脊柱侵袭性纤维瘤的外科治疗

目的探讨临床上少见的脊柱侵袭性纤维瘤的特点和外科手术联合放射治疗的效果。方法总结1998年1月至2006年9月收治的7例脊柱侵袭性纤维瘤患者,其中颈椎3例、胸椎2例、腰椎2例。所有病例肿瘤均侵犯椎弓根和附件,2例胸椎肿瘤侵犯硬膜囊。7例患者均采用手术治疗,术后进行正规放疗。结果所有患者局部疼痛消失,除1例胸腔镜辅助手术患者拔管后出现包裹性胸腔积液外,其余病例均无围手术期并发症。3例术前神经系统功能障碍者术后神经症状均得到不同程度的改善。术后平均随访17.6（13～23）个月,未见内固定失败和肿瘤复发。结论脊柱侵袭性纤维瘤是一种极为少见的局部侵袭性软组织肿瘤,临床上有侵袭性生长的生物学行为。外科手术切除肿瘤是脊柱侵袭性纤维瘤的主要治疗方法,但因该肿瘤具有较高的复发率,故需联合术后正规化疗才能获得满意的临床治疗效果。     

Surgical Treatment of Pediatric Desmoid Tumors. A 12-Year, Single-Center Experience

Background

Pediatric desmoid tumors (PDTs) represent a group of rare, distinct lesions. While sparse, available literature suggests that PDT are particularly aggressive and difficult to control when compared with their adult counterpart.

Methods

A retrospective review identified 39 patients who underwent treatment of PDT at St. Jude Children’s Research Hospital over a 12-year period. Clinicopathologic and treatment characteristics were analyzed to identify predictors of outcome.

Result

A total of 39 patients were treated during the study period, with a total number of 67 resections. Median age was 12.2 years; 49 % of patients were male, and 51 % were female. Median tumor size was 9.8 cm. PDT most commonly arose in the extremities (40 %), thorax (23 %), head and neck (21 %), and trunk (16 %). Also, 18 % of resections had negative margins (R0), 48 % were microscopic positive (R1), and 30 % were macroscopic positive (R2). The 1- and 5-year recurrence-free survival (RFS) was 97.1 and 73.1 %, respectively. Factors associated with worse RFS were patient age >12 years (HR = 5.08, p = 0.038) and tumor size >5 cm (HR = 1.22, p = 0.0597). Margin status did not affect RFS. Selective use of radiation therapy appeared to improve RFS.

Conclusions

Our study suggests that margin status alone at the time of extirpation is not a predictor of ultimate cure or likelihood of recurrence. Many patients received adjuvant therapy, with benefits suggested after analysis. For patients with PDT, surgical extirpation should not come at the expense of functional preservation, as overall survival is excellent.     

Desmoid Tumors (Fibromatoses) of the Breast: A 25-Year Experience

Background Breast desmoid tumors are rare and often clinically mistaken for carcinoma. We reviewed our 25-year institutional experience with breast desmoid tumors. Methods A search of pathology and sarcoma databases (1982–2006) identified 32 patients with pathologically confirmed breast desmoids. Records were retrospectively reviewed. Results Median presentation age was 45 years (range, 22–76). Eight patients (25%) had prior history of breast cancer and 14 (44%) of breast surgery, with desmoids diagnosed a median of 24 months postoperatively. All presented with physical findings. Mammography visualized the mass in 6/16, ultrasound in 9/9, and magnetic resonance imaging (MRI) in 8/8 patients in whom it was performed. In 15 patients with attempted needle biopsy, fine needle aspiration was inconclusive (9/9 patients), and core biopsy demonstrated a spindle cell lesion (6/7 patients). Treatment was surgical, with median tumor size of 2.5 cm (range, 0.3–15). Eight patients (29%) had recurring tumors at a median 15 months. Patients with recurring tumors were younger (median age: 28 vs. 46 years, p = 0.03). A trend toward more frequent recurrences in patients with positive (5/9 patients) versus negative (3/19 patients) margins (p = 0.07) and larger tumors (p = 0.12) was observed. Conclusions In our series, breast desmoids presented as palpable masses suspicious for carcinoma clinically and radiographically. Therapy remains primarily surgical, and core biopsy aided in operative planning. Recurrences are common, with younger age and possibly positive margin status and larger tumor size associated with increased risk of recurrence. As 5/9 patients with positive and 3/19 patients with negative margins experienced recurrences, clinical judgment should be used prior to extensive and potentially deforming resections.     

Spontaneous Regression of Primary Abdominal Wall Desmoid Tumors: More Common than Previously Thought

Purpose

The relevance of the initial observational approach for desmoid tumors (DTs) remains unclear. We investigated a new conservative management treatment for primary abdominal wall DTs.

Methods

Data were collected from 147 patients between 1993 and 2012. The initial therapeutic approaches were categorized as front-line surgery [surgery group (SG), n = 41, 28 %] and initial observation or medical treatment [nonsurgery group (NSG), n = 106, 72 %]. The cumulative incidence of the last strategy modification was estimated using competing risk methods with variable censoring times.

Results

Of the 147 patients, 143 were female (97 %). In the SG, 27 patients (66 %) required full-thickness abdominal wall mesh repair. In the NSG, 102 patients (96 %) underwent initial observation and four received medical treatment. In the NSG, the 1- and 3-year incidences of changing to medical treatment (no further changes during the follow-up) were 19 % [95 % confidence interval (CI) 11–28] and 25 % (95 % CI 17–35), respectively, and the 1- and 3-year incidences of a final switch to surgery were 14 % (95 % CI 8–22) and 16 % (95 % CI 9–24), respectively. An initial tumor size of >7 cm was associated with a higher strategy modification risk (p = 0.004). Of the 102 patients initially observed, 29 experienced spontaneous regression over a median follow-up period of 32 months. All second-intent resections were macroscopically completed, with R0 resections achieved in 82 % of patients.

Conclusions

This study supports an initial nonsurgical approach to abdominal wall DTs ≤7 cm, followed by surgery based on tumor growth in select cases.     

Desmoid tumors

We retrospectively studied the outcome in a consecutive series of 30 patients with desmoid tumors who were followed for more than 20 years after treatment (surgery in 29 patients). A local recurrence occurred in 12 patients and more than 1 recurrence occurred in 8. 3 patients had spontaneous complete regression of the tumor. At follow-up after mean 28 (20-54) years, all patients were tumor-free except 1 who had had a stable tumor for at least 11 years. Symptoms were related more to treatment than to the tumor. We conclude that desmoid tumors may have a high capacity for self-limitation and that conservative therapy should be considered in symptom-free patients.     

Desmoid tumors

Desmoid tumors are a rare form of malignancy with a great propensity for local extension and recurrence. They typically occur in the abdominal wall or within the abdomen but also may occur extra-abdominally. Most cases are sporadic but traumatic, hormonal, and genetic etiologies have been implicated. The only curative treatment is wide surgical excision, but the risk of local recurrence is high. Several adjuvant or complementary treatments have been proposed and the results show promise; the authors review all these therapies.     

Desmoid Tumors of the Anterior Abdominal Wall: Results from a Monocentric Surgical Experience and Review of the Literature

Background  Desmoid tumor, also known as aggressive fibromatosis, is a rare soft tissue tumor. For those cases localized in the anterior abdominal wall, radical resection and reconstruction with a mesh is indicated. Because the rarity of the disease, randomized trials are lacking, but in reported retrospective series, it is clear that although it is considered a benign lesion, local recurrence is not uncommon. Methods  We analyzed the records of 14 consecutive patients (3 men, 11 women, mean age 36 years, range 25–51 years) with desmoid tumor of the anterior abdominal wall treated at the European Institute of Oncology. The surgical strategy was the same in all cases: wide surgical excision and immediate plastic reconstruction with mesh after intraoperative confirmation by frozen sections of disease-free margins of >1 cm. We considered long-term outcomes by using the European Organization for the Research and Treatment of Cancer QLQ-C30 as an instrument to evaluate the overall quality of the treatment delivered to these patients. Results  No immediate postoperative complication was registered, and no patient developed recurrence after a median follow-up period of 55 months. Two women experienced mesh bulging within 1 year after the operation. The long-term mean global health status registered was 97 out of 100. Conclusions  Radical resection aided by intraoperative margin evaluation via frozen sections followed by immediate mesh reconstruction is a safe procedure and can provide definitive cure without functional limitations for patients with desmoid tumors of the anterior abdominal wall.     

Diagnosis and Treatment of Sporadic and Familial Adenomatous Polyposis (FAP) - Associated Desmoid Tumors: Literature Review

Desmoid tumors account for 3% of all soft tissue tumors and 0.03% of all neoplasms. In some cases, they appear as part of various syndromes, such as familial adenomatous polyposis (FAP). Desmoids are associated with CTNNB1and APC mutations, which exclude each other. Desmoid tumors appear as slowly growing masses that often do not cause symptoms. The natural course of desmoids varies and is unpredictable. Most patients feel relieved by the benign diagnosis and underestimate the aggressive behavior of the disease.Various imaging techniques have been used for diagnosis and monitoring of desmoids, including ultrasound (U/S), magnetic resonance imaging (MRI), computed tomography (CT) scan, positron emission tomography (PET) scan and plain X-ray. The definitive diagnosis requires biopsy.Desmoids usually express β-catenin and vimentin. Sporadic tumors are associated with CTNNB1 mutation of β-catenin. If this mutation is identified, the presence of any syndrome can be excluded. FAP is inherited in an autosomal dominant fashion and is caused by a mutation of the APC gene; 10-15% of patients with FAP develop desmoid tumors while 7.5-16% of desmoids are a manifestation of FAP.The basic therapeutic approaches are surgical excision, radiotherapy and medical treatment with anti-estrogen, nonsteroid anti-inflammatory drugs (NSAIDs), cytotoxic chemotherapy and molecular therapies. Close observation of the disease progress is imperative, whether the “wait and watch” strategy has been chosen or a specific treatment has been applied. Imaging, preferably MRI, should be performed every 3 months for the first year after diagnosis, every 6 months for the second year and annually thereafter.     

Desmoid tumors in three patients

Desmoids are rare tumors of the connective tissue. It develops about 1:1000 times more in patients with familial adenomatous polyposis (FAP, Gardner syndrome) compared to normal population. It has been shown in molecular genetic examinations, that different mutations of the APC gene are responsible for desmoid tumors in FAP. It means, that this disease is one of the extraintestinal manifestations of Gardner syndrome. This tumor has high recurrence rate and is growing rapidly, and as a result it is the second most common cause of death in FAP patients. That is why genetic examination for FAP patients is advised to decide if the patient has higher risk for desmoid formation. If the result of the genetic test is positive, it is advisable to try to slow the progression of polyposis with medical treatment, and so to delay the date of the colectomy because the surgical intervention--and connective tissue damage--can induce desmoid formation in these patients. At the same time it is reasonable to examine and regularly control patients with sporadic desmoid tumors searching for other manifestations of Gardner syndrome (colon, stomach and duodenum polyposis, tumor of papilla Vateri, retinopathy, etc.). Palliative surgery is not indicated in patients with inoperable intraabdominal desmoid tumors, because partial resections (R1, R2, debulking) result in further tumor progression. In these patients medical treatment (sulindac, tamoxifen), chemotherapy (doxorubicin, dacarbazin) and radiotherapy or combination of them can result tumor remission. We describe our three patients (an abdominal wall desmoid four years following Cesarean section; a desmoid tumor in the retroperitoneum and in the pelvis diagnosed three years after total colectomy; and a retroperitoneal and abdominal wall desmoid one year after total colectomy) and etiology, diagnosis and therapy of desmoid tumors are discussed.     

Desmoid tumour of lower abdomen

A desmoid tumour is slow growing fibromatosis with aggressive infiltration of adjacent tissue and extremely unusual systemic metastases. We report on a female patient aged 25 years who had a mass in lower abdomen for 2 years. There was no previous history of any surgical intervention. Preoperative evaluation included ultrasound and computed tomography. Patient underwent primary resection with wide margins. Histology revealed a desmoid tumour.