Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports
Abstract Background Intra-abdominal desmoid tumors are rare soft tissue tumors that arise mainly in the mesentery and pelvis. Their etiology may include genetic mutations, estrogen-associated changes after childbirth, and mechanical factors such as a history of abdominal surgery. However, there are...
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| Format: | Article |
| Language: | English |
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BMC
2021-09-01
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| Series: | Journal of Medical Case Reports |
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| Online Access: | https://doi.org/10.1186/s13256-021-03058-z |
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| author | Hiroshi Kuwabara Sou Katayanagi Itsuki Koganezawa Masashi Nakagawa Kenji Katsumata Akihiko Tsuchida Shigeyuki Kawachi |
| author_facet | Hiroshi Kuwabara Sou Katayanagi Itsuki Koganezawa Masashi Nakagawa Kenji Katsumata Akihiko Tsuchida Shigeyuki Kawachi |
| author_sort | Hiroshi Kuwabara |
| collection | DOAJ |
| description | Abstract Background Intra-abdominal desmoid tumors are rare soft tissue tumors that arise mainly in the mesentery and pelvis. Their etiology may include genetic mutations, estrogen-associated changes after childbirth, and mechanical factors such as a history of abdominal surgery. However, there are cases of intra-abdominal desmoid tumors that develop in the absence of such causes. Since they are rare, diagnosis is often difficult based on clinical findings. We encountered two cases of patients with sporadic intra-abdominal desmoid tumors with a very unusual onset and contrasting features. Case presentation The first patient was a 51-year-old asian man who presented with sudden onset of abdominal pain. He was referred to our department because of a giant tumor detected on abdominal ultrasonography. Imaging revealed a 19-cm tumor with internal tumoral hemorrhage; however, no definitive diagnosis was made. Tumor resection was performed for diagnostic and therapeutic purposes. The second patient was a 41-year-old asian man, and right hydronephrosis was detected on abdominal ultrasonography during a periodic medical checkup. We diagnosed invasion of the primary mesenteric tumor into the right ureter using diagnostic imaging and performed ileocecal resection with partial right ureteral resection for a definitive diagnosis and therapeutic purposes. Although the tumors of both patients had developed from the ileal mesentery, the tumors were substantially different from each other based on their imaging findings, macroscopic morphology, and progression pattern. Meanwhile, they showed similar pathological characteristics. Both consisted of bundles of collagen fibrils of spindle-shaped fibroblasts with low cell atypia. Moreover, they were diagnosed as desmoid tumors using positive immunohistochemical staining for β-catenin. Conclusions Neither patient had susceptibility factors for desmoid tumors, and to our knowledge, there have been very few reports to date of intra-abdominal desmoid tumors that were diagnosed because of acute abdominal pain caused by tumoral hemorrhage or asymptomatic obstructive uropathy. Furthermore, it is clinically interesting that the two patients showed contrasting progression patterns and imaging findings. Intra-abdominal desmoid tumors are rare and may present with various symptoms and findings similar to those observed in our patients. Diagnosis therefore requires experience and knowledge that is not bound by preconceptions. |
| first_indexed | 2024-12-14T18:32:07Z |
| format | Article |
| id | doaj.art-40f71cedf3524f419e15a35705eb6bec |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-12-14T18:32:07Z |
| publishDate | 2021-09-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-40f71cedf3524f419e15a35705eb6bec2022-12-21T22:51:45ZengBMCJournal of Medical Case Reports1752-19472021-09-011511910.1186/s13256-021-03058-zSporadic intra-abdominal desmoid tumor with a very unusual onset: two case reportsHiroshi Kuwabara0Sou Katayanagi1Itsuki Koganezawa2Masashi Nakagawa3Kenji Katsumata4Akihiko Tsuchida5Shigeyuki Kawachi6Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Gastrointestinal and Pediatric Surgery, Tokyo Medical UniversityDepartment of Gastrointestinal and Pediatric Surgery, Tokyo Medical UniversityDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterAbstract Background Intra-abdominal desmoid tumors are rare soft tissue tumors that arise mainly in the mesentery and pelvis. Their etiology may include genetic mutations, estrogen-associated changes after childbirth, and mechanical factors such as a history of abdominal surgery. However, there are cases of intra-abdominal desmoid tumors that develop in the absence of such causes. Since they are rare, diagnosis is often difficult based on clinical findings. We encountered two cases of patients with sporadic intra-abdominal desmoid tumors with a very unusual onset and contrasting features. Case presentation The first patient was a 51-year-old asian man who presented with sudden onset of abdominal pain. He was referred to our department because of a giant tumor detected on abdominal ultrasonography. Imaging revealed a 19-cm tumor with internal tumoral hemorrhage; however, no definitive diagnosis was made. Tumor resection was performed for diagnostic and therapeutic purposes. The second patient was a 41-year-old asian man, and right hydronephrosis was detected on abdominal ultrasonography during a periodic medical checkup. We diagnosed invasion of the primary mesenteric tumor into the right ureter using diagnostic imaging and performed ileocecal resection with partial right ureteral resection for a definitive diagnosis and therapeutic purposes. Although the tumors of both patients had developed from the ileal mesentery, the tumors were substantially different from each other based on their imaging findings, macroscopic morphology, and progression pattern. Meanwhile, they showed similar pathological characteristics. Both consisted of bundles of collagen fibrils of spindle-shaped fibroblasts with low cell atypia. Moreover, they were diagnosed as desmoid tumors using positive immunohistochemical staining for β-catenin. Conclusions Neither patient had susceptibility factors for desmoid tumors, and to our knowledge, there have been very few reports to date of intra-abdominal desmoid tumors that were diagnosed because of acute abdominal pain caused by tumoral hemorrhage or asymptomatic obstructive uropathy. Furthermore, it is clinically interesting that the two patients showed contrasting progression patterns and imaging findings. Intra-abdominal desmoid tumors are rare and may present with various symptoms and findings similar to those observed in our patients. Diagnosis therefore requires experience and knowledge that is not bound by preconceptions.https://doi.org/10.1186/s13256-021-03058-zAggressive fibromatosisMesenteric fibromatosisSurgeryTumoral hemorrhageObstructive uropathy |
| spellingShingle | Hiroshi Kuwabara Sou Katayanagi Itsuki Koganezawa Masashi Nakagawa Kenji Katsumata Akihiko Tsuchida Shigeyuki Kawachi Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports Journal of Medical Case Reports Aggressive fibromatosis Mesenteric fibromatosis Surgery Tumoral hemorrhage Obstructive uropathy |
| title | Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports |
| title_full | Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports |
| title_fullStr | Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports |
| title_full_unstemmed | Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports |
| title_short | Sporadic intra-abdominal desmoid tumor with a very unusual onset: two case reports |
| title_sort | sporadic intra abdominal desmoid tumor with a very unusual onset two case reports |
| topic | Aggressive fibromatosis Mesenteric fibromatosis Surgery Tumoral hemorrhage Obstructive uropathy |
| url | https://doi.org/10.1186/s13256-021-03058-z |
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