Desmoid Research Articles

Investigation of clinicopathological characteristics and prognosis of abdominal Desmoid tumors

BackgroundAbdominal desmoid tumors (DTs) are rare soft-tissue neoplasms known for their relatively high local recurrence rate. This study aims to elucidate the clinicopathological features and investigate prognostic factors related to abdominal DTs across various tumor locations.MethodsFrom January 2000 to January 2021, patients diagnosed with abdominal DTs who underwent complete resection at the Cancer Hospital, Chinese Academy of Medical Sciences, were included in this study. Thorough collection and review of clinicopathologic characteristics and follow-up data were performed. Prognostic factors, including age at presentation, sex, tumor location, size, and proximity to nerves or vasculature, were meticulously analyzed to assess their impact on recurrence-free survival.ResultsA cohort of 226 patients diagnosed with abdominal DTs was categorized into two groups based on tumor site: the abdominal wall group (n = 132) and the intra-abdominal cavity group (n = 94). Distinct clinicopathological features and prognoses were observed between abdominal wall DTs and intra-abdominal DTs. During a median follow-up of 60 months, 24 patients (10.2%) experienced local recurrence. Univariate and multivariate analyses identified intra-abdominal tumors, tumor size >10cm, and positive margins as independent risk factors associated with poor prognosis.ConclusionAbdominal wall DTs demonstrate different clinicopathological characteristics and better prognoses compared to intra-abdominal DTs. By achieving negative margins, patients with abdominal wall DTs can achieve favorable therapeutic outcomes and prognosis through curative resection.

Real-word experience of pazopanib and sorafenib in patients with desmoid tumors: A CanSaRCC multi-center study

BackgroundSorafenib and pazopanib, two tyrosine kinase inhibitors (TKI), are widely used in patients with progressive symptomatic desmoid tumors (DT). Limited real-word data is available on long-term outcomes of patients who progressed on, stopped, or continued TKIs. MethodsPatients diagnosed with DTs and treated with sorafenib or pazopanib between 2011 and 2022 at 11 institutions were reviewed. Patient history, response to therapy and toxicity were recorded. Statistical analyses utilized Kaplan-Meier and log-rank tests. Results142 patients with DT treated with sorafenib (n = 126, 88.7 %) or pazopanib (n = 16, 11.3 %) were analyzed. The median treatment duration was 10.8 months (range: 0.07- 73.9). The overall response rate and the disease control rate were 26.0 % and 95.1 %, respectively. The median tumor shrinkage was − 8.5 % (range −100.0 %- +72.5 %). Among responders, the median time to an objective response was 15.2 months (range: 1.1 to 33.1). The 1-year and 2-year progression-free survival rates were 82 % and 80 %. Dose reductions were necessary in 34 (23.9 %) patients. Grade 3 or higher adverse events were reported in 36 (25.4 %) patients. On the last follow-up, 55 (38.7 %) patients continued treatment. Treatment discontinuation (n = 85, 59.9 %) was mainly for toxicity (n = 35, 45.9 %) or radiological or clinical progression (n = 30, 35.3 %). For the entire cohort, 36 (25.4 %) patients required subsequent treatment. In the 32 responders, only 1 (3.1 %) patient required a subsequent treatment. In patients who discontinued TKI, 25 (44.6 %) with stable disease received subsequent treatment compared to 0 (0.0 %) of responders. ConclusionThis retrospective study represents the largest cohort of DT patients treated with sorafenib or pazopanib to date. Discontinuation of treatment in responders is safe. The optimal treatment duration in patients with stable disease remains to be defined.

Active surveillance and emerging medical treatment options for desmoid: when and for whom?

This article discusses the evolving approaches to desmoid tumors management, shedding light on recent developments. Active surveillance has become the primary approach for managing primary peripheral desmoid tumors. This strategy was initially based on evidence from retrospective studies. Roughly 50% of cases managed with active surveillance show spontaneous stabilization or regression. Recent prospective trials conducted in Italy, The Netherlands, and France (2022-2023) confirm the efficacy of active surveillance, revealing 3-year progression-free survival rates ranging from 53.4 to 58%. For the patients under active surveillance, decisions regarding treatment are based on significant tumor growth or progressive symptoms. Moreover, three contemporary randomized trials investigated medical treatments for progressive or recurrent desmoid tumors. Sorafenib, pazopanib, and nirogacestat demonstrated clinical activity, as evidenced by favorable progression-free survival and objective response rates. Active surveillance has solidified its position as the primary management approach for desmoid tumors, validated by three robust prospective studies. Three recent randomized trials explored medical treatment for progressive or recurrent desmoid tumors, revealing promising clinical activities.

