A rare case of signet ring cell carcinoma of the appendix

Caesar-Peterson, Shannon; Tulla, Katrina; Southall, Colton; Lin, Ying; Genelus-Dominique, Elvita

doi:10.1093/jscr/rjaa139

Cited by 7 publications

(5 citation statements)

References 7 publications

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“…Like the other well-defined malignant tumors, genetic mutation remains a fundamental principle in it. Pluripotent intestinal crypt epithelial stem cells have the potential function of secreting mucin droplets and forming neuroendocrine secretory granules, and gene mutations in it can contribute to the morphological feature of cytoplasm abundant with mucin vacuoles, which push the nucleus peripherally 8 . The transformation of this type of cell is thought to be linked with the tumorigenesis of signet-ring cell carcinoma.…”

Section: Discussionmentioning

confidence: 99%

Incidental diagnosis of primary appendiceal signet-ring cell adenocarcinoma after appendectomy for acute appendicitis, a case report

Xie,

2024

Annals of Medicine &Amp; Surgery

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Introduction: Appendiceal signet-ring cell adenocarcinoma (ASRA) is rare, and more aggressive in malignant appendiceal neoplasms. The presentation can be appendicitis, which is lack of specific symptom and makes early diagnosis difficult. There is no effective surveillance. Prognosis largely relies on timely detection. We report a case of ASCA incidentally diagnosed through pathological examination after appendectomy for appendicitis. Case presentation: The patient presented to our department with a progressive right lower quadrant abdominal pain lasting for 3 days. Physical examination revealed rigidity, tenderness and rebound tenderness on the right lower quadrant. CT scan showed a thickened, inflamed appendix with peri-appendiceal fat stranding without noticeable appendiceal mass at initial evaluation. The diagnosis was considered acute appendicitis, and an appendectomy was performed. The appendix was inflamed, gangrenous and perforated, and no mass was found during the surgery. Surgical specimen was sent for physiological examination, which incidentally detected signet-ring cell in H&E staining. And immunohistochemistry confirmed the diagnosis of appendiceal signet-ring cell adenocarcinoma with small amount of neuroendocrine neoplasms. Conclusion: Early diagnosis of ASCA can incidentally be made on pathological specimen following appendectomy for appendicitis. A routine pathological examination should be emphasized, and appendectomy may not be the endpoint of the treatment. Hemicolectomy and adjuvant therapy might ensue upon the diagnosis of appendiceal neoplasm. The poor prognosis of ASCA makes a timely diagnosis significant. Basic research is promising to unravel the molecular mechanisms of pathogenesis, finding typical tumor markers for screening and novel effective therapies for advanced cases.

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Section: Discussionmentioning

confidence: 99%

Incidental diagnosis of primary appendiceal signet-ring cell adenocarcinoma after appendectomy for acute appendicitis, a case report

Xie,

2024

Annals of Medicine &Amp; Surgery

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“…Primary appendiceal carcinomas are a rare entity. Contributing to an incidence rate of less than 0.5% of all gastrointestinal malignant neoplasms and accounting for 0.7%-1.4% of all the specimens collected after an appendectomy, they have a lower incidence rate among all digestive tract tumors [5,6]. Despite being a small vestigial organ, primary appendiceal carcinomas are composed of a wide variety of histological types, including adenocarcinoma (which are further subdivided into colonic adenocarcinoma, mucinous adenocarcinoma, and signet ring cell tumor), appendiceal neuroendocrine carcinoma, and mixed tumor containing both of these elements with goblet cells [1,2].…”

Section: Discussionmentioning

confidence: 99%

Rare Mucinous Adenocarcinoma of the Appendix Undergoing Multiple Recurrent Surgical Interventions

