|Year : 2015 | Volume
| Issue : 2 | Page : 61-65
Carcinoma in children at Ahmadu Bello University Teaching Hospital Zaria
Bello Usman, Abdullahi Mohammed
Department of Pathology, Ahmadu Bello University Teaching Hospital (ABUTH), Zaria, Niger
|Date of Web Publication||2-Mar-2016|
Department of Pathology, Ahmadu Bello University Teaching Hospital (ABUTH), Zaria
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Introduction: Carcinomas are an important cause of morbidity and mortality among adults but are relatively uncommon in children. However, a significant number of carcinomas do occur in children, with debilitating consequences.
Materials and Methods: This is a retrospective study of all the carcinoma diagnoses in children aged 15 years and below during the 17 years' study period. Laboratory records from the Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria were extracted and reviewed. The findings were compared with similar studies elsewhere.
Results: There were 41 children with confirmed cases of carcinoma, with 24 males and 17 females. The male-to-female ratio was 1.4:1. The peak age of incidence was 11-15 years. Patients aged less than 6 years constituted only 9.8% of cases. The commonest sites were the skin (29.3%), colon (22.0%), nasopharynx (14.6%), and major and minor salivary glands (14.6%).
Conclusion: Carcinomas in children do occur, but are rare. These tumors are diverse and site-specific in their epidemiology, histogenesis, natural history, biologic behavior, morphology, and prognosis.
Keywords: Carcinoma, children, histopathology
|How to cite this article:|
Usman B, Mohammed A. Carcinoma in children at Ahmadu Bello University Teaching Hospital Zaria. Ann Nigerian Med 2015;9:61-5
| Introduction|| |
Carcinomas are malignancies that originate in epithelial tissues. Epithelial cells cover the external surface of the body, line the internal cavities, and form the lining of glandular tissues and parenchyma of solid organs. Carcinomas are very rare among children, particularly before the adolescent years.  Cancers that originate from the epithelial cells, including those of the breast, lung, prostate, liver, and colon, are by far the most common types of cancer in adults.  Among children, the common types include leukemias; lymphomas; central nervous system cancers; sarcomas; and embryonal cancers such as neuroblastoma, retinoblastoma, and nephroblastoma; carcinomas are very rare, particularly before the adolescent years. , Despite their rarity, these tumors present a unique opportunity to assess theories of origin because the interval from birth to tumor onset is relatively short compared with that seen in adults. Nevertheless, a variety of carcinomas do occur in children, especially during late adolescence. According to a recent report, the commonest cancers in this study center are those arising from the epithelia of breast, cervix, prostate, skin, and head and neck. 
Comprehensive reports on childhood carcinomas are limited, and the few available reports were on the individual type as well as site-specific carcinomas including squamous cell carcinoma of the skin; anogenital mucous membrane mostly associated with human papillomavirus (HPV) infections; basal cell carcinoma associated with some congenital syndromes such as basal cell nevus syndrome, xeroderma pigmentosum, or nevus sebaceous; and in areas of intense ultraviolet (UV) radiation exposure. , Other commonly reported cases include nasopharyngeal carcinoma (NPC), mucoepidermoid carcinoma of the major salivary gland, renal cell carcinoma, and hepatocellular carcinoma (HCC). ,,, Less frequently reported were adenocystic carcinoma of the minor salivary gland, carcinomas of the oesophagus, larynx, thyroid, thymus, urothelium, sinunasal area, colorectal area, and ovarian surface epithelium. ,,,,,,,,
A retrospective study of carcinomas among children aged 0-15 years to determine the epidemiological and histopathological pattern of carcinomas among children in a northern Nigerian tertiary health institution.
| Materials and Methods|| |
This is a retrospective study of carcinoma in children aged 0-15 years in Ahmadu Bello University Teaching Hospital, Zaria, one of the referral tertiary institutions in northern Nigeria. Data obtained from the surgical bench book of the Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria for a period of 17 years (1994-2010), were reviewed. Those with histologically confirmed carcinoma were analyzed and presented in frequency distribution tables and figures, including photomicrographs with legends.
