35

Dear friends,

When I was given this book in order to translate it, I wanted to write down a

few words based on my experience and the pain I had felt believing that this way

I may soothe your pain.

Holding this booklet I am sure that your soul

and heart are upset and that you may be des-

perately looking to find a solution for the prob-

lem you never thought you are going to face.

All these feelings I have experienced person-

ally when two years ago I was surprised to hear

the doctor saying: �Mrs Perikou , your son has

cancer!�. My first reaction was to let myself fall

off the balcony and the next thought was �Dear

God why this to me ?�

As the time was going, living closely with

the disease and with the great support of my

child's doctors who were offering all their time

to us, I was able to face my child's future with

optimism and to believe that cancer is a dis-

ease that can be won if you only have faith to God and trust your child's doctors.

For these reasons, dear friends, don't be afraid , don't be disappointed ! In

the contrary fight for life, fight against cancer. Everyone who cares for your child

will stand next to you and they will fight with you.

Closing , I would like to thank the health care team who beside the efforts for

my child's treatment, they have provided me with moral support without which I

could barely move on. Also taking advantage of this opportunity I wanted to ex-

press my gratitude from the depth of my heart and say that I could not find better

supporters in my suffering.

Sincerely

Entela Perikou

36

CANCER IN CHILDREN

1. Are children affected by cancer?

Children are very rarely affected by cancer, (another word used is malignan-

cy) approximately 100 times less frequently than adults. Each year in every

100,000 children aged from birth to 15, 14 are affected by cancer, however

cancer is the commonest cause of death in children after the first year of life if

deaths attributed to accidents are excluded.

The types of cancer seen in children are leukemias, lymphomas and various

types of tumors which in Oncology are known as solid tumors.

The disease is not contagious and non typically hereditary although it is

known that certain types of cancers are seen among members of the same

family and that in certain families the incidence of cancer is increased. We may

therefore assume that cancer in children should be attributed to a variety of

factors, known or undetermined as yet.

2. Which are the most frequent types of malignancies inchildren?

The most frequent type of cancer in children is leukemia (approximately 35%

of the total) followed by tumors of the central nervous system, lymphomas (ma-

lignant tumors of the lymph nodes and other tissues), tumors of the kidney

(nephroblastoma) and the adrenal glands (neuroblastoma), sarcomas of the

soft tissues (such as rhabdomyosarcoma) and of the bones (such as osteogen-

ic sarcoma and Ewing�s sarcoma), tumors of the liver (hepatoblastoma), tumors

of the testes and ovaries (called germ cell tumors), tumors of the retina which is

part of the eye (retinoblastoma). Other types of tumors exist but they are very

rare in children.

Malignant tumors of the breast, lung, prostate, malignant melanoma are adult

type malignancies and they are very rarely seen in children.

37

Types of cancer

✔ Leukemia

✔ CNS tumors

✔ Lymphoma

✔ Neuroblastoma

✔ Nephroblastoma

✔ Sarcoma

✔ Other tumors

3. Which is the role of prevention?

Guidelines and rules in order to prevent cancer in children do not clearly

exist. Also the use of presymptomatic work up which is clinical and mainly la-

38

boratory investigation prior to the onset of clinical symptoms and signs (screen-

ing) is not applied in children. It is however crucial to provide the pediatrician

and the family physician the appropriate knowledge and sensitivity in order to

evaluate suspicious symptoms and findings and refer the child to the special-

ized center for further investigations. In children early diagnosis of cancer leads

to effective therapy and therefore better prognosis. It is also important to in-

struct children against smoking and unprotected exposure to sun and educate

them in favor of healthy nutrition.

39

4. What is leukemia?

Leukemia is a disease affecting the hematopoetic (blood) tissue which in-

cludes cells formed in the bone marrow which is found within every bone and in

particular the large bones such as femur, tibia, pelvis, sternum etc. The hemato-

poetic tissue includes cells with various picture (morphology) and function. These

cells are divided in 3 major groups (lines):

a. red cell line includes a variety of cells

which are forming the mature red blood

cells which are very important in trans-

porting oxygen to the tissues and play

major role in the well being of the indi-

vidual. If the amount of red blood cells is

decreased then anemia is documented.

b. white cell line includes a variety of cells

in various steps of maturation and morphology, the most mature being the

lymphocytes and the polymorphonuclear white cells also called neutrophils.

The latter are very important in the defense mechanisms of the child and the

successful attack of infections.

c. megakaryocytic cell line includes cells

which are producing the platelets which

are cells interfering in the clotting pro-

cess of the blood.

When a child gets sick with leukemia,

bone marrow is getting full of abnormal (in

morphology and function) white cells

(these cells are very immature and malig-

nant and are called leukemic cells or blasts) which cannot accomplish their

normal function which is the fight against infection.

When these leukemic cells fill the bone marrow, the production of the mar-

row�s normal cells (red cells, white cells, platelets) is dramatically decreased. As

the number of normal cells decreases, symptoms of leukemia become evident:

easy fatigue, weakness, pallor (decreased red blood cells), fever with or without

infection (decreased white cells), bleeding manifestations from the skin and the

mucosa (decreased platelet count).

40

5. Are there different types ofleukemia?

In children, leukemia is more fre-

quently acute (95%) and the malignant

cells in 85% of acute leukemias belong

in the lymphocytic type of the white

blood cells, called lymphoblasts. The

disease is therefore called acute lym-

phoblastic leukemia (ALL). The remain-

ing acute leukemias (15%) are called

acute non lymphocytic or myelogenous

leukemias (ANLL) and they include var-

ious subtypes. In children, chronic leu-

kemias are rare (5%) and the type seen

is the chronic myelogenous leukemia

(CML).

41

6. Which are the symptoms of leukemia?

Children suffering of leukemia and in particular of ALL experience symptoms

related to:

a) the decreased normal bone marrow cells and these are pallor, fever, fatigue,

42

bleeding manifestations from the skin (red spots or blue-black lesions called

petechiae and ecchymoses) or the mucosa (bleeding from the gums, the

nose and rarely the presence of blood in urine and stools)

b) the infiltration of bone marrow cavity and other organs by the malignant

cells and these are bone pain, limping and enlarged lymph nodes especially

in the neck.

On physical examination the child may be pale, may have bruises and en-

larged lymph nodes and/or liver and spleen.

7. How is leukemia diagnosed?

The diagnosis of leukemia is accomplished by obtaining sample from the

bone marrow cavity (mainly from the pelvic bones) with special needles. This

43

procedure is called bone marrow aspiration

and requires very good local anesthesia or

general anesthesia of short duration. The

sample is smeared on slides, stained and

is examined under the microscope. Part of

the sample is used for special studies which

are called immunophenotyping and cyto-

genetics.

8. How is leukemia treated ?

Children with leukemia should be re-

ferred to specialized pediatric oncology

centers and treated by specially trained pe-

diatric oncologists, experienced nursing personel, psychologist and social work-

er. Therapy includes the administration of specific pharmaceutic agents (che-

44

Cells as seen by a child

45

motherapy). The drug combination, the doses, the way of administration, the

time sequence constitute the so-called therapeutic protocol. Chemotherapeutic

agents are given intravenously via a special central venous catheter (Hickman

catheter) and occasionally by mouth or subcutaneously (under the skin). A very

important part of leukemia therapy is called central nervous system prophylaxis

(prevention) and includes the administration of chemotherapy within the central

nervous system. This is called intrathecal therapy and is given with the spinal tap

(a tap in the back between two lumbar vertebrae). Therapeutic protocols for

leukemia include an initial phase called induction and a final called maintenance.

46

In -between, reinduction and consolidation phases are usually part of the proto-

col. The majority of children with ALL have normal bone marrow after induction

therapy and this is called remission of the disease. Therapy may be given in the

hospital as in-patient, in the out patient department and at home. Total duration

of therapy for the majority of ALL is 2 years.

9. Which are the complications of therapy?

The most frequent and important complications (also called side-effects) of

chemotherapy are related to the mechanism of its action which is the destruc-

47

tion of rapidly proliferating cells.

