Although major cause of childhood morbidity and mortality in the developing world is still malnutrition and infections, pediatric neoplasms are also rising in number. Although pediatric neoplasms occur infrequently, they present a challenging diagnostic and therapeutic problem. Unfamiliarity with these conditions may lead to the erroneous diagnosis and unnecessary aggressive therapy. This was a retrospective analysis of 161 cases of pediatric tumors, both benign and malignant, in surgical pathology department excluding neurosurgery, cardiothoracic, and hemato-lymphoid malignancies (age group 0–12 years) encountered over a period of 5 years: January 2004–December 2008. The clinical, radiological, and therapeutic data were obtained from patients’ case paper records. Pattern of childhood tumors was studied with a focus on tumor incidence, age and sex distribution, demographic pattern, and histological type.

1. Spectrum of childhood neoplasms –
Evaluation of 161 cases in surgical
pathology department

2. Original Article
The spectrum of childhood neoplasms – Evaluation
of 161 cases in surgical pathology department
V.B. Gite *, M.A. Dhakane
Apollo Hospitals, Bilaspur, India
1. Introduction
Incidence of pediatric neoplasms is on the rise all over the
globe, though it is a small fraction of the overall global
tumor burden. Yet for children and their families, it can be
deeply distressing. Although pediatric neoplasms occur
infrequently, they present a challenging diagnostic and
therapeutic problem. Unfamiliarity with these conditions
may lead to the erroneous diagnosis and unnecessary
aggressive therapy.
Malignancy is the second most common cause of childhood
mortality in the developed world, accounting for 12.3% of all
childhood deaths in U.S.A.1
Although the major cause of
childhood mortality in the developing world is still malnutri-
tion and infections, pediatric neoplasms are also rising in
number. About 1/650 children develops malignancy before
their 15th birthday.2
Malignancies account for the major cause
of death in Indian children, next only to infection and
malnutrition. Approximately 35,000–40,000 children develop
malignancies each year in India.2
Thus, the appropriate management of pediatric tumors
requires detailed clinical history, tumor site, precise histo-
pathological diagnosis, and accurate grading and staging
wherever possible along with other clinical investigations.
Histological type is important for understanding etiology and
progression of disease. No histological diagnosis can be
accurate without a clinico-radio-pathological correlation.
a p o l l o m e d i c i n e x x x ( 2 0 1 5 ) x x x – x x x
a r t i c l e i n f o
Article history:
Received 13 May 2015
Accepted 27 July 2015
Available online xxx
Keywords:
Childhood neoplasms
Tumor incidence
Histological type
a b s t r a c t
Although major cause of childhood morbidity and mortality in the developing world is still
malnutrition and infections, pediatric neoplasms are also rising in number. Although
pediatric neoplasms occur infrequently, they present a challenging diagnostic and thera-
peutic problem. Unfamiliarity with these conditions may lead to the erroneous diagnosis
and unnecessary aggressive therapy. This was a retrospective analysis of 161 cases of
pediatric tumors, both benign and malignant, in surgical pathology department excluding
neurosurgery, cardiothoracic, and hemato-lymphoid malignancies (age group 0–12 years)
encountered over a period of 5 years: January 2004–December 2008. The clinical, radiological,
and therapeutic data were obtained from patients' case paper records. Pattern of childhood
tumors was studied with a focus on tumor incidence, age and sex distribution, demographic
pattern, and histological type.
Crown Copyright # 2015 Published by Elsevier B.V. on behalf of Indraprastha Medical
Corporation Ltd. All rights reserved.
* Corresponding author. Tel.: +91 7752248300.
E-mail address: vandanagite@gmail.com (V.B. Gite).
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Available online at www.sciencedirect.com
ScienceDirect
journal homepage: www.elsevier.com/locate/apme
http://dx.doi.org/10.1016/j.apme.2015.07.012
0976-0016/Crown Copyright # 2015 Published by Elsevier B.V. on behalf of Indraprastha Medical Corporation Ltd. All rights reserved.

