Children with cancer require long-term treatment. To administer intravenous drugs of chemotherapy, antibiotics or blood products, pricking every time is not a good option.
For this purpose, there are central lines, which are long tubes, placed through a peripheral vein or central vein into heart. These lines generally stay for longer duration & patient can move around with these catheters.
Types: 1. PICC (peripherally inserted central catheter)
2. Central venous tunneled catheter
3. Implantable ports
PICC- These catheters are inserted through a vein in elbow & advanced into heart. Advantages are they require local anaesthesia, easy to insert, not much expertise required, can be done bedside. Disadvantages are in very small child (less than year) peripheral veins are not big so difficult to put, require proper care time to time to avoid breakage & infection by a trained person.
Central venous tunneled catheter- Centrally inserted tunneled catheters are one of the mostly opted options. It is inserted under anaesthesia through a big vein in the neck or collarbone & the catheter is taken out through a tunneled subcutaneous tract. Advantages are it can be inserted in any age group, can be kept for longer duration until the treatment is over. Disadvantages are require anaesthesia, require aseptic care to avoid tract/ catheter related infection & require expertise to insert.
Implantable ports- Implantable ports are one of the best-suited options for child. There is small port along with a tube. The tube is inserted as any other central catheter & the attached port is placed in the subcutaneous space. Advantages are there is less risk of infection, no disturbances in day-to-day activities & require little care. The disadvantages are require anaesthesia, expertise & repeated needle pricks to access port.
MNTI was first described by Krompecker in 1918 as a congenital melanocarcinoma. It was known by many names as its cellular origin was not clear. These names included pigmented ameloblastoma, retinal anlagetumor, melanotic adamantinoma, retinal choristoma,melanotic progonoma, melanotic epithelial odontoma,pigmented teratoma, atypical melanoblastoma,pigmented epulis and retinoblastic teratoma. Someauthors proved that this tumor causes a high urinaryexcretion of vanillylmandelic acid (VMA), suggestinga neural crest origin. Hence, they coined the term 'melanocytic neuroectodermal tumor of infancy'.
About 200 cases of MNTI have been reported until now. It is found to characteristically occur in the maxilla, more so from the intraoral side. Bone destruction and displacement of teeth often occur because of the intraosseous location in the maxilla. Other sites are the skull, mandible and the brain. The lesion is usually solitary and the mucosa over the lesion is usually intact. It has got a typical bluish color due to the presence of melanin. Although it is a benign tumor with a 2% chance of malignancy, it is locally aggressive. The majority of MNTI patients (there is no sexual predilection) present in the 1 year of life. The children present with swelling in the oral cavity, which often hinders feeding. The differential diagnosis is ameloblastoma, odontoma, odontogenic myxoma, fibroma, rhabdomyosarcoma, EwingÌs sarcoma, LangerhansÌ cell histiocytosis (LCH), non-HodgkinÌs lymphoma.
The plain radiograph of MNTI shows a well-circumscribed radiolucent lesion. As the tumor advances, it destroys the bone suggesting a malignant process. In its typical premaxillary position, the tumor can displace or destroy the developing dentition. CT scanning with intravenous contrast is often used to delineate the margins of osseous involvement. Additionally, MRI can be used to evaluate the bony extent of the lesion. Most MNTIsappear as typical soft tissue tumors with nonenhancing heterogeneous tissue density.
Histopathology shows biphasic pattern with the larger pigmented, melanocyte-like cells and smaller, nonpigmented neuroblast-like cells. Immunohistochemistry (IHC) is positive for cytokeratin, synaptophysin, HMB45, NSE, epithelial membrane antigen, glial fibrillary acidic protein and Leu-7.
The treatment of choice in MNTI is usually complete surgical excision. This treatment can usually be accomplished with a partial maxillectomy by using a Weber-Fergusson incision and a facial degloving approach. The adjacent bone and developing teeth must be sacrificed to get an at least 5 mm margin of healthy tissue. The average local recurrence rate is 15-20%. Radiotherapy and combination chemotherapy including vinblastine, ifosphamide, etoposide, cyclophosphamide, doxorubicin and dactinomycin has been advocated for inoperable recurrence or margin-positive resection. A high index of suspicion is necessary to diagnose this tumor and close follow-up is necessary to detect recurrence. Permanent reconstruction can be done after growth is completed.
