Rare
Locations
of Malignant
Tumors
in Children
By DUBANBAJEC,PETARRAD~JKOVI~,IVANBEDEVIL, ANDKONSTANTIN BRANKOVAN
T
HE DIFFERENCE BETWEEN malignant neoplasms in children and similar lesions in adults lies in their biological characteristics, origin,
and location. During the past 5 years 211 children with primary malignant diseases were treated at the Institute of Child Health in Serbia, Yugolslavia. In this paper we will pay attention to the primary malignant neoplasms localized in the respiratory and gastrointestinal systems, where such tumors appear only rarely in children. Table 1 shows the list of tumors according to their incidence. It may be seen that the greatest number of children have been treated for leukemia and the smallest for carcinomas. We have selected 16 neoplasms from the last group of miscellaneous tumors with primary location in the respiratory and gastrointestinal systems, which we consider to be of special interest from the diagnostic and therapeutic points of view because of their exceptional occurrence and location. Primary Malignant Tumors of the Pleura, Bronchi, and Lungs From 1963 through 1968 we have observed 4 children with primary malignant tumors in the respiratory system, a pleural mesothelioma, one with primary anaplastic carcinoma in the bronchus: and two children with rhabdomyosarcoma of the lung. All these children have had surgical treatment, irradiation, and chemotherapy. Two children died and two are still alive. Table 2 presents symptoms and clinical signs manifested by these neoplasms. The 4-year-old child with malignant pleural mesothelioma didn’t show any serious signs in the beginning of the disease, except irritating cough and pain on inspiration. Only during the x-ray examination a shadow of the tumor was discovered, which was localized in chest wall. Complete surgical excision has been carried out together with a part of the thoracic wall. After 4 months the tumor reappeared at the same location. The tumor was excised and three ribs resected. Due to metastases in the pericardium and lungs the child died 6 months later. From the DU~AX BAJEC,
of Child Health
Serbia, Serbia,
in Pediatric Surgery, Postgraduate School of Medicine; Assistant Professor, Un~uersity of Belgrade. PETAR RADOJKOVIC,M.D.: Surgeonin-Chief, Second Division, Department of Pediatric Surgery; Lecturer in Pediatric Surgery, Postgraduate School of Medicine, Belgrade. Ivo BESEVIC, M.D.: Surgeon-in-Chief, First Division, Department of Pediatric Surgery; Lecturer in Pediatric Surgery, Postgraduate School of Medicine, Belgrade. COLONELKONSTANTIN BRANKOVAN, M.D.: Director, Institute of Pathology, Military n4edical Academy; Professor of Pathology M.M.A., Belgrade.
JOURNALOF PEDIATRIC SURGERY,VOL. 3, No. 6 (DECEMBER),1968
749
750
BAJEC
Table
2.-Primury
ET AL.
Tumors of the Pleura, Bronchi, and Lungs
Leukemia Tumors of sympathetic nervous system Tumors of central nervous system Nephroblastoma, Wilms Malignant tumors of lymphoid tissue, except rare localization tumors Soft tissue sarcoma and undifferentiated sarcoma Bone tumors Teratoma malignum, except rare localization Carcinoma, except rare localization Miscellaneous-malignant tumors with rare occurrence and localization
93
20 17 13 13 8 7 6 4 30
An S-year-old girl with anaplastic carcinoma of the left bronchus had been admitted to our hospital two months after the onset of symptoms. She complained of pain in the chest, cough, and bloody expectoration. The diagnosis of carcinoma had been established with bronchoscopy on which occasion a part of the tumor obstructing the bronchus was removed. Thoracotomy confirmed that the carcinoma was inoperable with pericardial and diaphragmatic metastases. The symptoms of the rhabdomyosarcoma of the lung in two children, 11 and 7 years old, have not been very characteristic. Both children had fever, undifferentiated chest pain, and irritating cough. A tumor shadow was discovered by x-ray examination in one child in the right, and in the second in the left lower lobe. We performed lobectomy in both cases, because no signs of tumor spread in the pleural space or lymph nodes could be found. One child died 2 years after the operation and the other child is still alive, without evidence of metastasis. Primary malignant neoplasms of the bronchial and lung tissues have rapid growth in children, although it seems that rhabdomyosarcoma is limited for a longer period to one of the lung lobes. Carcinoma with its intrabronchial growth brings about more rapid and complete obstruction of the bronchial tree than rhabdomyosarcoma with its extrinsic compression. The evolution of the malignant disease depends upon the speed and expanding growth of the tumor. In bronchial carcinoma, the diagnosis is established most safely with bronchoscopy; in rhabdomyosarcoma and pleural mesothelioma by x-ray examination. Neoplasms
of the Stomach
and Small Bowel
Malignant tumors appearing in the stomach and small bowel in children may develop inconspicuously until the signs of intestinal obstruction or hemorrhage appear. Before the appearance of such signs the child occasionally complains of abdominal pain. Such neoplasms could differ in their appearance, location, and development. They may grow into the lumen in which case they have the shape of a cauliflower with broad or narrow base. They may occasionally cause bleeding and intestinal obstruction.
