A Case of Medullary Carcinoma of the Jejunum Combined with the Intestinal Lymphangiectasia Accompanied by the Malabsorption Syndrome
O. A. Lobanova,
D. S. Trusova,
M. A. Afonina,
V. E. Varentsoy,
D. D. Protsenko,
N. V. Kretova,
N. B. Serezhnikova,
M. A. Peshkova,
N. V. Zharkov,
S. E. Kochetkova,
Yu. Yu. Stepanova,
Z. D. Shtanev,
K. A. Vekhova,
V. A. Yumasheva,
A. O. Kolesnikova,
M. Akan,
M. O. Chanturiya,
T. V. Serebrov,
A. S. Tertychnyy,
H. Guski,
E. E. Rudenko,
T. A. Demura,
E. A. Kogan https://doi.org/10.22416/1382-4376-2023-33-4-92-100
Abstract
Aim: to present a clinical and morphological observation of an extremely rare combination of medullary carcinoma of the jejunum and intestinal lymphangiectasia in a 33-year-old patient with clinical features of malabsorption syndrome over the 10 years.
Key points. An autopsy revealed a tumor formation spreading from the wall of the jejunum to the mesentery, with metastases to the mesenteric lymph nodes. The medullary carcinoma with positive expression of СD117, DOG1, EMA, PanCK, PDL-1, vimentin, mosaic non-intense expression of CA19-9, calretinin, CD10, CDX2, CEA, MUC-5AC, SATB2, and negative reaction to ALK, CD3, CD8, CD20, CD30, CD31, CD34, CD45, CD56, chromogranin, CK7, CK20, desmin. The proliferative index was high: Ki-67 > 80 %. Moreover, during the histological examination of the intestinal wall, intestinal lymphangiectasia complicated by the malabsorption syndrome was revealed.
Conclusion. The uniqueness of this clinical and morphological case is in the combination of medullary carcinoma of the jejunum metastasized to the mesenteric lymph nodes with the underlying intestinal lymphangiectasia accompanied by the development of malabsorption syndrome.
Keywords
small bowel medullary carcinoma,
medullary carcinoma,
intestinal lymphangiectasia,
malabsorption syndrome,
immunohistochemical analysis
Supporting agencies: the work was carried out with financial support from the Ministry of Science and Higher Education of the Russian Federation within the framework of state support for the creation and development of world-class Scientific Centers “Digital Biodesign and Personalized Healthcare” No. 075-15-2022-304.
About the Authors
O. A. Lobanova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Olga A. Lobanova — Junior Researcher of World-Class Research Center “Digital Biodesign and Personalized Healthcare”, Teaching Assistant of the Institute of Clinical Morphology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
D. S. Trusova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Daria S. Trusova — Resident of the Department of Forensic Medical Examination
119fi35, Moscow, Rossolimo str., 15/13, bld. 2
M. A. Afonina
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Maria A. Afonina — Teaching Assistant of the Institute of Clinical Morphology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
V. E. Varentsoy
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Vyacheslav E. Varentsov — Cand. Sci. (Med.), Associate Professor of the Department of Human Anatomy and Histology
125009, Moscow, Mokhovaya str., 11, bld. 10
D. D. Protsenko
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Dmitriy D. Protsenko — Cand. Sci. (Med.), Docent, Deputy Director for Academic Affairs at the Institute of Clinical Morphology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
N. V. Kretova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Natalia V. Kretova — Junior Researcher of the World- Class Research Center “Digital Biodesign and Personalized Healthcare”, Pathologist at the Institute of Clinical Morphology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
N. B. Serezhnikova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Natalia B. Serezhnikova — Cand. Sci. (Biol.), Senior Researcher at the Biobank of the Institute of Regenerative Medicine of the Scientific and Technological Park
119048, Moscow, Trubetskaya str., 8
M. A. Peshkova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Maria A. Peshkova — Junior Researcher of the World- Class Research Center “Digital Biodesign and Personalized Healthcare”
119435, Moscow, Bolshaya Pirogovskaya str., 6, bld. 1
N. V. Zharkov
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Nikolay V. Zharkov — Cand. Sci. (Biol.), Junior Researcher of the World-Class Research Center “Digital Biodesign and Personalized Healthcare”, Biologist at the Institute of Clinical Morphology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
S. E. Kochetkova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Svetlana E. Kochetkova — Student
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
Yu. Yu. Stepanova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Yulia Yu. Stepanova — Student
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
Z. D. Shtanev
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Zakhar D. Shtanev — Student
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
K. A. Vekhova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Ksenia A. Vekhova — Student
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
V. A. Yumasheva
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Valentina A. Yumasheva — Student
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
A. O. Kolesnikova
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Anastasia O. Kolesnikova — Student
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
M. Akan
