WILMS TUMOR

WILMS TUMORMost common renal malignancy in children <15yomedian age at diagnosis: 43 months in girls and 37 months in boys

ASSOCIATED WITH- Aniridia- Genitourinary abnormalities- Loss of function mutations of tumor suppressor and transcription genes

CLINICAL PRESENTATIONMost common: Asymptomatic abdominal mass or swelling (typically found by parents while bathing child)- Abdominal pain (30-40%)- Hematuria (12-25%)- Hypertension (25%)

PHYSICAL EXAMCareful!!! Vigorous palpation may rupture the renal capsule, resulting in tumor spillage, increasing the tumor stage and the need for more intensive therapy- firm, non-tender, smooth mass- eccentrically located - rarely crosses the midline

DIAGNOSIS1.      Abdominal ultrasound with evaluation of mass (assess flow)2.      CT/MRI abdomen can be used to further evaluate the lesion3.      Routine CT chest - Lung is the most common metastatic site4.      Definitive: biopsy with histologic confirmation

STAGINGBased on anatomic extent of tumor

MANAGEMENTRefer to a pediatric cancer centerTypically multimodal therapy involving chemotherapy, radiation therapy and surgery    -Surgical excision for respectable tumors    -Chemotherapy for all patients except extremely low risk    -Radiation therapy indicated by stage and histology

Overall five-year survival for Wilms tumor is 90%

DRESS

DRESS – Drug Reaction with Eosinophilia and Systemic Symptoms

THE SHORT VERSION:- Drug-induced hypersensitivity reaction        o Antiepileptics and allopurinol- Life threatening- Look for:        o Rash (morbiliform)        o Diffuse lymphadenopathy        o Diffuse facial edema (ask the pt as this may not be obvious on exam and something they forget to mention)        o Visceral involvement                - Liver (typically mild, can be severe liver failure)                - Kidneys (check Cr and BUN)                - Lungs (hypoxemia, pneumonitis, pleural effusion)- Typically resolves in weeks after offending agent is removed- Treat supportively (if renal/lung involvement consider corticosteroids)

THE LONG VERSION THAT YOU LIKELY WONT REMEMBER AND WILL HAVE TO LOOK UP WHEN YOU SEE A PATIENT WITH DRESS:

- Drug-induced hypersensitivity reaction

- Life threatening

        o 80% drug related

        o Occurs 2-8 weeks after initiation of medication

        o Antiepileptic agents (eg, carbamazepine, lamotrigine, phenytoin, phenobarbital) and allopurinol are the most frequently reported causes

        o 10-20% a drug relationship cannot be established

- Look for:

       1. Rash - starts as a morbilliform rash and rapidly (hours – days) progresses to a diffuse, confluent, and infiltrated erythema with follicular accentuation covering ≥50% of the body

                 a. Associated with ≥2 of the following: facial edema, scaling, purpura

       2. Diffuse lymphadenopathy

       3. Inflammation and pain of mucous membranes without lesions/erosions

       4. Labs

                a. Leukocytosis with eosinophils with >700/microL

                b. Large activated lymphocytes, lymphoblasts, or mononucleosis-like cells

       5. Organ involvement

                a. Liver (60-80%) – typically mild transient asymptomatic hepatitis (^LFTs)

                        i. Severe hepatitis is responsible for the majority of deaths associated with DRESS.

                        ii. Most important predictors of death: markedly elevated aminotransferase, bilirubin levels and jaundice

                b. Kidneys (10-30%) - acute interstitial nephritis (seen with allopurinol)

                c. Lungs (5-25%) – hypoxemia secondary to interstitial pneumonitis and/or pleural effusion. On broncho-alveolar lavage: Drug-specific T-lymphocytes and eosinophils may be found

- Clinical course:

        o Rash and visceral involvement resolve in 6-9 weeks after withdrawal of offending agent

        o 20% of cases symptoms persist for months with remission and relapse

- Diagnosis:

        o Pt who received new medication in the last 2-6 weeks with the following:

                - Morbilliform rash

                - Fever

                - Lymphadenopathy

                - Facial edema

                - Eosinophilia

        o Labs: CBC (eosinophilia), BMP (creatinine/BUN), viral hepatitis panel, dermatology referral for skin biopsy

        o CT chest if pulmonary symptoms

- Management:

        o Drug withdrawal and supportive measures

        o If suspected medication is antiepileptic, substitute with valproate

        o For pts with hepatic involvement refer to hepatologist

        o For pts with severe interstitial nephritis or interstitial pneumonia, consider corticosteroids