Pigmented Neuroepithelial Tumor

Introduction: Pigmented neuroepithelial tumor is a rare tumor. About 200 cases has been reported till now. It is characteristically occur in maxilla & has a typical bluish color due to the presence of melanin. It is benign tumor with 2% chances of malignancy. It is locally aggressive. Krompecker described it first in 1918 Many names given as the cell of origin was not clear ( Pigmented ameloblastoma, Retinal anlage tumor, Melanotic adamantinoma, Retinal choristoma)

Presentation: Majority present in1st year of life. Median age of occurrence is 4.3 months. There is no sexual predilection.

• Swelling in the region of oral cavity

• Often feeding & sucking impaired

Sites: More than 90%- head & neck region. Common site- anterior part of maxilla. Other sites are skull, mandible, & brain. The lesion is usually solitary & not multiple. Mucosa over the lesion is usually normal.

Investigations: There are no characteristic radiological findings so pathology remains the cornerstone of diagnosis.

Plain radiograph & CT/MRI: Well-circumscribed, low-density lesion without calcification typically arising from maxilla. As the tumor grows, bone is destroyed suggesting a malignant process.

Treatment: Treatment of choice is complete surgical excision. Developing teeth & surrounding tissues may need to be sacrificed to attain an adequate surgical margin.

Recurrence rate is 10-60%.Radiotherapy & Chemotherapy are used for inoperable recurrence or margin positive resection. Close follow up is necessary to detect recurrence

Permanent reconstruction can be done after growth is completed

Acute Leukemias In Children

Introduction: This is the commonest cancer in children. Types of leukemia seen in pediatric age group are
1. Acute lymphoblastic leukemia
2. Acute non-lymphoblastic leukemia
3. Chronic myeloid leukemia

Acute lymphoblastic leukemia:
Incidence: ALL is the most common type of leukemia in children.The incidence of ALL is about 30 cases per million people per year. The peak age of developing ALL is 2-6 years.

Etiology:
1. Genetic factor- It is seen in identical twins, Fanconi's anamia, Down's & Klinefelter's syndrome,
2. Immunologic factor- Ataxia telangiectasia
3. Environmental factor-Exposure to radiation, chemotherapeutic agents like alkalyting agents.
4. Viral infection- Epstein-Barr virus

Clinical features:
1. Anemia (fatigue, irritability, pallor)
2. Thrombocytopenia (bleeding, petechiae)
3. Neutropenia (fever)
4. Lymphadenopathy
5. Hepatospenomegaly
6. Joint pains
7. Headache, vomitings (Brain involvement)

Diagnosis:
1, Complete blood count-Eleveted leucocyte counts
2. Serum levels of uric acid, potassium, phosphorus, and calcium, and lactate dehydrogenase
3. Bone marrow aspiration & biopsy: The presence of 30% or more blasts in marrow is s/o acute leukemia
4. Lumbar puncture
5. Cytological & Molecular diagnosis
6. Ultrasonography: testis, kidneys
7. Chest X-ray

Chemotherapy: is the mainstay of treatment
Induction, CNS prophylaxis, consolidation & Maintenance therapy

Surgical role: Central line insertion for chemotherapy

Thyroid cancer in children

Introduction: Thyroid carcinomas in children represents 1-1.5% of all tumors before the age of 15 years. The incidence of thyroid carcinoma is 2-3 times more in girls than boys & commonly occurs between 7-12 years of age. Radiation to the neck in the childhood is established causative factor in development of thyroid cancer.

Clinical presentation:
1. anterior cervical lymphadenopathy
2. asymptomatic neck mass
3. vocal card palsy (rare)
4. breathlessness or dysphagia because of compression on trachea/ esophagus (rare)
5. Family h/o thyroid cancer (Especially in Medullary cancer)

Main Types:
1. Papillary (most common in children)
2.Follicular
3. Medullary
4. Anaplastic

Physical Examination:
1. Firm painless thyroid nodule in one or both lobes
2. may be associated with lymphadenpathy
3. signs of compression or immobility due to fixation is rare but can be present
4. hoarseness of voice due to vocal cord palsy is again rare in children

Diagnosis:
1. T3, T4, TSH ( Euthyroid)
2. Antithyroid antibodies (to rule out thyroidities)
3. Thyroglobulin levels (for postoperative monitoring)
4. Calcitonin (medullary carcinoma)
5. Ultrasonography of neck
6. Thyroid scan- cold nodules
7. needle biopsy or excision biopsy
8. Chest x-ray/ CT Chest to rule out metastasis

Treatment:
1. Surgery- is the mainstay of treatment
Total thyroidectomy
2. Radioiodine- For the cancer spread outside the thyroid gland
3. Chemotherapy containing low dose Doxorubicin & external irradiation are reserved for anaplastic carcinoma or recurrence of differentiated carcinomas.