Onset and resolution of ovarian toxicity with nirogacestat treatment in females with desmoid tumors: Updated safety analyses from the DeFi phase 3 study.

Nirogacestat is a targeted gamma secretase inhibitor approved in the United States for adults with progressing desmoid tumors. In the phase 3 DeFi study (NCT03785964) of nirogacestat, ovarian toxicity (OT) was identified as a safety signal among females of reproductive potential (FORP). This analysis further describes the incidence, presentation, and resolution of OT. Patients were randomized to twice-daily oral nirogacestat (150 mg) or placebo, taken in continuous 28-day cycles. Investigator-identified OT in FORP was based on abnormal reproductive hormone values or perimenopausal symptoms (or both). Adverse event follow-up was conducted to assess OT resolution. Post hoc analyses included return of menstruation and return of follicle-stimulating hormone (FSH) to within normal limits (WNL) (≤20.4 mIU/mL). Of 92 randomized females, 73 in the safety population were FORP (n=36 nirogacestat, n=37 placebo). OT was identified in 75% (27 of 36) receiving nirogacestat and 0% (0 of 37) receiving placebo. As of October 24, 2022, investigators reported OT resolution in 78% (21 of 27) of patients, with median OT duration of 19.1 weeks. Off-treatment resolution was reported in all 11 patients (100%) who stopped nirogacestat treatment; of these, all nine with available menstruation information experienced return of menstruation and eight had FSH WNL at last reported assessment. Resolution was reported in 10 of 14 (71%) while on nirogacestat; of these, all 10 experienced return of menstruation and seven had FSH WNL. Two patients were lost to follow-up. Most FORP treated with nirogacestat experienced OT, with the majority resolving, including all who stopped treatment, suggesting that OT is transient.

Parasitic Leiomyoma, A Less Well Known Entity: A Case Report

Parasitic leiomyomas, also known as free leiomyomas, occur outside the uterus and are rarely seen in clinical practice. They usually occur in genitally active women who have undergone hysterectomy or myomectomy. It is difficult to determine the nature of the mass on the basis of imaging studies alone. Diagnosis of certainty is based on anatomopathological examination. Management is essentially surgical. We report the case of a 44-year-old woman with a history of subtotal hysterectomy for fibroleiomyomatous uterus and a lumbar disc herniation, presenting with an extra uterine abdomino-pelvic leiomyoma diagnosed on histology, whereas the CT diagnosis suggested a GIST (gastrointestinal stromal tumour) or desmoid tumour. Our case highlights the importance of considering ectopic leiomyoma as a differential diagnosis in the face of an abdomino-pelvic mass after hysterectomy.

Abstract PO4-21-01: Recurrent Desmoid Tumor and Ductal Carcinoma In Situ in an African-American Woman: A Case Report and Discussion on Disparities for These Two Cancer Subtypes

