Ministry of public health and social development of the Russian Federation
Federal government-financed institution
Federal scientific and clinical Centre of child hematology, oncology and
immunology after Dmitry Rogachev
18 June 2012, Moscow
№ 893
Extract from the patient medical record № 2012/1394
18 June 2012
Sakharova Anna Mikhailovna, 21 September 2009 (aged 2,9).
Address: 27 Pionerskaya Street, Kineshma, Ivanovo region.
Anna is in clinical oncology unit from 21 May 2012 to nowadays with the
following diagnosis: neuroblastoma of retroperitoneal space on the right, the
4th stage, high risk class.
Condition after the 1st block of maintenance chemotherapy.
Complaints: hip joint ache, which requires anaesthetic (Nise), related to this
symptom gait malfunction (is lame mainly in the left leg); poor appetite, troubled
slumber, mild pyrexia (maximum temperature – 37,2 C).
Life anamnesis: the second pregnancy child, there was a threat of termination of
pregnancy from the 12th week, toxicosis 1,3 trimester. The second delivery at 37
weeks. Birth weight – 3800 grammes, body length – 52 centimetres. Prophylactic
inoculations: according to the individual schedule. Allergy: juices (orange),
chocolate – allergic appearance of skin rash. Drug allergy: gentamicin (allergic
appearance of skin rash). Heredity: great grandmother on the mother’s side –
stomach cancer; great grandfather on the mother’s side – stomach cancer. Had the
following illnesses: URTI (upper respiratory track infection), bronchitis,
pharyngolaryngitis, laryngotracheobronchitis.
Past medical history (disease anamnesis): Anna is sick from February 2012, when
stomach ache appeared, mainly in the right segments. She was hospitalized into
pediatrics ward of municipal children’s hospital in Kineshma, where according to
the results of US of abdominal cavity organs and kidneys (13 February 2012) there
was discovered space-occupying lesion of the right kidney. The patient was sent to
the planned urologist’s consultation and hospitalized into urology unit for
additional examination. From 12 March 2012 to 26 March 2012 she was on inpatient treatment in the children’s urology unit of Ivanovo regional hospital.
Carried examinations: 1) excretory urography – on the 6th minute after intravenous
contrast insertion it is possible to detect options of both kidneys. The right kidney
is situated lower than it is necessary, rotated, the lower pole is more medial than
the upper pole. Pyelocaliceal systems are not broadened from the both sides.
Ureters are not broadened, have cystoid structure. Contrast in the urinary bladder is
seen. 2) US of kidneys: the right kidney is typically situated, size 62 х 30 mm,
contour is accurate, parenchyma thickness – 12 mms, pyelocaliceal systems are not
broadened. In the projection of the upper pole there are several rounded formations
13,5 – 19,? – 20,5 mms in diameter, hyperechoic, with heterogeneous structure,
common size – 42 х38 mm. The left kidney is typically situated, size 68х33 mm,
contour is accurate, parenchyma thickness – 11 mm, pyelocaliceal systems are not
broadened. It id not possible to detect organ belonging of the formations in the
projection of the upper pole of the right kidney (maybe adrenal, maybe kidney).
Medical opinion: formation of retroperitoneal space. Medical advice: MPR of
retroperitoneal space organs with the aim of specification of diagnosis.
In connection with acute bronchitis the patient was discharged from hospital under
medical (pediatric) supervision.
Repeated hospitalization: 23 April 2012 – 14 May 2012. CT scanning of
abdominal cavity organs was conducted on 1 May 2012. According to it there is
more information for space-occupying lesion of the right adrenal. (There are discs
with the recording of the examination; the report of the examination is absent).
Blood test on Vanillyl mandelic acid (information from 14 May 2012): heightened
content of VMA in urine (positive response)++.
The patient is hospitalized into the federal scientific and clinical Centre of child
hematology, oncology and immunology after Dmitry Rogachev for conducting
additional examination and determination of further tactics of therapy.
On entrance to the department:
Temperature – 36.6C
Height – 95 cm
Weight – 13,9 kg
Cardiac rate – 105 per minute
Respiratory rate – 21 per minute
Serious condition of the underlying disease.