Prognosis: Prognosis of differentiated carcinomas is very good & more than 90% survival has been reported.

Sacrococcygeal Teratoma

Sacrococcygeal teratoma is the tumor arising in sacrococcygeal region & it is the commonest tumor found in newborns. It is also seen in infants, children & very rarely in adults. The SCT is more common in girls than boys with ratio of 3:1. The routine use of prenatal ultrasound has made the diagnosis early during fetal life.

Symptoms:
1. Sacral mass
2. Mass in the abdomen & perineum
3. Distension of abdomen
4. Displacement of anus due to sacral mass
5. Constipation
6. Sacral sinus.

Classification: Altaman's classification
Type 1- Entirely outside
Type 2- Mostly outside
Type 3-Mostly inside
Type 4- Entirely inside

Diagnosis:
1. Prenatal Ultrasound- Solid/ cystic mass occupying abdomen as well as perineum
2. CT Scan abdomino-pelvic region/ MRI abdomino-pelvic region
3. Tumor markers- AFP or Alfafetoproteins

Treatment:
1. Surgical excision in benign or mature teratoma
2. Associated with chemotherapy in malignant or immature teratoma

Chemotherapy:
Bleomycin, Etoposide & Cisplatin (BEP) protocol is the commonest first line protocol used.

Prognosis- Good if complete surgical excision is done along with removal of coccyx.

Infantile Fibromatosis

Infantile fibromatosis, originally described by Stout in 1954, seems to be genetic in origin although the mode of transmission is not clear. It refers to a family of soft tissue lesions characterized by proliferation of benign fibrous tissue, which is composed of uniform, elongated, fusiform, or spindle shaped cells surrounded and separated by abundant collagen. According to Enzinger and Weiss, they are classified into superficial and deep fibromatosis. Superficial tumors are usually purplish red as a result of intense vascularity. The intraabdominal organ involvement is also known which carries worse prognosis.

Fibromatosis is a group of related conditions having following common features; proliferation of well differentiated fibroblasts, infiltrative pattern of growth, presence of variable amount of collagen between the proliferating cells, lack of cytological features of malignancy and scanty or absent mitotic activity, aggressive clinical behavior characterized by repeated local recurrences but lack of capacity to metastasize distantly.

In infantile fibromatosis, the Characteristic feature is the presence of small, round intracytoplasmic inclusions. They are periodic acid- Schiff (PAS) negative and they apparently consist of actin filaments⁷. Morphologically, these lesions occur as unicentric gray white, firm poorly demarcated masses varying from 1 to 15 cm in greatest dimension.

Although histologically benign, this lesion tends to gradually infiltrate in skin subcutaneous tissue, muscles, nerves, blood vessels and even bone. They are rubbery, & tough. Clinically the presentation is of a slow-growing, firm, poorly circumscribed mass. Diagnosis is usually made histologically. The lack of malignant cells on histology differentiates infantile fibromatosis from infantile fibrosarcoma. It usually occurs in the anterior abdominal wall, buttocks, shoulder, upper arm and the head & neck. The scrotum & external genitalia are rare sites.

The progression is unpredictable. Wide local excision can provide cure if excision is complete with an adequate resection margin but this may be difficult due to infiltrative nature. Local recurrence occur usually within 18 months of original surgery. Wide local excision is the treatment of choice. Imaging is required for surgical planning. Radiographic examination reveals a soft tissue mass, usually noncalcified. CT appearances are nonspecific; MRI is the best modality for diagnosis & follow-up, specifically in deep fibromatosis.

The primary goal of treatment for fibromatosis is complete surgical resection to achieve negative margins. The management of children with unresectable or recurrent fibromatosis requires a multidisciplinary approach. Nonaggressive therapy with tamoxifen and diclofenac may be the first treatment choice in these patients, but in patients with progressive disease, cytotoxic chemotherapy is indicated. Weekly administration of vinblastine and methotrexate seems to be safe and effective in these children.