Such cells are the malignant cells

of leukemia and tumors and the

normal hematopoetic cells (bone

marrow cells) which are gradually

decreasing and the child presents

with anemia, bleeding diathesis

and infection. This bone marrow

status which follows the adminis-

tration of chemotherapy is called

bone marrow aplasia (empty marrow) and calls for special attention and thera-

py (transfusions, antibiotic therapy etc). Other side effects of chemotherapy is

the hair loss (alopecia) which is transient. It should be emphasized that pres-

ence or absence of alopecia does not correlate with chemotherapy effective-

ness. Nausea and vomiting are quite frequent side effects and should be pre-

vented and treated. Various chemotherapeutic agents cause specific side ef-

fects : kidney toxicity, liver toxicity, allergies, heart toxicity etc

48

10. Which is the prognosis of acute lymphoblastic leukemia?

The course and the evolution of ALL is called prognosis and depends on

various factors such as the age of the child (infants less than one year of age

and adolescents have the worse prognosis), the number of white cell blood

count at the time of diagnosis (as the number increases the prognosis is getting

worse), the child�s sex (boys have worse prognosis due to the risk of leukemia

in the testes) and other factors related to the malignant cell itself (size of the

cell,immunophenotype, cytogenetic analysis). Another very important prognos-

tic factor relates to the speed with which malignant cells respond to therapy in the

peripheral blood (day 8) and the bone marrow (days 15 and 33).

With current protocols more than 70% of children with ALL survive without

problems. Survival in continuous remission in the bone marrow and the central

nervous system 5 years from diagnosis is considered cure of the disease.

49

11. What does relapse of ALL mean?

The reappearance of leukemia (reccurence) is called relapse and may refer

to the bone marrow, the central nervous system and the testes in boys or any

combination of the above. Other rare types of relapse are documented in the

eye, the ovary, the kidney etc. When the leukemia is relapsed, the prognosis is

related mainly to the time of occurrence of the relapse being worse if it occurs

early in the course of the disease and while on chemotherapy.

Relapsed ALL is treated with aggressive chemotherapy followed by alloge-

neic bone marrow transplantation with an HLA compatible sibling if leukemia

relapses while on therapy in the bone marrow and if remission of the disease is

again accomplished. Siblings are HLA compatible in 25-30% of cases.

Bone marrow transplantation is not an operation but an intravenous infusion

(as transfusion) of the donor�s bone marrow or peripheral blood cells to the

patient. This infusion follows aggressive chemotherapy. If siblings are not HLA

compatible, then a donor is searched through the international donor marrow

pool.

50

12. Which are the more common tumors in children?

Following leukemia, tumors of the central nervous system (CNS) are the

commonest tumors in childhood affecting children from infancy to the adoles-

cence. These tumors are located in various sites of the CNS (which includes the

brain and the medulla) and mainly in the brain. Beside the location of the tumor,

tumor histology (the type of cells within the tumor), the way the tumor infiltrates

the normal brain tissue and the age of the child are important prognostic fac-

tors. Location of the tumor and the infiltrative nature of the tumor are closely

related to the adequate surgical removal of the tumor.

13. Which are the symptoms of tumors of the CNS?

Children with CNS tumors present with symptoms which mainly depend on

two main factors:

a) age of the child : children less than 3 years of age may experience general

symptomatology such as irritability or somnolence, crying, refusal of food

intake etc Older children may experience headache, vomiting, gait disorders

etc.

b) location of the tumor : symptoms are due to the direct infiltration or pressure

of the normal brain structures or may produce obstruction of the flow of the

cerebrospinal fluid and therefore increase of the intracranial pressure (pres-

sure within the brain) which is an indirect action. The symptoms due to the

increased intracranial pressure are morning headache, vomiting, vision dis-

turbance, weakness of arms or legs etc.

If the tumor is located in anatomic structures as is the cerebellum or the

brain stem (common sites of involvement), then symptoms include also difficul-

ty in walking, equilibrium disorders and palsies of certain cranial nerves (double

vision,slurred speech, difficulty in swallowing etc)

In other locations, other symptoms are headache, convulsions and various

palsies.

51

14. Which are the most common CNS tumors?

The most common CNS tumors are the gliomas which are subdivided in vari-

ous degrees of malignancies (grades) followed by the medulloblastomas which

are presently classified in the PNET group (primitive neuroectodermal tumors).

52

15. How is the diagnosis of the CNS tumors made?

CNS tumors are suspected when the above described symptoms are seen

and the imaging studies obtained (computerized tomography �CT-, magnetic

resonance imaging -MRI-) show the tumor. Surgical removal of the tumor (com-

plete, partial or simply biopsy) will lead to the final diagnosis. In certain

occasions,as is the case of brain stem tumors, biopsy is avoided and diagnosis

is made on clinical and imaging rounds.

16. How are CNS tumors treated ?

The most reliable and effective therapeutic approach is the surgical removal

of the tumor which is not always feasible as is the case of brain stem tumors,

optic pathway tumors etc

53

Radiotherapy is used in certain tumors however it should be taken into ac-

count that this therapeutic approach may cause serious damage in the deve-

loping brain whereas chemotherapy is limited in tumors of high degree of ma-

lignancy (such as medulloblastoma) or in certain tumors which cannot be treat-

ed surgically and when radiotherapy due to young age should be avoided such

as optic pathway tumors and in children age less than 3 years.

17. What type of tumor is the lymphoma?

Lymphomas are malignant tumors which are third in frequency after leuke-

mias and CNS tumors and are divided in Hodgkin�s disease and non Hodgkin�s

lymphomas.

Lymphomas are tumors which involve the peripheral lymph nodes and the

lymphoid tissue of various organs such as the tonsils, the adenoid tissue, the

spleen,the intestine and lymph nodes of the chest and of the abdomen.

18. Which are the manifestations of Hodgkin�s disease andhow is approached?

In the majority of children, Hodgkin�s disease is manifested with painless

swelling of the cervical lymph nodes associated in certain occasions with swel-

ling of the lymph nodes which are located in the chest,between the two lungs,

an anatomic area called mediastinum. Other sites of involvement are lymph

nodes located in the abdomen, the spleen and less frequently the liver. In

certain children, general symptoms such as fever, weight loss and excessive

sweating are noted.

Staging of Hodgkin�s disease includes investigations such as imaging stud-

ies of the chest, the abdomen, the bones and in some children the bone marrow

examination. Biopsy of suspicious lymph nodes is the main diagnostic tool.

Therapy of Hodgkin�s disease in children is the combination of chemotherapy

and radiotherapy of the involved areas. More extensive radiotherapy is chosen

if general symptomatology is also present. The prognosis of Hodgkin�s disease

is very good.

54

19. What is non-Hodgkin�s lymphoma ?

Non Hodgkin�s lymphoma is the commonest type of lymphoma in children.

The disease involves peripheral lymph nodes or the lymphoid tissue of various

organs which are infiltrated by malignant cells. Non-Hodgkin�s lymphomas are

divided according to the histological picture of the tumor, the immunopheno-

typing and cytogenetics and the site of involvement.

20. Which are the symptoms of non-Hodgkin�s lymphomas?

Depending on the site of the involvement the child suffering of non-Hodgkin�s

lymphoma presents with the following symptoms:

1. Thoracic involvement: the disease is usually located in the mediastinum

and the child presents with shortness of breath, cough, difficulty of breath-

ing especially when lying down, swelling in the neck and the face etc. This

type of lymphoma is called lymphoblastic lymphoma.

2. Abdominal involvement or other: children present with abdomilal pain and

55

swelling, vomiting or picture of bowel obstruction. In some other instances

the lymphoma is located in the lymphoid tissue of the head and neck and

children present with visible swelling in the involved area, difficulty in breath-

ing and occasionally with other symptoms such as change in the quality of

voice etc. This type of lymphomas are called undifferentiated lymphomas

most of which belong to the group of Burkitt�s lymphomas.

3. A third group of non-Hodgkin�s lymphomas is seen less frequently they may

involve any site such as skin, bones, lungs, abdomen and are called large

cell lymphomas.

