SOLID TUMORS

HODGKIN LYMPHOMA
It can affect children at any age, but is
more common in children over five years
old. Hodgkin lymphoma can also occur in
young adults. During childhood, boys are
more often affected, but in the teenage
years boys and girls are affected equally.
Hodgkin lymphoma was first described by
Dr Thomas Hodgkin in 1832. It is a cancer
that affects the lymphatic system.
 Hodgkin's lymphoma is rare in children under
5 years of age. In children under age 10, it is
more common in boys than girls. About 10%
to 15% of all cases of Hodgkin's are
diagnosed in children 16 and under
 What differentiates Hodgkin's lymphoma is the
presence of Reed-Sternberg cells (and variations on
this cell) in the cancerous area, a cell specific to
Hodgkin's Disease. There is definitive evidence that
the the cancerous cells are B-cell lymphocytes
(white blood cells). The Epstein-Barr virus (EBV)
also appears to be a factor, at least in some cases -
appearing in about 40-50% of Hodgkin's cases. It
has recently been found that Interleukin-13, a natural
cytokine in the body, may be overproduced by
Hodgkin's cancerous cells. What causes Hodgkin's
lymphoma is still being researched.
 Types of Hodgkin's Lymphoma:
Nodular sclerosis (NS). The lymph nodes in the lower neck,
chest and collarbone usually contain normal and reactive
lymphocytes and Reed-Sternberg cells separated by bands of
scar-like tissues.
Lymphocyte predominance (LP). The lymph nodes are
composed largely of reactive lymphocytes and malignant
L&H cells which have a "popcorn" appearance and very few
Reed-Sternberg cells.
Mixed cell (MC). The lymph nodes usually contain Reed-
Sternberg cells and inflammatory cells.
Lymphocyte depleted (LD). There are two different variations
of this classification: one with sheets of differing malignant
cells; the other with few Reed-Sternberg cells and
lymphocytes with scar-like tissue.
Nodular lymphocyte predominance Hodgkin's lymphoma
 SIGNS AND SYMPTOMS
The first sign of Hodgkin lymphoma is usually a painless
swelling of one gland, or a group of lymph glands, which
continues for some weeks. The first glands to be affected
are usually in the neck, most often on one side only, in a
small area above the collarbone.
Sometimes, enlarged glands can be felt in the armpit or
groin.
If glands in the chest are affected, this can cause a
troublesome cough or breathlessness.
Occasionally, a child with Hodgkin lymphoma may have a
high temperature (fever), night sweats, severe itching or
weight loss.
 POSITIVE DIAGNOSIS
biopsy
x-rays, CT and MRI scans and blood tests, are
carried out to find the exact size and position
of the lymphoma and whether it has spread. If
a blood test suggests that the bone marrow is
affected by the cancer, a bone marrow sample
may be taken.
 STAGING
Stage 1 One group of lymph nodes is affected but the
lymphoma is only on one side of the diaphragm
Stage 2 Two or more groups of lymph nodes are affected
and the lymphoma is only on one side of the diaphragm.
Stage 3 There are lymphoma cells in the lymph nodes
above and below the diaphragm. The spleen may also be
affected.
Stage 4 The lymphoma has spread beyond the lymph
nodes, for example to the liver, lungs or bone marrow
As well as giving each stage a number, there is a letter
code either A or B to show if the child has the
following specific symptoms: fever, significant weight loss
(more than a tenth of their body weight in the last six
months) or night sweats ;none of these the lymphoma
will be classified as A, and if they do have these symptoms
it is classified as B.