Abstract Introduction: Disparities in cancer care in patients from different races and ethnicities unfortunately do exist and are well described in literature. Hormonal-driven cancers, such as some soft-tissue tumors and DCIS, have been demonstrated to impact African-American women (AAW) disproportionately. These patients are at increased risk for not only development of these cancers, but also experience disparities in survival, treatment outcomes, and modalities of treatment. Clinical Case: A 47-year-old premenopausal AAW with past medical history of recurrent desmoid sarcoma of the abdomen and ductal carcinoma in-situ (DCIS) of the left breast presented to oncology clinic with recurrent abdominal mass. The patient was originally diagnosed with DCIS in the left breast in 2018 and subsequently underwent left breast mastectomy with TRAM reconstruction and fat graft later that year. In 2020, the patient noticed a left lower abdominal growth that quickly enlarged and became painful. She underwent imaging without biopsy and was taken to surgery in April 2021 with tumor removal and mesh placement. Pathology showed desmoid fibromatosis infiltrating skeletal muscle and involving the inked resection margin of the specimen. Conservative re-excision and additional therapy was recommended but not done at the time. In 2022, the patient had resection of the area again with pathology of the incisional scar showing skin and deep soft tissue foci suspicious for recurrent desmoid fibromatosis extending to the inked deep margin. No mesh was placed, and the wound closed almost completely. The patient was not aware of positive margins at the time. In January 2023, patient again notice a new bump that was bothersome. Initial CT and MRI without finding of defined mass, but with right rectus thickening and enlarged right external iliac lymph node. Core biopsy in June 2023 confirmed recurrent desmoid fibromatosis. In April 2023, annual screening with mammogram was performed with a finding of 2.9 cm cluster of heterogenous calcification in the right breast. Diagnostic mammogram and ultrasound performed. Core needle biopsy was subsequently performed in May with pathology consistent with high grade DCIS with low ER and PR positivity. Lymph node biopsy was negative, but clinically positive. She is currently undergoing further genetic testing with surgical workup for mastectomy. Discussion and Conclusion: AAW diagnosed with DCIS have higher all-cause and disease specific mortality in comparison to white women (WW) diagnosed with DCIS. AAW are also at increased risk of aggressive secondary breast cancers in both the ipsilateral and contralateral breast in comparison to other races. In regards to tumor biology, WW have been shown to have higher ER and PR positivity in both primary and primary breast cancers than AAW. In many of these large-scale studies higher mortality, recurrence, and metastasis in AAW have been identified regardless of treatment modality. Research into differences in treatment between AAW and WW has shown that AAW, in contrast to our patient case, are more likely to undergo endocrine and radiation therapy rather than mastectomy. Although there does not seem to be an increased risk of desmoid fibromatosis in AAW when compared to WW, other soft-tissue tumors and malignancies do show a predilection for AAW. Incidence of sarcoma in black patients is higher than white patients, and black patients also have poorer survival outcomes. This case provides an example of the intersectionality of two cancer subtypes that impact black patients disproportionately. More evidence is required to unearth the reasons why these two cancer subtypes which are driven by similar processes impact AAW to a greater degree. Citation Format: Vinita Akula, Jessica Jones. Recurrent Desmoid Tumor and Ductal Carcinoma In Situ in an African-American Woman: A Case Report and Discussion on Disparities for These Two Cancer Subtypes [abstract]. In: Proceedings of the 2023 San Antonio Breast Cancer Symposium; 2023 Dec 5-9; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2024;84(9 Suppl):Abstract nr PO4-21-01.

Sclerosing Mesenteritis: A Concise Clinical Review for Clinicians.

Sclerosing mesenteritis (SM), an idiopathic nonneoplastic condition affecting 0.18% to 3.14% of the population, is characterized by chronic fat necrosis, inflammation, and fibrosis most commonly of the mesentery of the small intestine. Sclerosing mesenteritis typically presents in the fifth or sixth decade of life, where patients with a history of abdominal surgery and/or autoimmune disease may be at higher risk. While many patients are asymptomatic, clinical features and complications are related to the mass effect resulting from the inflammation and fibrosis involved in the pathogenesis of SM. When present, common signs, symptoms, and complications include abdominal pain, weight loss, diarrhea, palpable abdominal mass on examination, bowel obstruction, chylous ascites, and mesenteric vessel thrombosis. Although SM was historically diagnosed predominantly by biopsy, current practice has shifted away from this to computed tomography imaging of the abdomen, given the invasive nature of biopsy. However, certain conditions, including mesenteric neoplasia (lymphoma, metastatic carcinoid tumor, desmoid tumor, mesenteric carcinomatosis), can mimic SM on imaging, and if clinical suspicion is equivocal, a biopsy may be warranted for definitive diagnosis. Asymptomatic patients do not require treatment. For patients with pronounced symptoms or complicated SM, the combination of tamoxifen 10 mg twice daily and prednisone 40 mg daily is the first-line pharmacotherapy; no randomized controlled trial of this regimen has been performed. Rarely, surgery may be necessary in cases of persistent bowel obstruction refractory to medical management. Sclerosing mesenteritis has an overall benign course in most cases, but disease progression and fatal outcomes have been reported.

Building the Evidence for Systemic Treatment in Desmoid Fibromatosis: Is a New Metronomic Regimen Another Layer in the Foundation?

