Multivisceral transplant is a viable treatment option for patients with non‐resectable intra‐abdominal fibromatosis

Chi, Zhikai; Mangus, Richard S.; Kubal, Chandrashekhar A.; Chen, Shaoxiong; Ji, Lin

doi:10.1111/ctr.13186

Cited by 5 publications

(2 citation statements)

References 24 publications

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“…The tumor cells of fibromatosis have been reported to be positive for SMA, desmin, calponin, and estrogen receptor β, but negative for CD34. At molecular levels, fibromatosis sporadically harbors CTNNB1 mutation, which encodes β-catenin protein [ 6 ]. In this case, CTNNB1 mutation was not detected.…”

Section: Discussionmentioning

confidence: 99%

A novel entity of HIPK2::YAP1 pulmonary fibromatosis

Liu,

Liang,

Chen

et al. 2024

BMC Pulm Med

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Background Pulmonary fibromatosis (PF) is a specific variant of fibromatosis, which is rarely reported occurring in the lung. PF with HIPK2-YAP1 fusion was a novel entity. Case presentation In this report, a 66-year-old male with PF had been smoking over 40 years. Multiple cords and small nodules in both lungs had been detected in a health examination two years earlier at our hospital. But approximately twofold enlarged in the lingual segment of the upper lobe in the left lung were disclosed in this year. Immunohistochemical analysis demonstrated that the vimentin and β-Catenin were positive in the largest nodule. After underwent a DNA/RNA panel next-generation sequencing (NGS), missense mutations and HIPK2-YAP1 fusion were found in this sample. Ultimately, the case diagnosis as PF with HIPK2-YAP1 fusion after multidisciplinary treatment. Currently, the patient is doing well and recurrence-free at 14 months post-surgery. Conclusions It’s difficult for patients with complex morphology to make accurate diagnosis solely based on morphology and immunohistochemistry. But molecular detection is an effective method for further determining pathological subtypes.

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Section: Discussionmentioning

confidence: 99%

A novel entity of HIPK2::YAP1 pulmonary fibromatosis

Liu,

Liang,

Chen

et al. 2024

BMC Pulm Med

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“…Meanwhile, successful small bowel transplantation after total bowel resection has also been reported in patients with intra-abdominal DTs recently, [24][25][26] bringing hope to more patients.…”

Section: Surgerymentioning

confidence: 99%

Update on Familial Adenomatous Polyposis-Associated Desmoid Tumors

Yang

Ding

2023

Clin Colon Rectal Surg

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Desmoid tumors (DT) represent the second high risk of tumor in familial adenomatous polyposis (FAP) patients. Although FAP-associated DTs (FAP-DT) are caused by germline mutations in the adenomatous polyposis coli (APC) gene, extracolonic manifestations, sex, family history, genotype, and the ileal pouch anal anastomosis procedure are all linked to the development of DTs in FAP patients. Multidisciplinary management has replaced aggressive surgery as the preferred treatment of DTs. There is growing evidence to support the use of active surveillance strategy as first-line treatment for FAP-DT patients. Radiotherapy for intra-abdominal desmoids is now rarely used because of severe late toxicity. Pharmacotherapy, however, represents a promising future with the improvement of traditional cytotoxic drugs and the investigation of targeted drugs. Although nonsurgery treatment has been used widely nowadays, surgery remains the mainstay when symptomatic or life-threatening DTs are present. Further research will be needed for more optimal clinical practice.

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