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Mikhail Akan — Student
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
M. O. Chanturiya
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Mamuka O. Chanturiya — Cand. Sci. (Med.), Surgeon, Head of the Surgical Department, University Hospital No. 4
1190fi8, Moscow, Dovatora str., 15, bld. 2
T. V. Serebrov
Russian State Research Center − Burnasyan Federal Medical Biophysical Center of Federal Medical Biological Agency
Russian Federation
Russian Federation
Tikhon V. Serebrov — Cand. Sci. (Med.), Head of the Department of Maxillofacial and Osteoplastic Surgery, Associate Professor of the Department of Oncology and Radiation Therapy with a Course in Medical Physics
123098, Moscow, Marshala Novikova str., 23
A. S. Tertychnyy
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Alexander S. Tertychnyy — Dr. Sci. (Med.), Professor, Pathologist, Oncomorphologist, Institute of Clinical Mor- phology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
H. Guski
Berlin University Medical Center
Germany
Germany
Hans Guski — Dr. Sci. (Med.), Professor, Pathologist, Oncomorphologist, Medizinische Fakultät Charite, Universitätsmedizin Berlin (Charite Faculty of Medicine, Berlin University Medical Center)
10117, Berlin, Charitéplatz, 1
E. E. Rudenko
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Ekaterina E. Rudenko — Cand. Sci. (Med.), Pathologist, Senior Lecturer, Deputy Director for Research at the Institute of Clinical Morphology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
T. A. Demura
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Tatiana A. Demura — Dr. Sci. (Med.), Professor, Director of the Institute of Clinical Morphology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
E. A. Kogan
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Russian Federation
Russian Federation
Evgeniya A. Kogan — Dr. Sci. (Med.), Professor, Pathologist, Oncomorphologist, Institute of Clinical Morphology and Digital Pathology
119435, Moscow, Abrikosovskiy lane, 1, bld. 1
References
1. Gonzalez HH, Sidhu S, Eisner T. A Rare Case of Medullary Carcinoma of the Ileum, Cureus, 2018; 10: 10–14
2. Scott OR, West NP, Cairns A. Is medullary carcinoma of the colon underdiagnosed? An audit of poorly differentiated colorectal carcinomas in a large national health service teaching hospital, Histopathology, 2020; 78: 963–969
3. Fatima Z, Sharma P, Youssef B, et al. Medullary Carcinoma of the Colon: A Histopathologic Challenge. Cureus, 2021; 13: e15831
4. Kumar S, Raja K, Gnanasekaran S, et al. Intestinal lymphangiectasia: a rare cause of intussusception in an adolescent. Ann. R. Coll. Surg. Engl., 2019; 101: e43
5. Haddar Z El, Sbia Y, Rkain M, et al. Complicated primary intestinal lymphangiectasia (Waldmann’s disease) in a child successfully treated with octreotide: A case report from a low-resource setting. Ann. Med. Surg., 2021; 68: 102588
6. Friedman K., Brodsky A, Lu S, et al. Medullary carcinoma of the colon: a distinct morphology reveals a distinctive immunoregulatory microenvironment, Mod. Pathol., 2016; 29: 528–541
7. Nguyen J, Coppola D, Shan Y, et al. Poorly differentiated medullary carcinoma of the colon with an unusual phenotypic profile mimicking high grade large cell lymphoma – a unique case report and review of the literature, Int. J. Clin. Exp. Pathol., 2014; 7: 828
8. Martinotti M. Cirillo F, Ungari M, et al. Microsatellite Instability in Medullary Carcinoma of the Colon, Rare Tumors, 2017; 9: 23–25
9. P. Giuffrida, Giovanni A, Grillo F, et al. PD-L1 in small bowel adenocarcinoma is associated with etiology and tumor-infiltrating lymphocytes, in addition to microsatellite instability, Mod. Pathol., 2020; 33: 1398–1409
10. Brcic I, Cathomas G, Vanoli A, et al. Medullary carcinoma of the small bowel, Histopathology, 2016; 69: 136–140
11. Fiehn A-M, Grauslund M, Glenthoj A, et al. Medullary carcinoma of the colon: can the undifferentiated be differentiated?, Virchows Arch., 2015; 466: 13–20
12. Remo A, Fassan M, Vanoli A et al., Morphology and molecular features of rare colorectal carcinoma histotypes, Cancers (Basel)., 2019; 11: 1–26
13. Kojima M, Miyake T, Ueki T et al. INI1-negative colorectal undifferentiated carcinoma with rhabdoid features and postoperative rapidly growing liver metastases: a case report and review of the literature, Surg. Case Reports, 2021; 7: 104
14. Morimoto Y, Takaoka M, Monobe Y, et al. Three Cases of Colon Medullary Carcinoma in Our Institution, Gan To Kagaku Ryoho, 2021; 7: 967-969
15. Lin F, Shi J, Zhu S et al. Cadherin-17 and SATB2 are sensitive and specific immunomarkers for medullary carcinoma of the large intestine, Arch. Pathol. Lab. Med., 2014; 138: 1015–1026
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For citations:
Lobanova O.A., Trusova D.S., Afonina M.A., Varentsoy V.E., Protsenko D.D., Kretova N.V., Serezhnikova N.B., Peshkova M.A., Zharkov N.V., Kochetkova S.E., Stepanova Yu.Yu., Shtanev Z.D., Vekhova K.A., Yumasheva V.A., Kolesnikova A.O., Akan M., Chanturiya M.O., Serebrov T.V., Tertychnyy A.S., Guski H., Rudenko E.E., Demura T.A., Kogan E.A. A Case of Medullary Carcinoma of the Jejunum Combined with the Intestinal Lymphangiectasia Accompanied by the Malabsorption Syndrome. Russian Journal of Gastroenterology, Hepatology, Coloproctology. 2023;33(4):92-100. https://doi.org/10.22416/1382-4376-2023-33-4-92-100
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