Musculoskeletal oncology and thermal ablation: the current and emerging role of interventional radiology.

The role of interventional radiology (IR) is expanding. With new techniques being developed and tested, this radiology subspecialty is taking a step forward in different clinical scenarios, especially in oncology. Musculoskeletal tumoral diseases would definitely benefit from a low-invasive approach that could reduce mortality and morbidity in particular. Thermal ablation through IR has already become important in the palliation and consolidation of bone metastases, oligometastatic disease, local recurrences, and treating specific benign tumors, with a more tailored approach, considering the characteristics of every patient. As image-guided ablation techniques lower their invasiveness and increase their efficacy while the collateral effects and complications decrease, they become more relevant and need to be considered in patient care pathways and clinical management, to improve outcomes. We present a literature review of the different percutaneous and non-invasive image-guided thermal ablation methods that are currently available and that could in the future become relevant to manage musculoskeletal oncologic diseases.

Laparoscopic gastric wedge resection and spleen-preserving distal pancreatectomy performed on a 6-cm gastric desmoid tumour with pancreatic invasion.

Desmoid tumours are rare tumours originating from fibroblasts, and are characterised by local infiltration and no metastasis. When complete resection is possible, surgical resection is considered a first-line treatment. In the case of large desmoid tumours, it is mainly performed by laparotomy, not laparoscopy. We report a case of a 43-year-old female patient presenting with a hypodense mass of approximately 5 cm in the posterior wall of the gastric antrum on computed tomography. There was no history of familial adenomatous polyposis, trauma or abdominal surgery. The patient underwent laparoscopic gastric wedge resection and spleen-preserving distal pancreatectomy without peri-operative complications. Pathological analysis revealed a desmoid tumour, which originated from the stomach and invaded the pancreas. Despite the large size and the locally infiltrative characteristics of these tumours, laparoscopic surgery can be an optimal treatment option due to its advantages.

Giant aggressive intra-abdominal desmoid-type fibromatosis - case report.

INTRODUCTION: Aggressive fibromatosis, also known as desmoid tumour (DT), is a locally invasive soft tissue malignancy originating from fascial planes, connective tissue, and musculoaponeurotic structures of the muscles. The symptoms greatly depend on the location and size of the tumour. CASE REPORT: A 68-year-old male patient without any comorbidities with a large, palpable mass in the abdomen underwent computed tomography (CT) of the abdomen during diagnostic examination in September 2017 in another centre. The CT scan revealed a giant intraperitoneal 30×40cm tumour without signs of infiltrating the surrounding organs and large vessels. The tumour biopsy revealed an aggressive DT. The patient was scheduled for tumour resection. Midline laparotomy was performed in the supine position under general anaesthesia. After gaining access to the abdominal cavity, 8 litres of clear ascites were evacuated. The tumour was not attached to the abdominal wall. Large omentum was freed from the DT. The perioperative finding confirmed the CT images of DT encapsulation of the medial colic artery, part of the small intestine, and transverse colon. The tumour was resected with part of the mesenteric radix, 30 cm of small intestine, and 2/3 of the transverse colon. After the DT was removed entirely, the small intestine was re-anastomosed end to end. The abdominal cavity and the liver were carefully checked for bleeding. The abdominal cavity was closed in a standard manner. RESULTS: The postoperative hospital stay was uneventful. The patient was discharged on the 7th postoperative day with prophylactic low weight molecular heparin for one month. Currently, we have five months of follow-up with no signs of DT recurrence based on CT examination. The histology of the resected tumour confirmed the diagnosis of a desmoid tumour (aggressive abdominal fibromatosis). CONCLUSION: Desmoid tumours are benign neoplasms with no metastatic potential. However, their treatment is challenging due to their aggressive growth, infiltrative behaviour, and a high tendency to recur.

Fertility preservation and PGT-M in women with familial adenomatous polyposis-associated desmoid tumours.

RESEARCH QUESTION: Is ovarian stimulation and pregnancy in women with familial adenomatous polyposis (FAP)-associated desmoid tumours safe? DESIGN: The study included women with FAP-associated desmoid tumours who underwent fertility treatments at the authors' tertiary medical centre between the years 2011 and 2021. Data were collected from the fertility unit's charts and from the oncological registries. The main outcome measures were the number of vitrified oocytes and embryos, and the number of live births in preimplantation genetic testing for monogenic/single gene defects (PGT-M) cycles. RESULTS: Overall, 17 women were identified suitable for this study. A total of 117 mature oocytes were vitrified for fertility preservation and 106 embryos were submitted to PGT-M. One patient returned to claim her cryopreserved oocytes, and five patients who underwent PGT-M embryo transfer reported three live births. A statistically significant decrease in selected fertility cycle parameters was observed in one woman who co-administered sorafenib (a multikinase inhibitor) during her first cycles of treatment, as the mean number of oocytes before and after was 2.7 (±1.3) versus 13.2 (±3.3) (P = 0.02), the mean number of metaphase II oocytes was 2.2 (±2.1) versus 7.7 (±2.6) (P = 0.007), and the mean number of two-pronuclei oocytes was 0.5 (±1.1) versus 3.5 (±1.7) (P = 0.09). Three patients had a median desmoid tumour growth on magnetic resonance imaging of 6.2 (2.9-7.2) cm when compared with prior ovarian stimulation imaging. CONCLUSIONS: Ovarian stimulation for women with desmoid tumours was characterized in some patients with an acceleration in tumour growth, regardless of the use of aromatase inhibitors. The use of sorafenib should be carefully considered during the course of fertility treatment.

Abdominal wall reconstruction for desmoid tumors following radical resection from the abdominoplasty incision: Case report.

Desmoid tumours are rare and locally invasive neoplasms that originate from the muscles and their aponeurosis. Incomplete excision causes recurrences; therefore, patients require aggressive resection that essentially entails tumour excision with a clear surgical margin. After radical resection, the resultant wide defect may lead to difficulty in closure of the anterior abdominal wall. Here, we report a case having surgery for large desmoid tumour of the anterior abdominal wall through an abdominoplasty incision followed by an abdominal wall reconstruction with a dual-sided composite mesh.

Mammary fibromatosis in a female dog: case report.

Fibromatosis, or desmoid tumour, is characterized by excessive and infiltrative proliferation of connective tissue originating from aponeurotic muscle structures. Mammary fibromatosis is rare in humans and animals and its precise aetiology is unknown. A 10-year-old mixed-breed female dog developed a mass in the right cranial thoracic mammary gland (M1) and underwent lumpectomy. The mass was firm, with an irregular surface and distinct limits. Microscopically, it was a neoplastic proliferation of fusiform cells with low atypia, interspersed with abundant dense collagenous tissue, confirmed by histochemical staining with Gomori's trichrome and Masson's trichrome and immunopositivity for vimentin and smooth muscle actin, confirming mammary fibromatosis. Mammary fibromatosis in dogs needs further studies to elucidate its clinical, epidemiological and aetiopathogenic aspects.

Developing knowledge, attitude and practice questionnaire in desmoid tumours (KAPID study): applying e-Delphi to rare diseases.

Background: Rare diseases are associated with unique challenges encountered in diagnosis, treatment and conduct of clinical research. Desmoid tumour (DT) is one such ultra-rare malignancy about which awareness among medical professionals remains limited. We developed a questionnaire to assess knowledge, attitude and practice (KAP) among medical professionals on DT. Methods: E-Delphi method was used for the assessment of KAP for DT amongst clinical experts (experience of >/= 3 years in DT). 22 open-ended statements were developed by the core research group using current consensus guidelines. In round 1, experts provided subjective feedback which was incorporated into a 35-item questionnaire. Round 2 entailed experts giving feedback as a 5-point Likert scale classified into agreement (median score >/=4), neutral (median score 3) and disagreement (median score <3). Feedback from Round 2 was incorporated and questions with neutral consensus were modified. Questions in Round 3 achieved consensus if >/= 75% participants agreed. Results: 11 (64.7%) of 17 contacted experts responded in Round 1 including 6 (54.4%) who gave additional inputs and 5 (45.6%) who agreed to all statements. In round 2, 8 out of 11 experts responded to the 35-item questionnaire on knowledge (n = 16), attitude (n = 8) and practice (n = 11). 32 questions obtained agreement and 3 (8.5%) had neutral consensus. These were modified for round 3, in which consensus on 2 (66.6%) was attained. The final questionnaire comprises 34 items with 15, 8 and 11 questions on in the sections of knowledge, attitude and practice (KAP), respectively.

Rare localisation of a recurrent desmoid tumour of the foot: A case report and review of the literature.

INTRODUCTION AND IMPORTANCE: Aggressive fibromatosis or desmoid tumour is a rare soft tissue tumour that develops from supporting tissues and fascia. Although benign, fibromatosis is a tumour that can be locally invasive, and surgical treatment is often difficult. The interest of this observation is to show the particular characteristics of desmoid tumours: the frequency, the major difficulties of excision and the role of adjuvant treatment in the management of these tumours. CASE PRESENTATION: The patient was 27 years old male, and had a pathological history of a recurrent desmoid tumour on the dorsal surface of the right foot, which had been present for 10 years, had been operated on 5 times and could not be completely removed. A radiological work-up confirmed the nature of the lesion, necessitating a biopsy, which confirmed the diagnosis of a recurrence of the desmoid tumour on the sole of the foot. CLINICAL DISCUSSION: Desmoid tumour is a rare tumour with less than 5 cases per million inhabitants, histologically benign and belonging to the deep fibromatosis group. The average age is 30, with a predominance of women. The most common location for desmoid tumours is the abdominal wall, and they are usually single and unilateral. The treatment of desmoid tumours is primarily surgical, but is fraught with technical difficulties due to the absence of any cleavage plane and the particularly tight adhesions to neighbouring structures. Radiotherapy or hormone therapy has been advocated as an adjunct to tumour resection, but its value is debated, and recurrence occurs in 50-80 % of cases. CONCLUSION: The desmoid tumour is a very rare tumour, with an infiltrating and aggressive benign character, whose surgical treatment is difficult, and whose evolution is marked by recurrences.

Two synchronous desmoids tumours of the thoracic wall: A case report.

INTRODUCTION AND IMPORTANCE: Desmoid tumour is a rare neoplasm that develops from the fascia and musculoaponeurotic tissue. These tumours tend to local invasion. Desmoid tumours are usually solitary. We present the first case of two synchronous desmoid tumours of the chest wall. CASE PRESENTATION: A 56-year-old male with no medical history presented a painless chest wall mass. CT scan showed a deep soft tissue mass infiltrating the pectoralis major and minor muscles with an invasion of the subclavian and axillary pedicles and a second tumour infiltrating the latissimus dorsi muscle. MRI has allowed for a better study of these two masses, and a surgical biopsy confirmed the diagnosis of a desmoid tumour. The surgical resection was intra-tumoural for the anterior mass to preserve the axillary and subclavian pedicles, and the tumour resection was marginal for the posterior tumour. The postoperative course was uneventful, and an adjuvant therapy based on Imatinib was performed. The tumour residue was stabilized for two years follow-up. CLINICAL DISCUSSION: Desmoid tumours are considered a locally aggressive disease. Ultrasound, CT scan, and MRI have different roles in their diagnosis. But pathological diagnosis is the "golden standard" diagnosis of desmoid tumours. The treatment of desmoid tumours is still not standardized. Surgery is the best primary treatment, but sometimes oncological resection may not be possible because of extension to the vital structure. Adjuvant therapy, like Imatinib, had demonstrated encouraging results. CONCLUSION: For desmoid tumours with vital or noble structure invasion, intra-tumoural resection associated with adjuvant therapy demonstrated encouraging results.

Physical symptom burden in patients with desmoid-type fibromatosis and its impact on health-related quality of life and healthcare use.

BACKGROUND: Desmoid-type fibromatosis (DTF) has a highly variable clinical course with varying intensity of symptoms. The objectives of this study were to identify subgroups of DTF patients based on physical symptom burden and to compare symptom burden subgroups on health-related quality of life (HRQoL) and healthcare use (univariate and multivariate). METHODS: Desmoid-type fibromatosis patients from the United Kingdom and the Netherlands received cross-sectional questionnaires on HRQoL (EORTC QLQ-C30), DTF-specific HRQoL (DTF-QoL) and healthcare utilisation. Latent class cluster analysis was performed to identify subgroups based on patients' symptom burden using EORTC QLQ-C30 and DTF-QoL physical symptom items. Multivariate linear and logistic regression analyses were conducted to examine associations of symptom burden with HRQoL and healthcare utilisation, respectively. RESULTS: Among 235 DTF patients, four symptom burden clusters were identified, with low symptom burden (24%), intermediate symptom burden-low pain (20%), intermediate symptom burden-high pain (25%) and high symptom burden (31%). DTF patients with high symptom burden had clinically relevant lower HRQoL scores compared to patients with low and intermediate symptom burden (p < 0.001) and reported more general and DTF-related visits to their general practitioner compared to the low symptom burden cluster (p < 0.01). In the multivariate analyses, symptom burden was independently associated with both HRQoL and healthcare utilisation. CONCLUSIONS: This study identified four distinct subgroups of DTF patients based on their level of symptom burden, with a considerable number of patients being highly symptomatic. Knowledge of the level of symptom burden DTF patients experience can help to identify patients at risk of poorer outcomes and tailor supportive care to the individual needs of DTF patients.

Desmoid tumour in an inguinal hernia in a patient with a previous diagnosis of melanoma.

A 68-year-old man, without a family history of cancer, was treated for primary cutaneous melanoma of the scalp. Two years later, a right lateral cervical lymph recurrence was observed and he was treated with lymphadenectomy and adjuvant nivolumab for 1 year. Four years from the initial melanoma diagnosis, a computer tomography scan showed a solid nodular lesion of 26 × 40 × 75 mm inside the previously known inguinoscrotal hernia. A new recurrence of melanoma was the most probable diagnosis and a right inguinal hernioplasty was performed. Notably, the histopathological examination revealed a mesenteric fibromatosis with the typical immunohistochemical pattern (strong nuclear staining of ß-catenin). Interestingly, this represents the first case of a patient with a mesenteric desmoid tumour presenting as an inguinal hernia masking a cutaneous melanoma recurrence.

Case report: Desmoid fibromatosis diagnosed in a 27-year-old male after being mistaken for a gastrointestinal stromal tumour.

Despite being distinct lesions, gastrointestinal stromal tumours (GISTs) and desmoid fibromatosis may appear similar on imaging when they involve the stomach wall or bowel. As a result, they may be confused with one another when initially diagnosed. This report aims to present a case where a desmoid tumour was mistaken for a gastric GIST in a 27-year-old gentleman despite extensive investigation prior to exploratory laparotomy, and why differentiation through pathology, with a focus on the immunohistochemistry profile, is key for proper prognostication and appropriate management, including timely investigation for associated diseases such as Familial Adenomatous Polyposis in patients with desmoid tumours.

Anterior abdominal wall metastasis following curative resection and chemoradiation of rectal cancer masquerading as a desmoid tumour: A clinical conundrum.

Desmoid tumour of the anterior abdominal wall (rectus sheath) commonly occurs in women post abdominal surgery. Metastasis from colorectal cancer to the anterior abdominal wall, on the other hand, is rare and produces a complex management dilemma. This Case study presents a 57-year-old woman who received a curative laparoscopic low anterior resection and adjuvant chemoradiation in 2013. Seven years later, she presented with an asymptomatic anterior abdominal wall lump. Clinically, the lump appeared to be a desmoid tumour. A wide local excision of the lump was carried out and the final histopathology showed a metastatic lesion (adenocarcinoma). With adjuvant chemotherapy, the patient is now disease-free and doing well. A possibility of distant metastasis must be kept in mind for all patients, even when they have undergone curative resection with adjuvant chemoradiation for colorectal cancer.

Desmoid tumour of the chest wall in paediatric post-operatory of heart transplant.

We will report a case of a desmoid tumour (DT), which developed at the surgical site of the pacemaker after a late childhood heart transplant. Patients with idiopathic dilated cardiomyopathy followed up in the paediatric cardiology service. It evolved with the dissociation of ventricular rhythm caused by severe heart failure, which led to the implantation of a cardiac resynchronization device prior to heart transplantation. The progression to end-stage heart disease culminated in a heart transplant at 12 years old. One year after the transplant, at the age of 13 years, he presented a progressively growing mass on the generator site of the resynchronization device. The initial decision was to remove the device. During the removal surgery, there was no haematoma or fluid collection. However, there was a progression of the lesion. The lesion was biopsied with the anatomopathological diagnosis of a DT. Resection surgery happened 4 months after the start of the mass growth. At that time, the tumour reached 20 cm in diameter. The lesion infiltrated the pectoralis major muscle and this muscle was resected partially en bloc with the lesion. The defect had primary closure. The patient evolved without postoperative complications and was discharged on the 14th postoperative day. The surgical specimen came with negative circumferential margins. However, the deep margin was microscopically positive. Due to deep involvement, the patient underwent adjuvant radiotherapy. Currently, the patient is under clinical follow-up and has no evidence of tumour recurrence. DT is a rare tumour, with unpredictable courses. Surgery can be considered in the progression of lesions. Treatment is justified by long survival after a heart transplant and in DT patients. DT is a differential diagnosis to be considered in progressive growth lesions.

CRYODESMO-O1: A prospective, open phase II study of cryoablation in desmoid tumour patients progressing after medical treatment.

BACKGROUND: Desmoid tumours (DTs) are rare tumours originating from musculoaponeurotic structures. Although benign, they may be locally aggressive, leading to pain and disability. European Society for Medical Oncology (ESMO) guidelines recommend frontline watchful waiting and medical treatment in progressing tumours. Cryoablation is an interventional radiology technique that is suitable for DT patients (pts) on the basis of repeated cycles of freezing, leading to cell death. METHODS: CRYODESMO-01 (ClinicalTrials.gov Identifier: NCT02476305) is a prospective, open-label, non-randomised, non-comparative, multicenter study assessing cryoablation in non-abdominopelvic progressing DT. Inclusion criteria were: pts ≥18 y.o., confirmed DT accessible to cryoablation (≥90% destruction), measurable lesion conforming to modified response evaluation criteria in solid tumours (mRECIST), progressive disease after ≥2 lines of medical therapy or with functional symptoms/pain, adequate biological parameters, informed consent, and affiliation to a medical insurance scheme. The primary end-point was the non-progression rate at 12 months; secondary end-points included safety, quality of life (QoL), assessment of pain and functional status. FINDINGS: 50 pts were enrolled (78% female) from four French centres and all were treated. The mean age was 41 y.o. (19-73). The median number of prior treatments was 2.00 [1-4] including non-steroidal anti-inflammatory drugs (NSAIDs), hormone therapy, chemotherapy, and anti-angiogenics. Tumour location included limbs (36%), trunk (60%), and cervical area (4%). The median tumour largest diameter was 89 mm. The rate of non-progressing disease at +12 months was 86% [confidence level (CI) 95% 73-94%]. Median PFS was not reached at a median follow-up of 31 months. Grade 1 and 2 toxicity occurred in 32.8% and 44.5% of patients, grade 3-4 in 22% and no Grade 5 toxicity was observed. Cryoablation significantly improved functional status and pain scores. INTERPRETATION: Cryoablation demonstrated feasibility in progressive DT pts. The study met is primary end-point with 86% of non-progressive disease at +12 months, with reduced pain, better functional status, and encouraging long-term disease control.

Invasive giant pancreatic desmoid-type fibromatosis with curative resection: A case report.

INTRODUCTION: Desmoid-type Fibromatoses (DTF) tumours are rare, benign fibrous tumours with aggressive invasive behaviour that account for approximately 0.03% of all neoplasms. We report the success in curing a rare, invasive, and huge pancreatic intraabdominal DTF. PRESENTATION OF CASE: A 42 years old male was medically free apart from recurrent left upper abdominal pain, anorexia, and nausea for more than ten years and no significant past surgeries, trauma, or family history of cancer. The patient has a non-tender large abdominal mass at the left hypochondria area extending down to the pelvis below the umbilicus with a rigid and smooth surface. The computed tomography scan showed a huge heterogeneous mass appears to be of pancreatic origin, measuring about 23 cm by 15 cm by 11 cm. The patient underwent radical antegrade modular pancreato-splenectomy, segmental transverse colectomy, adrenalectomy, and subsequent colo-colic anastomosis. The accurate gross size of the tumour specimen was 26 × 17 × 9 cm, and the weight was found to be 3.6 kg. Immunohistochemistry confirmed the diagnosis of pancreas DTF. The follow up to 5 years confirmed no recurrence reported clinically or by imaging. DISCUSSION: The Pancreas origin of DTF is a rarely reported subset with an incidence of around 5% of all DTF. Establishing the diagnosis is fundamentally based on the characteristic pathological and immunohistochemical studies, for the only available cure modality by complete radical resection to be promptly offered. CONCLUSION: Our case is rare and uniquely the largest pancreatic DTF reported in the literature with curative resection despite being locally invasive.

Desmoid tumours in the surveillance era: What are the remaining indications for surgery?

The treatment of desmoid tumours (DTs) has greatly evolved in recent years, and surgery is no longer considered a first-line treatment. Percutaneous biopsy with molecular analysis for beta-catenin or APC gene mutation provides a certain diagnosis. After imaging, a specialized multidisciplinary tumour board (MDT) addresses the following therapeutic strategy. As more than half of patients stabilize or regress, despite initial progression, active surveillance is offered to most patients as the first option. Surgery is proposed for complications such as perforation and occlusion, which sometimes are the first manifestations of the disease. In these cases, limiting surgery to the treatment of complications and leaving the tumour in place is possible if significant bowel sacrifice is needed, especially in patients with previous colectomy for polyposis. Medical therapy is discussed by the MDT in cases of functional or life-threatening masses and is preferred to local treatments that could be mutilating and often incomplete. First-line surgery is now contraindicated in cases of incomplete unplanned surgery, recurrence, pregnancy or DTs occurring in familial adenomatous polyposis (FAP). The best indications of second-line surgery are significantly progressing disease when morbidity is acceptable, such as parietal locations. Medical and other locoregional treatments (radiotherapy, isolated limb perfusion and cryotherapy) should be considered by the MTB when surgery might cause sequelae.

Successful outcomes after laparoscopic spleen-preserving pancreatic resection for a desmoid tumor: A case report.

INTRODUCTION: Desmoid tumors are slowly growing neoplasms that arise from fibroblasts. These tumors are locally aggressive and have a high rate of recurrence after surgery. Pancreatic desmoid tumors are extremely rare. The indications and outcomes of laparoscopic surgery for pancreatic desmoid tumors have not been fully elucidated. This report therefore aimed to describe a rare case of a pancreatic desmoid tumor in a patient who was successfully treated with laparoscopic spleen-preserving pancreatic resection. PRESENTATION OF CASE: We report a case of a 60-year-old man who presented with back pain. Contrast-enhanced computed tomography scan revealed a circumscribed tumor in the pancreatic tail measuring 3 cm. The patient underwent laparoscopic spleen-preserving distal pancreatectomy. Pathological analysis revealed a desmoid tumor infiltrating the pancreatic parenchyma. There was no evidence of recurrence at 36 months of follow-up. DISCUSSION AND CONCLUSION: Isolated sporadic pancreatic desmoid tumors are extremely rare. Laparoscopic radical resection could be a safe and effective treatment for this benign but locally aggressive tumor. To the best of our knowledge, this is the first study to report a patient with a pancreatic desmoid tumor who was successfully treated with laparoscopic surgery.

The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients.

Desmoid tumor (DT; other synonymously used terms: Desmoid-type fibromatosis, aggressive fibromatosis) is a rare and locally aggressive monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. Previously surgery was the standard primary treatment modality; however, in recent years a paradigm shift towards a more conservative management has been introduced and an effort to harmonise the strategy amongst clinicians has been made. We present herein an evidence-based, joint global consensus guideline approach to the management of this disease focussing on: molecular genetics, indications for an active treatment, and available systemic therapeutic options. This paper follows a one-day consensus meeting held in Milan, Italy, in June 2018 under the auspices of the European Reference Network for rare solid adult cancers, EURACAN, the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) as well as Sarcoma Patients EuroNet (SPAEN) and The Desmoid tumour Research Foundation (DTRF). The meeting brought together over 50 adult and pediatric sarcoma experts from different disciplines, patients and patient advocates from Europe, North America and Japan.

Abdominal wall reconstruction for desmoid tumour surgery: Case report.

INTRODUCTION: Desmoid tumours are rare benign neoplasms with strong tendency to local invasion and recurrence. They usually rise from muscles and aponeurosis and could be challenging for medical treatment. We here report the case of a patient requiring the excision of part of the abdominal rectus muscle and an abdominal wall reconstruction. PRESENTATION OF CASE: A 38 years old women referred to our institution complaining of a painful lump in the anterior abdominal wall. She underwent ultrasonography (US), magnetic resonance imaging (MRI) and a computed tomography (CT) biopsy who showed the presence of a desmoid tumour arising in the right abdominal rectus muscle. She underwent surgical excision of the mass and an abdominal wall reconstruction according to the posterior component separation technique, with a large polypropylene mesh reinforcement. The post-operative period was uneventful and at 6 months she shows no clinical nor radiological recurrence. DISCUSSION: Desmoid tumours are rare neoplasms rising in many anatomical spaces and requiring challenging decisions. A radical resection with free margins remains the principal determinant of outcome. Non-surgical approaches and adjuvant therapy may be useful for patients with unresectable lesions. CONCLUSION: The optimal treatment strategy for desmoid tumours still remains unclear. A multidisciplinary approach is always the best strategy to establish the correct treatment.