The patient feels rather well. Consciousness is clear. Reaction is adequate. Skin is
of the appropriate color, moderately moist. Isolated ecchymosis on the left knee on
the stage of solution. On the front surface of shanks and hips there are smallspotted elements (according to the patient mother’s words these are mosquito’s
bites). Turgor of skin is preserved.
Icteritiousness of skin and mucous membranes is absent.
Visible mucous membranes are pale-pink, clear, moist.
Skin hemorrhagic syndrome is absent.
Hypodermic adipose tissue is well-developed, uniformly distributed. Peripheral
edemata are absent.
Peripheral lymph nodes are palpated: anterior cervical, axillary, inguinal up to 1,0
cm, movable, painless, soft, flexible.
Musculoskeletal system is developed in concordance with age of the patient,
visible defects and deformations are absent.
Respiration through nose is not hindered. Short breath is absent. Under lungs
auscultation respiration is puerile, crackling is absent. Vesicular resonance.
The region of heart is without changes. Heart sounds are clear, loud. Systolic
murmur is under all spots of auscultation. Cardiac dullness is within the age limit.
Pulse of the peripheral arteries is normal.
Oral cavity is without visible changes. Enlarged tonsil without pathologic
superposition.
Abdomen is soft, painless in all segments. Liver +1 cm from the edge of costal
margin, spleen is not palpated.
Stool is once in two days, without pathologic admixture, shaped.
The region of kidneys is without changes. Kidneys are not palpated, the palpation
region is painless. Tapotement syndrome is negative from the both sides. Urination
is under control. Diuresis is adequate.
Neurological condition: without peculiarities.
Meningeal symptom is absent.
Conducted examination:
General blood analysis, 22 May 2012: white blood cells – 6,41х109/l, hemoglobin –
90 g/l, platelets - 146 х109/l; leukocytic formula: myelocytes 1%, segmental
leukocytes 21%, eosinocytes 5%, lymphocytes 69%, monocytes 4%.
Biochemical analysis of blood, 22 May 2012: uric acid 305 millimole/litre (0,143 –
0,339), lactate dehydrogenase 521 units/litre (90 – 330), ferritin 131
microkilogram/litre (6 – 60), other indicators are within the age limit.
Immune-enzyme analysis – cancer specific marker NSE, 22 May 2012: 65,262
microkilogram/litre (0-13).
Radiography of chest organs (straight projection), 22 May 2012:
Lung fields are without nidal and infiltrative changes. Lung marking is enriched at
the expense of interstitial component. Roots are overlapped by mediastinum
shadow. Mediastinum shadow is of normal configuration. Cupula of diaphragm
occupies typical position, it is clear, smooth. Sinuses are free.
ECG, 22 May 2012: vertical position of electrical axis. Moderate sinus arrhythmia.
Malfunction of the repolarization process in infarction ventricular (ST segment
upsurge in V4-V6 up to 0,5 mm).
General urine analysis, 23 May 2012: relative density 1,022, hydrogen indicator
6,5, protein and glucose are not discovered, red blood cells 0-2 in the visual field,
white blood cells 0-1-3 in the visual field, bacteria 24,3 х103/l, amorphous
phosphates are in quantity.
Echocardiogram, 23 May 2012: contractile myocardium function is preserved.
Cardiogram of chest organs and abdominal cavity under Kulenkampff’s anesthesia,
23 May 2012:
Chest: nidal changes inside lungs and enlargement of intrathoracic lymph nodes
are absent. Osteoplastic body affection of Th6 vertebra.
Abdominal cavity, pelvis: soft-tissue space-occupying lesion of the retroperitoneal
space on the right, which probably comes from the right adrenal – it may be
neuroblastoma. Multiple enlarged lymph nodes of the retroperitoneal space – Mts.
Polyaxes osteoplastic Mts-affection of lumbar vertebrae.
Description: CHEST: Examination was conducted according to standard methods.
In the obtained CT scans: in lungs there are no nidi and focuses of parenchymatous
infiltration. Symptoms of pulmonary ventilation malfunction are absent, airway
conductance is not infringed. The roots of the lungs are structural, not enlarged.
Mediastinum is not displaced, not enlarged. Mediastinum structures are clearly
distinguished. Lymph nodes enlargement and additional space-occupying lesions
in mediastinum and the roots of the lungs are absent. Free liquid in pleural cavity
and bone destructive changes are absent. Uneven chaotic infiltration (hardening) of
the body structure of the Th6 vertebra.
