common extracranial pediatric tumours

1. Pediatric Solid Tumours
Dr. Survesh Kumar Gupta
MBBS, MS(General Surgery), M. Ch.(Pediatric Surgery)
Assistant Professor,
Department of Pediatric Surgery, UPUMS, Saifai.

2. Introduction
Solid tumors make up about 30% of all cancers in children.
Major types of solid tumors
1.Sarcomas are tumors in a blood vessel, bone, fat tissue,
ligament, lymph vessel, muscle or tendon.
1. Ewing sarcoma and osteosarcoma, which are bone cancer
sarcomas.
2. Rhabdomyosarcoma, which is a soft tissue sarcoma found in
muscles.
2.Carcinomas are tumors that form in epithelial cells.
Epithelial cells are found in the skin, glands and the linings of
organs. Those organs includes the bladder, ureters and part
of the kidneys.
1. One common carcinoma is adrenocortical carcinoma. This is when
a tumor develops in one or both adrenal glands, located above
each kidney.

3. Nephroblastoma (Wilms’ Tumour)
• Malignant embryonal tumor of renal tissue.
• First described in 1899 by max wilms.

4. Epidemiology

5. Etiology
• Wilms tumor has been associated with loss of function mutations of
a number of tumor suppressor and transcription genes.
• These include mutations of the WT1, p53, FWT1, and FWT2 genes,
and at the 11p15.5 locus.
• Associated with some congenital syndromes. These syndromes
include:
–
–
–
WAGR syndrome.
Denys-Drash syndrome.
Beckwith-Wiedemann syndrome.

6. Types
• Histologically, the classic favorable histology Wilms
tumor is comprised of three cell types:

7. Symptoms & Signs

8. Staging

9. Diagnosis

10. Diagnosis
THE DEFINITIVE DIAGNOSIS OF WILMS
TUMOR IS MADE BY HISTOLOGIC
CONFIRMATION AT THE TIME OF EITHER
SURGICAL EXCISION OR BIOPSY.

11. Treatment
• Treatment plan is assigned based on results of the initial
staging, histological, and molecular studies.
• In General , lines of treatment include:
Surgery Chemotherapy Radiotherapy

12. Prognosis

13. Neuroblastoma
• Malignant embyronal tumor of precursor cells of sympathetic
ganglia and adrenal medulla.
• Commonly used to refer to a spectrum of neuroblastic tumors
(including neuroblastomas, ganglioneuroblastomas, and
ganglioneuromas)

14. Epidemiology

15. Etiology
• Etiology is unknown.
➢ Risk factors:
• Maternal factors: These include the following:
– Opiate consumption
– Folate deficiency
– Toxic exposures
– Congenital abnormalities
– Gestational diabetes mellitus
• Genetic factors:
– A higher incidence of neuroblastoma has been suggested in girls with Turner
syndrome, Hirschsprung's disease, central hypoventilation, and neurofibromatosis type 1
(NF1)

16. Types
• Neuroblastomas can arise anywhere throughout the
sympathetic nervous system.
• The common primary sites:
Adrenal gland
(40%)
Abdominal
(25%)
Thoracic
(15%)

17. Types

18. Symptoms & Signs

19. Staging

20. Diagnosis

21. Diagnosis
• Diagnostic criteria — Minimum criteria for establishing a diagnosis
of neuroblastoma have been agreed upon by an international
consensus panel. A definitive diagnosis of neuroblastoma requires
one of the following:
– An unequivocal histologic diagnosis from tumor tissue by light microscopy, with
or without immunohistochemistry, electron microscopy, or increased urine (or
serum) catecholamines or their metabolites.
– Evidence of metastases to bone marrow on an aspirate or trephine
biopsy with concomitant elevation of urinary or serum catecholamines or their
metabolites.

22. Treatment
• Treatment plan is assigned based on:
– Stage of the disease.
– Patient age.
– Histologic appearance of the tumor.
– Presence or absence of amplification of the MYCN oncogene.
– Quantitative DNA content of the tumor (DNA index or ploidy).

23. Treatment
•In General , lines of treatment include:
Surgery Chemotherapy Radiotherapy

24. Prognosis

25. Hepatoblastoma
• Hepatoblastoma is the most common primary
hepatic malignancy in early childhood.
• The majority of hepatoblastomas occur in the
first two years of life and rarely in children older
than five years.

26. Epidemiology
• One percent of all pediatric neoplasias
• The incidence of hepatoblastoma in boys is
twice that in girls.

