Intestinal transplantation for tumors

Purpose of review: Transplantation of the intestine is a vital component in the treatment of many intrabdominal tumors. This is a comprehensive review of current practice and outcome of intestinal transplantation for tumors.

Recent findings: Most commonly described for the intrabdominal desmoid tumor, excellent survival outcomes can be attained, particularly for the more aggressive desmoid tumors associated with familial adenomatous polyposis syndrome and early referral is critical in order to avoid diffuse tumor spread which would necessitate a more complex multivisceral transplant. Currently, consensus within the intestinal transplant community lists invasive intrabdominal desmoid tumor as a primary indication for intestinal transplantation. Rare tumors such as metastatic neuroendocrine tumors confined to the abdomen, and pseudomyxoma peritonei have been associated with acceptable survival and recurrence outcomes with intestinal transplantation in carefully selected patients who would otherwise have a dismal prognosis. In addition, intestinal transplantation is an option for patients with a history of abdominal malignancy who have been rendered with intestinal failure due to surgical complications from resection of a primary tumor.

Summary: Intestinal transplantation is a therapeutic option with excellent results in selected patients with intrabdominal desmoid tumors, neuroendocrine tumors and pseudomyxoma peritonei. Careful patient selection, technical expertise, and posttransplant oncologic surveillance is critical for successful long-term outcomes.

Pelvic desmoid fibromatosis is a rare locally aggressive benign neoplasm, typically presenting in the reproductive age group with lower abdominal pain. Extra-abdominal occurrence being predominant, the pelvic origin of the tumour makes the clinical management a multidisciplinary challenge. Previous surgeries, current or previous pregnancies, and familial adenomatous polyposis have been documented as associated risk factors. Our patient was a young woman with three previous caesarean sections, admitted with a large infiltrative lesion extending into the left pelvic sidewall up to the pelvic bone, causing ipsilateral severe hydroureteronephrosis. Malignancy was suspected due to the infiltrating nature of the lesion on imaging. Due to its proximity to the pelvic veins, preoperative embolisation of the feeding vessel was done. An exploratory laparotomy was performed by a multidisciplinary team. For this deep-seated disease, mass excision was performed along with total hysterectomy, left salpingo-oophorectomy and left ureteroneocystostomy. A part of the lesion infiltrating the presacral fascia was not removed during the primary surgery. Within 24 hours of surgery, she developed critical limb ischaemia in the left limb due to thrombosis in the ipsilateral external iliac artery. An emergency thrombo-embolectomy was performed by the vascular surgeons and thromboprophylaxis continued. Final histopathology confirmed desmoid fibromatosis. For the residual disease, the patient has been under close follow-up and is receiving targeted therapy with oral sorafenib to prevent local progression. Her follow-up imaging showed a decrease in the size of the residual disease and she is asymptomatic currently.

No abstract available

Introduction: Desmoid tumours are rare locally invasive soft tissue neoplasms arising from mesenchymal tissue that lack metastatic potential but have a high propensity for local recurrence. For unresectable mesenteric desmoid tumours requiring systemic therapy, the therapeutic landscape spans anti-hormonal combinations, chemotherapy, tyrosine kinase inhibitors, and gamma-secretase inhibitors. Imatinib is a multi-target tyrosine kinase inhibitor targeting PDGFR-alpha/beta and c-KIT that has demonstrated clinical activity in desmoid tumours with a favourable toxicity profile.

Case presentation: We present a case of a 27-year-old white Australian man who presented with a large abdominal mass and weight loss. Imaging revealed a 23 × 12 × 21 cm mass that was diagnosed as a desmoid tumour via biopsy. The tumour was deemed to be unresectable and radiotherapy was considered to carry an unacceptable risk of toxicity. Initial treatment with tamoxifen and sulindac was unable to halt disease progression. The patient was subsequently commenced on imatinib 400 mg daily. Imaging after four months showed partial response, and over six years, complete radiological remission was achieved despite intermittent medication compliance. Aside from one episode of grade 1 photosensitivity, no significant toxicities were observed. Imatinib was self-ceased after ten years of treatment, and the patient remains disease-free at eleven years post-diagnosis under active surveillance.

Conclusion: We describe a case of complete and durable remission achieved with imatinib monotherapy in an unresectable mesenteric desmoid tumour with the patient remaining disease-free at 10 years. This case highlights the potential for long-term complete response and underscores the need for prospective data to guide treatment duration and cessation criteria.

Aims: Low-grade spindle cell lesions of the breast (LGSCL-B) are uncommon, with malignant counterparts being particularly rare. Management recommendations for desmoid-type fibromatosis (DF), in particular, have evolved.

Methods and results: A retrospective review of cases from 2000 to 2024 was conducted using LGSCL-B-related keyword searches. Clinical features were assessed, and when available, core needle biopsy (CNB) findings were correlated with excision specimens and imaging. Of 68 LGSCL-B samples, DF was the most frequent subtype (28 cases, 41%). A total of six resections were negative for β-catenin expression, including three resections from cases diagnosed as DF and three resections from cases categorized as undetermined on CNB and subsequently finalized as DF on resection. Myofibroblastoma occurred in 12 patients, more often in men (58%), with lipid-rich (n = 3) and myxoid (n = 2) variants. One lesion initially diagnosed as a solitary fibrous tumour was reclassified as a palisaded myofibroblastoma. Malignancy was identified in two biopsies: a spindle cell sarcoma and a fibromatosis-like metaplastic carcinoma; the latter was reclassified at resection as a malignant phyllodes tumour with heterologous bone formation. Thirteen CNB (19%) were indeterminate, including one described with ambiguous wording, a spindle cell sarcoma with a differential encompassing both benign and malignant entities.

Conclusions: Most LGSCL-B can be classified on CNB, but approximately one-fifth yield indeterminate results. Diagnostic challenges include overlapping morphological features, immunohistochemical profiles and potential reclassification at resection. Awareness of these pitfalls, along with selective use of adjunct molecular testing, may enhance diagnostic accuracy and reduce overtreatment.

Spindle cell fibromatosis of the breast (SCFB) is a rare benign tumor that closely mimics malignancy on imaging and clinical examination. Accurate diagnosis relies on histopathology and immunohistochemistry, particularly β-catenin staining, to guide appropriate management and avoid overtreatment while addressing its locally aggressive behavior and associated conditions.