Introduction
Signet ring cell carcinoma (SRCC) of the small intestine is very rare. It is characterized by the presence of malignant cells that contain mucin that push nuclei to the periphery. It is more aggressive compared to other adenocarcinomas due to early metastasis and poorer prognosis.
Case Presentation
A 59-year-old male with a history of HIV/AIDS, presented with complaints of anorexia, vomiting and weight loss. Initial abdominal CT showed a retroperitoneal mass causing gastric outlet obstruction. The patient elected to go home after supportive treatment and follow up as an outpatient, however, he presented 10 days later with worsening symptoms. Evaluation with CT abdomen and pelvis showed enlarging soft tissue density in the retrocrural space extending into the retroperitoneum around the aorta, as well as a 1.5 cm intraluminal cystic lesion in the duodenum. Endoscopic ultrasound revealed lymphadenopathy of celiac and porta hepatis regions, along with duodenal stenosis, stent placement for decompression was not feasible and biopsies were inconclusive. The decision was made to proceed with laparotomy for decompression and additional biopsies from the retroperitoneal mass and omental lymph nodes, which confirmed poorly differentiated adenocarcinoma with signet ring cells. The presence of a mass in the duodenum strongly suggested adenocarcinoma of small intestine origin. As the patient’s symptoms worsened, imaging revealed progression with lung metastases. The patient continued to deteriorate rapidly requiring dialysis and gangrenous cholecystitis. Given his complex medical history, patient decided to transition to comfort care.
Discussion
SRCC can present with any GI symptoms. Most important step in diagnosing SRCC is biopsy. Current treatment options for small intestinal malignancies include wide resection that includes the mesentery and corresponding lymph nodes. The use of adjuvant chemotherapy has been described only in small retrospective studies. Due to its scarcity, there isn’t sufficient data for optimal treatment strategies compared to gastric SRCC.
Conclusions
This case report highlights the importance of how rare and aggressive signet ring cell adenocarcinoma of the small intestine. There are only a few cases documented in the literature, which is why we lack data on how to manage the disease.