 A physical exam including examining the lymph nodes
Collecting a medical history and history of symptoms
A complete blood work-up including checks for abnormal blood cell
count, blood chemistry, and abnormal sedimentation rate
A chest x-ray to view lymph nodes and to see if other organs are involved
A computerized tomography (CT or CAT) scan, positron emission
tomography (PET) scan and/or magnetic resonance imaging (MRI) scan
of the chest, pelvis, and abdomen to determine the possible spread of the
disease
A gallium scan to check for radioactive intake of gallium in the lymph
system indicating swelling and ultimately disease
A bone marrow aspiration and biopsy to determine if the bone marrow has
been affected by lymphoma; in this procedure the hip is numbed (ask for
EMLA cream or similar to numb the site beforehand at home) and a
needle is inserted into the bone; liquid bone marrow and a bone chip are
extracted and the tissue is examined under a microscope
Certain medical centers may perform additional tests, including
Exploratory surgery (staging laparotomy) to determine the extent of the
disease. In some cases the spleen is removed if it is the only organ
affected. A tissue sample may also be taken of the liver. The child may
receive medicine to prevent infection.
A lymphangiogram, a procedure during which a radio-opaque liquid is
injected into the lymph system through the feet; the fluid travels
throughout the lymph system and remains visible by x-rays for up to six
months
 Hodgkin lymphoma in children is treated with
chemotherapy, but sometimes radiotherapy is
also needed. For very localised disease (when the
cancer has not spread beyond its original site)
radiotherapy alone may be used. The type and
amount of treatment depends on the stage of the
disease at diagnosis.
Side effects can include nausea and vomiting,
hair loss, an increased risk of infection or
bruising and bleeding, tiredness and diarrhoea.
 Chemotherapy
The mainstream chemotherapy regimens is:
ABVD - Adriamycin (Doxorubicin), Bleomycin, Vinblastine
(Velban), Dacarbazine (DTIC)
Other regimens that have been or might be used are:
MOPP - Mechlorethamine, Vincristine, Prednisone,
Procarbazine
MOPP alternating with ABV
MOPP alternating with ABVD
ChlVPP - Chlorambucil, Vinblastine, Procarbazine,
Prednisone
Chemotherapy is given in measured doses. Often a
chemotherapy regimen will be given in cycles - treatments
spaced over the period of days. For example, it is common to
give ABVD in 4 week cycles. Two treatments, one every 2
weeks would be in one cycle. The number of cycles vary but
for example 6 cycles would consist of 12 treatments spaced
two weeks apart.
 Adriamycin 25 mg/m2 IV on days 1 and 15
Bleomycin 10 units/m2 IV on days 1 and 15
Vinblastine 6 mg/m2 IV on days 1 and 15
Dacarbazine375 mg/m2 IV on days 1 and 15
 NON HODGKONS LYMPHOMA
Non-Hodgkin's lymphoma is cancer in the lymphatic
system. According to estimates by the Leukemia and
Lymphoma Society, almost 85 percent of lymphomas
diagnosed in the US in 2006 were non-Hodgkin's lymphoma.
Lymphomas are the fifth most common childhood
cancer. They occur most often in children between the ages of
7 and 11, but can occur at any age from infancy to adulthood.
Non-Hodgkin's lymphoma affects males more often than
females, and is more common among Caucasian children than
among African-American children and children of other
races.
 Lymphoblastic non-Hodgkin's lymphoma accounts for about
30 percent of the cases, involves the T-cells, and usually
presents with a mass in the chest, swollen lymph node(s),
with or without bone marrow and central nervous system
involvement.
Burkitt's or non-Burkitt's lymphoma
Burkitt's or non-Burkitt's lymphoma is a non-Hodgkin's
disease in which the cells are undifferentiated and diffuse.
This has also been referred to as small non-cleaved cells.
Burkitt's and non-Burkitt's lymphoma accounts for about 40
to 50 percent of the cases and is usually characterized by a
large abdominal tumor and may have bone marrow and
central nervous system involvement.
large cell or diffuse histiocytic non-Hodgkin's lymphoma
Large cell or diffuse histiocytic non-Hodgkin's involves the
B-cells and T-cells and accounts for about 25 percent of the
cases. Children with this type of non-Hodgkin's lymphoma
usually have lymphatic system involvement, as well as a non-
lymph structure (i.e., lung, jaw, brain, skin, and bone)
involvement.

Diffuse Large B-cell Lymphoma
Burkitt Lymphoma
High-grade B-cell Lymphoma
(small non-cleaved) Burkitt-like
Lymphoma
Precursor T or B-cell
Lymphoblastic
Lymphoma/Leukemia
(Lymphoblastic Lymphoma
CLASIFICATION REAL
Cells are large, with prominent
nucleoli and abundant
cytoplasm and many mitoses.