3. 2. Materials and methods
This was a retrospective analysis of 161 cases of pediatric
tumors in surgical pathology department, excluding neuro-
surgery, cardiothoracic, and hemato-lymphoid malignancies
(age group 0–12 years), encountered over a period of 5 years:
January 2004–December 2008. Institutional ethics committee
permission has been taken before starting the study. Surgical
specimens and biopsy tissues received were fixed overnight in
10% buffered formalin and submitted for processing. Paraffin
sections were cut at 4–6 mm thickness and routine H&E
staining was performed. All cases were reevaluated histologi-
cally on sections from routinely processed formalin-fixed,
paraffin-embedded blocks. Special stains and immunohis-
tochemistry were studied wherever necessary. The clinical,
radiological, and therapeutic data were obtained from
patients' case paper records. Pattern of childhood malignan-
cies was studied with a focus on tumor incidence, age and sex
distribution, environmental and other etiological factors,
demographic pattern, and histological type (Tables 1–6).
3. Results
Average incidence of pediatric tumors in surgical pathology in
tertiary care unit was 0.36%. Males were affected more with
male to female ratio as 1.3:1. The commonest tumors in our
study were soft tissue tumors (49 cases out of 161), most
commonly seen in age group 5–10 years, with male:female
ratio as 1.4:1. Amongst soft tissue tumors, vascular tumors
(55.10%), including lymphangioma and angiofibroma, were
followed by lipoma and neurofibroma. In the vascular tumors,
hemangiomas were excluded, as these are clinically, radiolog-
ically distinct entities. Lymphangioma contributed 16 cases
out of 49 vascular soft tissue tumors followed by angiofibroma.
In the skeletal system, the most common tumor in children
was osteochondroma and the predominant age group affected
was the 10–12 years age group, with 13 out of 19 cases (68.42%)
belonging to this group. Osteochondroma had shown male
predominance. In the renal tumors, only Wilms tumors (9
cases) were seen, with classical triphasic tumors being more
common. The mean age of presentation was 3 years with the
age group 1–5 years being the commonest age group of
presentation (8 cases out of 9). Three of them had showed
unfavorable histology. Among the gonadal germ cell tumors,
there were noted three mature teratomas, one immature
teratoma, two yolk sac tumors of ovary, and one yolk sac
tumor in testis. Two mature sacrococcygeal teratomas were
also seen. Among the nonteratomatous dermoid cyst, peri-
orbital region (16 out of 27 cases) was the commonest location.
In the adrenal gland, adrenal medullary tumors were more
common than adrenal cortex, with neuroblastoma (4 of 10
cases) as the common individual tumor. 37 (22.98%) cases out
of 161 were diagnosed as malignant. The commonest tumor
was Wilms tumor (9 out of 37 cases) followed by neuroblasto-
ma (4 out of 37 cases). The common age of presentation was 1–
5 years with male predominance. Immunohistochemistry
performed on 10 of 11 round cell tumors revealed five cases of
lymphoma, three cases of rhabdomyosarcoma, and two cases
of Ewing's sarcoma/PNET.
4. Discussion
In literature, differences have been demonstrated in the
incidence rates of pediatric neoplasms, as they are studied by
anatomic site, age, race, or gender. They are also studied in
reference to various parameters, such as total pediatric tumors
against total pediatric hospital admissions or total autopsy
studies or total surgical pathology samples received. The
present study comprises of 161 cases (0.36%) of childhood
tumors from a total of 44,395 surgical specimens (both adults
and pediatrics) received over a period of 5 years. The incidence
of pediatric neoplasms from total pediatric surgical cases
received is 5.11% (161 cases of 3149). Data on the incidence of
childhood cancer in Queensland during the 10-year period,
1979–1988, show that the average annual crude incidence rate
and age-standardized incidence rate (to the world population)
for both sexes were 12.63 and 13.30 per 100,000, respectively.3
Kusumakumary et al. observed total number of pediatric
malignancies during a 10-year period and accounted for 4.5%
Table 2 – Incidence of pediatric tumors in total pediatric
cases (both biopsy and surgical specimens) received.