Introduction: It is rare benign soft tissue tumor arising from embryonal fat. It is usually seen in infants & children. There are two types, the encapsulated/ well circumscribed is called as lipoblastoma while noncapsulate, diffuse & infiltrating type is called as lipoblastomatosis.
Sites of Origin: The most common site is limb but it can occure in retroperitoneum, head & neck, mediastinum, trunk etc.
Clinical presentation:
1. Painless soft tissue tumor
2. Pressure symptoms
Signs:
1. soft tissue mass
2. pseudofluctuation
3. nontender
4. lobulated surface
Histological subtypes:
1. classic type
2. myxoid lipoblastomas
3. lipoma-like lipoblastomas
4. hibernoma-like lipoblastomas
Treatment: Complete surgical excision is the main modality of treatment. There is no adjuvant therapy is required. The chances of local recurrence is high especially in incomplete excision or when the margins are microscopically involved. So long follow-up is necessary.
Prognosis: Excellent.
Introduction:
Rhabdoid tumor is one of the most aggressive tumor in children. Initially it was considered as an aggressive form (Rhabdomyoblastic variant) of Wilms’ tumor as it arises in kidney but later it is separately classified. When the survival rate of Wilms’ tumor exceeds more than 85%, Rhabdoid tumor survival rate is only 20-25% inspite of all therapies. Apart from kidney, it also occurs in brain, liver, soft tissues, lung, skin, and heart. The median age of occurrence is 11 months & majority cases occur below 3 years of age. Here we will describe only Rhabdoid tumor of kidney.
Clinical Presentation:
1. Haematuria
2. Abdominal distension/ mass
3. Fever
4. Symptoms of brain involvement
Enlarged head
Vomitings
Convulsions
Signs:
1. A palpable mass in abdomen
2. High blood pressure
3. signs of raised intracranial pressure (if associated with brain involvement)
Diagnosis:
1. Complete blood count
2. Urine- routine & microscopy
3. Renal function tests
4. Ultrasound of abdomen
5. USG Doppler
6. CT/MRI abdomen
7. CT/MRI brain
8. CT chest
9. Bone scan
10. CT guided FNAC/ Biopsy of mass
Staging:
Stage I
Tumor is limited to the kidney and completely excised. The renal capsule is intact. The tumor is not ruptured or sampled for biopsy before it is removed. (Fine-needle aspiration is excluded from this restriction.) The vessels of the renal sinus are not involved. No evidence suggests tumor at or beyond the margins of resection.
Stage II
The tumor extended beyond the kidney, but it was completely excised. The tumor may regionally extend into the renal sinus or penetrate the renal capsule. Blood vessels outside the renal sinus may contain tumor, but the tumor must be removed en bloc with the tumor. No evidence of tumor at or beyond the margins of resection is present.
Stage III
Residual nonhematogenous tumor is confined to the abdomen. Any of the following may occur: (1) Tumor involves abdominal lymph nodes. (2) The tumor has penetrated the peritoneal surface. (3) Tumor implants are found on the peritoneal surface. (4) Gross or microscopic tumor remains after surgery. (5) The tumor is not completely resectable because of local infiltration of vital structures. (6) Tumoral spillage occurs before or during surgery. (7) Tumor biopsy was performed before resection.
Stage IV
Hematogenous metastases or lymph node metastases are present outside the abdominal and/or pelvic cavity.
Stage V
Tumors are bilateral.
Management:
1. Chemotherapy: Neoadjuvant chemotherapy (not standardized)
ICE (Ifosphamide + Carboplatin + Etoposide) alternating with VAC
(Vincristine + Adriamycin + Cyclophosphamide)
2. Surgery – Radical nephrectomy with retroperitoneal lymph node
Sampling.
3. Radiotherapy- Flank radiation & lung bath in cases of pulmonary
metastasis
Prognosis:
Despite of all the multimodality treatment, the prognosis remains poor.