RARE
LOCATIONS
OF
MALIGNANT
751
TUMORS
Table l.--Incidence
of MaEignant Diseases in Infancy and Childhood Treated at the Institute of ChiEd Health of Serbia, 1963-1967
Diagnosis
Pleural
mesothelioms
Carcinoma of the left main bronchus
Age
Sex
Cough
Hemoptysis
Chest Pain
FWW
1
M
+
0
+
0
Asthenia
0
8
I’
+++
+++
++
0
0
Rhabdomyosarcoma of the left lower lobe of the lung
11
M
+
0
++
++
0
Rhabdomyosarcoma of the right lower lobe of the lung
7
F
++
0
+
0
++
Such symptoms have been manifested by a leiomyosarcoma of the stomach in a I.5month-old child (Table 3). We have found at operation in two children \vith reticulosarcoma of the jejunum partial obstruction in one case and in the other, intussusception (Table 3). Lymphosarcoma shows more marked tendency to infiltrate the stomach or intestines. Because it develops very quickly, this neoplasm narrows the lumen and grows into mesentery and surrounding organs. Because of their extramural growth these neoplasms can sometimes be diagnosed through palpation of the abdomen. The diagnosis can be generally established by the symptomatology. In the clinical picture of all children with tumors of the stomach and small bowel. pain and signs of intestinal obstruction are dominant. The child with leiomyosarcoma of the stomach had hematemesis and the child with sarcoma of the ileum had melena. Tumor mass could be palpated in all children while the x-ray signs indicated that partial intestinal obstruction existed in three patients. Malignant neoplasms of the gastrointestinal tract in adults are chiefly carcinomas while in children sarcomas predominate. Although they are ver7 rare in children, such tumors are not histologically uniform. Tumors of the gastrointestinal tract should be removed radically and simultaneous block resection of the infiltrated mesentery should be made. This operation could not be performed in two children, one with lymphosarcoma of the stomach and other with lymphosarcoma of the ileum because of the involvement of mesenteric nodes and other intra-abdominal organs. The children received x-ray treatment after prior removal of the tumor with broad resection of the involved bowel. One child has died and the other is living, 2 years after surgery. However, as the children received chemotherapy at the same time it is difficult to evaluate the treatment. Primury Tum(ors of the Liver The heterogenous cell tissue and the different functions of the liver condition the appearance of various malignant primary neoplasms of this organ. Thev might consist of liver cells, biliary duct cells, connective tissue, etc. Tab& 4 indicates diagnosis and treatment of primary malignant tumors. There were malignant teratomas in two infants clearly localized in one child in the right, and in the other child in the left lobe of the liver. Both
M
F
M
8 yrs.
3 yrs.
3 yrs.
of
M
5 yrs.
of
Lymphosarcoma of the ileum Lymphosarcoma of the ileum and cecum
Reticulosarcoma the jejunum
M
6 yrs.
of
Lymphosarcoma the stomach Reticulosarcoma the jejunum
Sex
F
Age
15 mos.
