A rare case of primary signet‐ring adenocarcinoma of anorectal region in a young patient: Diagnostic challenges and therapeutic outcomes

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Publicado en:Clinical Case Reports vol. 12, no. 9 (Sep 1, 2024)
Autor principal: Shaikh, Bisma
Otros Autores: Gul, Areeba, Singh, Ajeet, Irfan, Hamza, Ali, Tooba, Karamat, Riyan, Akilimali, Aymar
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John Wiley & Sons, Inc.
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Acceso en línea:Citation/Abstract
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022 |a 2050-0904 
024 7 |a 10.1002/ccr3.9422  |2 doi 
035 |a 3107971368 
045 0 |b d20240901 
084 |a 239633  |2 nlm 
100 1 |a Shaikh, Bisma  |u Department of Internal Medicine, Jinnah Sindh Medical University, Karachi, Pakistan 
245 1 |a A rare case of primary signet‐ring adenocarcinoma of anorectal region in a young patient: Diagnostic challenges and therapeutic outcomes 
260 |b John Wiley & Sons, Inc.  |c Sep 1, 2024 
513 |a Journal Article Case Study 
520 3 |a Key Clinical Message Primary signet‐ring cell carcinoma of the anal canal and rectum is an extremely rare and aggressive malignancy. The present case underscores the importance of considering primary signet‐ring cell carcinoma in differential diagnoses for young patients with chronic anorectal symptoms. It highlights the need for a multidisciplinary treatment approach (including surgery, chemotherapy, and radiotherapy) and comprehensive follow‐up for managing this challenging condition and improving long‐term patient outcomes.Primary signet‐ring cell carcinoma of the anal canal and rectum is an exceedingly rare subtype of colorectal adenocarcinoma, often originating as an extension of rectal adenocarcinoma. This malignancy constitutes a small fraction of colorectal cancers and is scarcely reported in medical literature. We present the case of an 18‐year‐old male with a three‐year history of progressively worsening hematochezia, anorectal pain, and defecation‐associated prolapse. Initial conservative treatments failed, leading to further investigations that revealed a palpable, nodular anorectal mass. Imaging studies (including CT and MRI), and biopsy confirmed poorly differentiated adenocarcinoma with signet‐ring cell morphology. The tumor exhibited extensive lymphovascular invasion and involved perirectal lymph nodes, and was staged as pT3, N2a. Immunohistochemical staining was positive for CK 7, CK 20, and SATB2, supporting the primary anorectal origin. The treatment regimen included initial diversion colostomies for symptom relief, followed by neoadjuvant chemotherapy with a modified 5‐fluorouracil, leucovorin, irinotecan, and oxaliplatin (FOLFIRINOX) regimen and concurrent chemoradiation with Xeloda. The patient subsequently underwent an abdominoperineal resection (APR), which confirmed the diagnosis and achieved curative resection. Postoperative complications included transient ileus and wound infection, which were managed with supportive care. This case underscores the diagnostic and therapeutic challenges posed by primary signet‐ring cell carcinoma of the anorectal region, highlighting the need for a high index of suspicion and comprehensive diagnostic workup in atypical presentations. The multimodal treatment approach, incorporating surgery, chemotherapy, and radiotherapy, was crucial in managing this locally advanced tumor. The rarity and aggressiveness of this carcinoma necessitate a tailored treatment strategy to improve patient outcomes. Long‐term follow‐up, including regular imaging and surveillance, is vital for monitoring disease recurrence and evaluating treatment effectiveness. 
653 |a Infections 
653 |a Cancer 
653 |a Quality of life 
653 |a Hemoglobin 
653 |a Surgery 
653 |a Lymphatic system 
653 |a Anus 
653 |a Biopsy 
653 |a Ostomy 
653 |a Intestinal obstruction 
653 |a Rectum 
653 |a Females 
653 |a Potassium 
653 |a Surveillance 
653 |a Radiation therapy 
653 |a Hematology 
653 |a Chemotherapy 
653 |a Creatinine 
653 |a Colorectal cancer 
700 1 |a Gul, Areeba  |u Department of Internal Medicine, Jinnah Sindh Medical University, Karachi, Pakistan 
700 1 |a Singh, Ajeet  |u Department of Internal Medicine, Dow University of Health Sciences, Karachi, Pakistan 
700 1 |a Irfan, Hamza  |u Department of Medicine, Shaikh Khalifa Bin Zayed Al Nahyan Medical and Dental College, Lahore, Pakistan 
700 1 |a Ali, Tooba  |u Department of Internal Medicine, Dow University of Health Sciences, Karachi, Pakistan 
700 1 |a Karamat, Riyan  |u Department of Internal Medicine, Rahbar Medical and Dental College, Lahore, Pakistan 
700 1 |a Akilimali, Aymar  |u Faculty of Medicine, Official University of Bukavu, Bukavu, Democratic Republic of Congo 
773 0 |t Clinical Case Reports  |g vol. 12, no. 9 (Sep 1, 2024) 
786 0 |d ProQuest  |t Health & Medical Collection 
856 4 1 |3 Citation/Abstract  |u https://www.proquest.com/docview/3107971368/abstract/embedded/7BTGNMKEMPT1V9Z2?source=fedsrch 
856 4 0 |3 Full Text  |u https://www.proquest.com/docview/3107971368/fulltext/embedded/7BTGNMKEMPT1V9Z2?source=fedsrch 
856 4 0 |3 Full Text - PDF  |u https://www.proquest.com/docview/3107971368/fulltextPDF/embedded/7BTGNMKEMPT1V9Z2?source=fedsrch