Year No. of
pediatric
tumors
Total no. of
pediatric surgical
pathology cases
Percentage (%)
2004 15 602 2.49
2005 28 577 4.85
2006 33 685 4.82
2007 46 619 7.43
2008 39 666 5.86
Total 161 3149 5.11
Table 1 – Incidence of pediatric tumors in total surgical
specimens (both biopsy and surgical specimens of adults
and pediatric) received.
Year No. of
pediatric
tumors
Total no.
of surgical
pathology cases
Percentage
(%)
2004 15 9423 0.16
2005 28 9154 0.31
2006 33 8613 0.38
2007 46 8745 0.53
2008 39 8460 0.46
Total 161 44,395 0.36
Table 3 – Age incidence.
Age group
(years)
Total cases
(n = 161)
Percentage
(%)
0–1 10 6.22
1–5 52 32.29
5–10 56 34.78
10–12 43 26.71
Maximum cases were seen in children aged 5–10 years – 34.78%.
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4. of all cases.4
In Dibrugarh, in Northeastern India, and
Chandigarh, in the Northwestern India, the frequency was
2% and 4.8%, respectively.5
In the present study, 12 years was considered as the
pediatric age, with infancy as a separate age group. Marked
differences were found in the age distribution of various
tumors. The maximum number of tumors (56/161) was
observed in children aged 5–10 years, accounting for about
34.78% of total pediatric tumors. The second largest group of
tumors occurred in the age group of 1–5 years, amounting to
32.29%. Infancy contributed 6.22% of total childhood tumors.
The frequencies of neuroblastoma, Wilms tumor, retinoblas-
toma, and hepatoblastoma were strikingly more in children
younger than 5 years of age, similar to that observed by
Kusumakumary et al.4
However, round cell tumors and non-
neuroblastic adrenal tumors presented commonly in the age
group of 5–10 years. Tumors of the skeletal system were seen
predominantly in the age group of 10–12 years (13 out of 19
cases).
Pediatric neoplasms differ markedly in their age distribu-
tion.4
Most of these occur below the age of eight years,
although wide age variability exists in children.2
Age distribu-
tion varies with site. The principal embryonal tumors, namely
those of the CNS, neuroblastoma, retinoblastoma, Wilms
tumor, and hepatoblastoma, all had highest incidence in early
childhood, and about 40% of the cumulative incidence of
retinoblastoma and hepatoblastoma was observed in the first
year of the life. Contrastingly, incidence of some diagnostic
categories increases with age and more than two-thirds of the
cumulative incidence of childhood Hodgkin's lymphoma and
osteosarcoma occur at the age of 10–14 years. Congenital
lesions contribute to many of these pediatric neoplasms. A
patient's age at the time of presentation also is an important
consideration in determining the type of a particular pediatric
tumor, and this knowledge will guide the physician in further
evaluation and management of the patient. Age also has
strong prognostic relevance in certain tumors. It has been
observed that infants with neuroblastoma seemed to have
better prognosis than older children, even after minimal
therapy.6
However, the age <1 year at diagnosis has been
associated with a worse prognosis in rhabdomyosarcoma
(RMS).7
Table 4 – Age distribution of individual tumor.
