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Small Intestinal Medullary Carcinoma

MedGen UID:
328325
•Concept ID:
C1711326
•
Neoplastic Process

Definition

A carcinoma that arises from the small intestine. It is characterized by the presence of malignant epithelial cells with vesicular nucleus, distinct nucleolus, and abundant pink cytoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSmall Intestinal Medullary Carcinoma

Recent clinical studies

Etiology

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Frequency and clinicopathologic associations of DNA mismatch repair protein deficiency in ampullary carcinoma: Routine testing is indicated.
Xue Y, Balci S, Aydin Mericoz C, Taskin OC, Jiang H, Pehlivanoglu B, Muraki T, Memis B, Saka B, Kim GE, Bandopadhyay S, Knight J, El-Rayes BF, Sarmiento J, Reid MD, Erkan M, Basturk O, Adsay V
Cancer 2020 Nov 1;126(21):4788-4799. Epub 2020 Aug 28 doi: 10.1002/cncr.33135. PMID: 32857459
Approaches to Biopsy and Resection Specimens from the Ampulla.
Xue Y, Reid MD
Surg Pathol Clin 2020 Sep;13(3):453-467. Epub 2020 Jul 15 doi: 10.1016/j.path.2020.05.005. PMID: 32773194
Thyroid disorders in Brazilian patients with celiac disease.
da Silva Kotze LM, Nisihara RM, da Rosa Utiyama SR, Piovezan GC, Kotze LR
J Clin Gastroenterol 2006 Jan;40(1):33-6. doi: 10.1097/01.mcg.0000190756.63799.0f. PMID: 16340631
Apudomas.
Nakano PH, Bloom RR, Brown BC, Gray SW, Skandalakis JE, Kibbe JM
Am Surg 1987 Sep;53(9):505-9. PMID: 2820286

Diagnosis

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Approaches to Biopsy and Resection Specimens from the Ampulla.
Xue Y, Reid MD
Surg Pathol Clin 2020 Sep;13(3):453-467. Epub 2020 Jul 15 doi: 10.1016/j.path.2020.05.005. PMID: 32773194
Prognostic Significance of Stromal and Intraepithelial Tumor-Infiltrating Lymphocytes in Small Intestinal Adenocarcinoma.
Jun SY, Park ES, Lee JJ, Chang HK, Jung ES, Oh YH, Hong SM
Am J Clin Pathol 2020 Jan 1;153(1):105-118. doi: 10.1093/ajcp/aqz136. PMID: 31576398
Medullary carcinoma of the small bowel.
Brcic I, Cathomas G, Vanoli A, Jilek K, Giuffrida P, Langner C
Histopathology 2016 Jul;69(1):136-40. Epub 2016 Jan 13 doi: 10.1111/his.12908. PMID: 26599717
Prostaglandins and serotonin: nonpeptide diarrheogenic hormones.
Jaffe BM
World J Surg 1979 Sep 20;3(5):565-78. doi: 10.1007/BF01654761. PMID: 390899

Therapy

CABOCOL-01 trial: a single-arm phase II study assessing safety and efficacy of Cabozantinib for advanced or metastatic cervical carcinoma after platinum treatment failure.
Coquan E, Brachet PE, Licaj I, Leconte A, Castera M, Lequesne J, Meriaux E, Bonnet I, Lelaidier A, Clarisse B, Joly F
BMC Cancer 2021 Sep 25;21(1):1054. doi: 10.1186/s12885-021-08758-9. PMID: 34563169Free PMC Article
A water soluble tri-cationic porphyrin-EDTA conjugate induces apoptosis in human neuroendocrine tumor cell lines.
Schwach G, Thamyongkit P, Reith LM, Svejda B, Knör G, Pfragner R, Schoefberger W
Bioorg Chem 2012 Feb;40(1):108-113. Epub 2011 Oct 6 doi: 10.1016/j.bioorg.2011.09.006. PMID: 22024042Free PMC Article
Prostaglandins and serotonin: nonpeptide diarrheogenic hormones.
Jaffe BM
World J Surg 1979 Sep 20;3(5):565-78. doi: 10.1007/BF01654761. PMID: 390899
Histaminase activity: a biochemical marker for medullary carcinoma of the thyroid.
Baylin SB, Beaven MA, Buja LM, Keiser HR
Am J Med 1972 Dec;53(6):723-33. doi: 10.1016/0002-9343(72)90189-1. PMID: 4628882
Diarrhoea associated with medullary carcinoma of the thyroid.
Bernier JJ, Rambaud JC, Cattan D, Prost A
Gut 1969 Dec;10(12):980-5. doi: 10.1136/gut.10.12.980. PMID: 5366278Free PMC Article

Prognosis

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Medullary carcinoma of jejunum presenting as perforation peritonitis: A case report.
Talwar A, Ahuja A, Phulware RH
Indian J Pathol Microbiol 2022 Jul-Sep;65(3):679-682. doi: 10.4103/ijpm.ijpm_576_21. PMID: 35900501
CABOCOL-01 trial: a single-arm phase II study assessing safety and efficacy of Cabozantinib for advanced or metastatic cervical carcinoma after platinum treatment failure.
Coquan E, Brachet PE, Licaj I, Leconte A, Castera M, Lequesne J, Meriaux E, Bonnet I, Lelaidier A, Clarisse B, Joly F
BMC Cancer 2021 Sep 25;21(1):1054. doi: 10.1186/s12885-021-08758-9. PMID: 34563169Free PMC Article
Approaches to Biopsy and Resection Specimens from the Ampulla.
Xue Y, Reid MD
Surg Pathol Clin 2020 Sep;13(3):453-467. Epub 2020 Jul 15 doi: 10.1016/j.path.2020.05.005. PMID: 32773194
Prognostic Significance of Stromal and Intraepithelial Tumor-Infiltrating Lymphocytes in Small Intestinal Adenocarcinoma.
Jun SY, Park ES, Lee JJ, Chang HK, Jung ES, Oh YH, Hong SM
Am J Clin Pathol 2020 Jan 1;153(1):105-118. doi: 10.1093/ajcp/aqz136. PMID: 31576398

Clinical prediction guides

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Pitx2 is a useful marker of midgut-derived neuroendocrine tumours - an immunohistochemical study of 224 cases.
Soukup J, Manethova M, Faistova H, Krbal L, Vitovcova B, Hornychova H, Drugda J, Cesak T, Netuka D, Gabalec F, Ryska A
Histopathology 2022 Dec;81(6):799-807. Epub 2022 Sep 22 doi: 10.1111/his.14789. PMID: 36089904
Approaches to Biopsy and Resection Specimens from the Ampulla.
Xue Y, Reid MD
Surg Pathol Clin 2020 Sep;13(3):453-467. Epub 2020 Jul 15 doi: 10.1016/j.path.2020.05.005. PMID: 32773194
Prognostic Significance of Stromal and Intraepithelial Tumor-Infiltrating Lymphocytes in Small Intestinal Adenocarcinoma.
Jun SY, Park ES, Lee JJ, Chang HK, Jung ES, Oh YH, Hong SM
Am J Clin Pathol 2020 Jan 1;153(1):105-118. doi: 10.1093/ajcp/aqz136. PMID: 31576398
Medullary carcinoma of the small bowel.
Brcic I, Cathomas G, Vanoli A, Jilek K, Giuffrida P, Langner C
Histopathology 2016 Jul;69(1):136-40. Epub 2016 Jan 13 doi: 10.1111/his.12908. PMID: 26599717

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