Most are B-cell, but 20% are T-
cell phenotype
Intermediate sized B-
lymphocytes (small-noncleaved
cells)
Intermediate sized B-
lymphocytes (small non-cleaved
cells)
Intermediate sized lymphocytes
in a diffuse pattern
CD19, 20, 79a; some have
t(14;18); some have Bcl-2 and
Bcl-6 expression; linked to EBV
infection; negative TdT
CD10, 19, 20, 79a; t(8:14) is
characteristic; African form
linked to EBV infection;
negative TdT
CD19, 20
B-cells are CD19, 20, sometimes
CD10; T-cells are CD3 and 8;
all are TdT positive
Though often localized, they
tend to be aggressive extranodal
masses; seen in adults and
children, also in HIV infection
Endemic in Africa with
mandibular and abdominal
involvement; sporadic
elsewhere with abdominal
involvement; affects mainly
children and young adults
Sporadic; may be seen with
HIV infection
Seen in children and
adolescents; T-cell type often in
mediastinum; very aggressive
and can progress to acute
lymphocytic leukemia
B CELL LARGE
LYMPHOMA
 CLINICS
painless swelling of the lymph nodes in neck, chest,
abdomen, underarm, or groin
fever
sore throat
fullness in groin area from node involvement
bone and joint pain
night sweats
tiring easily (fatigue)
weight loss/decreased appetite
itching of the skin
recurring infections
 STAGING
stage I - involves the tumor at one site, either nodal
or elsewhere in the body.
stage II - involves the tumor at two or more sites on
the same side of the body.
stage III - involves tumors in any number that occur
on both sides of the body, but does not involve bone
marrow or the central nervous system.
stage IV - any stage of tumor that also has bone
marrow and/or central nervous system involvement.
Stage IV is also subdivided depending on the amount
of blasts (cancer cells) present in the bone marrow.
 TESTS
Blood ( CBC, ESR, Fg,ALT, BUN) , urine tests
x-rays of the chest
computed tomography scan of the abdomen, chest, and pelvis positron
emission tomography (PET) scan - radioactive-tagged glucose (sugar) is
injected into the bloodstream. Tissues that use the glucose more than
normal tissues (such as tumors) can be detected by a scanning machine.
PET scans can be used to find small tumors or to check if treatment for a
known tumor is working.
lymph node biopsy
lymphangiogram
bone marrow aspiration and/or biopsy examins the number, size, and
maturity of blood cells and/or abnormal cells.
lumbar puncture (to evaluate central nervous system disease for cancer
cells ) - a special needle is placed into the lower back, into the spinal
canal. This is the area around the spinal cord. A small amount of cerebral
spinal fluid (CSF) can be removed and sent for testing. CSF is the fluid
which bathes the brain and spinal cord.
 TREATMENT
chemotherapy
radiation therapy
surgery
close monitoring of blood work
bone marrow transplant
bone marrow examinations
lumbar punctures/spinal taps
antibiotics (to prevent or treat infections )
supportive care (for side effects of treatment )
long-term follow up care (to determine response to treatment,
detect recurrent disease, and manage late effects of treatment)
 WILMS' TUMOR
Over the past few decades, dramatic
improvement has occurred in the survival of
children with Wilms' tumor. This excellent
outcome is attributed primarily to the
availability of several active
chemotherapeutic agents; however, advances
in radiation oncology, supportive care, and
improved surgical and anesthetic techniques
also have a role.
 The total annual incidence of Wilms' tumor in
the United States is estimated at 450 to 500
cases.
The median age of presentation is 36.5
months for boys compared with 42.5 months
for girls with unilateral tumors.
Wilms' tumor presents almost equally in
males and females; however, there is a higher
incidence in African or African-American
children when compared with Caucasian or
Asian children.
 Several associated congenital anomalies, including
aniridia, hemihypertrophy, cryptorchidism, and
hypospadias, have been reported.