Tumor 0–1 year 1–5 years 5–10 years 10–12 years Total
Soft tissue tumors
Lymphangioma 1 5 9 1 16
Angiofibroma 3 8 11
Lipoma 1 5 6 5 17
Neurofibroma 1 1 3 5
Skeletal system
Aneurysmal bone cyst 1 1 2 4
Osteoid osteoma 2 2
Fibroma 1 3 4
Chondroblastoma 1 1
Osteochondroma 1 7 8
Genitourinary system
Mature teratoma 1 2 1 4
Immature teratoma 1 1
Yolk sac tumor 1 2 3
Wilms tumor 1 8 9
Bladder carcinoma 1 1
Skin
Pilomatrixoma 1 2 2 5
Naevus 2 3 3 8
Round cell tumors 1 2 6 2 11
Adrenal
Adrenocortical adenoma 1 1
Adrenocortical carcinoma 1 1
Pheochromocytoma 2 2
Ganglioneuroma 1 1
Ganglioneuroblastoma 1 1
Neuroblastoma 1 3 4
Larynx
Juvenile laryngeal papillomatosis 5 2 7
Eye
Retinoblastoma 3 3
Breast
Fibroadenoma 1 1 2
Liver
Hepatoblastoma 1 1
Dermoid cyst (nonovarian) 2 10 12 2 26
Sacrococcygeal teratoma 1 1 2
Total 10 52 56 43 161
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5. Gender distribution may help to guide the evaluation of the
tumor. In the present study, there is higher incidence in males,
with a male to female ratio of 1.3:1. In infancy, females were
more affected. In the children aged between 1 and 5 years, the
male to female ratio was 1.4:1. This ratio is 1.3:1 and 1.1:1 in the
age groups 5–10 and 10–12 years, respectively. Angiofibroma
and pilomatrixoma were exclusively seen in males whereas
ossifying fibromas were exclusively seen in females. Male
Table 5 – Gender distribution of individual tumor.
Male (M) Female (F) Total M:F
Soft tissue tumor
Lymphangioma 11 5 16 2.2:1
Angiofibroma 11 – 11 11(F)
Lipoma 11 6 17 1.8:1
Neurofibroma 3 2 5 1.5:1
Skeletal system
Aneurysmal bone cyst 2 2 4 1:1
Osteoid osteoma 1 1 2 1:1
Ossifying fibroma – 4 4 4(F)
Chondroblastoma – 1 1 1(F)
Osteochondroma 5 3 8 1.6:1
Genitourinary system
Wilms tumor 7 2 9 3.5:1
Bladder carcinoma 1 1 1(M)
Mature teratoma – 4 4 4(F)
Immature teratoma – 1 1 1(F)
Yolk sac tumor 1 2 3 1:2
Skin
Pilomatrixoma 5 5 5(M)
Naevus 3 5 8 1:1.7
Round cell tumor 9 2 11 4.5:1
Adrenal
Adrenocortical adenoma 1 1 1(M)
Adrenocortical carcinoma – 1 1 1(F)
Pheochromocytoma 1 1 2 1:1
Ganglioneuroma – 1 1 1(F)
Ganglioneuroblastoma 1 – 1 1(M)
Neuroblastoma 2 2 4 1:1
Larynx
Juvenile laryngeal papillomatosis 1 6 7 1:6
Eye
Retinoblastoma 2 1 3 2:1
Breast
Fibroadenoma – 2 2 2(F)
Liver
Hepatoblastoma 1 – 1 1(M)
Dermoid cyst (Nonovarian) 12 14 26 1:1.2
Sacrococcygeal teratoma – 2 – 2(F)
Total 91 70 161 1.3:1
Table 6 – Pediatric malignant tumor according to International Classification of Childhood Cancer (ICCC).
Group Number of malignant tumor Percentage (%)
(I) Leukemias Excluded –
(II) Lymphomas and reticuloendothelial neoplasms Excluded –
(III) CNS and miscellaneous intracranial and intraspinal neoplasms Excluded –
(IV) Sympathetic nervous system tumors 5 13.51
(V) Retinoblastoma 3 8.11
(VI) Renal tumors 9 24.32
(VII) Hepatic tumors 1 5.41
(VIII) Malignant bone tumors – –
(IX) Soft tissue sarcomas – –
(X) Germ cell, trophoblastic, and other gonadal neoplasms 5 13.51
(XI) Carcinomas and other malignant epithelial neoplasms 3 5.41
(XII) Other and unspecified malignant neoplasms 11 (Round cell tumors) 29.73
Total 37 100.00
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6. preponderance was seen in Wilms tumor and round cell tumor
and female preponderance was seen in juvenile laryngeal
papillomatosis. There were equal incidences of neuroblastic
tumors in males and females.
