Kaleva, V.

Kertikova, S.

Radeva, D.

Tzaneva, V.

English

Varna Medical University Press: Journals

DIAGNOSTIC PROBLEMS AND THEARAPEUTIC BEHAVIOUR IN CHILDREN WITH NEUROBLASTOMA V. Kaleva, S. Kertikova, V . Tzaneva, D. Radeva Key-words: neuroblastoma — diagnosis — treatment — prognosis — children Neuroblastoma is an embryonal tumour originating from stem cells of the sympathetic nervous system and adrenal medulla. That is why it is numbered among neoplasms of the nervous and endocrine system as well. It is the most frequent solid malignant tumour in childhood and presents at the average 6 — 10 per cent of all the malignant neoplasms in children (2, 6, 8). Receatl;, its increasing incidence rate as well as difficulties for early diagnosis and timely treatment pose the problem of this neoplastic process in the centra of the atten-tion of all the oncohaematological departments in our country and in the world. The aim of the present work is to systematize our own experience with the clinical specificity, diagnostic problem and therapeutic behaviour in children with neuroblastoma. Material and methods Case records of 21 children hospitalized in the Pediatric Clinic, Higher Institute of Medicine, Varna, for a 10-year period with a diagnosis of neurobla-stoma were retrospectively analyzed. Patients' distribution according to sex is almost equal: 11 girls (52 per cent) towards 10 boys (48 per cent). 14 children are in the age group up to 4 years (67 per cent), 2 ones are in the age group bet-ween 5 and 8 years (10 per cent) and 5 ones (23 per cent) are 5 years old. Cases with intraabdominal localization predominate — a total of 17 childrei. Neuro-blastoma originates from the right adrenal gland in 8 cases (38 per cent), from the left one in 2 (9 per cent), from paravertebral ganglia in 3 (15 per cent) but its localization is not specified in 4 cases (20 per cent). Two eas^s(9 per cent) are of mediastinal origin and another two (9 per cent) with unspecified locali-zation. Children's distribution according to clinical stages is done after the classi-fication of Evans et al . (1971). A l l the children were admitted to hospital in an advanced phase of the disease: 16 children (75 per cent) were in the fourth and 5 ones (24 per cent) in the third phase. Histological biopsies (intraoperati-vely or post-mortem) were performed in 13 patients. Neuroblastoma was the only one histological result (in 100 per cent of the cases). Results and discussion Clinical symptomatics is determined by localization and size of primary tumour and by metastatic dissemination. Anemia dominates in the clinic al picture in all the cases. Anemic syndrom is persistent and precedes the rest Diagnostic problems and. . . 95 symptoms of the illness for a long period of time. Febrility is observed in 18 children, abdominal pain with intraabdominal localization — in 15 appetite . ] o s s — } п 15, polyadenopathia — in 14, palpebral oedema with haematoma — in 9 (fig. 1, 2); hepatomegalia — in 9, headache, diarrhoe and bone pains — in W oach, exophthalmin 6 (fig- 1, 2), subcutaneous lesions (fig, 2), palpable tu-ig. 1. Palpebral oedema with haematoma, Fig'. 2. Subcutaneous metastatic lesion, xophthalmus. meur in the abdomen — in 4 each, syndrome of radiculitis — in 3, exanthema — in 2, pareses, obstipation and icterus — in one case each. Concerning routine laboratory examinations, it stresses that there is a per­sistent anemia of hypochromic type as well as E S R elevation. Biochemical and other examinations of the peripheral and venous blood did not indicate any significant abnormalities. We present the number of positive results from the examinations by means of all the methods applied in our study of different num­ber of patients in order to reveal the diagnostic possibilities for every single method: Roentgenologic examination: mediastinum — 1 whole abdomen — 2 bone metastases —6 (out of 17 cases) venous urography — 12 (out of 17 cases) Echoscopy of the abdomen: tumour — 10 (out of 17 cases) 96 V. Kaleva, S. Kertikova, V. Tzaneva, D. Radeva liver and- lymph node metastases Urinary metabolites (vanillo-mandelic acid and homo-vanillic acid) Myelogram: metastases reactive changes Fine-needle aspiration biopsy: lymph nodes subcutaneous lesion liver — 9 8 10 bone lesion — 2 (out of 5 cases) — 4 (out of 4 cases) — 1 (out of 1 case) — 1 (out of 1 case). Roentgenography of the whole ab-domen proves seldom a tumour shadow which is rater on more precicely loca-lized by echoscopy and venous urography (fig. 3). The latter almost always indi-cates a renal dislocation in caudal and-lateral direction as well as a conside-rable deformation, rotation and devia-tion of ureters with outwards convexity. Rcentgenologically, bone metastases show numerous osteolytic foci involving most frequently cranial, pelvic and femcral bones (fig. 4). Bom:-marrow metastases are estab-lished in 45 per cent of myelograms per-formed in a total of 18 children (fig. 5). Cytological finding does not present and differential-diagnostic difficulties with other systemic blood diseases. Fine-needle aspiration biopsy has been introduced as a routine method for examination of peripheral lesions and enlarged lymph nodes since 1987. Despite the small number of investigations the results obtained confirm its diagnostic importance and role for clinical stage determination (fig. 6) (9, 10). j A complex treatment: surgical eli-mination of the tumour, radiation the-rapy and chemotherapy is carried out in 4 patients only. Three of them are in the third and one in the fourth stage of the illness. Only chemotherapy is admini-stered in 5 children in the fourth stage but combination of chemotherapy and radiation therapy in 2 children in the third and 10 children in the fourth stage. Therapeutic behaviour is conformed to the clinical stage at the first place as well as to the age of the child, primary tumour size and localization and meta-stases (4, 7). A triple combination of cyclophosphamide (at a doses of 10 mg/kg b. w,), vincristine (1.5 mg/njf2) and adriamycine (60 mg/m2) was applied. A total radiation dosage of 15 — 60 Qry was realized. Fig. 3. Venous urography the right kidney-is dislocated in external and caudal di-rection by neuroblastoma originating from the corresponding adrenal gland. 7 Scripta scientica medica V. Kalcva, S. Kcrtikova, V. Tzaneva, D. Kadeva Prognosis depending mainly on the clinical stage, age, primary tumour localization and histological result is confirmed as extremely poor concerning the patients in the third and fourth stage of the disease. There is a 3-month survival in 19 children, a 6-month one — in 13, one year one — in 8, 18-month Fig. 6. Fine-needle aspiration biobsy of a peripheral lymph node — metastasis of well-d} -fferentiated neuroblastoma (Slain after Giemsa Microphoto x 40Э). one — in 4, and over two-years survival — in one patient (his survival already is longer than 10 years) with primary mediastinal tumour localization. It has to be noted that our results coincide with literature data available about the distribution of the disease according to sex, age and localization. However, there are some differences concerning the absence of initial stages and other histological varitions except neuroblastoma. 90 per cent of the patients are rather late hospitalized (after the third month since the initial symptoms ) ( 1 - 3 , 5). We can conclude that analogous clinical symptomatics is presented in any patients. The highest specificity is demonstrated by: anemia (in 100 per cent of the cases), febrility (in 85 per cent), pain (71 per cent), palpebral oedema (in 43 per cent) and exophthalm (in 28 per cent). Diagnosis is realised by the com­bination of roentgenological, sonographic and cytomorphological examination in the greatest percentage (75) of the cases. Concerning the fine-needle aspira­tion biopsy it is to be outlined that cytologic data about distant metastases are established in 100 per cent of the cases with peripheral subcutaneous le­sions and in 40 per cent of the peripheral lymph noies. Combination of chemo­therapy and radiation therapy is the most frequently used therapeutic approach (in 55 per cent of the cases) in conformitv with the clinical stage of the disease, Diagnostic problems and. R E F E R E N C E S 1. Б о б е в , Д . , M. К а м е н о в а , I I . Х р и с т о з о в а , P. С а в о в а , Ш. Н и н ь о. Педиатрия, 1984, № 4, И—16. — 2. Д о н ч е в а, Н. Докт. дис. София, 1979. — 3. М и т р о в , Г. (ред.) Злокачествени новообразувания. София, Медицина и физ­култура, 1983. — 4. Р у с е в а , Ц. Канд. дис. София, 1980. - 5. Х р и с т о з о в а , И. Канд. диес. Москва, 1983. —- 6. X р и с т о з о в а, И. Педиатрия, 1988, № 2, 4—7. — 7. Х р и с т о з о в а , I I . Педиатрия, 1988, № 2, 1—4—8. E v a n s , A. E., G. J . D' A n g i o , J . R a n d o l p h . Cancer. 27. 1971, No 2, 374-378. — 9. Miller, T. R. , K. B o t t l e s , J . S. A b e l e , J . H. B e c k s t e a d . Acta Cytol., 29, 1985,461—468. — 10. Perikala, V. , Kumar., Acta Cytol., 31, 1987, No 5, 583—586. Д И А Г Н О С Т И Ч Е С К И Е П Р О Б Л Е М Ы И Т Е Р А П Е В Т И Ч Е С К О Е П О В Е Д Е Н И Е П Р И Л Е Ч Е Н И И Д Е Т Е Й С Н Е Й Р О Б Л А С Т О М О Й ' * ". { и i I* ; . - ! В. Ka.ieei, С. Кфтл&озл, В. Цаиеоа, Д . Радева : 1 Р Е З Ю М Е Изучаются начальные проявления, клинические особенное!и, диагностические про­блемы и терапевтическое поведение при лечении двадцати одного ребенка, лечившихся в Детской клипике при Высшем медицинском институте в Варне в течение десяти лет с диаг­нозом: ненробластома. У всех дете~г установлена аналогическая начальная симптоматика. Клинический поли­морфизм обусловлен преимущественно злокачественными метастазами, находящимися в четвертой стадии в 75% случаев, что ограничивает комплексный подход при лечении. Диагностические проблемы связаны на первом месте с установлением первичной ло­кализации опухоли. Обсуждается диагностическая ценность топкоигольнон аспирационнон биопсии и ^хоскопии с целью определения клинического стадия заболевания. 

DIAGNOSTIC PROBLEMS AND THEARAPEUTIC BEHAVIOUR IN CHILDREN WITH NEUROBLASTOMA

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DIAGNOSTIC PROBLEMS AND THEARAPEUTIC BEHAVIOUR IN CHILDREN WITH NEUROBLASTOMA

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