Genitourinary anomalies (hypospadias,
cryptorchidism, other genital anomalies, renal fusion
anomalies) are present in 4.5% of patients with
Wilms' tumor.
The specific association of male
pseudohermaphroditism, renal mesangial sclerosis,
and nephroblastoma is known as Denys-Drash
syndrome.
Hemihypertrophy may occur alone or as part of the
Beckwith-Wiedemann syndrome,
 The incidence of aniridia in patients with
Wilms' tumor is 1.1%.
Aniridia and Wilms' tumor are most
commonly associated in patients with the
WAGR syndrome ( Wilms tumor, aniridia,
genital anomalies, and mental retardation).
Most affected individuals have a
constitutional deletion on chromosome 11,
and the incidence of Wilms' tumor is 42%.
 Histology is one of the most important determinants
of outcome in children with Wilms' tumor.
Wilms' tumor simulates the development of a normal
kidney with blastemal, epithelial (tubules), and
stromal components
Favorable histology is defined as the lack of findings
of anaplasia
Blastemal-predominant tumors behave more
aggressively and present with early metastasis and
advanced disease.
 CLINICAL EVALUATION AND
STAGING
Most patients are diagnosed by their primary care physician
after an abdominal enlargement or mass is noted in routine
care of the child.
The child may also present with acute or chronic abdominal
pain, hematuria, or fever. The child should be examined
carefully, with particular attention to associated anomalies
such as aniridia, hemihypertrophy, macroglossia, or
genitourinary malformations.
The patient should be examined for signs of venous
obstruction, such as varicocele, prominent veins over the
abdominal wall, or leg swelling.
The abdominal examination should attempt to differentiate
this mass from benign causes such as hepatomegaly or
splenomegaly and from other malignancies such as
neuroblastoma.
 Imaging
Preoperative diagnosis through accurate imaging is an
important part of the evaluation of a child with Wilms' tumor.
Although CT is often performed, the most useful initial study
is ultrasonography. The goal of imaging should be to localize
the source of the mass, identify associated genitourinary
anomalies, confirm the presence of a functioning contralateral
kidney, and exclude extension of tumor into the inferior vena
cava. Ultrasound is useful in determining the origin of the
mass and in assessing the inferior vena cava but is of limited
value in assessing renal function.
Chest radiography should be performed to ensure that there is
no metastatic disease. CT of the chest may detect lesions not
seen on plain film, and the management of these patients is
somewhat controversial
 Stage I. The tumor is limited to the kidney and was completely excised.
The renal capsule has an intact outer surface.The tumor was not ruptured
or manipulated for a biopsy prior to removal (fine-needle aspiration
biopsies areexcluded from this restriction). The vessels of the renal sinus
are not involved.
Stage II. The tumor extends beyond the kidney but was completely
excised. There may be regional extension oftumor (i.e., penetration of the
renal capsule or extensive invasion of the renal sinus).
Stage III. Residual nonhematogenous tumor is present and confined to the
abdomen. Any one of the following mayoccur: (1) Lymph nodes within
the abdomen or pelvis are found to be involved by tumor (renal hilar,
para-aorticor beyond). Lymph node involvement in the thorax or other
extra-abdominal sites would be a criterion for stageIV. (2) The tumor has
penetrated through the peritoneal surface. (3) Tumor implants are found
on the peritonealsurface. (4) Gross or microscopic tumor remains
postoperatively (e.g., tumor cells are found at the margin ofsurgical
resection on microscopic examination). (5) The tumor is not completely
resectable because of localinfiltration into vital structures. (6) Tumor spill
not confined to the flank occurred either before or during surgery.
Stage IV. Hematogenous metastases (e.g., lung, liver, bone, brain), or
lymph node metastases outside theabdominopelvic region are present.
Stage V. Bilateral renal involvement is present at diagnosis. An attempt
should be made to stage each side accordingto the above criteria on the
basis of the extent of disease prior to biopsy or treatment.