@Thalcin explains how a metronomic regimen for desmoid tumors fits in the current treatment landscape.

Scar endometriosis as an easily misdiagnosed abdominal mass: a case report and review of the literature

Scar endometriosis, which is characterized by ectopic endometrial-like glands and stroma surrounding scar tissue, is rare and presents diagnostic challenges due to its inconsistent presentation. We report a case of a 40-year-old woman who had previously undergone two cesarean sections and a hysterectomy, in whom a mass in the subcutaneous layer of the lower abdomen was incidentally discovered by computed tomography (CT). A physical examination revealed a linear cesarean scar with a palpable, pigmented mass. An enhanced abdominal CT scan revealed an approximately 3.5-cm ill-defined soft tissue mass infiltrating the rectus abdominis muscle. A desmoid tumor was suspected based on the radiological findings, and endometriosis was also considered in light of the patient’s medical history. Wide mass excision was performed, ensuring 5-mm margins. The mass was completely excised, and a histopathological examination revealed endometriosis. Due to its rarity, scar endometriosis in the abdominal area can be easily misdiagnosed. Therefore, a heightened suspicion of scar endometriosis should be maintained in female patients with a history of abdominal or pelvic surgery presenting with an abdominal mass. Although imaging modalities can play a supportive role in the diagnosis, an extensive medical history assessment and comprehensive physical examination remain crucial.

A large inflammatory fibroid polyp of jejunum with unusual presentation: very difficult to diagnosis in paediatric patient

Inflammatory fibroid polyps (IFP), also known as Vanek tumours, are one of the rarest groups of gastrointestinal tract polyps. They represent 0.1-3.0% of all polyps in this organ system. Most common location is the stomach, mainly the antrum (70%), ileum (19%), and colon (6%). Large polyps of the stomach can cause intermittent obstruction, described as “ball valve syndrome”. This case report, reported in SMS medical college Jaipur in one year six months old child. We report a case of a 1 year 6 months female child with complaint of abdominal lump noted since 7 days, due to this reduced oral intake. On examination single, around 5×5 cm size non-tender lump is situated in left hypogastric region. On CECT abdomen shows mesenteric origin, may be desmoid tumour, Castleman disease, mesenteric haemangioma or inflammatory myo-fibroblastic tumour. Further MRI abdomen suggestive of inflammatory myo-fibroblastic tumour or NHL. Tumour marker study shows serum ferritin and LDH level raised. On exploration revealed a single, 6×5×4 cm lobulated jejunal mass encircle the jejunum, for this resection and anastomosis done. On histopathological report, suggestive of inflammatory fibroid polyp. Patient discharged successfully on 6th pod without any complication. Exploration done and a mass, which encircle the jejunum found. Resection and anastomosis done and patient discharged on 6th pod without any complications. Such an occurrence was incidental in the reported case, which can confuse our diagnosis, so knowledge about this type of disease is very important, especially in paediatrics population.

The use of vascularized fibula flap with allograft in post-oncologic microsurgical bone reconstruction of lower limbs in pediatric patients.

Post-oncologic surgical reconstruction of lower limbs in pediatrics remains a challenging topic. Microsurgical techniques allow reconstructions of large bony defects. The use of vascularized fibular flap with allograft has proven to be an ideal biologic construct. We aim to assess the success rate of this operation, including flap survival, bony union, weight-bearing ambulation, and complications in a long-term follow-up in our case series compared to the literature. Our case-series includes 18 femoral resections (9 osteosarcomas, 8 Ewing sarcoma, and 1 desmoid tumor) and 15 tibial resections (10 osteosarcoma, 4 Ewing sarcoma, and 1 Malignant Fibrous Histiocytoma). We collected patients' demographics, type of tumor, type of resection, defect size, fibula-flap length, method of fixation, anastomosis site, follow-up data, complications, and their management. All survivals were examined by X-ray and CT-scan to evaluate the morphological changes of the vascularized fibula and follow-up. The functional evaluation was performed by the 30-point Musculoskeletal Tumor Society Rating Score (MSTS) for the lower limb (Enneking et al., Clinical Orthopaedics and Related Research 1993(286):241-246). The mean age of the femur resection patients' group was 11.2 years with a mean defect size of 14 cm and a mean length of the fibular flap of 18 cm; for the tibia the mean age was 12 years with a mean defect size of 14 cm and a mean length of the fibular flap of 16.6 cm. The overall survival of the reconstructions at 5 years follow-up was 17 out 18 cases for the femur and 13 out of 15 cases for the tibia. MSTS score was 28.2 for the femur and 23.7 for the tibia. The average time of union of the fibula was seen after 5 months, while allograft consolidation was observed around 19.7 months. The mean time of follow-up was 144.5 months for the femur and 139.2 months for the tibia. The complication rate observed was 7 out of 18 for the femur and 7 out of 15 for the tibia reconstructions. The viability of the fibula is a cornerstone in the success of reconstruction as well as the successful management of complications in intercalary defects after tumor resection in pediatrics to restore good functionality. Our results are in line with those reported in the literature in terms of overall complication rates. The high primary union of allograft, the high MSTS score obtained, and the low rate of severe complications reflect the mechanical role of this reconstructive technique over a long follow-up.