According to the literature, male predominance is a salient
feature of many childhood tumors.2
McWhirter et al.3
found
that the incidence of pediatric tumors in males is unusually
high, as is the sex ratio of male to female, that is 1.57:1. Sex
ratio also varies with the site. The incidence of germ cell
tumors of certain sites, thyroid tumors, and malignant
melanoma were markedly in excess in the female child.
Sacrococcygeal teratoma is usually associated with female
predominance. Several studies indicate that the prognosis for
survival is poor in males.8
The present study had 49 out of 161 cases involving soft
tissue contributing to 30.43% of total tumors, most commonly
seen in age group 5–10 years, with male:female ratio as 1.4:1.
Amongst soft tissue tumors, vascular tumors (55.10%),
including lymphangioma and angiofibroma, were followed
by lipoma and neurofibroma. In the vascular tumors, heman-
giomas were excluded, as these are clinically, radiologically
distinct entities. Lymphangioma contributed 16 cases out of 49
vascular soft tissue tumors followed by angiofibroma. The
second largest group of tumors involved the skeletal system,
contributing 11.80%. The most common tumor in children was
osteochondroma and the predominant age group affected was
the 10–12 years age group, with 13 out of 19 cases (68.42%)
belonging to this group.
Other major systems weregenitourinary system,skin, round
cell tumors, adrenal glands, and larynx, which contributed
11.18, 8.07, 6.83, 6.21, and 4.35%, respectively. In the renal
tumors, only Wilms tumors (9 cases) were seen, with classical
triphasic tumors being more common. The mean age of
presentation was 3 years with the age group 1–5 years being
the commonest age group of presentation (8 cases out of 9).
Three of them had showed unfavorable histology. Among the
gonadal germ cell tumors, there were noted three mature
teratomas, one immature teratoma, two yolk sac tumors of
ovary, and one yolk sac tumor in testis. Two mature
sacrococcygeal teratomas were also seen. In the adrenal gland,
adrenal medullary tumors were more common than adrenal
cortex with neuroblastoma (4 of 10cases) as common individual
tumor.Immunohistochemistry performed on10of 11round cell
tumors revealed five cases of lymphoma, three cases of
rhabdomyosarcoma, and two cases of Ewing's sarcoma/PNET.
The classification of childhood cancer is based on primary
site and tumor morphology, with an emphasis on morphology
rather than only primary site, as for adults. The classification
scheme for childhood cancer is based according to the
recommendation by International Classification for Child-
hood Cancer (ICCC) definitions for malignant neoplasms.9
Childhood neoplasms include a diverse array of malignant
tumors and nonmalignant tumors. In the present study,
benign tumors (77.02%) incidence was more than malignant
tumors (22.98%). Among the malignant tumors, Wilms tumor
with 9 cases (24.32%) was the largest group (9 out of 37 cases),
followed by neuroblastoma (4 out of 37 cases). The common
age of presentation was 1–5 years with male predominance.
Childhood malignant neoplasms account for no more than
2% of all cancers. Malignancies of the hematopoietic system
are the largest subgroup of childhood malignancies, account-
ing for 30–60% of all tumors. They are followed by tumors of
the brain and nervous system (10–20%), bone (3–10%), and liver
and others (1–3%).10
The tumors encountered by Sebastian
et al.11
were lymphomas 44.3%, Wilms' tumor 20.1%, sarcomas
11.5%, neuroblastomas 8.6%, retinoblastoma 8.0%, teratomas
4.6%, and hepatoma 2.9%. Upadhyay et al.12
did a retrospective
analysis of the prevalence of malignant childhood tumors in
children up to the age group of 15 years. Leukemias (29.2%),
malignant lymphomas (15.4%), bone tumors (12.6%), and soft
tissue sarcomas (8.2%) were the common malignancies
encountered. Central nervous system tumors (5.4%) were less
frequently seen, in contrast to certain western studies.
So to conclude, the likelihood of a given type of tumor being
present in a particular age or sex group or particular site may
heighten the index of suspicion and ultimately influences
etiology, biology, natural history, relative incidence and
distribution frequency, clinical presentation and manifesta-
tions, and response to therapy and outcome.
Conflicts of interest
The authors have none to declare.
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