ABDOMINAL CAVITY, PELVIS: Examination was conducted according to
standard methods with usage of intravenous bolus vascular opacification. In the
obtained CT scans: there is massive soft-tissue space-occupying lesion of the
anomalous shape in retroperitoneal space on the right (size 90 х40х53 mm), which
probably comes from the right adrenal and spreads on the vascular pedicle of the
right kidney. Contours of the lesion are clear, uneven, symptoms of rooting of the
lesion into the right kidney are absent. Its structure is heterogeneous at the expense
of multiple small calcifications. The lesion broadly intimate belongs to the right
segment of the liver, sharply deforms its contour – we don’t think there is invasion
of liver capsule, however it can not be excluded. The lesion is small and
accumulate contrasting substance unevenly. In the retroperitoneal space there are
multiple enlarged lymph nodes (from 5-6 mm up to 34х28), which are mainly
situated in the hepatoduodenal ligament projection and along the vascular pedicle
of the right kidney. The structure of the enlarged lymph nodes conglomeration is
identical to the structure of the space-occupying stomach lesion described above.
There are no authentic symptoms of nidal affection of spleen, liver, kidneys and
pancreas. Urinary bladder is not enlarged, its wall looks thickened, nested. Free
liquid in the abdominal cavity and pelvis cavity is absent. Bone destructive
changes are absent. There is uneven chaotic infiltration (hardening) of the body
structure of all vertebrae.
23 May 2012: in the operating theatre under anesthetic the central venous catheter
with bone-marrow puncture of 4 points. Material was referred to the
morphological, cytogenetic and molecular-genetic examinations MYCN.
Postoperative period is without peculiarities.
Myelogram, 23 May 2012: malignant cells, which are normally situated, and
creating lesions are visualized in all points, in №3 – near-total metaplasia.
Cytogenetic analysis of identification of the gene NMYC amplification by PCR
method, 23 May 2012: additional copies of the gene NMYC are not discovered
(bone marrow, 4 points).
Cytogenetic analysis of identification of the gene NMYC amplification by FISH
method, 23 May 2012: the gene NMYC amplification is absent. 1p deletion (67%
of nuclei. Also there is add 11q 17q.
Results of scintigraphy with MIGB, 24 May 2012: presence of active specific
tissue of neurogenic character (nidi of heightened accumulation of radioactive
drugs) in the projection of retroperitoneal space on the right, in the projection of
cranial bones, vertebral column, pelvis bones, sternum, shoulder-blades, humeral
bones, femoral bones, fibulae.
28 May 2012: at the first stage diagnostic operation was conducted: trepanobiopsy
of the neoplasm. Postoperative period is without peculiarities.
The results of histologic study: quantity of material is not enough to make a
conclusion.
MRT-scan of brain with contrast enhancement, 31 May 2012:
Description: in the number of MR-scans of brain there are no pathologic spaceoccupying lesions, nidi of pathologic accumulation of contrast medium in
substance of the cerebral hemispheres, brain stem and cerebellum. There are small
(up to 2-3 mm) multiple segments liquid-based MP-signals – dilation of
perivascular space – in the region of nucleus basales and hippocampus, not
numerous ones in white substance of the cerebral hemispheres and the cerebral
peduncle. Midline structures are not displaced. Ventricular system is not deformed,
no material dilation. In the front segments of the right lateral ventricle in T2FLAIR it is possible to detect the lesion of high signal, of oval shape, size 8х5 mm,
with clear contours. In the ordinary T2-VI (vi) and native T1-VI (vi) the lesion is
not visualized, so as it does not differ from liquor. The lesion does not accumulate
contrast medium. Convexital subarachnoid space, fissures of the cerebral cortex,
cerebral hemisphere is not enlarged. Cerebellar tonsils do not substantially go
beyond Chamberlain line. There are diffuse changes in the structure of the
cuneiform bone and the occipital bone and moderate enlargement of their volume.
