Analytical Report Colorectal carcinoma in a ten-year-old girl: A case report ABSTRACT Colorectal carcinoma is very rare in childhood In this case report, we depict a ten-year-old girl who presented with features of intestinal obstruction which turned out to be due to poorly differentiated mucin secreting adenocarcinoma of descending colon Only increased awareness of this malignancy in this age-group and a high index of suspicion can help when a child complains of persistent pain of abdomen, altered bowel habits or rectal bleeding, and may provide diagnosis at an earlier stage, thereby improving the prognosis KEY WORDS: Adenocarinoma, children, colon cancer INTRODUCTION Colorectal region is the most common gastrointestinal malignancy and represents about 15% of all cancer-related deaths The incidence peaks around seventh decade of life and less than 20% presents before the age of 50 But colorectal carcinoma in children is very rare, with an incidence of only 1.3 per million.[1] Limited numbers of colorectal carcinoma in children have been reported, mostly presenting in teens, though, the youngest one reported so far was a 9-month-old baby.[2] Only increased awareness and a high index of suspicion of this malignancy in the pediatric age-group may provide diagnosis at an earlier stage, thereby improving the prognosis when a child presents with non-specific complaints like persistent pain of abdomen or altered bowel habits CASE REPORT In September 2010, a 10-year-old girl presented with features of sub-acute intestinal obstruction and had a history of recurrent vague abdominal pain for last two months for which anti-spasmodic drugs were taken irregularly with little effect Routine hematological tests and chest X-ray were within normal limits Ultrasonogrphy and straight X-ray of abdomen in erect posture confirmed lower GI tract obstruction without any other significant abnormality The girl did not improve on conservative management and on exploratory laparotomy, a constricting growth was detected at descending colon near its junction with sigmoid colon and left hemicolectomy was done The resected specimen consisted of a 19 cm long segment of descending and sigmoid colon, 120 showing an ulcero-proloferative circumferential growth measuring cm in longest axis located cm from the distal resection margin and without any macroscopic involvement of the serous coat No polyp-like lesion was seen in the resected specimen, thereby excluding polyposis syndromes Histopathological examination of the growth showed features of poorly differentiated mucin secreting adenocarcinoma Signet ring cells were seen infiltrating through intestinal wall diffusely and in single file with presence of tumor infiltration in serous coat [Figures and 2] Lymphatic emboli [Figure 3] and perineural invasion were also present (Stage Duke C, Astler–Coller C2) Resection margins were free from tumor Sections from the non-tumorous areas of the specimen showed normal histology without any evidence of inflammatory bowel disease All five resected lymph nodes showed complete replacement by metastatic deposit [Figure 4] No family history of colorectal carcinoma in first or second degree relative was found Serum CEA after surgery was ng /ml Six cycles of adjuvant chemotherapy were administered with Inj 5-FU (370 mg / m2 / day and Inj Leucovorin (500 mg / m2 / day) i.v for days to be repeated every 29 days After eight months of treatment, USG of abdomen, serum carcinoembryonic antigen level remained within normal limits and colonoscopy showing no evidence of recurrence Sarbani Chattopadhyay, Phalguni Gupta1, Ranen Kanti Aich1, Asit Ranjan Deb1 Departments of Pathology and Radiotherapy, NRS Medical College, Kolkata, India For correspondence: Dr Sarbani Chattopadhyay, 254A, Kalicharan Ghosh Road, Kolkata - 700050, India E-mail: sarbanichattopadhyay@ yahoo.co Access this article online Website: www.cancerjournal.net DOI: 10.4103/0973-1482.95189 PMID: *** DISCUSSION Quick Response Code: Colon cancer is very rare in pediatric population Hereditary non-polyposis colon cancer and other familial syndromes account for a few, but most childhood cases appear sporadically Predisposing Journal of Cancer Research and Therapeutics - January-March 2012 - Volume - Issue Chattopadhyay, et al.: Colorectal carcinoma in a ten-year-old girl Figure 1: Microphotograph showing mucin secreting adenocarcinoma infiltrating into intestinal wall from mucosa Figure 2: Microphotograph showing signet ring cells infiltrating in muscle coat Figure 3: Microphotograph showing tumor emboli in lymphatics Figure 4: Microphotograph showing metastatic deposit of adenocarcinoma in lymph node factors like familial polyposis of colon, other polyposis syndromes, ulcerative colitis, familial multiple cancer syndromes are noted in about 10% of colorectal cancers in pediatric population, which is far higher than in adults.[3] SalasValverde et al,[4] reported presence of predisposing factors in three out of 11 cases but in many others, no predisposing factor can be found.[5,6] The present case was also a sporadic one In cases with predisposing factors also, carcinoma usually develops in adulthood In pediatric population colorectal carcinoma shows male preponderance (M: F= 2:1).[5] Presenting symptoms of colorectal carcinoma in children vary from bloody stools, pain of abdomen, and altered bowel pattern to unexplained weight loss Since all the symptoms and signs are vague, early diagnosis is often missed and the disease presents at an advanced stage, often with intestinal obstruction Pandey et al, noted bleeding per rectum as the presenting symptom along with weight loss in all four cases they reported.[5] In the present case also, the child presented with sub-acute intestinal obstruction Because of limited number of cases being reported in the literature, there is controversy about tumor distribution in the colon in children Redkar et al,[7] noted a higher incidence of the right and transverse colon involvement compared with adults and a far lower rate of rectal involvement, whereas recto-sigmoid region was the most common site (65%) in the 20 case series of Karnak et al.[8] In the present case, the growth was situated at the descending colon In adults, colorectal adenocarcinoma usually shows tubular differentiation But in children mucinous or signet ring appearance predominate.[5,8,9] In mucinous carcinoma, lakes of extracellular mucin are formed in which relatively bland looking tumor cells float The mucin absorbs water, swells and invades tissues, thereby promoting spread of malignant cells This tumor may grow to large size because of the pooled mucin It interferes with immune recognition of carcinoma cells caused by mucopolysaccharide coating.[10] Signet ring cell carcinoma causes early metastasis to lymph nodes, peritoneal surfaces and ovary, rather than liver and has extremely poor prognosis This variant accounts for only 1% of primary colon carcinoma in adults In the present case, Journal of Cancer Research and Therapeutics - January-March 2012 - Volume - Issue 121 Chattopadhyay, et al.: Colorectal carcinoma in a ten-year-old girl cases will also help the clinicians to improve the awareness and management histology showed predominating signet ring histology Most of the pediatric patients presented at an advanced stage, mostly Dukes C or D.[6] Karnak et al also recorded advanced stage at presentation in all cases (stage C: cases, D: 13 cases).[8] The present case was also at stage C All the studies published so far, described a poor outcome, for childhood colorectal carcinoma.[6,8,9] The five-year-survival rate for children ranges from 7% to 12%, and is similar for both developed and developing countries.[6] Advanced stage at presentation and mucinous/signet ring cell histology contribute to this outcome Among all prognostic parameters, tumor stage, lymph node metastasis and vascular and perineural invasions are most important All of them are usually present when the histology is a signet ring type, as in the present case, making the prognosis extremely poor Because the disease remains unsuspected, presentation is delayed with up to 60% having luminal obstruction as opposed to 18% in adults At operation, complete resection is possible in less than 40% of cases as against 90% possible resection in adults.[6] Since detection at earlier stage can lead to a better cure rate and screening programs for adults are not applicable to children, a high index of suspicion is helpful when a child complains of persistent pain of abdomen, altered bowel habits or rectal bleeding Careful clinical and rectal examination along with colonoscopic evaluation and awareness of steadily increasing incidence of colorectal carcinoma in children may lead to diagnosis at an earlier stage, thereby improving the prognosis Registration of all childhood colo-rectal carcinoma 122 REFERENCES Afroza A, Hasan S, Rukunuzzaman M, Hussain SA, Amin R Carcinomarectum in an 11 years old boy Mymensingh Med J 2007;16 (2 Suppl):S70-72 Kern WH, White WC Adenocarcinoma of the colon in a 9-month-old infant; report of a case Cancer 1958;11:855-7 Angelini C, Crippa S, Uggeri F, Bonardi C, Sartori P, Uggeri F Colorectal cancer with neuroendocrine differentiation in a child Pediatr Surg Int 2005;21:839-40 Salas-Valverde S, Lizano A, Gamboa Y, Vega S, Barrantes M, Santamaría S, et al Colon carcinoma in children and adolescents: Prognostic factors and outcome-a review of 11 cases Pediatr Surg Int 2009;25:1073-6 Pandey A, Gangopadhyay A, Sharma S, Kumar V, Gupta D, Gopal S, et al Pediatric carcinoma of rectum Varanasi experience Indian J Cancer 2008;45:119-22 Radhakrishnan CN, Bruce J Colorectal cancers in children without any predisposing factors A report of eight cases and review of the literature Eur J Pediatr Surg 2003;13:66-8 Redkar RG, Kulkarni BK, Naik A, Borwankar SS Colloid carcinoma of rectum in a 11 year old child J Postgrad Med 1993;39:218-9 Karnak I, Ciftci AO, Senocak ME, Bỹyỹkpamukỗu N Colorectal carcinoma in children J Pediatr Surg 1999;34:1499-504 Ladd AP, Grosfeld JL Gastrointestinal tumours in children and adolescents Semin Pediatr Surg 2006;15:37-47 10 Symmonds DA, Vickery AL Mucinous carcinoma of the colon and rectum Cancer 1976;37:1891-900 Cite this article as: Chattopadhyay S, Gupta P, Aich RK, Deb AR Colorectal carcinoma in a ten-year-old girl: A case report J Can Res Ther 2012;8:120-2 Source of Support: Nil, Conflict of Interest: None declared Journal of Cancer Research and Therapeutics - January-March 2012 - Volume - Issue Copyright of Journal of Cancer Research & Therapeutics is the property of Medknow Publications & Media Pvt Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission However, users may print, download, or email articles for individual use ...Chattopadhyay, et al.: Colorectal carcinoma in a ten- year- old girl Figure 1: Microphotograph showing mucin secreting adenocarcinoma infiltrating into intestinal wall from mucosa Figure... clinical and rectal examination along with colonoscopic evaluation and awareness of steadily increasing incidence of colorectal carcinoma in children may lead to diagnosis at an earlier stage,... Therapeutics - January-March 2012 - Volume - Issue 121 Chattopadhyay, et al.: Colorectal carcinoma in a ten- year- old girl cases will also help the clinicians to improve the awareness and management histology