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.

Germ Cell Tumor

Introduction:
Germ Cell Tumors are the neoplasm arising from primordial germ cells which produces specialized cells in the body like sperm and egg cells. With the recent advances in cisplatin based chemotherapy, the cure rate of germ cell tumors is increased if diagnosed in early stages. It frequently occurs in three modal peaks of life, infancy, 25-40 yrs and around 60 yrs.

Sites:
Testes
Ovaries
Paratesticular area
Abdomen (retroperitoneum)
Mediastinum
Brain

Types: 
Malignant GCTs: The tumors such as yolk sac tumors, choriocarcinoma, and immature tearatomas encompass this type. The elevated tumor markers, rapid growth signifies malignant transformation.

Benign GCTs: Teratomas are benign tumors. They have characteristic appearance where there are teeth, bone, hair is found inside the tumor.

Symptoms:
Painless scrotal mass
Abdominal mass
Abdominal pain
Breathlessness
Sacral mass
Mediastinal mass

Investigations:
Tumor markers like AFP, β-HCG, LDH
Biopsy
CT Scan/ MRI

Treatment: 
Surgery: The location of the tumor may influence the need for surgery. When possible, the first choice is usually to try and remove the entire tumor. This can be enough to cure most teratomas and immature teratomas. The coccyx needs to be removed in case of sacrococcygeal teratomas.

Chemotherapy: BEP (Bleomycin, Etoposide & Cisplatin) is the first line chemotherapy for the malignant germ cell tumors. This tumors are very much chemosensitive & the fall in tumor marker gives the idea about the response. If the complete resection is not possible initially then neoadjuvant chemotherapy is advisable.

Radiotherapy: It is indicated as a local therapy when the surgery is not possible.

Prognosis:
The 5-year survival rate is about 95% in germ cell tumors.

Phimosis

Phimosis is narrowing of prepucial opening. In infancy, prepucial skin is adherent to the glans.Over the period it gets separated. This is physiological phimosis. Pathological phimosis generally occurs in older children because of repeated infection also called as balanoposthitis.

The child will present with urinary complaints like crying during micturition, redness at glans/meatus, ballooning of prepuceal skin while micturating, repeated urinary tract infection, failure to thrive.

Circumcision is the procedure of choice where the excess prepucial skin is cut under local/general anesthesia.In some religions like muslim, it is done as a ritual custom.

It is day care procedure & within 8-10 days child recovers & wound dries up. The sutures are absorbable like catgut, vicryl rapid etc.Oral antibiotics, analgesics,local ointment & local sitz bath is required  after the procedure. Complications are immediate bleeding, recurrent adhesion (if the excess skin is not cut adequately), excess cutting of the prepucial skin, meatal stenosis etc.

Hodgkin's Disease

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%.

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.