27. Etiology
•
•
Exact etiology is unknown.
Syndromes with an increased incidence of
hepatoblastoma include:
– Beckwith Wiedmann syndrome.
– Trisomy 18 & 21.
– Acardia syndrome.
– Li-Fraumeni syndrome.
– Goldenhar syndrome (a type of craniofacial microsomia).
– Type 1a glycogen storage disease (von Gierke’s disease).
– Familial adenomatous polyposis (FAP).

28. Types

29. Symptoms & Signs

30. Staging
Stage
Stage I
Stage IIA
Stage IIB
Stage III (any of the following)
Stage IV
Characteristics
• The tumor is completely resectable via
wedge resection or lobectomy.
• The tumor has PFH results.
• The AFP level is within reference range
within 4 weeks of surgery.
• The tumor is completely resectable.
• The tumor has histologic results other than
PFH (UH).
• The tumor is completely resectable.
• AFP findings are negative at time of diagnosis
(ie, no marker to follow).
• The tumor is initially unresectable but is
confined to one lobe of liver.
• Gross residual disease is present after
surgery.
• Tumor ruptures or spills preoperatively or
intraoperatively.
• Regional lymph nodes are involved.
Distant bone or lung metastasis is present.

31. Diagnosis

32. Treatment
• Treatment plan is assigned based on results of the initial
staging, histological, and molecular studies.
• In General , lines of treatment include:
Surgery Chemotherapy Radiotherapy

33. Prognosis

34. Germ cell tumour
•Germ cell tumors (GCT) can be gonadal or extragonadal
in origin.
•These lesions are presumed to originate from the
primordial germcell.
•Overall incidence of GCT is about 1 in 100,000 children
less than 15 years of age

35. • Extragonadal site predominates(60%) compared
with gonadal locations . Ovarian germ tumors
(30%), and testicular germ cell tumors(10%)
•Sacrococcygeal site is the commonest, the other
sites being the head and neck, brain, mediastinum,
and retroperitoneum.
•80% benign,20% malignant.
•The most common benign germ cell tumors are
teratomas and malignant histology is yolk sac
tumor, which has alpha-fetoprotein (AFP) as a
sensitive marker.

36. Clinical Presentation
• Ovarian tumors – Painless lower abdominal mass. 10 %
with torsion.
• Testicular tumors – Usually painless mass in the scrotum.
Occasionally with pain.
• Sacrococcygeal teratoma (SCT) – Most common neoplasm of
the fetus and newborn with an incidence of 1 in 20,000–
40,000 live births and has a female predominance. Most
SCTs are histologically benign; however, approximately one-
fifth exhibit malignant features. Antenatal diagnosis is also
freguent. SCT in newborns is predominantly benign.
• Older children and intrapelvic presentation are associated
with a higher rate of malignancy.

37. Altman Classification
Based on its local extent.
•Type I lesion has only external components.
•Type II lesion has primarily external components but
also has pelvic extension.
•Type III lesion has little external component and has
significant abdominopelvic extension.
•Type IV lesion does not have an external component.
The risk of malignancy is highest in type IV lesions,
probably because they present late.

38. Investigations
• CECT - evaluation of local extent and metastatic workup.
• Infants of less than 2 months of age with sacrococcygeal
teratoma, metastatic workup avoided.
• Testicular tumor - ultrasonography of the scrotum is sufficient.
• Plain radiograph. - Teratomas often show calcification
• Biochemical markers: Serum (AFP) and (βhCG) - malignant
germ cell tumors markers. Choriocarcinoma, although less
common, has human chorionic gonadotropin (hCG) as an
easily identifiable serum marker.

39. Staging
•Stage I – Complete resection at any site,
coccygectomy for sacrococcygeal site, negative
tumor margins
•Stage II – Microscopic residual, lymph nodes
negative
•Stage III – Lymph node involvement with metastatic
disease. Gross residual tumor or biopsy only;
retroperitoneal nodes negative or positive
•Stage IV – Distant metastases, including the liver
and lung.

40. Treatment
•Excision of the tumor is the primary treatment.
•Extremely chemo-sensitive tumors and respond to
PEB (cisplatin, etoposide, and bleomycin).
•In the case of immature teratoma grade III, some
advocate additional chemotherapy due to the high
risk of recurrence and malignant transformation.

41. Surgery
• Sacrococcygeal teratoma. Coccygectomy should be performed along
with excision of the sacrococcygeal teratoma.
• Testicular tumor - High inguinal orchidectomy Scrotal incision
avoided
Ovarian tumors.
• Contralateral ovary Ascitic fluid Examine peritoneal surface
and the liver, The ovary is removed without violating the
tumor capsule, Examine omentum Inspection of
retroperitoneal lymph nodes and biopsy of enlarged nodes
The fallopian tube, without spilling .

42. Thank you

44. References