 TREATMENT
patients with stage I favorable histology Wilms' tumor were
treated successfully with either a 10-week or 18-week
regimen of vincristine and actinomycin D
Preoperative treatment can produce a dramatic reduction in
the size of the primary tumor, facilitating surgical excision;
however, the staging information obtained following
prenephrectomy chemotherapy does not reflect the original
tumor stage
The surgeon has an important role in the management of
Wilms' tumor. Careful removal of the tumor without rupture
or spill is mandatory because these patients have a sixfold
increase in local abdominal relapse
 NEUROBLASTOMA
Neuroblastoma is the most common extracranial
solid tumor in infancy. It is an embryonal
malignancy of the sympathetic nervous system
arising from neuroblasts (pluripotent sympathetic
cells). In the developing embryo, these cells
invaginate, migrate along the neuraxis, and populate
the sympathetic ganglia, adrenal medulla, and other
sites. The pattern of distribution of these cells
correlates with the sites of primary disease
presentation.
In the U.S., approximately 650 children are
diagnosed with neuroblastoma each year. It is often
present at birth, but not detected until the tumor
begins to grow and compress the surrounding
organs. Most children affected by neuroblastoma
have been diagnosed before the age of 5.
 Origin and migration pattern of neuroblasts during fetal
development explains the multiple anatomic sites where these
tumors occur; location of tumors appears to vary with age.
Tumors can occur in the abdominal cavity (40% adrenal, 25%
paraspinal ganglia) or can involve other sites (15% thoracic,
5% pelvic, 3% cervical tumors, 12% miscellaneous). Infants
more commonly present with thoracic and cervical tumors,
whereas older children more frequently have abdominal
tumors.
Stage of the tumor at the time of diagnosis and age of the
patient are the most important prognostic factors. Although
patients with localized tumors (regardless of age) have an
excellent outcome (80-90% 3-year event-free survival [EFS]
rate), patients older than 1 year with metastatic disease fare
poorly. Generally, more than 50% of patients present with
metastatic disease at the time of diagnosis, 20-25% have
localized disease, 15% have regional extension, and
approximately 7% present during infancy with disseminated
disease limited to the skin, liver, and bone marrow (stage 4S).
 HISTOLOGY
The undifferentiated neuroblastomas histologically present as
small, round, blue cell tumors with dense nests of cells in a
fibrovascular matrix and Homer-Wright pseudorosettes.
These pseudorosettes, which are observed in 15-50% of
tumor samples, can be described as neuroblasts surrounding
eosinophilic neuritic processes. The typical tumor shows
small uniform cells with scant cytoplasm and hyperchromatic
nuclei. A neuritic process, also called neuropil, is a
pathognomonic feature of neuroblastoma cells. NSE,
chromogranin, synaptophysin, and S-100
immunohistochemical stains are usually positive
 CLINICS
Tumors that arise from the paraspinal sympathetic ganglia can grow
through the spinal foramina into the spinal canal and impinge on the
spinal cord. This may result in the presence of neurologic symptoms,
including weakness, limping, paralysis, and even bladder and bowel
dysfunction.
Thoracic neuroblastomas (posterior mediastinum) may be asymptomatic
and are usually diagnosed by imaging studies obtained for other reasons.
Presenting signs or symptoms may be insignificant and involve mild
airway obstruction or chronic cough, leading to chest radiography.
Because more than 50% of patients present with advanced-stage disease,
usually to the bone and bone marrow, the most common presentation
includes bone pain and a limp. However, patients may also present with
unexplained fever, weight loss, irritability, and periorbital ecchymosis
secondary to metastatic disease to the orbits. The presence of bone
metastases can lead to pathologic fractures.
Approximately two thirds of patients with neuroblastoma have abdominal
primaries. In these circumstances, patients can present with an
asymptomatic abdominal mass that usually is discovered by the parents or
a caregiver.
 TESTS
More than 90% of patients have elevated homovanillic acid (HVA) and/or
vanillylmandelic acid (VMA) detectable in urine.
Serum LDH (useful as biologic marker)
Ferritin (useful as biologic marker)
CBC count and differential (Anemia or other cytopenias suggest bone
marrow involvement.)
