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We present the case of a man in his 60s with hypertension, who had a 3-year history of an irreducible mass in the left inguinal area. The patient presented at the emergency room with left lower quadrant pain and scrotal pain. The clinical examination was not suggestive of an acute abdomen. A CT scan was done showing an anterior abdominal wall defect at the left inguinal region. The patient underwent exploratory laparotomy, ileal resection and anastomosis, mesh hernioplasty left for the mesenteric fibromatosis mass mimicking as an irreducible inguinal hernia. Histopathology and immunohistochemistry showed desmoid-type fibromatosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2025. No commercial re-use. See rights and permissions. Published by BMJ Group.)

Rationale: Desmoid-type fibromatosis (DTF), also known as aggressive fibromatosis, is a rare neoplasm originating from the fascial or musculoaponeurotic tissues. While benign and characterized by slow growth, it exhibits local aggressiveness and lacks specific clinical characteristics. However, in a considerable percentage of patients, it could be asymptomatic and discovered by accident during routine clinical examinations. Only a few cases of DTF arising from the psoas major muscle have been reported in the medical literature., Patient Concerns: A 24-year-old male, asymptomatic and without significant personal or family medical history, was diagnosed with grade 2 hydronephrosis by abdominal ultrasonography during a routine physical examination. This diagnosis was made 15 days after undergoing uncomplicated open-heart surgery to repair an atrial septal defect., Diagnosis: Intravenous pyelogram revealed hydronephrosis with dilation of the pelvicalyceal system. Ureteroscopy ruled out any intrinsic lesions of the ureter. Contrast-enhanced computed tomography identified a 3.5 × 2 × 5.2 cm mass in the retroperitoneum, closely associated with the psoas muscle and enveloping the ureter adjacent to the iliac artery. Postoperative pathological analysis confirmed a definitive diagnosis of sporadic DTF., Interventions: The patient underwent exploratory abdominal surgery, during which the tumor was resected without any intraoperative complications., Results: After close monitoring over a 5-year follow-up period, which included periodic physical examinations, magnetic resonance imaging, and ultrasonography, no local recurrence was detected., Lessons: Achieving an accurate preoperative diagnosis presents a challenge in cases involving retroperitoneal tumors originating from the psoas major muscle and encasing the ureter. However, the insertion of a double J stent is deemed a crucial step in the surgical process, facilitating the dissection and isolation of the ureter from the tumor while preserving kidney function., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Aggressive fibromatosis (AF) is a fibroblastic/myofibroblastic tumor known for its locally aggressive properties. Intra-abdominal AF primarily occurs in the small intestine mesentery, ileocolic mesocolon, omentum, retroperitoneum, and pelvis, and rarely originates from the intestinal wall. Here, we report a rare case of small bowel obstruction caused by duodenum-derived AF with β-catenin (CTNNB1) T41A mutation., Patient Concerns: A 35-year-old male had a 4-month history of abdominal pain, nausea, and vomiting, which gradually worsened over time., Diagnoses: Based on the results of CT examination, histopathology and Sanger sequencing, the patient was diagnosed with small bowel obstruction caused by duodenum-derived AF., Interventions: Due to the extensive adhesion between the tumor and surrounding tissue, it is extremely challenging to completely remove the tumor through surgical resection with negative margins in this case. In order not to damage the function of surrounding vital organs, gastrojejunostomy was performed to relieve the symptoms of small bowel obstruction., Outcomes: The patient experienced a successful recovery. It is important to note that this patient is still at risk of local recurrence and requires regular follow-up., Lessons: The best treatment should be taken based on the individual patient to relieve symptoms and improve quality of life. Moreover, histopathology plays a crucial role in diagnosing and differentiating duodenum-derived AF. The detection of mutations in exon 3 of the CTNNB1 has become strong evidence for diagnosing duodenum-derived AF., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: We have recently described circumferential nerve involvement of neuromuscular choristoma associated with desmoid-type fibromatosis (NMC-DTF) in cases involving the sciatic nerve, supporting a nerve-derived mechanism for the DTF. We wondered whether a similar growth pattern occurs in cases involving the brachial plexus (BP)., Methods: We reviewed all available magnetic resonance (MR) imaging in patients diagnosed at our institution with NMC or NMC-DTF of the BP. We also performed a literature search of patients with NMC or NMC-DTF of the BP., Results: In our clinical records, four patients with NMC of the BP were identified, and three developed NMC-DTF. All three patients had MR imaging evidence of circumferential encasement of the BP. In the literature, we identified 15 cases of NMC of the BP, of which 12 had identified NMC-DTF. Four published cases included MR images, and only two were of sufficient quality for review. The single provided image in both cases demonstrated a similar pattern of circumferential encasement of the BP by the NMC-DTF. One additional case report was published without MR images but described circumferential involvement in the surgical findings. One unpublished case of NMC-DTF of the BP from an international radiology meeting also had this circumferential pattern pattern on MRI., Conclusions: The MRI findings of circumferential nerve involvement in patients with NMC-DTF of the BP are similar to our previously reported data in patients with NMC-DTF of the sciatic nerve, providing further imaging-based support of a nerve-driven mechanism. Clinical implications are presented based on the proposed pathogenetic mechanism., (Copyright © 2024 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: Clinical experience teaches that intraperitoneal adhesions are more severe in patients with familial adenomatous polyposis than in patients without it. This impression may come from the common association of familial adenomatous polyposis with desmoid disease., Objectives: This study aimed to determine whether patients with familial adenomatous polyposis and desmoid disease develop more severe adhesions than those without desmoid disease., Design: Prospectively collected data study., Settings: Hereditary colorectal cancer center in a tertiary referral hospital., Patients: Patients undergoing first reoperative intra-abdominal surgery for familial adenomatous polyposis; controls were those having their initial abdominal surgery., Interventions: Surgery and adhesiolysis., Main Outcome Measures: Presence and type of desmoid disease; presence and severity of nondesmoid intraperitoneal adhesions. Where patients had multiple operations, only the first reoperative surgery was chosen. Desmoid disease was noted as reaction (sheet) or mass. Adhesions were graded as none, mild (<10 min for mobilization), average (10-30 min), and severe (>30 min or significant bowel damage). Patients having their first abdominal surgery for familial adenomatous polyposis were used as a control group., Results: A total of 211 patients had no prior surgery; 5% had desmoids and 1% had adhesions. One hundred thirty-seven patients underwent reoperative surgery: 39% had desmoid disease ( p < 0.05 vs no prior surgery), the highest rate being in patients after IPAA (57%), and 45% had severe adhesions ( p < 0.01 vs no prior surgery), worst after Koch pouch (89%), and total proctocolectomy with ileostomy (82%). Thirty-six percent of patients without desmoid disease had severe adhesions. Desmoid reaction was associated with severe adhesions in 47% of cases and desmoid tumors in 66% of cases., Limitations: Possible limitations include the potential overlap between desmoid adhesions and nondesmoid adhesions and the potential for inaccuracy in defining the time of adhesiolyses., Conclusions: Familial adenomatous polyposis is associated with severe postoperative adhesions after reoperative abdominal surgery, especially in patients who develop desmoid disease. See Video Abstract ., Correlacin Entre La Gravedad De Las Adherencias Y La Enfermedad Desmoidea En Pacientes Con Poliposis Adenomatosa Familiar Estudio Prospectivo De Cohortes: ANTECEDENTES:La experiencia clínica demuestra que las adherencias intraperitoneales son más graves en pacientes con poliposis adenomatosa familiar que en pacientes sin enfermedad desmoidea. Esta impresión puede provenir de la asociación común de poliposis adenomatosa familiar con enfermedad desmoidea.OBJETIVOS:Ver si los pacientes con poliposis adenomatosa familiar y enfermedad desmoidea desarrollan adherencias más graves que aquellos sin enfermedad desmoidea.DISEÑO:Estudio de datos recolectados prospectivamente.AJUSTES:Centro de cáncer colorrectal hereditario en un hospital de referencia terciario.PACIENTES:Pacientes sometidos a una primera cirugía intraabdominal de caracter reoperatorio por poliposis adenomatosa familiar: los controles fueron los que se sometieron a su cirugía abdominal inicial.INTERVENCIONES:Cirugía y adhesiolisis.PRINCIPALES MEDIDAS DE RESULTADO:Presencia y tipo de enfermedad desmoidea; presencia y severidad de adherencias intraperitoneales no desmoideas. Cuando los pacientes tenían múltiples operaciones, solo se eligió la primera cirugía reoperatoria. La enfermedad desmoidea se anotó como reacción (hoja filamentosa) o masa. Las adherencias se calificaron como ninguna, leve (<10 minutos para la movilización), promedio (10 a 30 minutos) y severa (>30 minutos o daño intestinal significativo). Los pacientes sometidos a una primera cirugía abdominal por poliposis adenomatosa familiar se utilizaron como grupo de control.RESULTADOS:211 pacientes no tenían cirugía previa: 5% desmoideos y 1% adherencias. 137 pacientes se sometieron a cirugía reoperatoria: 39% tenía enfermedad desmoidea ( p < 0,05 frente aquellos sin cirugía previa), la tasa más alta se presentó en aquellos pacientes después de una anastomosis ileoanal con reservorio (57%) donde el 45% tenía adherencias graves ( p < 0,01 frente aquellos sin cirugía previa), peores resultados se observaron después de la confección de un reservorio de Koch (89%) y luego de proctocolectomía total con ileostomía (82%). El 36% de los pacientes sin enfermedad desmoidea tenían adherencias graves. La reacción desmoidea se asoció con adherencias graves en el 47% de los casos, y los tumores desmoides se asociaron con adherencias graves en el 66% de los casos.LIMITACIONES:Superposición potencial entre adherencias desmoideas y adherencias no desmoideas. Posible inexactitud en la definición del tiempo de adhesiolisis.CONCLUSIONES:La poliposis adenomatosa familiar se asocia con adherencias postoperatorias graves después de una cirugía abdominal reoperatoria, especialmente en pacientes que desarrollan enfermedad desmoidea. (Traducción-Dr. Xavier Delgadillo )., (Copyright © The ASCRS 2023.)

Purpose: To investigate the safety and efficacy of iodine-125 seed implantation in the treatment of abdomen-thorax desmoid tumors (DTs)., Methods and Materials: Data from 14 DT patients who received brachytherapy with iodine-125 seeds were retrospectively collected from 2014 to 2020. The operation was completed using CT guidance and the treatment plan system (TPS). The number of lesions and the target dosimetry parameters were recorded. After brachytherapy, the lesions were evaluated using response evaluation criteria in solid tumors (RECIST)., Results: Fourteen patients with 18 lesions were enrolled in this study; eleven lesions were in the thorax, seven were in the abdomen, and the lesion gross tumor volume (GTV) was 82.10 cc (interquartile range [IQR]: 40.37, 203.42 cc). The median number of seeds was 88 (IQR: 35, 158), and the median prescription dose was 120 Gy (IQR: 115, 120 Gy). The D 90 was 123 ± 16.7 Gy, the V 90 was 97% (IQR: 95.00, 97.25%), and the V 200 was 27% (IQR: 14.50, 33.00%). The median follow-up time for each lesion was 34 (IQR: 23, 67) months, and the local response rate was 100%. Following brachytherapy, the overall survival was 52.3 ± 30.72 months. One year after brachytherapy, one patient experienced persistent worsening of a brachial plexus injury; another received a ureteral stent. No brachytherapy-related complications were observed in the remaining patients., Conclusions: Iodine-125 brachytherapy is a novel treatment option for DT of the abdomen and thorax. Although it is a safe and effective treatment, the radiation dose of iodine-125 brachytherapy for DT-embedded organs at risk must be investigated further., (Copyright © 2023. Published by Elsevier Inc.)

The aim of this study is to evaluate the prevalence, determinants and prognostic value of pain at diagnosis in patients with desmoid-type fibromatosis (DF). We selected patients from the ALTITUDES cohort (NCT02867033), managed by surgery, active surveillance or systemic treatments, with pain assessment at diagnosis. Patients were invited to fill QLQ-C30 questionnaire and Hospital Anxiety Depression Scale. Determinants were identified using logistic models. Prognostic value on event-free survival (EFS) was evaluated using the Cox model. Overall, 382 patients were included in the current study (median age: 40.2 years; 117 men). The prevalence of pain was 36%, without significant difference according to first-line treatment (P = .18). In the multivariate analysis, pain was significantly associated with tumor size >50 mm (P = .013) and tumor site (P < .001); pain was more frequent in the neck and shoulder locations (odds ratio: 3.05 [1.27-7.29]). Pain at baseline was significantly associated with poor quality of life (P < .001), depression (P = .02), lower performance status (P = .03) and functional impairment (P = .001); we also observed a nonsignificant association with anxiety (P = .10). In the univariate analysis, baseline pain was associated with poor EFS; the 3-year EFS was 54% in patients with pain compared to 72% in those without pain. After adjustment for sex, age, size and line of treatment, pain was still associated with poor EFS (hazard ratio: 1.82 [1.23-2.68], P = .003). One third of recently diagnosed patients with DF experienced pain, especially those with larger tumors and neck/shoulder locations. Pain was associated with unfavorable EFS after adjustment for the confounders., (© 2023 The Authors. International Journal of Cancer published by John Wiley & Sons Ltd on behalf of UICC.)

Objective: Neuromuscular choristoma (NMC) is a rare developmental malformation of peripheral nerve that is frequently associated with the development of a desmoid-type fibromatosis (DTF). Both NMC and NMC-DTF typically contain pathogenic CTNNB1 mutations and NMC-DTF develop only within the NMC-affected nerve territory. The authors aimed to determine if there is a nerve-driven mechanism involved in the formation of NMC-DTF from the underlying NMC-affected nerve., Methods: Retrospective review was performed for patients evaluated in the authors' institution with a diagnosis of NMC-DTF in the sciatic nerve (or lumbosacral plexus). MRI and FDG PET/CT studies were reviewed to determine the specific relationship and configuration of NMC and DTF lesions along the sciatic nerve., Results: Ten patients were identified with sciatic nerve NMC and NMC-DTF involving the lumbosacral plexus, sciatic nerve, or sciatic nerve branches. All primary NMC-DTF lesions were located in the sciatic nerve territory. Eight cases of NMC-DTF demonstrated circumferential encasement of the sciatic nerve, and one abutted the sciatic nerve. One patient had a primary DTF remote from the sciatic nerve, but subsequently developed multifocal DTF within the NMC nerve territory, including 2 satellite DTFs that circumferentially encased the parent nerve. Five patients had a total of 8 satellite DTFs, 4 of which abutted the parent nerve and 3 that circumferentially involved the parent nerve., Conclusions: Based on clinical and radiological data, a novel mechanism of NMC-DTF development from soft tissues innervated by NMC-affected nerve segments is proposed, reflecting their shared molecular genetic alteration. The authors believe the DTF develops outward from the NMC in a radial fashion or it arises in the NMC and wraps around it as it grows. In either scenario, NMC-DTF develops directly from the nerve, likely arising from (myo)fibroblasts within the stromal microenvironment of the NMC and grows outward into the surrounding soft tissues. Clinical implications for patient diagnosis and treatment are presented based on the proposed pathogenetic mechanism.

Diarrhea is a common condition seen among soldiers in both garrison and deployed environments. Although the vast majority of soldiers with diarrhea will recover uneventfully with supportive care, clinicians should also maintain suspicion for less common causes and perform a thorough physical exam. We report the case of a young, healthy soldier with chronic diarrhea and progressively worsening abdominal distention that began during his deployment to Honduras who was subsequently found to have a large intra-abdominal desmoid tumor. Desmoid tumor is a rare and benign neoplasm that typically appears on the extremity, abdominal wall, intra-abdominal space, and occasionally in the chest wall. This tumor may be associated with abdominal distension and gastrointestinal complaints. A large tumor can compress organs, causing local tissue damage and, in rare cases, death., (© The Association of Military Surgeons of the United States 2021. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Re-expansion pulmonary edema is a potentially life-threatening situation following thoracic surgery of a compromised lung. We report the case of a 24-year-old female scheduled for a resection of a large intrathoracic desmoid tumor that presented with re-expansion pulmonary edema at the conclusion of her surgery and discuss the clinical presentation, mechanism and predictors of this entity and review similar cases reported in the literature.

Objective: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial., Aim: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables., Methods: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation., Results: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients., Conclusion: Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (Copyright © 2022. Published by Elsevier España, S.L.U.)

Desmoid fibromatosis is a rare connective tissue malignancy. It can occur in a variety of locations, including the abdominal wall, extremities and abdominal cavity. There has been an association with development in a prior surgical scar. Common symptoms can vary depending on the location and can include being painless to having pain at the site, functional impairment and bowel obstruction from intra-abdominal masses. In the following report, we discuss a case in which a patient's abdominal pain was attributed to a postoperative haematoma based on CT radiographic features; however, further work-up and biopsy yielded desmoid fibromatosis, a rare locally aggressive malignancy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Meckel's diverticulum (MD) is among the many known congenital malformations of intestine. It is seen in approximately 2% of the population. Desmoid tumors are unusual. They are unique, well-differentiated, and fast-growing musculoaponeurotic fibromatosis tumors, contemplated as Grade 1 fibro sarcoma. They offer exclusive management challenges to surgeons. The most common presentation of desmoid tumors is of painless masses with sluggish growth. Rarely, they may present as intestinal obstruction leading to surgical emergency. We present the only case in history till date along with review of the relevant literature of a 65-year-old gentleman who presented to us with features of intestinal obstruction who successfully underwent elective segmental resection of ileum containing MD with its desmoid tumor. A multimodality approach is needed to tackle such kind of diseases with a team comprising oncosurgeons, oncophysicians, and radiation oncologist to design a standard treatment protocol., Competing Interests: None

Introduction: Desmoid-type fibromatosis (DF) are locally infiltrative, non-metastasizing tumours associated with significant morbidity and mortality if located intra-abdominally, retroperitoneally or in head and neck localisation. They are mostly sporadic, due to somatic CTNNB1 mutations. Alternatively, they can be associated with germline pathogenic variants in APC causing Familial Adenomatous Polyposis (FAP). Germline APC variants and somatic CTNNB1 mutations are mutually exclusive., Aims and Methods: We conducted a retrospective descriptive analysis of patients with DF seen at the Royal Marsden NHS Foundation Trust Sarcoma Unit in London. We aimed to describe the methods of screening for FAP in patients with DF from a specialist unit. Patients diagnosed between 1992 and 2020 were selected from the prospectively maintained Sarcoma Unit database., Results: 226 patients were identified and 67% (n = 152) were female. Median age at diagnosis was 37.5 (range 2-81) years. Tumour localisation was limbs/pelvis in 30.9% (N = 70), intra-abdominal 16.8% (N = 38), abdominal wall 23.5% (N = 53), thorax 18.6% (N = 42), head and neck 3.1% (N = 7) and vertebral/paravertebral 7.1% (N = 16). Colonoscopy was requested in 65 patients (28.8% of all cases) and was completed in forty-six (20.4%). Molecular testing of CTNNB1 testing was requested in 35 cases (15.5%). APC germline test was requested in 12 cases. Four patients in our cohort had an FAP-associated DF., Conclusions: CTNNB1 ± APC testing and colonoscopy are useful tools for the screening of patients with DF. CTNNB1 molecular testing should be performed in all cases of newly diagnosed DF. Negative CTNNB1 results, alongside clinical assessment, should prompt APC testing and/or colonoscopy., (© 2021. The Author(s), under exclusive licence to Springer Nature B.V. part of Springer Nature.)

Background: Desmoid fibromatosis (DF) is a rare and locally infiltrative monoclonal fibroblastic proliferation arising from connective tissues, with lack of metastatic potential. About 10% of all DF cases are intra-abdominally sited. Complications in this site, due to the locally infiltrative nature of the disease, may be severe and potentially life threatening. However, data on incidence, management, and outcome of these complications are limited., Aim: Data of patients with sporadic or FAP-related intra-abdominal DF treated at Istituto Nazionale dei Tumori (INT) in Milano from 2005 to 2020 who developed a serious complication during the course of their disease were retrospectively collected and analyzed with a descriptive statistics., Methods and Results: Out of 72 intra-abdominal DF, 8 cases were identified (M/F: 5/3, median age: 35 years, FAP-related/sporadic: 2/6): 3 with bowel obstruction, 5 with bowel perforation. In 4 cases the serious complication was the first evidence of disease; in the other 4 cases it occurred at a time interval from diagnosis in the range of 4-44 months (during an active surveillance program in one case and during chemotherapy in the other 3 cases). A surgical treatment was feasible and successful in 5 cases. In 3 surgically unmanageable patients, all progressing and experiencing acute complications while on chemotherapy, a non-surgical approach with intensive supportive treatment and with a prompt change of chemotherapy regimen was implemented, being successful in two, the other patient dying due to a concomitant progressive lymphoma thereafter., Conclusion: In this series of intra-abdominal DF, the incidence of serious complications was 11%. Most patients were successfully treated with surgery. When surgery was deemed to be unfeasible, a conservative management with intensive supportive care and a careful choice of chemotherapy was adopted, ensuring a favorable outcome in most., (© 2021 The Authors. Cancer Reports published by Wiley Periodicals LLC.)

Rationale: Familial adenomatous polyposis (FAP) associated with desmoids tumors (DTs) complicated by abscess formation is rare. The management is not easy and the choice of the best treatment may be controversial., Patient Concerns: A 33-year-old man was admitted to our hospital for abdominal pain, fever, chills, nausea, and vomiting. He had a family history of FAP, and history of abdominal surgery., Diagnoses: An abdominal enhanced chest computed tomography (CT) scan revealed a soft tissue mass in the abdominal wall and an irregular mesenteric soft tissue mass with internal fistula and intra-abdominal abscess. A CT-guided biopsy of the abdominal wall mass revealed DTs., Interventions: The patient was given oral antibiotics for 6 months, and ultimately underwent surgery., Outcomes: The patient had no evidence of recurrence on follow-up at 10 months., Lessons: This case indicates that for patients with FAP who have a history of abdominal surgery and a progressively enlarging mass and abscess in the abdomen, it is necessary to consider the possibility of DTs. FAP-related DTs are rarely complicated by abscess formation. Antibiotic therapy plus surgical resection of the tumor may be effective and make good prognosis., Competing Interests: The authors have no conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Desmoid-type fibromatosis is a rare disease that can result in hydronephrosis. Hydronephrosis associated with desmoid-type fibromatosis often requires surgery or ureteral stent insertion. Although radiation therapy is recommended for inoperable cases of desmoid-type fibromatosis, there has been no report of treatment for hydronephrosis associated with desmoid-type fibromatosis by radiation therapy alone. We herein report a case of successful treatment for inoperable recurrence of desmoid-type fibromatosis with unilateral hydronephrosis by radiation therapy alone., Case Presentation: A 43-year-old Japanese female underwent resection of desmoid-type fibromatosis in the right inguinal region and combined resection of the right external iliac vein 5 years before. Other treatment was not performed because of her pregnancy. Four years after surgery, desmoid-type fibromatosis recurred in the right pelvic wall. Cyclooxygenase-2 selective inhibitor treatment was given for 1 year, but her desmoid-type fibromatosis enlarged to more than 10 cm, and she had swelling of her right leg and hydronephrosis of her right kidney. The patient received 50.4 Gy in 28 fractions of prophylactic irradiation using 10 MV X-ray and 9 Gy in five fractions of a sequential boost for the recurrent desmoid-type fibromatosis. Although there was temporary tumor progression at 1 month after radiation therapy, slow regression of the tumor was seen. At 5 years after radiation therapy, there was no disease progression or severe complications., Conclusion: We experienced successful treatment for an inoperable case of desmoid-type fibromatosis with hydronephrosis. Moderate-dose radiation therapy alone is an effective and feasible approach for the management of hydronephrosis associated with desmoid-type fibromatosis., (© 2021. The Author(s).)

Abstract: We present an unusual case of a 6-year-old boy who presented with the sudden presence of left neck mass and acute hypoxemic respiratory failure, whose subsequent imaging demonstrated a previously undiagnosed anterior mediastinal mass (AMM) extending into the left neck. Biopsy of the mass was consistent with a desmoid tumor, which is a rare cause of AMM in children. Desmoid tumors are locally aggressive, often invading and enveloping surrounding tissues, but overall slow growing. The sudden growth of the neck mass suggests a very aggressive desmoid tumor, causing an unexpected respiratory compromise. Anterior mediastinal masses may cause symptoms by compressing the heart, great vessels, and airways. However, the patient may adapt and develop compensatory mechanisms to counter the compressive effects. Emergency care of the patient with an AMM who presents with acute respiratory distress includes optimizing oxygenation through promoting a calm environment, oxygenating while minimizing positive end-expiratory pressure, maintaining the patient's compensatory mechanisms by minimizing sedation and muscle relaxation, positioning the patient to minimize compressive effects of the mass on the vital thorax structures, and early consultation with pediatric specialists to develop a shared-emergency treatment strategy and to secure an expedited disposition to the appropriate venue of care., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)

A 70-year-old man was admitted to our hospital with acute abdominal pain. Abdominal CT showed a 6-cm-sized tumor near the third portion of the duodenum, and a duodenal GIST was suspected. Although the abdominal pain was intense and the tumor was suspected to be ruptured, the vital signs were stable; therefore, we initially planned to perform an elective surgery. However, because the pain could not be controlled, the surgery was performed on the 6th day of hospitalization. The tumor appeared to be a duodenal GIST because it was pulling the third portion of the duodenum inwards. It had a strong tendency to infiltrate the surrounding organs; therefore, forced resection of the right colon, which is the surrounding organ, was performed. Pathological findings showed that the resected specimen was a desmoid tumor and the surgical margins were negative. The postoperative course 1 year after surgery was favorable, and no tumor recurrence occurred. We report a case of desmoid tumor, which caused acute abdominal pain.

A 17-year-old adolescent boy was referred by an orthopaedic physician to physical therapy for chronic left shoulder pain and a clinical diagnosis of labral tear. The physician ordered current radiographs, which were noncontributory. Findings during physical therapy examination warranted referral back to the physician, with a request for magnetic resonance imaging. Magnetic resonance imaging revealed a soft tissue abnormality in the infraclavicular and subcoracoid spaces. The patient was referred to orthopaedic oncology, where biopsy confirmed a desmoid tumor. J Orthop Sports Phys Ther 2020;50(8):467. doi:10.2519/jospt.2020.9596 .

Background: With recent conservative strategies, prognosis of patients with desmoid-type fibromatosis (DTF) is about function preservation. We analyzed the long-term quality of life (QoL) of pediatric patients with DTF., Methods: All French young patients (<21years) treated between 2005 and 2016 for a DTF in the EpSSG NRSTS-05 study were analyzed. A first wait-and-see strategy was recommended. Patients' QoL was analyzed with the internationally validated Child Health Questionnaire (CHQ). We focused on the relevant subscales scores: physical functioning (PF), role social limitations physical (RP), bodily pain (BP), general health perception (GH) and physical (PhS) and psychosocial (PsS) summary measures., Results: Among the 81 patients, 52 families answered the CHQ (median delay since diagnosis = 6.2years; min2.2-max13.3 years). Median age at diagnosis was 11.5 years. Primary site: limbs (52%), head/neck (27%), or trunk (21%). Five year-Progression Free Survival was 39.1% (95%CI: 27.7-50.5%). As initial management for these 52 patients, 30 patients were first observed (57%), 13 had surgery (25%) and 9 received chemotherapy (18%). Total burden of therapy was exclusive surgery (9pts/18%), exclusive chemotherapy (18pts/35%), surgery + chemotherapy (13pts/25%), chemotherapy + radiotherapy (1 pt), surgery + chemotherapy + radiotherapy (1 pt), wait and see (10 pt). Regarding the parent forms, patients have significant lower PF (86.0vs.96.1; p = 0.03), RP (82.0vs.93.6; p = 0.04), GH (60vs.73; p < 0.005) and PhS (46.2 vs.53; p = 0.02) scores compared to healthy population. Comparison of QoL subscales scores according to initial strategy (wait-and-see vs.surgery/chemotherapy) did not reveal any difference (PF = 87.3vs.84.9; p = 0.80/RP = 83.4vs.78.7; p = 0.72/BP = 78.9vs.78.2; p = 0.95/GH = 59.7vs60; p = 0.97). Similar results were found using the children or adult forms., Conclusions: Initial wait-and-see strategy does not affect long term functional impairment., Competing Interests: Declaration of competing interest All authors disclose any actual or potential conflict of interest., (Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Competing Interests: Competing interests: None declared.

After an adapted imaging, the diagnosis of a desmoid tumor (DT) is provided by a percutaneous microbiopsy, with a molecular analysis for beta-catenin or APC gene mutation. The therapeutic strategy must be decided in a specialized multidisciplinary tumor board (MTB). Surgery is no longer the first-line treatment for a DT. Except within a surgical complication, active surveillance is offered to the majority of patients, since more than half stabilize or regress after an initial progression, whether the location is peripheral or intra-abdominal. If the localization and/or volume are likely to be functional or life-threatening, medical induction therapy is discussed in MTB, before a local treatment whose potential sequelae would be definitive. Incomplete unplanned resection, recurrence, pregnancy or desmoids occurring in a polyposis context are no longer routine surgical indications. In an emergency setting (occlusion, peritonitis), it is discussed to treat only the mechanical complication and leave the DT in place, if its resection would lead to too much digestive resection, especially in patients who have already undergone colectomy for polyposis. The best indications for surgery are patients who have parietal locations with significant and documented progression, because surgery can be easily completed at the cost of an acceptable morbidity. In localizations where surgery would cause sequelae, medical treatment or other regional loco treatments are discussed in MTB., (Copyright © 2019 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Objective: Cryoablation is being more and more often used to treat desmoid tumors. We report our experience of cryoablation as a local and analgesic treatment for inoperable or recurring desmoid tumors., Methods: This study includes 34 patients who underwent percutaneous cryoablation of 41 desmoid tumors between July 2012 and September 2016. All patients underwent pain assessment using a visual analog scale (VAS) and preoperative imaging. All cryoablation procedures were performed using the same cryoablation system. Patients received clinical and radiological follow-up at 6 months post-procedure, with pain-rating and adverse events being recorded. A long-term follow-up was achieved until 31 December 2018. Disease-free survival at 3 years was also recorded. Radiological tumor response was determined by tumor measurements using RECIST 1.1., Results: Twelve patients benefitted from curative treatment on 100% of the tumor volume, but 22 patients received debulking treatment because of the risk of neighboring structures. Two patients had a postoperative hematoma grade 2 of the CIRSE classification system for complications, and two patients had grade 4 complications involving palsy of the common fibular nerve. Disease-free survival at 3 years was 42.2%. The mean VAS pain scores were 5.7 and 2.4 at pretreatment and 6 months, respectively, showing a mean reduction of 3.3 (p < 0.001). At 6 months, all measured tumor dimensions were significantly lower than pretreatment., Conclusion: Cryoablation is an effective therapeutic option for the local treatment and for the analgesic management of desmoid tumors.

Desmoid fibroma (DF) is a rare connective tissue tumor comprising about 0.03-0.13% of all neoplasms. DF has a low potential for malignant transformation, but it is characterized by aggressive course and unfavorable prognosis. The main contingent of patients consists of women of reproductive age. Despite the nearly two-century history of study, there are certain unsolved problems including endocrine problems associated with this disease. We report a 30-year-old female with DF and diabetes mellitus type 1. Total resection of the affected right rectus abdominis muscle was performed in a single block with aponeurotic sheath and peritoneum after normalization of carbohydrate metabolism. Muscular aponeurotic defect 27�10 cm was closed after separation of abdominal wall structures and implantation of polypropylene prosthesis. There are no X-ray and clinical signs of DF recurrence or postoperative hernia after 8 months.

Background: Desmoid tumors, also known as aggressive fibromatosis, are extremely rare, accounting for less than 3% of soft-tissue sarcomas and less than 0,03% of all neoplasms. The diagnosis is usually delayed because of the lack of specific symptoms, and can sometimes lead to serious and, even fatal complications., Case Presentation: We report the case of a 27-year-old male patient presenting with the clinical picture of acute appendicitis. During the operation, we found a tumor in the jejunum with a necrotic zone and perforation on its surface, causing hemorrhagic effusion into the abdominal cavity and subsequent peritonitis. The tumor was removed with negative margins via resection of the small bowel. The final histological result showed aggressive fibromatosis., Conclusions: Aggressive fibromatosis remains a serious problem with the possibility of locally aggressive behavior with high rates of recurrence. Sometimes, its clinical and macroscopic recognition can be immensely tricky. As shown by our patient, on rare occasions, desmoid tumors can lead to acute surgical abdomen requiring an emergency operation.

Background: Rupture of a superior mesenteric artery pseudoaneurysm is a rare but potentially lethal complication in patients with familial adenomatous polyposis and desmoid disease., Objective: We report our experience in the management of such patients with a rare but significant and life-threatening condition., Design: This is a descriptive study of a small series of patients., Settings: Data were obtained from their medical charts and from the Cologene Database of the David G. Jagelman Polyposis Registry in the Sanford R. Weiss, M.D., Center for Hereditary Colorectal Cancer at the Cleveland Clinic Foundation., Patients: Of 227 patients with mesenteric desmoid disease, there were 4 cases of superior mesenteric artery pseudoaneurysm rupture., Main Outcomes Measures: We reviewed the patients with mesenteric desmoid tumors in our desmoid registry. The registry is approved by the institutional review board of the Cleveland Clinic., Results: The patients were young (aged from 22 to 28 y at presentation), with otherwise minimal comorbidities. Two patients had a previous proctocolectomy and J-pouch, and 2 had a total colectomy and ileorectal anastomosis. Two patients had preemptive endoluminal stenting and fared better than the 2 who had damage control embolization. One patient died and, in the others, recovery was prolonged and complicated. Two of the 4 patients have ended up with a reasonable quality of life., Limitations: We acknowledge that this is a rare complication of an uncommon disease and, as such, any case series will be limited by small numbers; therefore, a tailored approach is warranted when managing such complex patients., Conclusions: We advocate an increased awareness of the possibility of visceral pseudoaneurysms in patients with familial adenomatous polyposis who have desmoid disease encasing the superior mesenteric artery. See Video Abstract at http://links.lww.com/DCR/A914.

Background: Opioids are currently offered as first-line treatment for chronic pain from cancer. Continuous regional analgesia could be an alternative to opioids. However, the required duration of catheterization and the sustained analgesic effects of this technique after catheter removal have yet to be clarified., Case Report: We report the case of a patient with a shoulder desmoid tumour for which monitoring of tumour progression was the sole therapeutic strategy. Analgesia took the form of patient-controlled infusion of local anaesthetics through an interscalene catheter. Due to the need of an MRI control 45 days later, the pump was stopped. The persistence of pain relief 48 hr later led to the decision to remove the perineural catheter. No pain was reported by the patient over the following 42 days., Conclusions: In this patient, it would seem that continuous analgesia allowed for a sustained resolution of pain from the shoulder-located tumour. One hypothesis is that local anaesthetics play a direct role in the erasure of pain memory. This hypothesis needs to be tested with a large patient cohort., Significance: This case report provides new insights into the treatment of cancer pain. The most interesting finding is that the pain did remained absent after 45 days of continuous infusion of local anaesthetics through an interscalene catheter. We postulated that local anaesthetic drugs have an impact on pain memory., (© 2018 European Pain Federation - EFIC®.)

The aim of this retrospective cohort study was to review urological complication rates arising from familial adenomatous polyposis associated desmoid tumours and their management. All patients over a 35-year period were identified from a prospectively maintained polyposis registry database and had an intra-abdominal desmoid tumour. Those without ureteric complications (n = 118, group A) were compared to those that developed ureteric obstruction (n = 40, group B) for demographics, treatment interventions and survival outcomes. 158 (56% female) patients were identified. Median age at diagnosis was 31 years and desmoids typically occurred 3.6 years after colectomy for familial adenomatous polyposis. Ureteric obstruction secondary to tumour growth occurred in 25% of cases. There was no significant difference in gender distribution or overall age at desmoid diagnosis between the two groups. In group B, the median age at desmoid diagnosis was significantly younger in women compared to men (25 and 43 years, respectively) (p = 0.01). Thirty-eight percent of patients already had ureteric obstruction at desmoid diagnosis, the remainder occurred after 48.6 months, but 20 years in two cases. Seventy-three percent (29/40) had ureteric stenting, a long-term requirement for most. Permanent renal injury occurred in six cases but survival between the two groups was not significantly different. Ureteric obstruction occurs frequently in patients with familial adenomatous polyposis and an intra-abdominal desmoid tumour. Those most at risk are the young following colectomy. Clinicians should actively survey the renal tract at regular intervals after a diagnosis of an intra-abdominal desmoid tumour as complications can arise insidiously, at any stage.

Background: Here we report a very rare entity of an infratemporal region aggressive fibromatosis in a 23-year-old Tharu man who had presented with the symptoms of painless but progressive trismus., Case Presentation: We describe a case of aggressive fibromatosis in a 23-year-old Tharu man. Radiological imaging as well as an immunohistochemistry panel from a biopsy indicated a diagnosis of an aggressive fibromatosis. Since there was no aggravation in his trismus following surgery and because of his poor socioeconomic status, he was advised to attend regular follow-up visits without any adjuvant therapy., Conclusions: This case report adds to the notion of keeping the differential diagnosis of an aggressive fibromatosis in all patients presenting with progressive but painless trismus. The characteristic imaging findings as well an immunohistochemistry panel will help us clinch the correct diagnosis.

Desmoid-type fibromatosis (DF) is a rare mesenchymal lesion with high risk of local recurrence. Specific β-catenin mutations (S45F) appeared to be related to this higher risk compared to T41A-mutated or wild-type (WT). We explored the influence of both mutations and WT on structure stability and affinity of β-catenin for α-catenin and the pattern of gene expression that may influence DF behavior. Using 33 surgically resected primary DFs harboring T41A (n = 14), S45F (n = 10), or WT (n = 9), we performed a comparative molecular analysis by protein/protein interaction modeling, gene expression by DASL microarrays, human inflammation gene panel, and assessment of immune system-based biomarkers by immunohistochemistry. Mutated proteins were more stable than WT and formed a weaker complex with α-catenin. Consensus unsupervised gene clustering revealed the presence of two DF group-mutated (T41A + S45F) and WT (P = 0.0047). The gene sets 'Inflammatory-Defense-Humoral Immune Response' and 'Antigen Binding' were significantly enriched in T41A. The deregulation of 16 inflammation-related genes was confirmed. Low numbers of T cells and tumor-associated macrophages (TAM) infiltrating the tumors and low/absent PD-1/PD-L1 expression were also identified. We demonstrated that mutated DFs (T41A or S45F) and WT are two distinct molecular subgroups with regard to β-catenin stability, α-catenin affinity, and gene expression profiling. A different inflammation signature characterized the two mutated groups, suggesting mediation either by T41A or by S45F. Finally, all mutated cases showed a low number of TIL and TAM cells and a low or absent expression of PD-1 and PD-L1 consistent with β-catenin activation insensitive to checkpoint blockade., (© 2017 The Authors. Published by FEBS Press and John Wiley & Sons Ltd.)

Aggressive fibromatosis (AF) or desmoid tumors is an aggressive fibroblastic proliferation which is locally invasive but can not metastasize. The treatment of AF is challenging. Surgery was the main treatment modality for AF in the past, other strategies including radiotherapy, systemic therapies and wait-and-see policy. The use of non-steroidal anti-inflammatory drugs (NSAIDs) and targeted therapies has demonstrated good results. In the case report, a 39-year-old man presented with progressive chest wall pain. Computed tomography (CT) showed an approximately 46× 13 mm soft-tissue mass between the inside of the fifth and sixth rib on the right side. The entire mass was excised and an AF was confirmed based on histopathology. Four months after surgery, magnetic resonance imaging (MRI) showed a soft-tissue mass in surgical areas and biopsy confirmed local recurrence. Therefore, Tomotherapy was administered. However, two months later, an (18)F-fluorodeoxyglucose (FDG) Positron Emission Tomography combined with CT (PET-CT) revealed the presence of an FDG-avid mass in the area of local recurrence. Genetic testing reported the presence of a p.T41A mutations on the CTNNB1 gene, which predicted that he is sensitive to the COX-2 inhibitor celecoxib. The tumor regressed rapidly after the application of celecoxib. Within the 20-month follow-up period, the patient showed remarkable regression without any signs and symptoms. Our case report provides further evidence for the efficacy of celecoxib in AF with CTNNB1 gene mutations. To our knowledge, this is the first report of AF treated with celecoxib under the guidance of the genetic testing. However, further studies are required.

A 19-year-old woman presented with painless swelling of the abdomen. During surgery, a giant mass measuring 37 cm×26 cm×12 cm within the distal pancreas invading the spleen was noted. The clinical diagnosis of a solid cystic pseudopapillary tumor of the pancreas was suspected. Distal pancreatectomy, splenectomy, and debulking surgery were performed. Histological examination showed that the tumor infiltrated the spleen and pancreatic parenchyma, and sections of the solid areas revealed a proliferation of spindle-shaped or stellate cells growing in fascicular and storiform patterns within a myxoid intercellular matrix. Cystic areas were representing the entrapped excretory pancreatic ductules. Interestingly, there were two ectopic adrenal tissues found incidentally in the peripheral portion of the tumor. The histopathologic and immunohistochemical features were consistent with a solid cystic desmoid tumor of the pancreas. Desmoid tumors of the pancreas are very rare, and if they present as a solid cystic lesion, their diagnosis may be difficult. We report the case for its rarity and huge size and to emphasize a regular follow-up because the long-term prognosis is currently unknown.

Patients with familial adenomatous polyposis require surgical intervention at some point in their lives. The diagnosis is often apparent from their phenotype and family history, however, this is not always the case. Many factors can influence the surgical strategy although the polyposis burden and distribution remain the main consideration. While prophylactic removal of the rectum and colon is often required, sparing the rectum at the index surgery is safe in select patients. This article aims to dispel misconceptions in the diagnosis and treatment of patients with familial adenomatous polyposis., (© 2017 Royal Australasian College of Surgeons.)

Patients with Down syndrome (DS) have a markedly higher incidence of childhood leukemia, but a lower incidence of most solid tumors, compared with age-matched euploid individuals. Trisomy 21 might be protective against tumorigenesis because of several tumor suppressive mechanisms. Desmoid-type fibromatosis (DF) is a rare monoclonal, fibroblastic proliferation characterized by a variable clinical course. In recent reports, almost all cases of DF involved genomic alterations associated with activation of the Wnt/β-catenin pathway. Here, we report the case of a boy with DS who developed DF without activation of the Wnt/β-catenin pathway. To the best of our knowledge, this is the first case of DS involving DF., (© 2017 Japan Pediatric Society.)

Purpose: Desmoid tumor (DT) is a rare myofibroblastic proliferative neoplasm, but can occur in up to 26% of patients with familial adenomatous polyposis (FAP). Therefore, DT may be an extraintestinal manifestation of FAP that precedes the development of advanced malignancies. The aim of this study was to identify the incidence of polyps in DT patients and investigate value of colonoscopy for diagnosis of FAP in DT patients without gastrointestinal symptoms., Materials and Methods: The records of patients diagnosed with DT were retrospectively reviewed using the clinical data warehouse (CDW) system. Clinical data, including location of tumor, type(s) of treatment, and colonoscopic findings, were collected., Results: Sixty-five patients were diagnosed with DT during the study period; 10 patients received colonoscopies before diagnosis of DT, and 20 patients received colonoscopies after diagnosis of DT. The mean age at diagnosis of DT was 41.9 ± 16.7 years old (range 17-74). Most tumors were extra-abdominal (n = 24, 80.0%), and fewer were intra-abdominal (n = 4, 13.3%) or in an abdominal wall (n = 2, 6.7%). The colonoscopy results revealed one or two tubular adenomas in 11 patients (36.6%), although none of the patients had colorectal polyposis or colorectal cancer., Conclusion: None of the patients in our study population had colorectal polyposis. Routine colonoscopy of DT patients without gastrointestinal symptoms seems to have low diagnostic yield for detection of FAP.

Desmoid tumors, also known as aggressive fibromatosis, are locally infiltrating musculoaponeurotic neoplasms arising in connective tissues. Desmoid tumors may be associated with familial adenomatous polyposis (FAP), a genetic disorder that presents with hundreds to thousands of precancerous colorectal polyps. The authors report the case of an 18-month-old boy who underwent resection of a right temporal desmoid tumor (initially diagnosed as cranial fasciitis) and developed a bilateral frontoparietal calvarial desmoid tumor 2 years later. The patient underwent gross-total resection of the tumor that required a large cranioplasty. He was subsequently diagnosed with FAP. The patient has been without tumor recurrence for 9 years afterwards and has not required revision of his cranioplasty. This is the first report describing a recurrent cranial desmoid tumor in a pediatric patient with FAP. The authors believe, however, that some of the cases previously reported as cranial fasciitis are likely desmoid tumors pathobiologically and genetically.

Background: The efficacy of the classical treatment modalities surgery and radiotherapy in the treatment of aggressive fibromatosis is presently disputed and there is a shift towards a more conservative approach. The aim of the present study is to objectify tumor growth in patients with extra-abdominal or abdominal wall aggressive fibromatosis, while adhering to a "watchful waiting" policy. Other objectives are to investigate quality of life and to identify factors associated with tumor growth, in particular the relation with the presence of a CTNNB1-gene mutation in the tumor., Design and Methods: GRAFITI is a nationwide, multicenter, prospective registration trial. All patients with extra-abdominal or abdominal wall aggressive fibromatosis are eligible for inclusion in the study. Main exclusion criteria are: history of familiar adenomatous polyposis, severe pain, functional impairment, life/limb threating situations in case of progressive disease. Patients included in the study will be treated with a watchful waiting policy during a period of 5 years. Imaging studies with ultrasound and magnetic resonance imaging scan will be performed during follow-up to monitor possible growth: the first years every 3 months, the second year twice and the yearly. In addition patients will be asked to complete a quality of life questionnaire on specific follow-up moments. The primary endpoint is the rate of progression per year, defined by the Response Evaluation Criteria In Solid Tumors (RECIST). Secondary endpoints are quality of life and the rate of influence on tumor progression for several factors, such as CTNNB1-mutations, age and localization., Discussion: This study will provide insight in tumor behavior, the effect on quality of life and clinicopathological factors predictive of tumor progression., Trial Registration: The GRAFITI trial is registered in the Netherlands National Trial Register (NTR), number 4714 .

A 25 years married women having one child delivered vaginaly presented in the department of Obs & Gynae, Bangabandhu Sheikh Mujib Medical University, Bangladesh on April 2013 with pelvic pain and discomfort. No history of previous pelvic trauma was present. Patient examination showed a isolated mass in the right lower abdomen, right adnexa, extending to the pelvic wall upto lower end of ureter. Tumor markers were within normal limit. Intravenous pyelogram (IVP) showed mild right hydroureter and hydronephrosis with obstruction at the lower end of ureter. She was diagnosed as a case of adnexal mass with mild hydroureter & mild hydronephrosis and it was decided to operate on the patient. The surgical approach was transabdominal. On laparotomy a pseudocystic lesion 12×10cm in size was found over the right paracolic gutter and extending down into the pelvis involving the right parametrium. No abnormality was found in the uterus or tubes. The histological examination revealed a desmoid tumor of the pelvis. The patient's recovery was uneventful.