Wims' tumor

Introduction:
There are variety of tumors arising from kidney in children but the most common is Wilms' tumor named after Karl Max Wilms who is the first person to review the literature & described the unique properties/ So it is named after him. It is also called as nephroblastoma. It occurs in children upto 6 years with peak at 3 years.It is extremely uncommon after 6 yrs.

Syptoms:
1. mass or Lump or distension of abdomen ( most common)
2. abdominal pain.
3. hematuria (blood in the urine or smoky urine)
4. fever
5. refusal of feeds
6. lethargy.
7. weight loss

Investigations:
The following investigations are proposed for whole work up when one suspect the Wilms tumor.
1. CT scan of abdomen & CT guided FNAC of tumor
2. X-ray chest
3. Comlete Blood Counts
4. Biochem profile

Treatment:
Treatment for wilms' tumor is multidisciplinary
It includes surgery, chemotherapy & radiotherapy

Surgery- includes radical nephrectomy with lymph node sampling

Chemotherapy- combination of drugs like Vincristine / Actinomycin- D/ Cyclophosphamide.

Radiotherapy- is given to the flank in advanced disease or to the chest if there are metastasis.

Prognosis:This is one of the most curable cancer. the cure rate is more than 80% with multimodality treatment.

Other tumors of kidney in childhood
1. Clear cell sarcoma
2. Rhabdoid tumor
3. Renal cell carcinoma
4. Primitive neuroectodermal tumor
5. Infantile osteoid producing kidney tumor.

Hodgkin’s disease in children

Introduction:

Hodgkin’s disease is a cancer of the lymphatic system. It is also called as Hodgkin’s lymphoma. Hodgkin’s disease accounts for 5% of cancers diagnosed in children. It is rare before the age of five years. The number of cases increases significantly in the second decade of life.

Symptoms:

1. enlarged lymph nodes, called lymphadenopathy
(Painless, firm, rubbery, and movable)
2. loss of appetite
3. loss of weight
4. fever
5. lethargy
6. lump in abdomen
7. pain in abdomen
8. itching
9. night sweat
10. cough or breathlessness.

Investigations:

1. X-ray Chest
2. Ultrasonography / CT scan of abdomen
3. lymph node biopsy
4. Complete Blood Count (specially ESR)
5. Biochemistry ( specific- LDH, β2 macroglobulin, serum albumin)
6. Bone marrow / Bone scan in advanced stage
7. PET scan

Treatment:

Hodgkin’s disease is treated with chemotherapy & radiotherapy. The surgery is done only for the diagnosis.

Chemotherapy:

The following drugs are used
1. Adriamycin
2. Bleomycin
3. Vinblastine
4. Dacarbazine

Radiotherapy:

Radiotherapy is used after 4 or 6 cycles of chemotherapy.

Prognosis: Excellent. 5 year survival rate is >85%.

Liver cancers in children

Introduction:

The liver is a large, very important organ situated in the right upper quadrant of abdomen. The normal functions of the liver in the body are:
To help store nutrients from food
To break down and remove harmful chemicals from the body
To build chemicals that the body needs to stay healthy

Hepatoblastoma: The most common type of liver cancer in children is Hepatoblastoma. it occurs most frequently in infants or very young children between 2 months and 2 year.

Symptoms:

• Lump in the abdomen (most common)



• Reduced appetite



• lethargy



• nausia / vomiting



• Pain in abdomen



• Jaundice

Investigations:

The following minima investigations are proposed to diagnose hepatoblastoma

• Tumor markers- serum AFP



• CT scan of abdomen with Ct guided FNAC or Biopsy



• X-ray chest



• Complete blood count



• Biochem profile

Treatment:

Surgery & chemotherapy are two tratment options

Surgery: Liver resection according to the location & extent of the tumor. It will be either

• Rt hepatectomy



• Lt hepatectomy



• Extended Rt or Lt hepatectomy



• Bi or trisegmentectomy

If liver resection is not possible because of multicentric disease then liver transplant is the only treatment of option.

Chemotherapy:
Chemotherapy is given before or after surgery. Surgery is done either as a sandwitch surgery (in between cycles of chemotherapy) or it is done at the start & then chemotherapy is given. Antitumor drugs dispersed in a lipid lymphographic medium are intraarterially administered unresectable hepatoblastoma.The more information about intra-arterial chemotherapy can be read in Rare Adult & Pediatric Cancer Web Blog

The drugs used are

• Cisplatin



• Doxorubicin



• Vincristine



• 5-Fluorouracil

Other less common malignant liver tumor in children:

Hepatocellular carcinoma.