CBC, ESR,BUN.ALT, Total bilirubin
Alkaline phosphatase
Total protein
Albumin
Prothrombin time (PT)/activated prothrombin time (aPTT)
Electrolytes
Calcium
Magnesium
Uric acid
 Obtain chest and abdominal radiographs to evaluate for the
presence of a posterior mediastinal mass or calcifications.
A CT scan of the primary site is essential to determine tumor
extent. The main body of the tumor is usually
indistinguishable from nodal masses.
In cases of paraspinal masses, MRI aids in determining the
presence of intraspinal tumor and cord compression.
I123/131-methyliodobenzylguanadine (MIBG) accumulates
in catecholaminergic cells and provides a specific way of
identifying primary and metastatic disease if present.
Increasing numbers of institutions have access to MIBG
scanning.
A technetium-99 bone scan can also be used to evaluate bone
metastases. Especially in patients with negative MIBG study
findings.
Skeletal surveys may also be useful, especially in patients
with multiple metastatic lesions.
Positron emission tomography (PET) scan are under
evaluation.
 TREATMENT
SURGERY
CHEMOTHERAPY
RADIOTHERAPY
Classification of Histiocytosis
EM demonstrating ultrastructural Birbeck granules
(cluster between two arrows).
 Unifocal LCH (eosinophilic
granuloma of bone)
Age: 5-15 years
Solitary calvarial lesion in young adults;
other sites of involvement include the
vertebra, the rib, the mandible, the
femur, the ilium, and the scapula .
Asymptomatic or painful.
 Multifocal LCH (Hand-
Schuller-Christian disease)
Age: 2-10 years
Fever, diffuse eruption(scalp, ear canal), otitis
media, mastoiditis, URI, bone lesions, mild
lymphadenopathy, hepatomegaly, and
splenomegaly.
Diabetes insipidus (posterior stalk of the
hypothalamus)
Hand-Schller-Christian triad: calvarial bone
defects, diabetes insipidus, exophthalmos
 Acute disseminated LCH
(Letter-Siwe disease)
Age: <2 y/o
Aggressive systemic disorder
fever; anemia; thrombocytopenia; pulmonary
infiltrates; skin lesions; and enlargement of
the lymph nodes, the spleen, and the liver
Rapid fatal if untreated
With intensive chemotherapy, 5-year survival is about
50%
 Clinical Presentation
Bone
Skin
Hypothalamic/Pituitary axis
Other endocrinopathies
CNS
Lymph nodes
Hepatic enlargement
Others
 Bone
The most common manifestation (80~100% )
Skull (27%), femur (13%), mandible/maxilla (11%),
pelvis (10%), vertebral bodies (8%), ribs (8%),
humerus (5%), and tibia (3%)
The bones of the hands and feet usually spared.
 Skin
Up to 50% with multisystem disease may initially
present with a rash.
Often the first sign of multisystem LCH
Scaly, erythematous, seborrhea-like brown to red
papules, presenting in a fashion similar to contact
dermatitis
 Hypothalamic/Pituitary axis
Hypothalamic involvement:disturbances in behavior,
appetite, temperature regulation, or sleep patterns.
Posterior pituitary involvement: DI
 Other endocrinopathies
Growth retardation
Thyroid hormone deficiency
Precocious or delayed puberty, amenorrhea,
and hypocortisolism
 CNS
Cognitive impairment, emotional lability, changes in
behavior, neurologic dysfunction, pyramidal signs,
cerebellar symptoms, and cranial nerve palsy
(causing difficulties in speech and swallowing)
The most common manifestation is cerebellar
symptoms, followed by pyramidal signs and cranial
nerve palsy
 Lymph node
Lymph nodes are sometimes enlarged in LCH
patients (less than 10%), with those from the
head and neck region preferentially affected
 Hepatic enlargement
Very common in people with disseminated disease
(up to 1/3 to 1/2 of children with disseminated
disease have hepatomegaly) Signify a later stage of
multisystemic disease or a manifestation of a more
fulminant disease process
 Lung disease
Respiratory distress with tachypnea, retraction, and
persistent cough
lung disease is most common in adult LCH patients.