Sphenoidal sinus is not detected – not formed (aplasia) or filled with extraosseous
pathologic soft-tissue component (thickness is up to 5 mm), which accumulates
contrast medium intensively. There are also small (maximum thickness is up to 3
mm) number of soft-tissue component is detected on the fundus of lateral segments
of the middle cranial fossa from the both sides.
Conclusion: small contrast negative additional lesion in the body of the right
lateral ventricle – cyst of vascular plexus? Structural changes of the cuneiform
bone and occipital bone, with the presence of small intracranial soft-tissue
component – probably secondary neoplastic lesion; it is reasonable to conduct CTscanning of the state of the skull base bones.
General blood analysis, 17 June 2012: white blood cells – 4,04х109/l, hemoglobin –
81 g/l, platelets - 45 х109/l.
Treatment that was conducted in the department:
From 30 May 2012 to 2 June 2012 one N5 block according to NB-2004
protocol was conducted:
Vincristine 1,5 mg/m2 the 1st day intravenous (single dose = 0,9 mg, daily dose
(per diem) = 0,9 mg),
Cistplatin 40 mg/m2 1 – 4 days intravenous (single dose = 24 mg, daily dose =
96mg),
Vepesid 100 mg/m2 1 – 4 days intravenous (single dose = 60 mg, daily dose =
240mg).
The block was conducted together with standard accompanying therapy; was
moderately sustained.
From 28 May 2012 to 9 June 2012 (because of episode of febrile fever):
Amoksiklav 140 mg х 3 times per twenty-four hours intravenous within an hour.
From 7 June 2012 to 15 June 2012 (because of multiple liquid stool):
Amikacin 100 mg х 2 times per twenty-four hours per os (5 ml = 5% of glucose).
Transfusion of erythrocytic material, 9 June 2012 (130 ml).
Thrombocyte concentrate: 10 June 2012 (2 doses), 11 June 2012 (1 dose), 12 June
2012 (1 dose).
From 5 June 2012 up to nowadays – parenteral nutrition – kabiven 20 ml/kg.
From 28 May 2012 up to nowadays – infusion therapy.
Conclusion:
Diagnosis substantiation:
1. on the ground of the anamnesis data, visualization (US, CT-scanning), there
is the tumourous lesion in retroperitoneal space on the right (which probably
comes from the right adrenal) with metastatic affection of retroperitoneal
lymph nodes and lumbar vertebrae;
2. taking into account child’s age at the moment of the beginning of disease
(aged 2,8);
3. typical location of the lesion (retroperitoneal space on the right, coming
from the right adrenal);
4. taking into account the presence of typical genetic marker – 1p deletion,
which was received after cytogenetic analysis of bone marrow by FISH
method;
5. taking into account positive blood response on catecholamines’ metabolites,
considerable increase of the level of the cancer marker NSE;
6. body scintigraphy with MIGB – the presence of active specific tissue of
neurogenetic nature (nidi of heightened accumulation of radioactive drugs)
in the projection of the retroperitoneal space on the right, in the projection of
skull bones, vertebral column, pelvis bones, sternum, shoulder-blades,
humeral bones, femoral bones, fibulae;
7. myelogram data – tumourous cells in all points of the bone-marrow
puncture. It is possible to talk about neuroblastoma of retroperitoneal space
on the right.
Taking into account the presence of disseminated tumour (metastatic affection of
skeleton bones, skull, lumbar vertebrae, retroperitoneal lymph nodes, bone
marrow) there is the 4th stage of the disease.
In concordance with child’s age, more than 1 year (at the moment of the diagnosis
ascertainment – aged 2,9), the stage of the disease (the 4th), it is obligatory to
include the child into the group of high risk in concordance with the NB-2004
protocol.
To child, Sakhorova Anna Mikhailovna, 21 September 2009 (aged 2,9), with
the diagnosis: neuroblastoma of retroperitoneal space on the right, the 4th
stage, high risk class.
Condition after the 1st block of maintenance chemotherapy – it is necessary to
conduct medico-social examination for the ascertainment of the status
“disabled child” in concordance with the lost of the functions of health.
The extract from the patient medical record is sent to give it at the place of abode
for conducting medico-social examination for the ascertainment of the status
“disabled child” in concordance with the lost of the functions of health.
Deputy head doctor
Acting division superintendent of clinic oncology
Attending medical doctor
Litvinov
Moiseenko
Shumilina