Signet Ring Cell Carcinoma, Ileal Crohn Disease or Both - A Case of Diagnostic Challenge

Abstract

ResumoO carcinoma de células em anel de sinete é uma forma rara de adenocarcinoma que afeta predominantemente o estômago. A forma primária do íleon é muito rara e apresenta mau prognóstico. Apresentamos um caso de doença de Crohn do íleon cuja forma de apresentação cursou com quadro oclusivo associado a adenocarcinoma de cé-lulas em anel de sinete do íleon. A doente apresentava história prévia de diarreia crónica e tinha história familiar de doença inflamatória. Foi submetida a ressecção cirúr-gica e os achados histopatológicos revelaram a presença de aspetos compatíveis com doença de Crohn do íleon e adenocarcinoma de células em anel de sinete. Foi proposta uma associação com esta forma rara de tumor e infla- Keywords Signet ring cell carcinoma · Crohn disease · Ileum AbstractSignet ring cell carcinoma is a rare form of adenocarcinoma that predominantly affects the stomach. Signet ring cell carcinoma originated from the ileum is extremely rare and the prognosis is poor. We present a case of small bowel obstruction with features suggesting Crohn disease of the ileum. The symptoms were chronic diarrhea and abdominal pain with a family history of inflammatory bowel disease. The patient underwent surgery and histopathology revealed both aspects of signet ring cell carcinoma and Crohn disease of the ileum. An association between long-standing inflammation and the development of this subtype of tumor has been proposed but there are no established surveillance guidelines for small bowel neoplasm in inflammatory bowel disease. 48 mação de longa duração, contudo, não existem recomendações estabelecidas de vigilância de neoplasia na doença inflamatória intestinal com atingimento ileal.

Ileal Signet Ring Cell Carcinoma Masked by Crohn Disease

“…13 One patient received a simultaneous diagnosis of CD and ileal SRCC. 3 Clinically, our patient had relatively well-controlled disease, suggesting that even subclinical inflammation may be contributory and that aggressive medical therapy (eg, biologic agents such as anti-tumor necrosis factor-alpha, antiintegrins, anti-interleukin-12, and anti-interleukin-23) and close surveillance may be beneficial, even in the presence of symptomatic remission. In our patient, the overlap in symptomology between SRCC and CD led to delayed diagnosis.…”

“…A review of the literature yielded 8 cases. 3,[7][8][9][10][11][12][13] Including our patient, the mean age was 50.9 years (range, 31 to 67 years), 55% were female, 89% presented with abdominal pain, and the mean CD duration (duration was not reported in 1 case 13 ) was 20.4 years (range, 0 to 44 years). One patient had a history of right ileocolectomy for intestinal obstruction from CD before the SRCC diagnosis.…”

“…2 Signet ring cell carcinoma (SRCC) is a rare adenocarcinoma that generally involves the stomach but can involve other organs, including the small intestine. 3 SRCC is poorly differentiated and has a poor prognosis. 4,5 Crohn disease (CD) is a well-known risk factor for intestinal cancer, 3 arguably because of CD-associated inflammation.…”

“…3 SRCC is poorly differentiated and has a poor prognosis. 4,5 Crohn disease (CD) is a well-known risk factor for intestinal cancer, 3 arguably because of CD-associated inflammation. 6 We report a case of ileal SRCC in a patient with longstanding, clinically controlled CD.…”

“… 2 Signet ring cell carcinoma (SRCC) is a rare adenocarcinoma that generally involves the stomach but can involve other organs, including the small intestine. 3 SRCC is poorly differentiated and has a poor prognosis. 4 , 5 …”