Children with uncontrolled LCH may develop
chronic respiratory failure, presenting with cysts or
bullae
 Treatment
Single-agent chemotherapy is the first
line therapy.(carboplatin, 2-
chlorodeoxyadenosine, chlorambucil,
cyclophosphamide, cytosin, arabinoside, daunomycin,
etoposide, mercaptopurine, methotrexate,
mechlorethamine, procarbazine, vinblastine,
vincristine, vindesine)
Topical steroid, intralesional injection of
steroids, NSAID, phototherapy, bone marrow
allografting, hematopoietic stem cell
transplantation, cyclosporin A,prednisoe.
 Unifocal LCH (eosinophilic
granuloma of bone)
Age: 5-15 years
Solitary calvarial lesion in young adults;
other sites of involvement include the
vertebra, the rib, the mandible, the
femur, the ilium, and the scapula .
Asymptomatic or painful.
 Multifocal LCH (Hand-
Schuller-Christian disease)
Age: 2-10 years
Fever, diffuse eruption(scalp, ear canal), otitis
media, mastoiditis, URI, bone lesions, mild
lymphadenopathy, hepatomegaly, and
splenomegaly.
Diabetes insipidus (posterior stalk of the
hypothalamus)
Hand-Schller-Christian triad: calvarial bone
defects, diabetes insipidus, exophthalmos
 Acute disseminated LCH
(Letter-Siwe disease)
Age: <2 y/o
Aggressive systemic disorder
fever; anemia; thrombocytopenia; pulmonary
infiltrates; skin lesions; and enlargement of
the lymph nodes, the spleen, and the liver
Rapid fatal if untreated
With intensive chemotherapy, 5-year survival is about
50%
 Clinical Presentation
Bone
Skin
Hypothalamic/Pituitary axis
Other endocrinopathies
CNS
Lymph nodes
Hepatic enlargement
Others
 Bone
The most common manifestation (80~100% )
Skull (27%), femur (13%), mandible/maxilla (11%),
pelvis (10%), vertebral bodies (8%), ribs (8%),
humerus (5%), and tibia (3%)
The bones of the hands and feet usually spared.
 Skin
Up to 50% with multisystem disease may initially
present with a rash.
Often the first sign of multisystem LCH
Scaly, erythematous, seborrhea-like brown to red
papules, presenting in a fashion similar to contact
dermatitis
 Hypothalamic/Pituitary axis
Hypothalamic involvement:disturbances in behavior,
appetite, temperature regulation, or sleep patterns.
Posterior pituitary involvement: DI
 Other endocrinopathies
Growth retardation
Thyroid hormone deficiency
Precocious or delayed puberty, amenorrhea,
and hypocortisolism
 CNS
Cognitive impairment, emotional lability, changes in
behavior, neurologic dysfunction, pyramidal signs,
cerebellar symptoms, and cranial nerve palsy
(causing difficulties in speech and swallowing)
The most common manifestation is cerebellar
symptoms, followed by pyramidal signs and cranial
nerve palsy
 Lymph node
Lymph nodes are sometimes enlarged in LCH
patients (less than 10%), with those from the
head and neck region preferentially affected
 Hepatic enlargement
Very common in people with disseminated disease
(up to 1/3 to 1/2 of children with disseminated
disease have hepatomegaly) Signify a later stage of
multisystemic disease or a manifestation of a more
fulminant disease process
 Lung disease
Respiratory distress with tachypnea, retraction, and
persistent cough
lung disease is most common in adult LCH patients.
Children with uncontrolled LCH may develop
chronic respiratory failure, presenting with cysts or
bullae
 Treatment
Single-agent chemotherapy is the first
line therapy.(carboplatin, 2-
chlorodeoxyadenosine, chlorambucil,
cyclophosphamide, cytosin, arabinoside, daunomycin,
etoposide, mercaptopurine, methotrexate,
mechlorethamine, procarbazine, vinblastine,
vincristine, vindesine)
Topical steroid, intralesional injection of
steroids, NSAID, phototherapy, bone marrow
allografting, hematopoietic stem cell
transplantation, cyclosporin A,prednisoe.