Metastatic Primary Signet Ring Cell Carcinoma of Rectum: A Case Report of 10-Year-old Male Child

Singh, Kumkum; Singh, Amit; Bhutra, Shyam; Pachori, Geeta; Jangir, Mahesh Kumar

doi:10.7860/jcdr/2014/6988.4051

Cited by 8 publications

(9 citation statements)

References 10 publications

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“…13,14,22 Similarly to adults, SRCC type in children has the worst prognosis of all, except for undifferentiated carcinoma. 3,17,23,[70][71][72] Interestingly, signet-ring cell CRC may arise or be confined within a solitary polyp, 10 the same as has been occasionally seen in adults. 72 Instead, to the best of our knowledge, no case of linitis plastica carcinoma has been fully reported in children so far: we are just aware that a wide age range occurs (17 to 90 y) in primary colorectal linitis plastica carcinoma (diffuse schirrous carcinoma), with a frequency of 0.1% to <0.9% of all tumors of the colorectum according to different series, presenting in 1 of the 3 following histologic patterns: with abortive gland formation, signet-ring cells, or undifferentiated cells.…”

Section: Authors' Commentmentioning

confidence: 99%

Selected Case From the Arkadi M. Rywlin International Pathology Slide Club

Galliani

Sanchez

D’Errico

et al. 2015

Advances in Anatomic Pathology

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We report a case of a 14-year-old female with primary adenocarcinoma of the transverse colon. She was hospitalized after presenting with abdominal pain and signs of intestinal obstruction. There was no health antecedent or family history of neoplasia. Physical examination revealed a distended abdomen. Tenderness was elicited to palpation of the right lower quadrant. Magnetic resonance imaging of the abdomen revealed obstructive signs, with a constricting lesion in the mid-transverse colon of probable neoplastic nature. Laparoscopic segmental resection of the colon was followed by standard right hemicolectomy. A circumferential mid-transverse tumor was diagnosed as primary colorectal carcinoma (CRC) of signet-ring cell type, AJCC stage IIIC, Dukes' C stage. On the basis of immunohistochemistry and clinical data, hereditary nonpolyposis and hamartomatous colorectal cancer syndromes were excluded. Involvement of either the p53, BRAF, or K-RAS genes was ruled out by immunohistochemistry profiling and genetic testing. The neoplasm was categorized as sporadic. The possibility of activation of the Wnt signaling pathway was suspected, because of a defective turnover of the β-catenin protein. Postoperatively, the patient was treated with both systemic and intra-abdominal adjuvant chemotherapy, including oxaliplatin. Between 18 and 24 months after diagnosis, intra-abdominal tumor recurrences were detected. The patient underwent bilateral oophorectomies for Krukenberg tumors and received salvage chemotherapy. Recently, additional recurrent metastatic retroperitoneal disease caused hydronephrosis. The retroperitoneal mass was debulked and a ureteric stent was placed. At the time of this writing, 43 months after diagnosis, the patient is receiving FOLFOX chemotherapy combined with panitumumab. CRC of childhood is exceedingly rare, generally develops in the setting of unrecognized genetic predisposing factors to cancer, presents with advanced disease, is high grade, and tends to have dismal prognosis.

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Section: Authors' Commentmentioning

confidence: 99%

Selected Case From the Arkadi M. Rywlin International Pathology Slide Club

Galliani

Sanchez

D’Errico

et al. 2015

Advances in Anatomic Pathology

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“…While colon adenocarcinomas are observed at an advanced age, signet ring cell carcinomas generally occur in young adults (2). Signet ring cell carcinoma of the colon was found in a 17-year-old patient in the study of Marone et al (6), a 19-year-old patient in the study of Pamukçu et al (7), and a 10-year-old patient in the study of Singh et al (8).…”

Section: Discussionmentioning

confidence: 92%

Mixt Signet-ring Cell Mucinous Carcinoma in the Large Bowel

Balcı¹,

Eken²,

Tayfur³

et al. 2016

JAREM

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Carcinomas of the large bowel are the third most commonly seen cancer in both males and females and are the second reason of deaths resulting from cancer. The most frequently seen microscopic type of large bowel cancer is adenocarcinoma. Signet-ring cell carcinoma is a rarely seen microscopic subtype of large bowel carcinomas and may be accompanied by other microscopic types. The diagnosis of signet-ring cell carcinomas is made while the count of signet ring cells is over 50% of the tumor cells. The presence and ratio of signet-ring cells should be noted because the prognosis of signet-ring cell carcinoma is very bad. In our case, it is viewed mixt mucinous signet-ring cell carcinoma including mucinous components in the large bowel. This report is presented because it is rarely seen and because the prognosis is very bad and is necessary be specified in the report. (JAREM 2016; 6: 117-8)

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“…It is defined as the subtype of colorectal adenocarcinoma with prominent intra-cytoplasmic mucin in more than 50% of tumor cells, according to the World Health Organization (WHO) classification of tumors [3]. SRCC has higher malignant potential, is commonly diagnosed at a late stage, and is characterized by distant metastasis with an extremely poor prognosis [4,5].…”

Section: Introductionmentioning

confidence: 99%

Diagnosis and Progression of Rectal Signet Ring Cell Carcinoma in a 15 Year Old Lebanese Boy: A Case Report

Farhat

Abtar²,

Shibli

et al. 2023

Int J Clin Res

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Introduction: Colorectal cancer (CRC) is a rare entity in children and adolescents compared to adults. In the young, it is mostly detected in the right and transverse parts of the colon. Among the variants of CRC are the uncommon Signet Ring Cell Carcinoma (SRCC) which has a late presentation and pessimistic prognosis. Patients are asymptomatic for a long time and suddenly develop changes in bowel habits or obstruction. Case Report: A 15-year-old boy with no known health issues presented with recent rectorrhagia and weight loss. He was stable but pale with abdominal tenderness and no rectal mass on digital rectal examination (DRE). Colonoscopy unveiled a 13 centimeters segment of circumferential ulcerated blackish mucosa extending from the upper rectum to the rectosigmoid junction. Pathology studies revealed SRCC of the rectum and sigmoid. Metastatic workup showed rectosigmoid wall thickening and denoted the tumor a stage IV with ascites and intraperitoneal implants. Palliative treatment with chemotherapy was initiated, and a follow-up CT was done later to assess disease progression and response to treatment. The disease had worsened and the patient deteriorated. Conclusion: In children, colorectal SRCC is scarce and usually presents at a late stage due to the lack of characteristic symptoms and findings. It is then usually missed by physicians and not considered in the differential thus delaying the diagnosis and rendering the prognosis poorer. To improve the outcome, pediatricians ought to keep CRC in mind when facing obstructive symptoms or refractory abdominal pain.

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Metastatic Primary Signet Ring Cell Carcinoma of Rectum: A Case Report of 10-Year-old Male Child

Cited by 8 publications

References 10 publications

Selected Case From the Arkadi M. Rywlin International Pathology Slide Club

Selected Case From the Arkadi M. Rywlin International Pathology Slide Club

Mixt Signet-ring Cell Mucinous Carcinoma in the Large Bowel

Diagnosis and Progression of Rectal Signet Ring Cell Carcinoma in a 15 Year Old Lebanese Boy: A Case Report

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