| Results|| |
There were 41 children with confirmed diagnoses of carcinoma over the study period: 24 males and 17 females. The male-to-female ratio was 1.4:1. A total of 491 cases of cancers in the children aged 0-15 years within the 17-year period under review were recorded. Carcinomas constituted about 8.4% of all these childhood cancers and it formed 0.78% of all malignancies diagnosed during the period. The peak age of incidence was 11-15 years. Patients aged less than 6 years constituted only 9.8% of cases. The commonest sites were the skin (29.3%), colon (22.0%), nasopharynx (14.6%), and major and minor salivary glands (14.6%). Other sites affected were buccal mucosa (4.8%) and liver, with a single case of HCC in a 15-year-old female. Metastatic carcinoma to the lymph node and soft tissues constitute 9.8% and 2.4%, respectively, as shown in [Table 1] and [Table 2].
|Table 2: Age distribution of the various site of affectation of carcinomas in children|
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There were 12 cases of carcinomas of the skin involving 7 males and 5 females with a male-to-female ratio of 1.4:1. Out of this, 10 cases were well to moderately differentiated squamous cell carcinomas involving the skin of the conjunctiva (3 cases), scalp (2 cases), lower legs (3 cases), and 1 case each in the trunk, perineum and orbit. Other histologic variants were 1 case each of basal cell carcinoma and verrucous carcinoma affecting 8-year-old and 15-year-old female patients, respectively [Table 3]. [Figure 1] and [Figure 2] show micrographs of well-differentiated and moderately differentiated squamous cell carcinoma respectively.
Colonic adenocarcinomas accounted for 22.0% of cases in this series with 9 cases and a slight female preponderance (male-to-female ratio of 1:1.2). Eight of the 9 patients with colonic adenocarcinomas had typical moderately to poorly differentiated adenocarcinomas, while a 13-year-old female patient had mucinous adenocarcinoma. One of the metastatic lymph nodes was also an adenocarcinoma from the colon. Poorly differentiated adenocarcinomas tend to have less favorable prognosis, especially with nodal metastasis, and tend to occur in younger patients, even among the adult populations. 
Primary epithelial malignancies of the nasopharynx and salivary gland constituted 14.9% each in this study. Four of the 6 patients with NPCs were of the undifferentiated type; this was further confirmed with the expression of the immunohistochemical epithelial markers in some of the cases, since this site is notorious for non-Hodgkin's lymphoma in this age group.  Two cases had typical squamous cell carcinoma. Among the salivary gland carcinomas, mucoepidermoid carcinoma of the parotid gland formed half of the cases from 2 female patients and 1 male 15-year-old patient, while 2 patients had adenoid cystic carcinoma of the parotid gland. One case of adenoid cystic carcinoma was seen in the minor salivary gland of the oropharynx.
Other primary carcinomas seen in this study were 2 cases of squamous cell carcinoma involving the tongue of an 8-year-old male child and buccal mucosa of a 10-year-old female patient and a single case of HCC in a 15-year-old female patient.
Metastatic carcinomas accounted for 12.2% of the cases and all affected the lymph nodes except for 1 case of metastatic carcinoma to the soft tissue of the neck. The primaries of such metastatic diseases were the nasopharynx, colon, and two unknown sites including 1 case of papillary adenocarcinoma.
Squamous cell carcinoma was the most common form of carcinoma, accounting for 34.1% of all the cases studied. Ten (71.4%) of these were from the skin, while there were 2 cases each (14.3%) from the oral mucosa and the nasopharynx.
| Discussion|| |
Carcinoma in children represents only a small fraction of all pediatric cancers diagnosed in this study. The peak age of distribution showed that these cancers were predominant in children above 10 years of age and comparable with the available documented literatures. ,,,,,,,
Squamous cell carcinoma is the predominant histopathologic diagnosis in this series and this is comparable to the report by Chow et al. in Australia. They reported 19 cases of squamous cell carcinoma of the skin and mucous membrane in children over a 30-year period.  They demonstrated that these significantly high figures are associated with improved survival of cancer patients, therapeutic irradiation, immunosuppression, and possibly, increased prevalence of HPV in the community. Though the exact role of HPV in the causation of these cancers was not demonstrated in this study, histologically such infections were seen in many of our cases as koilocytotic atypia with viral incursions. Possible additional risk factors in this environment include immunosuppression from malnutrition and human immunodeficiency virus infections, which are endemic. We also found a single case of basal cell carcinoma, which was not associated with any of the known hereditary associated factors. Le Seur et al. reported 3 cases of basal cell carcinoma of children not associated with basal cell nevus syndrome, xeroderma pigmentosum, nevus sebaceous, or intense UV radiation exposure. 
Colorectal carcinoma is the second most common pediatric carcinoma recorded in this series. This can be attributed to the rising frequency of colorectal carcinoma in younger individuals in our environment, as reported by Abdulkareem et al., unlike in the developed world where these tumors are seen in the older age group.  Colorectal carcinomas in children also lack the typical natural history and biologic behavior of dysplasia, adenoma, and carcinoma sequence.  Generally, colorectal carcinomas are associated with established risk factors. These include genetic, environmental, and dietary factors; high content of red meat and animal fat; low content of unabsorbable fiber in diet; low overall fruit and vegetable intake; low intake of protective micronutrients, alcohol and tobacco consumption, obesity, and sedentary habits. Among genetic factors, familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer syndrome (HNPCC) are the commonly associated precursor lesions in colon cancer. In children, these predisposing factors were absent in most cases, as reported by Radhakrishnan et al. 
NPC is the next common carcinoma seen in this study. NPC is epidemiologically and histologically different from other head and neck tumors. It is an Epstein-Barr virus (EBV)-associated carcinoma of epidermoid origin endemic in southern China, North Africa, and the Mediterranean basin. It commonly has poorly or undifferentiated morphology with high incidence of cervical lymph node metastasis and great radiosensitivity and chemosensitivity. It has been demonstrated that EBV is harbored in almost all NPC tumors regardless of degree of differentiation and geographic distribution.  Rauf et al. reported 3 cases over a period of 8 years in a 8-year, 9-year, and 11-year-old boys. This is comparable to our 6 cases within the study period. These authors reported that all their cases were histologically undifferentiated carcinomas, and that keratinization is hardly seen in children and young adults.  This is also comparable with the index study, since none of our cases had keratinizing carcinoma.
Neoplastic salivary glands epithelial tumors are fairly common, though the majority are benign. A frequency of 3.5-5.5% has been reported.  Fang et al. reviewed 122 salivary gland tumors in children and only 17 were malignant; mucoepidermoid carcinoma cases constituted 47.1% of the malignant group, the parotid gland was the common site of involvement, and none of the malignant tumors affected the submandibular gland.  This is comparable with this series, since mucepidermoid carcinoma comprises 50% of the series and all cases affect the parotid gland. A case of adenocystic carcinoma of the minor salivary gland and 2 cases in the parotid gland were seen in our series. In a population-based study, Sultan et al. reported that from 1973 to 2006 there were 263 children/adolescents with a reported diagnosis of carcinoma of the salivary glands, constituting 0.5% of all malignancies reported in children/adolescents, with slight female preponderance (58.0%).  In our study, the male-to-female ratio was 1:1; however, the commonest malignancy had female preponderance.
Primary liver cell carcinoma is a common malignancy in our environment in adults, but very infrequent in the pediatric age group. Akinyinka et al. in Ibadan reported 19 cases spanning a 35-year period and all of the cases were associated with hepatitis B viral (HBV) infection.  Chronic infection with HBV, hepatitis C virus (HCV), or both is the most common cause globally. Among Western populations, alcohol-induced liver injury is a leading cause of liver cirrhosis and constitutes the most important HCC risk. In southern China and sub-Saharan Africa, dietary ingestion of high levels of aflatoxin may present a special environmental hazard, particularly in individuals chronically infected with HBV. Other exogenous factors have also been incriminated, including iron overload, long-term use of oral contraceptives, and high-dose anabolic steroids. The development of liver cirrhosis, particularly in association with inherited genetic diseases such as alpha-1-antitrypsin deficiency or hemochromatosis places the individual at a greater risk of HCC development.  Risk is also increased if etiological risk factors exist in combination, e.g., HCV infection and alcohol use or HBV infection and exposure to aflatoxin. In children, perinatal transmission during childbirth accounts for 90% of cases.  This tumor has guarded prognosis. All the cases reported by Akinyinka died within 2 weeks of presentation. 
| Conclusion|| |
Carcinomas in children do occur, but are rare. The commonest are squamous cell carcinoma of the skin and mucous membrane; colorectal carcinoma; NPC; and carcinomas of the salivary glands. These tumors are diverse and site-specific in their epidemiology, histogenesis, natural history, biologic behavior, morphology, and prognosis.
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]