21. How is this group of lymphomas approached?

The diagnosis of non-Hodgkin�s lymphoma is made by using the least inva-

sive procedures. Major surgical operations such as thoracotomy and abdominal

operations are to be avoided, if possible. The reason is that a) there is always

danger when administering general anesthesia in large thoracic tumors and b)

risk of delaying therapy when the abdomen is operated. It is therefore important

to obtain diagnosis by using biologic material such as chest or abdominal fluid

and if not possible by biopsing peripheral lymph nodes or other tissues with fine

needle (FNA).

Treatment of choice is aggressive chemotherapy the type and the duration

of which depend on the type of the tumor. In all types, CNS prophylaxis is im-

portant. Lymphoblastic lymphomas are treated more or less as ALL and undif-

ferentiated lymphomas require short but very aggressive therapy. The progno-

sis of non Hodgkin�s lymphomas is very good.

22. Which are the commonest abdominal tumors of children?

In children, the most common abdominal tumors are kidney tumors and

tumors arising from cells of the sympathetic nervous system.

1. The tumor of the kidney is called nephroblastoma (Wilms� tumor) and it

usually affects children of age less than 5 years. Children present with ab-

dominal mass (which is usually seen or felt by the mother), abdominal pain,

discomfort and less frequently with fever and hematuria (bloody urine).

If the clinical and imaging studies are in favor of nephroblastoma, then de-

56

pending on the therapeutic protocol, either chemotherapy precedes surgi-

cal removal of the tumor (nephrectomy) or vice versa. After nephrectomy,

therapy depends on various factors such as the extension of the disease

within the kidney and the surrounding tissues, the specific histologic type of

the tumor and the presence or absence of metastatic disease especially in

57

the lungs. In certain occasions, besides chemotherapy and surgery, radio-

therapy is added.

Children with nephroblastoma respond very well to therapy and most of them

are cured of their disease.

2. Tumors arising from the sympathetic nervous system are called neuroblas-

tomas and are located either in the adrenal gland (endocrine gland located

on top of the right or left kidney) or in the ganglionic cells of the sympathetic

chain (located in the right or left side of the spine).Neuroblastoma may also

be located in the thorax or neck. The disease affects young children less

than 5 years of age. Children older than one year of age, very frequently

present with distant metastases in the bones and in the bone marrow. In the

majority of children, there is increased amount of certain substances in the

urine which are called catecholamines such is the urinary VMA. The exten-

sion of the disease is evaluated with imaging studies, examination of the

bore marrow and with a special scanning of primary and metastatic disease

called MIBG. Therapy of children with neuroblastoma depends on the age of

the child, the location of the tumor and the stage of the disease. Chemothe-

rapy is the treatment of choice in the majority of children followed by surgical

removal of the tumor and autologous bone marrow or peripheral stem cell

transplantation in certain instances. The prognosis is very good in infants

less than one year of age (in whom treatment is minimal if any) and poor in

older children with metastatic disease.

23. What type of tumors are the sarcomas?

Sarcomas are malignant tumors of fetal origin located in the soft tissues

(called soft tissue sarcomas) or in the bones. Soft tissue sarcomas are further

subdivided in rhabdomyosarcomas and in the non rhabdo-soft tissue

sarcomas.The second group is more frequently diagnosed in older children

and adolescents and the disease is named after the tissue of origin ie liposarco-

ma (arising from the fatty tissue), leiomyosarcoma (arising from the smooth

muscles), fibrosarcoma (arising from the fibrous tissue), synovial sarcoma (aris-

ing from the synovium) etc. These tumors are best treated with complete surgi-

cal excision.

58

24. What is the rhabdomyosarcoma?

Rhabdomyosarcoma is soft tissue tumor whose the cells resemble cells of

the muscles of the fetus. The clinical symptoms depend on the site of involve-

ment at the time of diagnosis. Children with tumors in the head and neck re-

gion, which is the commonest site of involvement, are presented with swelling

of the orbit, of the cheek, of the neck, they may complain of nasal congestion or

obstruction and in certain types, called parameningeal tumors, may develop

neurological signs (double vision, squint, or other nerve palsies). Children with

involvement of the urogenital tract or the abdomen may develop abdominal

swelling, pain, urination problems, constipation etc. Finally children with tumors

of the thoracic wall or the extremities, which are less common sites of involve-

ment, may present with local swelling with or without pain. The site of the dis-

ease, the extension of the tumor (stage) and the specific histology pattern are

the main prognostic factors. For example tumors involving the head and neck

area if they are not parameningeal have better prognosis as have tumors with

histology called non alveolar.