Ilyas¹,

Umar²,

Pansuriya³

et al. 2023

Cureus

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Primary appendiceal carcinomas are rare and often found incidentally when the appendix is surgically removed. Adenocarcinoma predominates the histological types of malignancies, with mucinous adenocarcinoma being the most prevalent of the various subtypes. Pseudomyxoma peritonei (PMP), a complication seen in mucinous adenocarcinoma of the appendix (MAA), is the collection of mucinous ascites in the intra-abdominal cavity and the thickening of the surrounding viscera by mucin-producing tumor cells. PMP initially presents with increased abdominal discomfort and girth and, in later stages, presents with obstructive abdomen symptoms. These symptoms are nonspecific and can be a challenge to pinpoint. Such was the case for our patient, in this case report, who initially presented with dyspepsia and later demonstrated compressive symptoms and weight loss, raising concern for malignancy. An appendiceal pathology was of concern when his right lower quadrant pain acutely worsened during an abdominal ultrasound, and imaging and biopsy confirmed MAA with PMP. The aim of this report is to shed light on the management of recurrent MAA. Our patient's recurrent MAA was managed with debulking procedures and three rounds of hyperthermic intraperitoneal chemotherapy (HIPEC) and was managed postoperatively with folinic acid, fluorouracil, and irinotecan (FOLFIRI) and bevacizumab, which in its totality helped achieve a progression-free survival of more than two years. We believe that cytoreduction and intraoperative chemotherapy prolong survival in patients with recurrent disease, as was the case with our patients. Our patient also demonstrated benefit as his disease stabilized after starting bevacizumab; however, more studies need to be performed at a larger scale to show a consistent relationship.

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“…[1] Although with the progress of medical technology, the 5-year survival rate of SRCC is only 20.5%, and if there is metastasis, it will be reduced to 6.7% to 14%. [32] Regardless of stage, right hemicolectomy was associated with better survival and prognosis than neither surgery nor appendectomy alone. Both preoperative neoadjuvant therapy and postoperative adjuvant chemoradiotherapy can reduce the recurrence rate and improve the survival rate, improve the prognosis, and bring clinical benefits to patients.…”

Section: Discussionmentioning

confidence: 99%

Clinical benefits of FOLFOXIRI combined with bevacizumab for advanced-stage primary signet ring cell carcinoma of the appendix: A case report

Huang

Wang

et al. 2023

Medicine

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Rationale: Signet-ring cell carcinoma, which is an infrequent type of colorectal cancer. Abdominal pain is the primary presenting complaint of patients with acute appendicitis. It is difficult to diagnose patients with appendiceal carcinomas accompanying with symptoms of acute appendicitis. Patient Concerns: A 33-year-old female patient was admitted to our hospital, with chief complaints of “bilateral pelvic space-occupying lesions for 1 month, aggravated abdominal distension, and she accompanied with diarrhea for 3 days.” Diagnosis: The patient was with primary signet ring cell carcinoma of the appendix, presented with acute appendicitis, as well as bilateral ovarian metastasis and peritoneal implantation metastasis. Interventions: She was then treated with irinotecan, oxaliplatin, calcium folinate, 5-FU combined with bevacizumab, surgical treatment, and postoperative adjuvant treatment with oxaliplatin, capecitabine regimen to consolidate the efficacy. Outcomes: The patient is in good conditions, and postoperative adjuvant chemotherapy is in progress as well. Conclusion: The outcomes highlighted the importance of strict histopathologic assessment for appendiceal adenocarcinoma, and provided new ideas for the diagnosis and treatment of advanced-stage signet ring cell carcinoma of the appendix.

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A rare case of signet ring cell carcinoma of the appendix

Cited by 7 publications

References 7 publications

Incidental diagnosis of primary appendiceal signet-ring cell adenocarcinoma after appendectomy for acute appendicitis, a case report

Incidental diagnosis of primary appendiceal signet-ring cell adenocarcinoma after appendectomy for acute appendicitis, a case report

Rare Mucinous Adenocarcinoma of the Appendix Undergoing Multiple Recurrent Surgical Interventions

Clinical benefits of FOLFOXIRI combined with bevacizumab for advanced-stage primary signet ring cell carcinoma of the appendix: A case report

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