The advent of the first electric driven EUS-guided 17 gauge core needle biopsy – A pilot study on subepithelial lesions

Background and aims This pilot study aimed to evaluate safety and tissue sampling from subepithelial lesions (SEL) in the upper gastrointestinal tract with a novel electric motor driven endoscopic ultrasonography (EUS)-guided 17-gauge (G) size core needle biopsy (CNB) instrument. Methods An investigator-led prospective open label, performance and safety control study, including seven patients (female n = 4, median 71 y, range 28–75) with a determined SEL (median size 30 mm, range 17–150 mm) in the upper digestive tract (stomach n = 6, duodenum n = 1) were eligible and later followed up 14 days after index procedure. All investigations were completed according to protocol with three FNB 22-G passes with four fanning strokes and two EndoDrill® 17-G passes with three fanning strokes. Results Quality of samples as ‘visible pieces’ (>5 mm): FNB (n = 5/7) (fragmented/blood imbibed n = 1, poor tissue quantity n = 1) compared with 17-G CNB (n = 7/7). Histological result which led to final diagnosis (leiomyoma n = 2, adenocarcinoma n = 1, schwannoma n = 1, neuroendocrine tumour n = 1, desmoid tumour n = 1 and gastrointestinal stromal tumour (GIST) n = 1) could be obtained with the 17-G CNB instrument in all seven patients. FNB technique reached correct diagnosis in six patients. No serious adverse event were recorded. Conclusions By using an electric driven 17-G biopsy device, a true cylinder of core tissue can be obtained in one single puncture from the area of interest reducing the need for a second sampling. The absolute benefit of EUS-guided CNB is that the sample can be handled and histologically prepared in the same manner as standard percutaneous core needle sample, e.g., breast and prostate cancer.

Safety and efficacy of cryoablation of Soft-Tissue tumors.

To assess the safety and efficacy of percutaneous cryoablation (CA) of soft-tissue tumors (desmoid tumors (DT), vascular malformations (VM), and abdominal wall endometriosis (AWE)). This systematic review of studies published before January 2024 encompassed a detailed analysis of CA techniques and technical aspects for the treatment of soft-tissue tumors. Data concerning CA efficacy, complication rates, and other relevant metrics was extracted and included for analysis. The analysis included 27 studies totaling 554 CA procedures. For DT (13 studies, 393 sessions), CA showed an average pain reduction of 79 ± 17% (range: 57-100) and a lesion volume decrease of 71.5 ± 9.8% (range: 44-97). VM (4 studies, 58 sessions) had a 100% technical success rate and an average pain reduction of 72 ± 25% (range: 63-85). The average pain reduction for AWE (6 studies, 103 sessions) was 82 ± 13% (range: 62-100). Overall, the complication rate for CA was low, with minor adverse events (AE) in about 20% of patients and major events in less than 5% of patients. Showing substantial efficacy in pain reduction and lesion volume decrease, as well as low incidence of severe AE, CA presents as a highly effective and safe alternative for the treatment of soft-tissue tumors. CA is effective and safe in treating soft-tissue tumors, particularly DT, VM, and AWE.

Unraveling the Mystery of Desmoid Tumors: Insights From a Moroccan Tertiary Center.