25. How is rhabomyosarcoma treated ?

Rhabdomyosarcoma as all childhood tumors, is a tumor best approached

and treated by a team of specialists: pediatric oncologist, pediatric surgeon,

pathologist (the doctor who �reads� biopsies), radiologist and in certain occa-

sions (such as parameningeal tumors, inoperable tumors, alveolar tumors etc)

radiotherapist. Chemotherapy is the initial treatment of choice and results de-

pend on the site, stage and histology of the tumor and the possibility of com-

plete surgical removal of the tumor.

26. Which tumors are involving the bones?

The most common tumors of the bones among children are the osteogenic

sarcoma and the Ewing�s sarcoma.

Ewing�s sarcoma is a tumor of the adolescents and young adults which less

commonly affects younger children as well. Boys are more frequently affected

than girls and the disease involves mainly the pelvic bones, the thorax, the femur

and less commonly the other bones. Children experience pain in the involved

area, have local swelling and dysfunction and may have fever. The disease may

59

give metastases to the lungs and less frequently to the other bones. Imaging

studies include plain films, computed tomography, magnetic imaging, evaluation

of the lungs and bone scan. The diagnosis is made with biopsy of the involved

60

area.Therapy includes chemotherapy followed by local surgery and radiotherapy

if there is residual tumor and/or inadequate necrosis (dead cells) of the tumor.

Osteogenic sarcoma is a tumor seen in adolescents 10-20 years of age

and boys are, as in Ewing�s, affected more commonly than girls.The disease

involves the bones around the knee joint and presents with local pain and swell-

ing whereas fever is very uncommon. The disease frequently spreads to the

lungs and diagnosis is made as described in Ewing�s sarcoma. Therapy also

follows the rules of Ewing�s tumor with the exception of radiotherapy which is

not active in osteogenic sarcoma.

It should also clearly noted that radical surgical procedures as is the ampu-

tation is as possible avoided in both types of tumors although it may be chosen

in certain rare occasions.

27. Are tumors of the liver seen in children?

Liver tumors are very rare in children and may be benign or malignant. Among

malignant, hepatoblastoma is more common whereas less frequent is hepato-

cellular carcinoma which is a tumor seen in the adult life. The most frequent

clinical manifestation is the presence of enlarged abdomen with or without pain

in the right side. Hepatoblastoma usually affects young children whose the gen-

eral condition may be affected (fever, loss of appetite, weight loss, jaundice).

Imaging studies are very helpful in showing tumor in the right or left or both liver

lobes. In these tumors a laboratory blood test may confirm the diagnosis and

this is the measurement of a substance called alpha �fetoprotein. This is a pro-

tein produced by the embryonal (fetal) liver and the cells of the yolk sac in the

embryo (fetus) and the production stops at the time of birth. Increased levels of

alpha �fetoprotein is seen in malignant liver tumors and in another type of tu-

mors called germ cell tumors. If present, biopsy of the liver is not necessary and

the child is placed on chemotherapy followed by surgical excision of the liver. If

tumor responds to therapy this protein is gradually dropping and is a marker for

monitoring the disease since it increases again if the tumor recurs. Hepatocel-

lular carcinoma is more aggressive tumor and is seen in older children.

