暂无摘要。

UNASSIGNED: Desmoid-type fibromatosis (DF) is characterized by a rare monoclonal fibroblast proliferation that exhibits variable and unpredictable clinical presentation. DF can be classified into sporadic and hereditary types. Despite extensive research efforts, the exact etiology of DF remains elusive.
UNASSIGNED: A 31-year-old male patient presented to the hospital with a progressively growing mass in the right lower abdomen, accompanied by abdominal discomfort. Symptoms are discovered 1 week before admission. Enteroscopy revealed no evidence of colonic abnormalities, and blood tests did not indicate any abnormalities. Due to the indeterminate nature of the mass during surgery, a partial resection of the ileum and cecum was performed, followed by ileocolonic end-to-end anastomosis, with no postoperative complications. The final pathological diagnosis confirmed primary desmoid-type fibromatosis of the distal ileum (invasive fibromatosis). To effectively manage DF, we recommend a follow-up schedule for patients. This includes appointments every 3 months in the first year following surgery, followed by appointments every 6 months up to the fifth year, and then once a year thereafter. The follow-up examinations should include collection of the patient\'s medical history, physical examination, blood tests, ultrasounds, CT scans, and other relevant assessments. During the first year of the follow-up period, no further treatment was administered, and the patient remained disease-free.
UNASSIGNED: Desmoid-type fibromatosis (DF) originating from the small intestine is an extremely rare condition that exhibits local invasiveness and can be life-threatening. Despite its benign histology, DF has a high local recurrence rate and lacks metastatic potential. Diagnosis of DF remains challenging, especially in cases where surgical intervention is not feasible due to asymptomatic patients or partial organ impairment. In such cases, a \"watchful waiting\" approach is recommended as the initial treatment strategy. However, when preoperative diagnosis is difficult, surgery is typically considered the best option. Given the potential for local recurrence and the uncertain long-term prognosis, regular follow-up is necessary.

OBJECTIVE: Although the treatment of abdominal wall desmoid-type fibromatosis (DF) has evolved over the past decades, surgical treatment remains an important approach. Previously, surgeries for abdominal DF were mostly performed by laparotomy, which involves massive dissection and significant trauma. Here, we report our single-center experience of the laparoscopic management of abdominal wall DF in young female patients.
METHODS: The clinical data of nine patients diagnosed with abdominal wall DF during January 2020-April 2022 at the Qilu Hospital of Shandong University were retrospectively analyzed. All patients underwent laparoscopic resection of abdominal wall DF and immediate abdominal wall reconstruction (AWR) with mesh augmentation via the intraperitoneal onlay mesh (IPOM) technique.
RESULTS: Laparoscopic DF resection and AWR were successfully performed in all patients. The mean operation time was 175.56 ± 46.20 min. The width of abdominal wall defect was 8.61 ± 3.30 cm. Full- and partial-thickness myofascial closure and reapproximation were performed in five, two, and two patients, respectively. The average mesh size was 253.33 ± 71.01 cm2. The total and postoperative lengths of hospital stay were 11.00 ± 3.46 and 4.89 ± 2.03 days, respectively. Tumor recurred in one patient after 20 months of the resection. Nonetheless, death, herniation, or bulging were not observed in any patient during a mean follow-up of 16.11 ± 8.43 months.
CONCLUSIONS: Laparoscopic resection of abdominal wall DF and immediate AWR with IPOM mesh reinforcement is safe and reliable for young female patients. Management of such patients should be decided according to the biological behavior, size, and location of tumors.

UNASSIGNED: Aggressive fibromatosis is a rare and locally infiltrative monoclonal fibroblastic proliferation with lack of metastatic potential. We describe a rare case of intra-abdominal aggressive fibromatosis on young female with hyperemesis.
UNASSIGNED: A 23-year-old female was admitted with hyperemesis and loss of weight.
UNASSIGNED: According to imaging findings and immunohistology findings, a diagnosis of intra-abdominal aggressive fibromatosis was formulated.
UNASSIGNED: After the surgery, no evidence of local recurrence was noted during the 6 months of follow-up.
UNASSIGNED: AF may explain why pregnant women may have severe hyperemesis.

暂无摘要。

BACKGROUND: Neuromuscular choristomas (NMCs), are extremely rare developmental lesions that, have been previously established associated with recurrent fibromatosis after surgery, leading to several operations or even amputation. However, reports on the ultrasound imaging features and clinical conditions of NMCs are rare. The purpose of this study is to describe the ultrasound features and clinical analysis of NMCs to provide suggestions to identify the optimal management strategy.
METHODS: From September 2020 to September 2021, 7 patients with a confirmed diagnosis of NMC who underwent ultrasound examination in our department were enrolled in our study. Physical examinations were performed to detect motor deficits, sensory deficits, neuropathic pain, limb undergrowth, muscular atrophy, cavus foot and bone dysplasia. Ultrasound imaging was performed and investigated both in affected nerves and neuromuscular choristomas associated desmoid-type fibromatosis (NMC-DTF). All patients had a definite history and regular follow-up. The clinical course, physical examinations, ultrasound features and pathologic results of NMC patients were analyzed.
RESULTS: Seven patients with an average age of 7.0 ± 7.2 years (range: 2-22 years) were enrolled in our study. The affected nerves included the sciatic nerve (6 cases) and the brachial plexus (1 case). Six patients (85.7%) presented with limb undergrowth, 6 (85.7%) with muscular atrophy, and 5 (71.4%) with cavus foot deformity. Based on ultrasound findings, all the visibly affected nerve segments presented with hypoechoic and fusiform enlargement with intraneural skeletal muscle elements. Five patients (71.4%) had NMC-DTFs at the site of the affected nerve. All NMC-DTFs were shown as hypoechoic solid lesions adjacent to the nerve and were well circumscribed. In the subset of the surgery group, all 5 patients presented with progression to NMC-DTFs at the site of the NMCs. No fibromatosis was detected in the other two nonsurgical patients.
CONCLUSIONS: Understanding the typical ultrasound features and clinically associated conditions would support the early diagnosis of this rare disease. When a potential diagnosis is determined, an invasive procedure such as biopsy or resection might not be a good choice given the frequent occurrence of complications such as aggressive recurrence.

A 65-year-old man underwent excision of a giant mesenteric fibromatosis (MF) via combined splenectomy and partial transverse colectomy. Pathological examination confirmed the presence of MF, whereas genetic testing indicated that the tumor was sensitive to tamoxifen. Over a 1-year follow-up, no symptoms of abdominal discomfort or recurrence was noted.

暂无摘要。

This study aimed to describe the characteristics of superficial desmoid fibromatosis (DF) using two-dimensional and contrast-enhanced ultrasonography, intending to improve diagnostic accuracy. We retrospectively analyzed 19 cases of superficial DF confirmed by surgery or core-needle biopsy in our hospital from January 2018 to August 2020. All patients underwent two-dimensional and contrast-enhanced ultrasound (CEUS) examination. Nineteen patients included 15 women and 4 men, with an average age of 33.37±12.13 years old. The mean size of lesions was 4.78±1.99 cm. On ultrasound, all lesions presented as solitary heterogeneous hypoechoic masses; 13 presented with ill-defined margins. Ten lesions (52.63%) presented with fusiform shapes, and 11 lesions (57.89%) presented with the \"fascial tail\" sign. CEUS suggested the tumors were hyperenhanced, with an enhanced pattern of rapid wash-in and slow wash-out. Four lesions (21.05%) showed an enlarged scope in the CEUS image compared with the grayscale ultrasound image. In conclusion, an ill-defined heterogeneous hyperechoic appearance with fusiform-shaped and \"fascial tail\" signs on US and heterogeneous hyperenhancement with an enlarged scope on CEUS are valuable clues in the diagnosis and treatment of superficial DF.

UNASSIGNED: To evaluate the long-term effectiveness of patients received surgical treatment under the guidance of \"West China Classification\" of desmoid-type fibromatosis (DTF) in the shoulder girdle.
UNASSIGNED: The clinical data of 32 patients with DTF in the shoulder girdle admitted between June 2003 and December 2016 were retrospectively analyzed, including 14 males and 18 females, aged 14-56 years with an average age of 36.8 years. The maximum diameter of the tumor was 7-19 cm, with an average of 11.1 cm. According to the \"West China Classification\" of DTF in the shoulder girdle, there were 4 cases of region Ⅰ, 3 cases of region Ⅱ, 6 cases of region Ⅲ, 3 cases of region Ⅳ, 5 cases of regions Ⅰ+Ⅱ, 5 cases of regions Ⅱ+Ⅲ, and 6 cases of regions Ⅰ+Ⅱ+Ⅲ. In addition, the involvement of blood vessels and nerves was also taken into consideration for choosing a surgical approach. Finally, 12 cases were operated via anteroposterior approach (group A), 14 via posterior approach (group B), and 6 via combined anterior-posterior approach (group C). The 1993 Musculoskeletal Tumor Society (MSTS93) score (including pain, limb function, satisfaction, hand position, hand flexibility, and lifting ability), Japanese Orthopedic Association (JOA) score, range of motion (ROM) of shoulder joint (including flexion, extension, abduction, and adduction), and complications of patients in the 3 groups were recorded and compared.
UNASSIGNED: All the 32 patients were followed up 30-190 months, with an average of 94.6 months. At last follow-up, complications occurred in 5 cases (15.6%), including 2 cases (16.6%) in group A, 2 (14.3%) in group B, and 1 (16.6%) in group C. There was no significant difference in the incidence of complications among the 3 groups ( P=1.000). Tumor recurrence occurred in 5 (15.6%) cases, including 1 (8.3%) case in group A, 2 (14.3%) in group B, and 1 (16.6%) in group C. No significant difference was found in the recurrence rate among the 3 groups ( P=1.000). At last follow-up, MSTS93 score of pain, limb function, satisfaction, hand flexibility, and hand position in groups A and B were significantly better than those in group C ( P<0.05), even though no significant difference existed between group A and group B ( P>0.05). The lifting ability score in group C was significantly lower than in group A ( P<0.05), and no significant difference was found between other groups ( P>0.05). The JOA score and flexion, extension, abduction, and adduction activities of shoulder in groups A and B were significantly better than those in group C ( P<0.05). The extension activity in group A was significantly better than that in group B ( P<0.05), the flexion activity in group B was significantly better than that in group A ( P<0.05). There was no significant difference in other indexes between groups A and B ( P>0.05).
UNASSIGNED: Taking a rational approach to fully expose and completely remove the tumor is the key point of surgical treatment for patients with DTF in the shoulder girdle. At the same time, preservation of vital structures and reconstruction of soft tissues should also be taken into consideration. Overall, surgical treatment under the guidance of \"West China Classification\" of DTF in the shoulder girdle has achieved satisfactory long-term effectiveness.
UNASSIGNED: 评估肩胛带韧带样纤维瘤（desmoid-type fibromatosis，DTF）华西分区指导外科治疗的远期临床疗效。.
UNASSIGNED: 回顾分析 2003 年 6 月—2016 年 12 月收治的 32 例肩胛带 DTF 患者临床资料，男 14 例，女 18 例；年龄 14～56 岁，平均 36.8 岁。肿瘤最大径 7～19 cm，平均 11.1 cm。根据肩胛带 DTF 华西分区，肿瘤位于 Ⅰ 区 4 例、Ⅱ 区 3 例、Ⅲ 区 6 例、Ⅳ 区 3 例、Ⅰ+Ⅱ 区 5 例、Ⅱ+Ⅲ 区 5 例、Ⅰ+Ⅱ+Ⅲ 区 6 例。根据肩胛带 DTF 华西分区及血管神经受累情况，12 例采用前路手术（A 组），14 例采取后路手术（B 组），6 例采用前-后联合入路手术（C 组）。记录并比较 3 组患者 1993 美国骨肿瘤学会评分系统（MSTS93） 评分（包括疼痛、肢体功能、满意度、手的位置、手灵活度、举物能力 6 方面）、日本骨科协会（JOA）评分、肩关节活动度（包括前屈、后伸、外展、内收）及并发症发生情况。.
UNASSIGNED: 32 例患者均获随访，随访时间 30～190 个月，平均 94.6 个月。随访期间共 5 例（15.6%）患者发生并发症，其中 A 组 2 例（16.6% ）、B 组 2 例（14.3%）、C 组 1 例（16.6%），各组并发症发生率比较差异均无统计学意义（ P=1.000）。至末次随访时，共 4 例（12.5%）患者肿瘤复发，其中 A 组 1 例（8.3%）、B 组 2 例（14.3%）、C 组 1 例（16.6%），各组复发率比较差异均无统计学意义（ P=1.000）。末次随访时，A、B 组 MSTS93 评分的疼痛、肢体功能、满意度、手灵活性、手的位置评分及总分均显著优于 C 组（ P<0.05），A、B 组间差异无统计学意义（ P>0.05）；C 组举物能力评分显著低于 A 组（ P<0.05），其余组间比较差异无统计学意义（ P>0.05）。A、B 组 JOA 评分及肩关节前屈、后伸、外展、内收活动度均显著优于 C 组（ P<0.05）；A 组后伸活动度优于 B 组，B 组前屈活动度优于 A 组，差异有统计学意义（ P<0.05），其余指标 A、B 组间差异均无统计学意义（ P>0.05）。.
UNASSIGNED: 根据肩胛带 DTF 华西分区选择适当的手术入路，充分暴露并完整切除肿瘤，取得了较满意的远期临床疗效。.