Desmoid tumors (DTs) are rare, aggressive malignancies developing from clonal fibroblastic proliferation originating from soft tissues. Despite their low metastatic potential, their invasiveness towards neighboring organs and a high recurrence rate contribute significantly to morbidity and mortality, thereby impacting the quality of life of patients. Several therapeutic options are available, but standardized protocols are lacking. In this study, we reviewed 14 cases of DT retrospectively over a period of 15 years, from September 2008 to December 2023. The most prevalent tumor locations were in the extremities, and the majority of patients were female. We identified risk factors in two patients, those being surgical trauma and familial adenomatous polyposis (FAP). Half of the patients underwent surgery for DT, and two received salvage radiotherapy. Systemic therapy was used in the first and second lines and comprised of chemotherapy, endocrine therapy, and non-steroidal anti-inflammatory drugs (NSAI). Active surveillance was proposed in three patients. This is the first retrospective study to assess the characteristics of DT in Moroccan patients in a tertiary care setting. It aims to shed light on the challenges faced in treating these rare tumors in the context of a lack of therapeutic standardization.

68 Ga-FAPI-04 PET/CT Imaging of Mesenteric Aggressive Fibromatosis.

Aggressive fibromatosis is a relatively rare disease. We describe 68 Ga-FAPI-04 PET/CT findings in a case of histologically proved mesenteric aggressive fibromatosis. 68 Ga-FAPI-04 PET/CT revealed a mass in the mesentery with increased FAPI activity. This case indicates that FAPI PET may be useful for evaluation of aggressive fibromatosis.

Contrast-enhanced Susceptibility Weighted Imaging (CE-SWI) for the Characterization of Musculoskeletal Oncologic Pathology: A Pictorial Essay on the Initial Five-year Experience at a Cancer Institution

Susceptibility-weighted imaging (SWI) is based on a 3D high-spatial-resolution, velocity-corrected gradient-echo MRI sequence that uses magnitude and filtered-phase information to create images. It SWI uses tissue magnetic susceptibility differences to generate signal contrast that may arise from paramagnetic (hemosiderin), diamagnetic (minerals and calcifications) and ferromagnetic (metal) molecules. Distinguishing between calcification and blood products is possible through the filtered phase images, helping to visualize osteoblastic and osteolytic bone metastases or demonstrating calcifications and osteoid production in liposarcoma and osteosarcoma. When acquired in combination with the injection of an exogenous contrast agent, contrast-enhanced SWI (CE-SWI) can simultaneously detect the T2* susceptibility effect, T2 signal difference, contrast-induced T1 shortening, and out-of-phase fat and water chemical shift effect. Bone and soft tissue lesion SWI features have been described, including giant cell tumors in bone and synovial sarcomas in soft tissues. We expand on the appearance of benign soft-tissue lesions such as hemangioma, neurofibroma, pigmented villonodular synovitis, abscess, and hematoma. Most myxoid sarcomas demonstrate absent or just low-grade intra-tumoral hemorrhage at the baseline. CE-SWI shows superior differentiation between mature fibrotic T2* dark components and active enhancing T1 shortening components in desmoid fibromatosis. SWI has gained popularity in oncologic MSK imaging because of its sensitivity for displaying hemorrhage in soft tissue lesions, thereby helping to differentiate benign versus malignant soft tissue tumors. The ability to show the viable, enhancing portions of a soft tissue sarcoma separately from hemorrhagic/necrotic components also suggests its utility as a biomarker of tumor treatment response. It is essential to understand and appreciate the differences between spontaneous hemorrhage patterns in high-grade sarcomas and those occurring in the therapy-induced necrosis process in responding tumors. Ring-like hemosiderin SWI pattern is observed in successfully treated sarcomas. CE-SWI also demonstrates early promising results in separating the T2* blooming of healthy iron-loaded bone marrow from the T1-shortened enhancement in bone marrow that is displaced by the tumor. SWI and CE-SWI in MSK oncology learning objectives: SWI and CE-SWI can be used to identify calcifications on MRI. Certain SWI and CE-SWI patterns can correlate with tumor histologic type. CE-SWI can discriminate mature from immature components of desmoid tumors. CE-SWI patterns can help to assess treatment response in soft tissue sarcomas. Understanding CE-SWI patterns in post-surgical changes can also be useful in discriminating between residual and recurrent tumors with overlapping imaging features.

