to try to shrink tumors before surgery, making them easier to remove at the time of surgery to treat larger tumors that are causing serious problems (such as trouble breathing) and do not respond quickly to chemotherapy as part of the treatment regimen (along with high-dose chemotherapy) before a stem cell transplant in children with high- risk neuroblastoma to help relieve pain caused by advanced neuroblastoma mild skin reactions, nausea, diarrhea, or fatigue
MIBG 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 radiation child will need to stay in a special room for a few days nausea and vomiting, lower blood cell counts
Retinoid 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 transplant 6 month therapy drying and cracking of the lips, dry skin or eyes, nosebleeds and changes in the nails
Hydronephrosis (Grading) Society of Fetal Urology (SFU)Grading System Mild hydronephrosis (Grade I or II) Moderate hydronephrosis (Grade III) Severe hydronephrosis ( Grade IV) Fernbach SK, Maizels M, Conway JJ. Ultrasound grading of hydronephrosis: introduction to the system used by the Society for Fetal Urology. Pediatr Radiol. 1993;23: Grade 0No splitting of the central renal echo complex ISlight splitting of the central renal echo complex IIDilated renal pelvis and some fluid in calyces IIIPelvis dilated beyond sinus, calyces uniformly dilated IVPelvis and calyces dilated, parenchyma thin
Hydronephrosis: Therapeutic Regimen
Treatment (Medical) The role of medical treatment is limited to pain control and treatment or prevention of infection Most 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 fibrosis Vourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
Treatment (Medical) Neonates with hydronephrosis are at high risk for pyelonephrosis Therefore, all neonates diagnosed with unilateral or bilateral hydronephrosis should be started on antibiotic prophylaxis – Example: Amoxicillin 10 mg/kg per 24 h Schwartz’s Manual of Surgery 8 th Ed.
Treatment (Surgical) The specific treatment of a patient with hydronephrosis depends on the etiology of the process Any signs of infection within the obstructed system warrant urgent intervention because infection with hydronephrosis may progress rapidly to sepsis The potential for loss of renal function also adds to the urgency Vourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
Treatment (Surgical) Urethral catheterization to help rule out a lower tract cause for hydronephrosis and hydroureter – Difficulty in placing a Foley catheter may suggest urethral stricture or bladder neck contracture Ureteral stent placement in cases of intrinsic and extrinsic causes of hydronephrosis. – Stents can bypass an obstruction and dilate the ureter for subsequent endoscopic treatment Vourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
Treatment (Surgical) Nephrostomy tube – Using the Seldinger technique, a tube ranging from 8-12F can be placed – Placed when a retrograde stent cannot be passed because of anatomic changes in the bladder or high-grade obstruction in the ureter Vourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
Treatment (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 removal Vourganti, S. (2008). Hydronephrosis and Hydroureter: Treatment & Medication
Sacrococcygeal Teratoma: Staging
Sacroccocygeal 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 stalk – Altman type II — mostly outside – Altman type III — mostly inside – Altman type IV — entirely inside; this is also known as a presacral teratoma or retrorectal teratoma
AAPSS Staging Classification of Sacrococcygeal Teratomas TypeDescription ICompletely external, no presacral component IIExternal component and internal pelvic component IIIExternal component and internal component extending into abdomen IVCompletely internal and no external component Fetology: diagnosis & management of the fetal patient By Diana W. Bianchi, Timothy M. Crombleholme, Mary E. D'Alton
Sacrococcygeal Teratoma: Therapeutic Regimen
Treatment Chemotherapy – Cisplastin – Bleomycin – If serum AFP levels remain elevated, chemotherapy containing cisplastin for four cycles is recommended – Carboplatin – Etoposide Avery's diseases of the newborn By H. William Taeusch, Roberta A. Ballard, Christine A. Gleason, Mary Ellen Avery
Treatment JEB Regimen – Etoposide 120 mg/m 2 i.v. over 1 hour – Carboplatin i.v. over one hour on day 2 in a dose calculated as 600 mg/m 2 – Bleomycin 15 mg/m 2 i.v. over 15 minutes S.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. Oakhill Sacrococcygeal teratomas: the UK Children’s Cancer Study Group’s experience. I. Neonatal
Treatment Surgery – complete surgical excision through a chevron- shaped buttock incision – most tumors can be completely removed using a sacral approach If preoperative imaging demonstrates significant intra-abdominal extension of the tumor – combined abdominal-sacral approach Townsend: Sabiston Textbook of Surgery, 18th ed.
Care must be taken to individually ligate the vessels supplying the tumor, including the middle sacral artery and branches of the hypogastric arteries After the tumor is excised, the levator muscle complex is secured to the presacral fascia, and the remaining wound is closed in layers Townsend: Sabiston Textbook of Surgery, 18th ed. Mature sacrococcygeal teratoma: case report JN Legbo, WEk Opara, and JF Legbo Department of Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria