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Brief Reports Indian Pediatrics 2000;37: 1353-1358 |
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Colorectal Carcinoma in Indian Children |
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Carcinoma of the colon and rectum is a relatively uncommon malignancy in India as compared to the western world. In India, colorectal carcinoma does not figure amongst the 10 most common malignancies(1). The age-standardized rates of colorectal cancer in India have been estimated to be 4.2 and 3.2 /1,00,000 for males and females, respectively as compared to 60.8 and 42.3 respectively in the USA(1,2). Considering such a low incidence in adults it would be rare to find colorectal carcinoma in the pediatric age group. A literature search could not reveal much information on colorectal cancer in the Indian pediatric population. In the US, the incidence of colorectal cancer in children is estimated to be 2 cases per million children and is the second most common gastro-intestinal malignancy in children and adole-scents, after liver tumors(3,4). We, retro-spectively, reviewed the records of pediatric patients at our institute to document the profile of colorectal carcinoma patients and analyze our experience in the management of this condition.
On reviewing the records of our patients from 1985 to 1999, i.e., for period of 15 years and using an arbitrary cut off age of 15 yr, we found 7 cases of colorectal cancer. The presenting signs and symptoms, location of primary tumor, stage of disease at diagnosis, histology, diagnostic and treatment proce-dures undertaken and outcome were recorded.
A brief summary of the clinical profile of these patients is presented in Table I. The most common symptoms were bleeding per rectum (5 patients), abdominal pain (4 patients) and abdominal distension (4 patients). One patient had fecal incontinence that was due to spurious diarrhea, resulting from impaction of feces above the tumor. Another patient had urinary frequency due to mass of tumor pressing on the urinary bladder, resulting in diminished bladder capacity. The average duration of symptoms prior to diagnosis in 6 patients was 4 months while one patient presented with acute obstruction. The work-up of patients was primarily directed towards assessing the extent of disease intra-abdominally and in lungs.
Treatment modalities used consisted of surgery, chemotherapy and radiotherapy with the aims of cure or palliation depending on the individual case. Surgery: The extent of surgery varied from biopsy only to resection of colon along with its mesentery. Curative resection was possible only in one patient. Of the remaining, two patients required colostomy for palliation, one had palliative resection for obstruction and in 3 patients only biopsy was done. The outcome of patients correlated with extent of surgery is presented in Table II.
Chemotherapy: The patient who had curative resection received adjuvant chemotherapy (5-FU + levamisole for 25 cycles). Chemo-therapy was given with the aim of palliation in 3 patients for an average of 3.6 cycles with no documentable clinical response in any of the 3 patients. Radiation therapy: One patient received palliative radiotherapy for persistent fixed backache. The dose was 3000 cGy delivered over 15 days with no resultant relief of symptoms. Histopathology Histopathological verification was obtained in all patients either by biopsy or by examination of the resected bowel specimen. The histology was poorly differentiated mucinous adeno-carcinoma-signet ring cell type in 6 patients and well-differentiated adenocarcinoma in 1 patient. Survival Out of 7 patients, one patient who had curative resection was free of disease for 1 year before he was lost to follow-up. One patient refused treatment in any form after the diagnosis. In the remaining 5 patients, the mean survival was 6.4 weeks.
Colorectal cancer is rare in children with an incidence of 1.3 to 2 cases per million children(4). Most of these cases in children occur in the second decade of life, although a nine-month-old infant with colon cancer has also been reported(3,5,9). A review of literature published during the last decade revealed scant information on the subject from India. The poor prognosis of pediatric patients with very few five year survivors is attributed to the advanced stage of the disease at presentation(3,7). This results from a delay in diagnosis and aggressive biological behavior of the disease(3,6,8,10,11). The symptomatology of vague abdominal pain, constipation, vomiting, nausea, rectal bleed-ing, etc. are usually not thought of as being due to malignancy and patients are often treated for other causes like amebiasis and worm infestation which are more prevalent in our country. In 6 of our patients, the average duration of symptoms, prior to diagnosis was 4 months and one patient presented with acute obstruction, prompting exploration. Steinberg et al. reported a higher incidence of Duke’s D lesion in the patients who were diagnosed later than 3 months after development of symptoms(9). Some reports attribute the delay in diagnosis to more right sided lesions in this population(3,10,11), although, there was not a single patient with right sided colon cancer in our study. Five patients, who had the tumor in the lower third of rectum, could easily have been diagnosed if a digital rectal examination had been performed. The other cause of poor prognosis is thought to be increased proportion of mucinous histology(12-14). This type of cancer accounts for only 5 to 15% of adult colorectal cancer and is believed to be clinically aggressive(6,12-14). Rao et al. noted that most of their patients had mucinous histology, which correlated with our study where 6 out of 7 had mucinous histology, and one patient had well differentiated adeno-carcinoma(3). This last patient survived for one year before being lost to follow up. For the rest of the patients, survival ranged from 2 weeks to 13 weeks for five patients and one being lost to follow up immediately after diagnosis. The primary treatment for colorectal cancer in children continues to be a curative surgical resection. However, this is rarely feasible because of the advanced stage at diagnosis. No patient with rectal cancer was deemed to be resectable at presentation in our study. One patient with the lesion in the descending colon underwent palliative resec-tion to relieve obstruction. Curative resection was possible only in one patient, who had a growth in sigmoid colon. Other reports also indicate less than optimal rates of complete resection(3). Palliative chemotherapy elicited no response in five patients and one patient who received palliative pelvic radiotherapy for pain also did not have any relief of symptoms. The value of chemotherapy as a means of palliation has been controversial and responses have been less than optimal(3,7). In rectal cancers, preoperative radiotherapy has been utilized extensively to convert unresect-able lesions to resectable ones(15,17). However, in our study, the presence of disseminated abdominal disease and/or poor performance status precluded delivery of effective radiotherapy. In contrast to the review of literature, which designates an even site distribution of the childhood colorectal carcinoma, in our study, all the cases were on the left side. In fact five of these cases were located in the lower third rectum. A high level of suspicion coupled with a simple digital rectal examina-tion followed by sigmoidoscopy and/or colonoscopy if required, can result in early diagnosis which will go a long way in providing effective therapy. Contributors: MSB collected and analyzed the data and drafted the manuscript. SC co-ordinated the analysis and helped in drafting the manuscript. RS helped in drafting the paper. DDP permitted the usage of clinical material and reviewed the draft. Finding:
None.
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