Anterior abdominal wall desmoid tumors: Reconstructive considerations and challenges in an oncoplastic unit in a low- and middle-income country: A case report

Desmoid tumors (DTs) are rare, locally aggressive tumors. When left untreated, DT can rapidly proliferate and occupy a significant portion of the anterior abdomen, making postresection reconstruction challenging. We present a 32-year-old female with a previous history of cesarean section and a 10-month history of two rapidly growing anterior abdominal wall (AAW) masses. There was no clinical or radiologic evidence of distant metastases. Abdominopelvic computed tomography scan showed no intra-abdominal extension. She had a wide local excision of the masses using an “abdominoplasty” technique and reconstruction of the extensive musculofascial defect with fascia lata grafts and polypropylene mesh. A histologic diagnosis of desmoid-type fibromatosis was made. The patient is scheduled for adjuvant radiotherapy and desires future pregnancies. This case report highlights the reconstructive considerations and challenges associated with surgical resection of huge AAW DT in an oncoplastic unit in a low- and middle-income country.

Local Treatment of Desmoid Tumors: An Update.

Desmoid tumors (DTs) are rare fibroblastic proliferations, characterized by infiltrative growth and a propensity for local recurrence. Traditional strategies such as surgery, radiotherapy, and chemotherapy are the mainstays of treatment, each with its limitations and associated risks. The trend in DT management leans toward a "wait-and-see" strategy, emphasizing active surveillance supported by continuous MRI monitoring. This approach acknowledges the unpredictable nature of the disease, and a multidisciplinary management of DT requires a nuanced approach, integrating traditional therapies with emerging interventional techniques. This review highlights the emerging role of minimally invasive interventional radiological technologies and discusses interventional radiology techniques, including chemical, radiofrequency, microwave, cryoablation, and high-intensity focused ultrasound ablations as well as transarterial embolization.

Favorable clinical efficacy of cytotoxic chemotherapy in patients with progressive desmoid tumors: a retrospective real-world study.

The real-world evidence about the efficacy of cytotoxic chemotherapy in desmoid tumors is still limited. We investigated the efficacy of chemotherapy in the treatment of recurrent or progressive desmoid tumors. The patients with desmoid tumors who had received cytotoxic chemotherapy between November 2007 and June 2020 in two tertiary hospitals in Korea were reviewed. A total of 25 patients were included in the analysis. The most common primary tumor site was the intra-abdominal or pelvic cavity (56%), followed by the trunk and abdominal wall (24%), extremities (16%), and head and neck (4%). Sixty percent of the patients had familial adenomatous polyposis and 76% received doxorubicin plus dacarbazine. The objective response rate and disease control rate was 64% (95% confidence interval [CI]: 40.7-82.8) and 96% (95% CI: 77.2-99.9), respectively. With the median follow-up time of 55months (95% CI: 41.0-68.2), the 3-year PFS rate was 65% (95% CI: 41.1-80.5), and the 3-year OS rate was 89% (95% CI: 63.8-97.3). Grade 3 or 4 hematologic adverse events were reported in 14 patients, all of which were manageable. Our real-world evidence suggests that doxorubicin-based cytotoxic chemotherapy can be an effective treatment option for recurrent and progressive desmoid tumors with respect to favorable clinical outcomes.

Surgical treatment of locally advanced desmoid tumor of the anterior abdominal wall

Desmoid tumor (DO) is a rare monoclonal connective tissue tumor arising from deep soft tissues, characterized by invasive growth and a tendency to local relapses and incapable of metastasis [1].The purpose of presenting this clinical case is to assess the significance of timely diagnosis, the choice of an individually correct treatment method, tactics of further observation, therapy.To achieve this goal, a number of literature sources were studied, a clinical case of surgical treatment of a desmoid tumor of the anterior abdominal wall was presented in our medical institution (Northwestern State Medical University named after I.I.Mechnikov, St. Petersburg).The difficulty of the case was the significant size of the indolent tumor, insensitive to systemic therapy, in a young man, and therefore the decision on surgical treatment tactics was also ambiguous. In each clinical case, it is worth taking an individual approach to treatment tactics. When choosing observational tactics, strict outpatient monitoring and regular follow-up should be performed.