of
Leiomyosarcoma the stomach
Diagnosis of the
Resection of small bowel, chemotherapy, leukeran, irradiation Resection of small bowel chemotherapy, antimit and oncovin Resection of the tumor Chemotherapy, irradiation Biopsy only
Partial gastrectomy, recurrence, resection stomach Biopsy only
Therapy
Table 3.Etomach
++ 0
+
++ Tntussusception
+++
+++
Intestinal Obstruction
+ 0
0
0
0
++-I-
Gastrointestinal Hemorrhage
and Small Bowel Neoplaems
i-+
0
-t
++
++
++
Abdominal Pain
++ +-t-t
++-t
+++
++-t
+-t-
Abdominal Mass
0 0
0
+++
0
0
Destructive Jaundice
RARE LOCATIONSOF MALIGNANTTUMORS
753
tumors were of solid consistencyand sharply delineated SO that we could remove them completely. Both children are healthy 2 and 3 years postoperatively without signs of metastasis. In two children a carcinoma of the liver has been found, one histologically verified as hepatoma (hepatoblastoma) which was biopsied only because the tumor had infiltrated the biliary ducts. In the other child in whom adenocarcinoma of the liver has been diagnosed, lobectomy of the left lobe was performed. In spite of chemotherapy both children died in the early postoperative period. Reticulosarcoma, which is considered as one of rarest of primary tumors of the liver, was observed in only one child. We have excised three separate tumors from the liver tissue. Although radiation and chemotherapy were given, the child died of lung metastasis. A case of mesenchymoma of the liver, consisting of various mesodermal tissue deserves special attention. This tumor was well localized in the left lobe, with a broad base, below Ghsson’s capsule. Two years before, the tumor had been completely removed only to reappear more than a year later. The tumor weighing 7 lbs. was removed again together with the left lobe of the liver and gallbladder. During a third operation a tumor weighing 16 lbs., which had grown into the left diaphragm and hilus of the spleen, was again removed along with the spleen and a part of diaphragm. Neoplasms of the liver usually present as a mass in the right half of the abdomen. The child usually complains of pain (Table 4). An enlarged liver could be palpated in all our cases. The tumor compressed the stomach and duodenum in three cases and the biliary ducts in two cases when clinical signs of jaundice appeared as well. In 4 children ascites were found due to the compression of the tumor on the portal circulation. CONCLUSION The neoplasms presented here deserve attention because they are considered exceptional. The diagnosis is usually not established early enough when favorable results could be expected from surgical treatment. We have presented 16 cases of primary tumors in rare locations. If the biology and evolution of such tumors were better known it could be expected that the diagnosis might be established earlier. Thus the surgical results could be improved and the mortality decreased. With the present knowledge of the biology of malignant neoplasms in children, surgical treatment is most efficient. Since the child has a Iong life expectancy there is no sense in palliative surgery. Whenever there exists a material chance to remove a malignant tumor completely, radical excision should be performed even at the price of significant mutilation of the organ from which the tumor originates. It is difficult to evaluate the significance of postoperative irradiation and chemotherapy of these malignant tumors in children because experience is insufficient. Because tumors of childhood grow so rapidly, surgery is a relative emergency.
3 yrs. 14 yrs.
10 mos.
Adenocarcinoma Mesenchymoma
teratomn
Malignant
F F
M
F
M
mos.
8
4 yrs.
teratoma
Malignant
M
Sex
7 mos.
Age
Reticulosarcoma
t’eratoma
Malignant
Diagnosis
Malignant
1 Resection of the tumor 2. Partial resection of liver, chemotherapy Biopsy only, metastasis Excision of tumor, chemotherapy Extirpation of three tumors, chemotherapy L. Iobectomy 1. Excision of the tumor 2. L. lobectomy, cholecystectomy (Weight 16-lb)
Therapy
Table 4 .-Primary of
+t-f
++ -I-
+++
+-t-i++
++
+++ +++
++
++ +++
Ast,henia
the Liver
Enlargement of the Liver
Tumors
0 0
+++
++
++
0
0 0
++ a
Gastric Symptoms
+++
0
Jaundice
+ + -I-
-I-+ +
0
I-f
0
Ascites
RARE
LOCATIONS
OF
MALIGNANT
SUMMARIO In le curso de1 passate
cinque
755
TUMORS
annos,
IN INTERLINGUA 211
juveniles
con primari
morbos
mahgne
esseva
tractate al instituto pro le sanitate de juveniles in Jugoslavia. Le serie includeva 16 cases de primari tumores maligne in le systemas respiratori e gastrointestinal, i.e., in organos in que tal tumores es rar in le population pediatric. Viste que quasi omne le patientes esseva tractate post le chirurgia con agentes difficile evalutar differente modos de tractamento.
chimotherapeutic
e con
irradiation,
il es
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J. C.: Tu-
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3. Womack, Mixed tumors 195, 1938.
E.: 26:
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