61

28. What are the germ cell tumors?

Germ cell tumors are relatively rare childhood tumors originating from very

primitive cells found in the normal fetus and are called germ cells. These cells

�migrate� in the fetus and form the testes or the ovaries or are �misplaced� in

the midline of the body. These tumors are therefore located in the testes or the

ovaries and in the midline as is the posterior aspect of the abdomen such is the

sacrococcygeal region, the area between the two lungs in the thorax (called

mediastinum) and the brain in an area called epiphysis. Symptoms depend on

the location and tumors in the testis present with painless swelling, in the ovary

with pain and abdominal swelling, in the mediastinum with cough or shortness

of breath, in the sacroccygeal region with a visible and palpable mass (some-

times diagnosed during pregnancy with ultrasound), in the epiphysis with symp-

toms from the central nervous system. Alpha-fetoprotein is very valuable sub-

stance in this group of patients as in the liver tumors. An additional substance is

measured in this group of patients the beta chorionic gonadotropin which is

normally produced by the placenta. Therapy depends on the location of the

tumor, the age of the patient, the presence or not of these two blood substan-

ces, the histologic type of tumor (biopsy) and the operability of the tumor. Che-

motherapy and surgery are the main types of therapy. Among these tumors,

benign teratomas requiring only surgical removal, are also included.

29. What type of tumor is the retinoblastoma?

Retinoblastoma is a malignant

tumor of the eye arising from the

retina and is seen exclusively in

children. This is a type of tumor

that may be seen in more than one

member within the same family

and therefore may be hereditary.

Hereditary type is usually affect-

ing both eyes and is diagnosed

earlier in life. The child with reti-

noblastoma may have white eye in the dark called leukocoria or cat�s eye reflex,

squint, red eye etc. The eyes must then be examined under general anesthesia

and the diagnosis is made without requiring biopsy. The extend of the disease

62

within the affected eye, the other eye and outside the eye globe (bone marrow,

bones, spinal fluid etc) is important in defining the type of therapy chosen. Enu-

cleation may be the treatment of choice followed by placement of prosthetic

eyeball. Chemotherapy, radiotherapy and other types of local therapy such as

cryotherapy, laser etc may be used as well. It should be emphasized that eye

examination of siblings is obligatory.

30. What type of disease is the histiocytosis Langerhans ?

Langerhans cell histiocytosis, in the past called Histiocytosis X, is a disease

characterized by accumulation of cells called dendritic in various tissues and

organs and is rather a reactive process and not a definite malignant disorder.

The disease may affect

any organ in single or

multiple sites. Bones,

mucous membranes and

skin are affected as are

lymph nodes, l iver,

spleen and less com-

monly bone marrow,

lungs and brain. When

the disease affects

bones, children complain

of pain or have swellings especially in the skull or ear drainage. Affected skin

may resemble acne and dermatitis which may become ulcerative in moist areas

such as the axillae, the groin etc and lymph nodes may be swollen. General

symptoms include fever, weakness, loss of appetite, weight loss etc. A distinct

manifestation of the disease is the so � called diabetes insipidus which is pre-

sented with extreme thirst and water drinking followed by frequent urination

with increased quantity of urine, requiring therapy with the missing pituitary

hormone (antidiuretic) throughout life. Treatment (chemotherapy) and progno-

sis depend on various factors such as age, number of organs involved and

dysfunction of the organs (such is low blood counts, abnormal liver function).

The disease may have ups and downs without making the prognosis bad with

the exception of infants and young children with organ dysfunction whose the

prognosis is poor.

63

31. What is the final conclusion for cancer in children?

Cancer in children is a very rare disease with various clinical manifestations.

If the disease is suspected, then diagnosis is made without delay and most

children are successfully treated. Children with cancer should be referred in

specially organized pediatric oncology units and treated by a team of well trained

physicians and sensitive nurses who collaborate in order to offer the best avai-

lable medical and psychosocial care not only to the suffering child but to the

entire family. This care should be provided not only during therapy but also after

the completion of therapy and the achievement of the goal (which is the cure of

the child), the period of the reintegration. Children who were treated for cancer

require long term follow up for investigation of late effects of therapy the com-

monest of which are toxicities of the endocrine glands (thyroid, testes, ovaries),

heart, skeletal and neurological system. New (second) types of cancer may

rarely be seen among cured children.

With current therapeutic modalities 2 out of 3 children with cancer are cured

of their disease. Also today 1 out of 570 young individuals as a child has suf-

fered of cancer and is now cured.