Autopsy and Case Reports
https://app.periodikos.com.br/journal/autopsy/article/doi/10.4322/acr.2023.434
Autopsy and Case Reports
Autopsy Case Report

Fatal sclerosing mesenteritis: a 7-year-old male autopsy case report

Juan Carlos Celis Pinto; Lucía Hernández Peláez; Guillermo Mendoza Pacas; Juan Mayordomo Colunga; Milagros Balbín; Ana Pitiot; Héctor-Enrique Torres-Rivas; Verónica Blanco Lorenzo

Downloads: 13
Views: 919

Abstract

Sclerosing Mesenteritis (SM) is a rare diagnosis, particularly in pediatric patients, and is typically non-fatal when appropriately treated. Although molecular and immunohistochemical alterations have been described, no pathognomonic signature has been identified for this entity. This report presents a case of a seven-year-old boy who suffered sudden cardiorespiratory arrest. Upon autopsy, he was found to have multicentric SM on the upper mesentery, which led to bowel wall thinning and abdominal bleeding with bacterial translocation. We performed comprehensive morphological, immunohistochemical, and molecular analyses. SM is an atypical disorder with diverse clinical manifestations, including a rare but potentially fatal course. Early diagnosis is critical, given its potential severity. To our knowledge, this is the first case report of pediatric mortality linked to SM. Our findings emphasize the importance of increased awareness and early detection of SM in pediatric patients.

Keywords

Panniculitis, Peritoneal, Autopsy, Fatal Outcome, Pediatrics, Case Reports

References

1 Jura V, editor. Sulla mesenterite retractile sclerosante. Rome: Policlinico; 1924.

2 Kuhrmeier A. Mesenteric lipodystrophy. Schweiz Med Wochenschr. 1985;115(36):1218-24. PMid:2413530.

3 Daskalogiannaki M, Voloudaki A, Prassopoulos P, et al. CT evaluation of mesenteric panniculitis: prevalence and associated diseases. AJR Am J Roentgenol. 2000;174(2):427-31. http://dx.doi.org/10.2214/ajr.174.2.1740427. PMid:10658720.

4 Sharma P, Yadav S, Needham CM, Feuerstadt P. Sclerosing mesenteritis: a systematic review of 192 cases. Clin J Gastroenterol. 2017;10(2):103-11. http://dx.doi.org/10.1007/s12328-017-0716-5. PMid:28197781.

5 Kipfer RE, Moertel CG, Dahlin DC. Mesenteric lipodystrophy. Ann Intern Med. 1974;80(5):582-8. http://dx.doi.org/10.7326/0003-4819-80-5-582. PMid:4499963.

6 Emory TS, Monihan JM, Carr NJ, Sobin LH. Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity? Am J Surg Pathol. 1997;21(4):392-8. http://dx.doi.org/10.1097/00000478-199704000-00004. PMid:9130985.

7 Coulier B. Mesenteric panniculitis. Part 2: prevalence and natural course: MDCT prospective study. JBR-BTR. 2011;94(5):241-6. http://dx.doi.org/10.5334/jbr-btr.659. PMid:22191288.

8 Green MS, Chhabra R, Goyal H. Sclerosing mesenteritis: a comprehensive clinical review. Ann Transl Med. 2018;6(17):336. http://dx.doi.org/10.21037/atm.2018.07.01. PMid:30306075.

9 Danford CJ, Lin SC, Wolf JL. Sclerosing mesenteritis. Am J Gastroenterol. 2019;114(6):867-73. http://dx.doi.org/10.14309/ajg.0000000000000167. PMid:30829677.

10 Mullen JT, Delaney TF, Kobayashi WK, et al. Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series. Ann Surg Oncol. 2012;19(13):4028-35. http://dx.doi.org/10.1245/s10434-012-2638-2. PMid:22965569.

11 Meazza C, Belfiore A, Busico A, et al. AKT1 and BRAF mutations in pediatric aggressive fibromatosis. Cancer Med. 2016;5(6):1204-13. http://dx.doi.org/10.1002/cam4.669. PMid:27062580.

12 Alman B, Attia S, Baumgarten C, et al. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020;127:96-107. http://dx.doi.org/10.1016/j.ejca.2019.11.013. PMid:32004793.

13 Razanamahery J, Humbert S, Malakhia A, et al. AB1056 symptomatic sclerosing mesenteritis revealing erdheim-chester disease: a rare condition mediated by braf. Ann Rheum Dis. 2020;79(Suppl 1):1818. http://dx.doi.org/10.1136/annrheumdis-2020-eular.2447.

14 Trautmann M, Rehkämper J, Gevensleben H, et al. Novel pathogenic alterations in pediatric and adult desmoid-type fibromatosis - A systematic analysis of 204 cases. Sci Rep. 2020;10(1):3368. http://dx.doi.org/10.1038/s41598-020-60237-6. PMid:32099073.

15 Davis JL, Lockwood CM, Albert CM, Tsuchiya K, Hawkins DS, Rudzinski ER. Infantile NTRK-associated Mesenchymal Tumors. Pediatr Dev Pathol. 2018;21(1):68-78. http://dx.doi.org/10.1177/1093526617712639. PMid:28683589.

16 Albert CM, Davis JL, Federman N, Casanova M, Laetsch TW. TRK fusion cancers in children: a clinical review and recommendations for screening. J Clin Oncol. 2019;37(6):513-24. http://dx.doi.org/10.1200/JCO.18.00573. PMid:30592640.

17 Akram S, Pardi DS, Schaffner JA, Smyrk TC. Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. Clin Gastroenterol Hepatol. 2007;5(5):589-96, quiz 23-4. http://dx.doi.org/10.1016/j.cgh.2007.02.032. PMid:17478346.

18 Daumas A, Agostini S, Villeret J, Ah-Soune P, Emungania O, Granel B. Spontaneous resolution of severe, symptomatic mesocolic panniculitis: a case report. BMC Gastroenterol. 2012;12(1):59. http://dx.doi.org/10.1186/1471-230X-12-59. PMid:22672224.

19 Liang CP, Yang M, Chen PY, Geng LL, Li DY, Gong ST. Sclerosing mesenteritis in a 5-year-old Chinese boy: a case report. BMC Pediatr. 2017;17(1):179. http://dx.doi.org/10.1186/s12887-017-0931-8. PMid:28764764.

20 Iannaccone SF, Švajdler P, Sopková D, Fröhlichová L, Farkaš D. Incidental idiopathic focal sclerosing mesenteritis in a 4-month-old child. Cesk Patol. 2019;55(4):235-8. PMid:31842555.
 


Submitted date:
02/21/2023

Accepted date:
04/29/2023

Publication date:
05/31/2023

6477611ea953957c0d4a6de2 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections