Abstract
Background: Acute abdominal syndrome can be caused by several possible reasons. The most common causes are perforation of a gastroduodenal ulcer, peritonitis, intestinal obstructions, and perforation of an appendix or fallopian tube. Fever and pain can be caused by an appendicitis or sigmoiditis. Appendiceal lymphoma is a rare disease that is usually found incidentally during appendectomy. Most of the cases are non-Hodgkin’s lymphomas. Mantle cell lymphoma is an aggressive B-cell non-Hodgkin’s lymphoma with a poorer prognosis than other B-cell lymphomas; thus, a definitive diagnosis is essential. Case Summary: A 60-year-old man presented with right lower quadrant pain. He denied any nausea, vomiting or anorexia and was afebrile. The physical examination revealed right lower quadrant abdomen tenderness. The computed tomography scan revealed periappendiceal fatty stranding with a swollen appendix, approximately 2 cm in diameter and prominent paraaortic, portacaval and mesenteric lymph nodes. A diagnosis of acute appendicitis was made, and laparoscopic appendectomy was performed immediately. The subsequent pathological examination revealed severe congestion with lymphoid hyperplasia. The immunohistochemistry stains revealed positive staining for cluster of differentiation (CD) CD20, B-cell lymphoma-2 (Bcl-2), cyclin D1, SRY-box transcription factor-11 (SOX-11), immunoglobulin D (IgD) and immunoglobulin M (IgM) but negative staining for CD3, CD5, CD10 and CD23. 18F-FDG positron emission tomography showed peripheral lymph node involvement, while the bone marrow biopsy showed negative findings. Therefore, a diagnosis of mantle cell lymphoma, Ann Arbor stage IVA, was made. The patient received postoperative combination chemotherapy and remained in a stable condition over a 1-year follow-up period. Conclusion: We report an uncommon case that initially presented as acute appendicitis, for which a final diagnosis of mantle cell lymphoma was made. In comparison with other B-cell lymphomas, mantle cell lymphoma has a poorer prognosis, and positive immunochemical staining of cyclin D1 and SOX-11 is useful for differentiating mantle cell lymphoma from other appendiceal lymphomas and treating patients appropriately. Physicians and nursing staff should be also aware of the associated complications and management in these patients.
Highlights
Acute abdominal syndrome can present with a variety of clinical conditions from benign and self-limited disease to surgical emergencies
NHLthat thatusually usuallyoccurs occursininmiddle-aged middle-aged adults, has a male predominance and presents with advanced stage at diagnosis. It typically adults, has a male predominance and presents with advanced stage at diagnosis. It typipresents with generalized lymphadenopathy, and extranodal involvement is common, incally presents with generalized lymphadenopathy, and extranodal involvement is common, cluding bone blood, spleen, and Waldeyer’s ring involvement and inincluding bonemarrow, marrow,peripheral peripheral blood, spleen, and involvement and vasion into the gastrointestinal tract, which may present as a distinctive symptom of multiinvasion into the gastrointestinal tract, which may present as a distinctive symptom of ple lymphomatous polyposis of the intestine
Rare cases of diffuse large B-cell lymphomas could be positive for cyclin D1, but they frequently lack CCND1 gene translocation and lack immunoreactivity for SRY-box transcription factor-11 (SOX-11) [30]
Summary
Acute abdominal syndrome can present with a variety of clinical conditions from benign and self-limited disease to surgical emergencies. The gastrointestinal tract is the most common extranodal site involved in lymphomas, accounting for 5–20% of all cases [2]. MCL typically initially present as lymphadenopathy, and up to one-third of patients have systemic B symptoms and frequently have disease involvement in extranodal sites. Common sites of extranodal involvement include the gastrointestinal tract, breast, pleura, and orbit [6]. We report a case of mantle cell lymphoma that initially presented as acute appendicitis, emphasizing the need to consider differential diagnoses with appropriate healthcare and management. In this manuscript, a literature search was performed in PubMed and Scopus for all publications from inception published before 31 October 2020.
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