Surprising finding of desmoid in a young man by an urologist

Česká verze

Authors: J. Marešová ¹; J. Kočárek- ^{1 3}; M. Čermák ^1,2; L. Plincelnerová ^1,4
Authors place of work: Urologické oddělení, Oblastní nemocnice, Kladno, a. s., Nemocnice, Středočeského kraje ¹; Urologická klinika 1. LF UK, a ÚVN – VFN Praha ²; Urologická klinika 3. LF UK, a FN Královské Vinohrady, Praha, ⁴ Urologická klinika 1. LF UK, a VFN v Praze ³
Published in the journal: Rozhl. Chir., 2025, roč. 104, č. 2, s. 61-66.
Category: Kazuistika
doi: https://doi.org/10.48095/ccrvch202561

Summary

We present a case report of a 26-year-old patient with an incidental finding of intra-abdominal fibromatosis (desmoid) in the small pelvis who presented to the urology outpatient clinic for erectile dysfunction. Ultrasound examination revealed hydronephrosis of the right kidney with parenchymal reduction, and a bulky tumor was detected in the small pelvis, which was compressing the right-sided ureter. The patient was followed up with a CT scan which confirmed a solid tumour in the small pelvis of unclear origin, the nature of the tumour and the treatment were discussed by the multidisciplinary team and a decision was made to resect the tumour. Pathologist confirmed sporadic variant of desmoid. Desmoid tumors are characterized by both locally aggressive carcinomas mimicking growth and frequent recurrences after resection, which may require subsequent oncological treatment. For these reasons, patients with desmoids should be followed up by an oncologist, ideally at centers experienced in treating this rare disease.

Keywords:

hydronephrosis – Nephrectomy – fibromatosis – desmoid – Gardner syndrome

Zdroje

1. Ganeshan D, Amini B, Nikolaidis P et al. Current update on desmoid fibromatosis. J Comput Assist Tomogr 2019; 43(1): 29–38. doi: 10.1097/RCT.0000000000000790.

2. Bektas M, Bell T, Khan S et al. Desmoid tumors: a comprehensive review. Adv Ther 2023; 40(9): 3697–3722. doi: 10.1007/s12325-023-02592-0.

3. Kumar SS, Rajeevan K, Devarajan E. Desmoid-type fibromatosis –⁠ clinical study of an uncommon disease. Indian J Surg Oncol 2020; 11(1): 71–74. doi: 10.1007/s13193-019-00985-8.

4. Ophir G, Sivan S, Hana S et al. Abdominal desmoid: course, severe outcomes, and unique genetic background in a large local series. Cancers (Basel) 2021; 13(15): 3673. doi: 10.3390/cancers13153673.

5. Kasper B, Baumgarten C, Garcia J et al. An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol 2017; 28(10): 2399–2408. doi: 10.1093/annonc/mdx323.

6. Grochol L, Cibulková I, Hrudka J et al. Desmoidní fibromatóza jako vzácný nádor mezenteria. Gastroent Hepatol 2022; 76(1): 67–71. doi: 10.48095/ccgh202267.

7. Kyriakidis F, Kogias D, Venou TM et al. Updated perspectives on the diagnosis and management of familial adenomatous polyposis. Appl Clin Genet 2023; 16 : 139–153. doi: 10.2147/TACG.S372241.

8. Huh SY, Shin JW, Na JI et al. The efficacy and safety of 4-n-butylresorcinol 0.1% cream for the treatment of melasma: a randomized controlled split-face trial. Ann Dermatol 2010; 22(1): 21–25. doi: 10.5021/ad.2010.22.1.21.

9. Napolitano A, Mazzocca A, Spalato Ceruso M et al. Recent advances in desmoid tumor therapy. Cancers (Basel) 2020; 12(8): 2135. doi: 10.3390/cancers12082135.

10. Nuyttens JJ, Rust PF, Thomas CR et al. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer 2000; 88(7): 1517–1523. doi: 10.1002/(SICI)1097-0142(20000401)88 : 7<1517::AID-CNCR3>3.0.CO;2-9.

11. Garbay D, Le Cesne A, Penel N et al. Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG). Ann Oncol 2012; 23(1): 182–186. doi: 10.1093/annonc/mdr051.

12. Toulmonde M, Pulido M, Ray-Coquard I et al. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non -⁠ -comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol 2019; 20(9): 1263–1272. doi: 10.1016/S1470-2045(19)30276-1.

13. Novotný R, Mendl J, Kočík M et al. Obří agresivní intraabdominální fibromatóza desmoidního typu kazuistika. Rozhl Chir 2022; 101(7): 337–341. doi: 10.33699/PIS.2022.101.7.337–341.

MUDr. Jiřina Marešová

Urologické oddělení

Oblastní nemocnice Kladno, a.s.

Nemocnice Středočeského kraje

Vančurova 1548

272 59 Kladno

uro@nemk.cz