142例侵袭性纤维瘤病的临床特点及预后分析

目的 分析侵袭性纤维瘤病的临床特征及预后因素,为临床治疗提供依据.方法 回顾分析本院1983-2009年治疗的142例侵袭性纤维瘤病患者的临床资料,观察临床特点及治疗方式对预后影响.采用Logrank单因素分析及Cox多因素回归分析评估可能影响局部预后的危险因素.结果 随访率为93.7%,随访满5、10者分别为63例、6例.本组病例男女比例为1∶1.8,18～35岁女性为高发人群(25.4%).病变部位发生于躯干(55.6%)及四肢(31.7%)多见.5、10年局部复发率分别为24.4%、31.1%,生存率均为99.3%.单因素分析发现肿瘤大小(χ2=4.37,P=0.037)和切缘情况(χ2=12.36,P=0.002)为肿瘤复发的危险因素.多因素分析发现切缘情况为独立的复发危险因素(RR=2.129;χ2=9.47,P=0.002),放疗为侵袭性纤维瘤病的保护因素(RR=0.360;χ2=4.95,P=0.026).放疗后切缘阳性患者10年局部复发率从70.1%降至20.7%(χ2=4.22,P=0.040);而切缘阴性患者从19.8%降至10.4%(χ2=0.90,P=0.344).结论 根治性切除为侵袭性纤维瘤病的首选治疗,术后放疗可以降低切缘阳性患者的局部复发率,但对切缘阴性患者的意义尚需大样本临床研究证实.

Abstract：

Objective Aggressive fibromatosis is a rare kind of soft tissue tumor and was evaluated by few large studies. This study was to evaluate the clinical characteristics and identify the prognostic factors of this disease. Methods One hundred and forty-two patients with aggressive fibromatosis treated from January 1983 to August 2009 in Tianjin Medical University Cancer Hospital were retrospectively reviewed.The prognostic value of clinical and treatment factors was analyzed. Univariate analysis was performed with Log-rank test and Multivariate analysis was performed with Cox regression model. Results The follow-up rate is 93.7% and the median follow up time was 54 months (range,6 -208 months). Sixty-three patients had a minimum follow up time of 5 years and 6 patients had a minimum follow up time of 10 years. The male/female ratio was 1/1.84. The disease was most popular in women aged from 18 to 35 years old. The disease frequently occurred in the trunk (55.6%) and extremity (31.7%). All patients received surgery,and 46 received radiotherapy. The 5-year and 10-year local recurrence rates were 24. 4% and 31.1%,respectively. The 5-year and 10-year overall survival rates were both 99. 3%. Univariate analysis revealed that factors correlated with local recurrence were tumor size ( χ2 = 4. 37, P = 0. 037 ) and margin status (χ2 = 12. 36,P =0. 002). Multivariate analysis revealed that margin status was an independent risk factor (RR = 2. 219; χ2 = 9. 47,P = 0. 002) and radiotherapy was an independent protective factor ( RR = 0. 360;χ2 = 4. 95, P = 0. 026 ) for disease recurrence. When radiotherapy was delivered, the 10-year local recurrence rate decreased from 70. 1% to 20. 7% in patients with positive margin ( χ2 = 4. 22, P = 0. 040 )and decreased from 19.8% to 10.4% (χ2= 0.90, P= 0.344) in patients with negative margin.Conclusions Radical resection is the mainstay of treatment for aggressive fibromatosis. Postoperative radiotherapy can reduce the recurrent rate for patients with positive margin. In patients with negative margin,the role of radiotherapy should to be further evaluated in large clinical trials.     

Implantation of 125Ⅰ seeds for recurrence cervical node of head and neck tumor after external beam radiotherapy

Objective To summarize the efficacy and the feasibility of 125I seed implantation for recurrence cervical lymph node of head and neck tumor after radiotherapy or radiotherapy plus neck dissection. Methods Thirty-six patients with the recurrence cervical lymphnode of head and neck tumor after radiotherapy (17 patients) or radiotherapy plus neck dissection (19 patients) were treated with 125I seed implantation guided by ultrasound or CT under local anesthesia. The median number of seeds was 27( range from 3 to 78 ). Postoperative quality evaluation were routinely obtained for all patients. The actuarial D90 ranged from 90-160 Gy (median, 130 Gy). Results The follow-up rate was 100%. The number of the patients who were followed up over 1-and 2-year were 11 and 3. The overall response rate was 81%. The 1-and 2-year over local control rates, over survival rates were 69% and 35%, 50% and 22%, respectively.The 1-and 2-year local control rates in patients with recurrence node after radiotherapy plus neck dissection were 72% and 54%, while those were 67% and 50% in patients with recurrence node after radiotherapy,respectively (χ2=00,P=0.965). The 1-and 2-year survival rates in two groups were 48%, 13% , and 51%, 39%, respectively (χ2=0.17, P=0.676). Conclusions 125I seed implantation is a safe,minimal invasive with low morbidity and high efficacy salvage treatment method for cervical lymph node recurrence of head and neck tumor after radiotherapy with or without neck dissection.     

74例隆突型皮肤纤维肉瘤的治疗与预后分析

Objective： To study treatment and prognostic factor in 74 patients with dermatofibrosarcoma protuberans. Methods： From August 1990 to November 1999, 74 patients with dermatofibrosarcoma protuberans （DFSP） confirmed by pathology were treated in Cancer Hospital of Sun Yat-sen University. 72 cases were given wide excision and 2 cases were given local excision. All of 74 cases, 52 cases had surgical resection alone, and 22 cases had surgical resection combined with radiotherapy. Total dose of radiotherapy was 50-70 Gy. Results： The rate of recurrence was 36.1% for all patients. The 5-year recurrence-free survival （RFS） rate was 66%. The 5-year recurrence-free survival rates for resection alone and combined with radiotherapy were 58% and 90%, respectively （P=-0.0187）. The 5-year recurrence-free survival rates for positive microscopic margins and negative microscopic margins were 57% and 75%, respectively （P=0.0468）. Conclusion： Post-operation radiotherapy is an effective treatment to decrease the recurrence rate for the patients with positive microscope, or the patients without suitable surgical treatment.     

Prognostic Factors for Patients with FIGO Stage-IB Cervical Squamous Cell Carcinoma：Does the Tumor Size（≤4 cm or〉4 cm）Really Matter？

OBJECTIVE To investigate the factors that can accurately predict the prognosis for patients with FIGO stage-IB cervical squamous cell carcinoma treated with radical surgery. METHODS A retrospective analysis of clinical data from 174 cases of FIGO Stage-IB cervical squamous cell carcinoma treated in our institute was conducted. RESULTS The 5-year overal disease-free survival of the patients was 79.4%and the recurrence rate was 16.7%.Seventy-five percent of the 60 patients with a tumor>4 cm and 28.1%of the 114 patients with a tumor≤4 cm received preoperative radiotherapy,resuting in a significant difference be- tween the two groups(P<0.001).The 5-year disease-free survival rate for the groups with a tumor≤4 cm without and with preoperative radiotherapy, and with a tumor>4 cm without and with preoperative radiation therapy were 80.5%,85.2%,69.3%and 77.1%,respectively.There was no significant dif- ference between any of the groups(P>0.05).A univariate analysis showed that pelvic node metastasis,a positive parametrial surgical margin and post- operative adjuvant therapy were al significantly correlated with the 5-year disease-free survivals(P<0.05).Multivariate analysis revealed that pelvic node metastasis(P=0.004)and a positive parametrial surgical margin(P= 0.040)were independent factors that influenced the prognosis.The 5-year disease-free survivals for the cases with a tumor≤4 cm and>4 cm were 57.4%and 44.7%respectively in the high-risk group(patients with pelvic lymphatic metastasis and/or positive parametrial surgical margin)(P=0.575) and the recurrence ratio was 7/18 and 6/14 for the cases of the two tumor sizes in the same risk group.There was no significant difference between the two groups(P=0.821).The 5-year disease-free survivals for the cases with a tumor≤4 cm and>4 cm were 86.5%and 82.9%respectively in the low-risk group(patients without pelvic lymph-node metastasis and/or positive para- metrial surgical margin),respectively(P>0.05)and the recurrence ratio was 9/95 and 7/47 for the cases of the two tumor sizes in the same risk group. There was no significant difference between the two groups(P>0.05). CONCLUSIONS For FIGO Stage-IB cervical squamous cel carcinoma patients with radical surgery as the major means of treatment,the features of pelvic lymph-node metastasis and a positive parametrial surgical margin are independent factors that influence the prognosis.The tumor size can not be used as a criterion for predicting the prognosis.     

The recurrence and survival of oral squamous cell carcinoma: a report of 275 cases

Oral squamous cell carcinoma （OSCC） is a common malignant tumor of the head and neck, and recurrence is an important prognostic factor in patients with OSCC. We explored the factors associated with recurrence of OSCC and analyzed the survival of patients after recurrence. Clinicopathologic and follow-up data of 275 patients with OSCC treated by surgery in the Cancer Institute and Hospital of Tianjin Medical University between 2002 and 2006 were analyzed. Recurrence factors were analyzed with Chi- square or Fisher＇s exact test and multivariate analysis. The prognosis of patients after recurrence was analyzed with the Kaplan-Meier method and log-rank test. The recurrence rate was 32.7%. The recurrence time ranged from 2 to 96 months, with a median of 14 months. Univariate analysis showed that T stage, degree of differentiation, pN stage, flap application, resection margin, and lymphovascular invasion were factors of recurrence （P 〈 0.05）. Multivariate analysis showed that T stage, degree of differentiation, and pN stage were independent factors of recurrence （P 〈 0.001）. The differences in gender, age, tumor site, region of lymph node metastasis, and perineural invasion between the recurrence and non-recurrence groups were not significant （P 〉 0.05）. Kaplan-Meier and log-rank tests showed that the 2- and 5-year survival rates were significantly lower in the recurrence group than in non-recurrence group （67.6% vs. 88.0%, 31.8% vs. 79.9%, P 〈 0.001）. Therefore, to improve prognosis, we recommend extended local excision, flap, radical neck dissection, and adjuvant chemoradiotherapy for patients more likely to undergo recurrence.     

High-risk endometrial cancer may be benefit from adjuvant radiotherapy plus chemotherapy

Objective:To present patterns of practice and outcomes in the adjuvant treatment of intermediate-and high-risk endometrial cancer.Methods:Retrospective data on 224 women with intermediate-risk and high-risk endometrial cancer from 1999 to 2006 were reviewed.All patients underwent surgical staging.Patterns of adjuvant treatment,consisting of pelvic radiotherapy,chemotherapy,and radiotherapy plus chemotherapy,were assessed.The 3and 5-year disease-specific survival(DSS) rates were calculated using the Kaplan-Meier method.Results:The difference in 5-year DSS rate was statistically significant between adjuvant group and non-adjuvant group(80.65% vs.63.80%,P=0.040).In 110 high-risk patients who underwent adjuvant treatment,both 5-year DSS rate and recurrent rate were significantly different in combined radiotherapy and chemotherapy group compared with radiotherapy alone and chemotherapy alone groups(DSS rate,P=0.049;recurrent rate,P=0.047).In 83 intermediate-risk women who underwent adjuvant treatment,there was no significant difference in 5-year DSS rate and recurrence rate among the combined radiotherapy and chemotherapy,radiotherapy alone and chemotherapy alone groups(DSS rate,P=0.776;recurrent rate,P=0.937).Conclusions:Adjuvant radiotherapy plus chemotherapy is associated with a higher 5-year DSS rate and lower recurrence rate compared with radiotherapy alone and chemotherapy alone in high-risk endometrial cancer patients.Patients with intermediate-risk endometrial cancer may be not likely to benefit from adjuvant combined radiotherapy and chemotherapy.     

Analysis of recurrence for locally advanced gastric or gastroesophageal cancer patients after receiving curative gastrectomy ( ＞ D1 ) and its indication for adjuvant chemoradiotherapy

Objective The benefit of adjuvant chemoradiotherapy remains controversial for gastric cancer patients treated with more than D1 dissection. This retrospective analysis is to distinguish the first site of recurrence in patients treated with curative resection and more than D1 dissection and to find any feasible adjuvant concurrent chemoradiotherapy recommendation for them. Methods All patients treated between January 2002 and December 2004 who met the following criteria were analyzed: primary gastric or gastroesophageal cancer, underwent curative gastrectomy ( UICC R0 ) and more than D1 lymphadenectomy,pathologically staged as T3-4N0-1 M0, or any Tx N2-3M0. There were 297 patients analyzed and 19.5%,52. 2%, 17. 8% , 10. 4% of patients had stage Ⅱ ( T3 N0 M0, T1 N2 M0 ), Ⅲa, Ⅲb and Ⅳ ( M0 ) diseases,respectively. 76. 1% of patients received adjuvant chemotherapy, while Only 2 patients underwent adjuvant radiotherapy. Failure patterns and the prognostic factors for locoregional recurrence were analyzed. Results The median follow-up time was 61 months and the follow-up rate was 92. 3%. 145 patients developed recurrence with a median recurrent time of 26 months. Locoregional recurrence was observed in 82 patients and distant metastasis in 79 patients. Gastric stump, anastomosis, intra-abdominal lymph nodes were the most common sites of locoregional recurrence. Liver and lung were the most frequent sites of distant metastasis. Prognostic variables for locoregional recurrence were identified after univariate analysis,including pathologic type ( χ2 = 11.50, P = 0. 009 ), total number of dissected lymph nodes ( χ2 = 6. 65,P =0. 010), the number of positive lymph node ( χ2 =5. 80,P =0. 016), lymph node capsular invasion ( χ2 =pathologic type, total number of dissected lymph nodes, lymph node capsular invation, AJCC TNM stage and Borrmann type were independent prognostic factors for locoregional recurrence ( χ2 = 6. 77,19. 33,17. 84 and 6. 02,P =0. 009,0. 000,0. 000 and 0. 014). Conclusions Locoregional recurrence remains the main cause of failure for locally advanced gastric or gastroesophageal cancer patients even though the patients have had more than D1 lymphadenectomy. The role of adjuvant concurrent hemoradiotheray for those patients is warranted.     

早期鼻腔NK/T细胞淋巴瘤治疗方法和预后分析

Objective To investigate the prognosis of patients with nasal NK/T cell lymphoma receiving different treatment modalities. Methods From 1990 to 2004, 85 patients with stage ⅠE and ⅡE primary nasal NK/T cell lymphomas were retrospectively studied. Twenty patients received chemotherapy of CHOP regimen alone, 11 patients received radiotherapy only, 6 patients received radiotherapy followed by more than 2 cycles of chemotherapy, and 48 patients received more than 2 cycles of chemotherapy followed by radiotherapy. Survival analysis was performed by the Kaplan-Meier method, the difference between groups was evaluated by the Log-rank test, and the Cox regression model was used for multivariate analysis. Results The 5-year overall survival rate (OS) was 40%. The 5-year OS was 57% and 28% for limited stage ⅠE and extended stage ⅠE(X2 =8. 87, P =0. 003), and 23% for stage ⅡE, which was similar to extended stage ⅠE (X2 =0. 19, P-0. 664). The 5-year OS was 13%, 54% and 47% for chemotherapy alone, radiotherapy followed with or without chemotherapy, and chemotherapy followed by radiotherapy, respectively. The last two groups had better OS than chemotherapy alone (P = 0. 030 and 0.049). The 5-year OS was 58% and 12% for patients achieving complete response (CR) and uncomplete response (X2 = 30.68, P = 0. 000).The CR rate was 56% and 86% for radiotherapy of ≤50 Gy and >50 Gy (X2 =6.11, P=0. 013). The corresponding 5-year relapse-free survival rate was 89% and 84% (X2 =0.36, P=0.551). Of 68 patients receiving initial chemotherapy, the CR rate of those who received ≤2, 3-4 and ≥5 cycles was 0, 20%and 3 3 % , respectively (X2 = 7.65 , P = 0. 022) . For 5 0 patients who received ≥ 3 cycles of initial chemotherapy and 17 patients who received initial radiotherapy of ≥40 Gy, the CR rate was 28% and 88%(χ2= 18. 75, P= 0. 000). In patients with pathological nodular and ulcer type, the CR rates with radiotherapy were higher than with chemotherapy (100%: 38%, χ2 = 7.92, P = 0. 005; and 100%: 11%,χ2 = 14.40, P = 0. 000). Multivariate analysis showed that stage and recent effect were the independent prognostic factors. Conclusions The initial radiotherapy with 50 Gy is appropriate for early stage nasal NK/T cell lymphomas. Combined chemotherapy could be used for extended stage ⅠE and ⅡE, but the outcome of CHOP regimen is poor.     

早期鼻腔NK/T细胞淋巴瘤治疗方法和预后分析

Objective To investigate the prognosis of patients with nasal NK/T cell lymphoma receiving different treatment modalities. Methods From 1990 to 2004, 85 patients with stage ⅠE and ⅡE primary nasal NK/T cell lymphomas were retrospectively studied. Twenty patients received chemotherapy of CHOP regimen alone, 11 patients received radiotherapy only, 6 patients received radiotherapy followed by more than 2 cycles of chemotherapy, and 48 patients received more than 2 cycles of chemotherapy followed by radiotherapy. Survival analysis was performed by the Kaplan-Meier method, the difference between groups was evaluated by the Log-rank test, and the Cox regression model was used for multivariate analysis. Results The 5-year overall survival rate (OS) was 40%. The 5-year OS was 57% and 28% for limited stage ⅠE and extended stage ⅠE(X2 =8. 87, P =0. 003), and 23% for stage ⅡE, which was similar to extended stage ⅠE (X2 =0. 19, P-0. 664). The 5-year OS was 13%, 54% and 47% for chemotherapy alone, radiotherapy followed with or without chemotherapy, and chemotherapy followed by radiotherapy, respectively. The last two groups had better OS than chemotherapy alone (P = 0. 030 and 0.049). The 5-year OS was 58% and 12% for patients achieving complete response (CR) and uncomplete response (X2 = 30.68, P = 0. 000).The CR rate was 56% and 86% for radiotherapy of ≤50 Gy and >50 Gy (X2 =6.11, P=0. 013). The corresponding 5-year relapse-free survival rate was 89% and 84% (X2 =0.36, P=0.551). Of 68 patients receiving initial chemotherapy, the CR rate of those who received ≤2, 3-4 and ≥5 cycles was 0, 20%and 3 3 % , respectively (X2 = 7.65 , P = 0. 022) . For 5 0 patients who received ≥ 3 cycles of initial chemotherapy and 17 patients who received initial radiotherapy of ≥40 Gy, the CR rate was 28% and 88%(χ2= 18. 75, P= 0. 000). In patients with pathological nodular and ulcer type, the CR rates with radiotherapy were higher than with chemotherapy (100%: 38%, χ2 = 7.92, P = 0. 005; and 100%: 11%,χ2 = 14.40, P = 0. 000). Multivariate analysis showed that stage and recent effect were the independent prognostic factors. Conclusions The initial radiotherapy with 50 Gy is appropriate for early stage nasal NK/T cell lymphomas. Combined chemotherapy could be used for extended stage ⅠE and ⅡE, but the outcome of CHOP regimen is poor.     

The 10-Year Local Recurrence and Partial Breast Radiotherapy for Early Breast Cancer Tmarted by Conservative Surgery

To study the local recurrence and the role of whole breast radiotherapy for early breast cancer treated by conservative surgery. METHODS From April 1990 to December 2000, 49 patients with early primary breast cancer were treated by conservative surgery in our hospital. The cases were comprised of Stage 0, 1; Stage Ⅰ, 31; and Stage Ⅱa, 17. Forty cases underwent quadrantectomy plus axillary lymph node dissection, and the other 9 cases had lumpectomy alone. Irradiation, which was received by 39 patients, was administered by using low tangential half fields with 6 MV X-ray to decrease the pulmonary irradiative volume. The dose to the whole breast was 45 Gy/22 ～23f/4.5W, then a 15 Gy boost dose was delivered to the tumor bed by an electron beam. The other patients underwent an irradiated regional field according to postoperative pathology. RESULTS All patients were followed-up for 10 years or more. The 10- year local recurrence rates, distant metastasis rates and survival rates were 6.1%, 4.1% and 98.0% respectively. All of the 3 patients who had a local recurrence had infiltrative carcinomas and negative lymph nodes. The 10-year local recurrence rate was higher （2.6% vs. 20.0%） with nonpostoperative whole breast radootherapy, but the statistical difference was not marked because of the low number of cases. All of the recurrent lesions localized within 3 cm of the primary lesion. CONCLUSION Original recurrence of the tumor was the main type of local recurrence. Radiotherapy after conservative surgery is very essential. After conservative surgery it is feasible that irradiation can be delivered alone to the neighboring region of the tumor bed. Partial breast radiotherapy can substitute for whole breast radiotherapy.     

Familial plantar fibromatosis

A family in which three siblings developed plantar fibromatosis is described. This occurrence confirms the importance of heredity in the etiology of plantar fibromatosis.     

腹外型韧带样型纤维瘤病的临床、病理及影像学表现

目的 探讨腹外型韧带样型纤维瘤的影像学表现.方法 回顾性分析经病理证实的15例腹外型韧带样型纤维瘤病患者的临床及影像资料.其中MRI检查11例,增强10例;CT检查5例,增强4例.结果 13例位于四肢、胸壁、颈部及臀部软组织内,2例位于骨.形态上,13例呈类圆形或分叶状肿块,2例呈不规则斑片状影,10例边界不清或部分不清,5例边界尚清.肿块直径较大,平均直径约9.7 cm.CT显示,5例病灶均呈略低于肌肉的低密度灶,其中1例伴囊变、坏死;MRI显示,11例病灶与肌肉比较均呈不均匀等或稍长T1、稍长T2信号,内均伴条片状长T1、短T2信号,其中1例伴囊变、坏死.MRI增强10例呈不均匀明显强化;CT增强4例,3例轻度欠均匀强化,1例不均匀明显强化,2例静脉期呈渐进性强化.病理镜下瘤细胞呈长梭形,无明显异型性,核分裂少见,呈侵袭性生长,浸润周围的横纹肌及脂肪组织,伴胶原纤维增生及程度不等的透明变性、黏液变性.结论 腹部外型韧带样型纤维瘤的影像学表现具有一定的特征性,且MRI对手术切除范围的判断具有优势.     

Hormonal manipulation with toremifene in sporadic desmoid-type fibromatosis

IntroductionMany patients affected by desmoid-type fibromatosis (DF) are treated with a course of hormonal therapy as front line. So far, tamoxifene has been the preferred choice. Toremifene is an anti-oestrogen agent, but possible further mechanisms of action in desmoids are related to its role in regulation of transforming growth factor-beta and β-catenin pathways.Material and methodsWe retrospectively reviewed all patients treated with toremifene between 2005 and 2012 at a reference institution. Indication to toremifene was radiologically progressive disease and/or symptomatic deterioration. Progression-free survival (PFS), clinical benefit (CB) and safety profile were analysed.ResultsForty-four patients were treated with toremifene 180 mg daily, 20 for radiological progression, 16 for pain and 8 for both. In 28 patients, toremifene was offered as front-line therapy, while in 11 after tamoxifen failure. PFS was 89.6% at 2 years. According to Response Evaluation Criteria in Solid Tumours, partial response, stable disease and disease progression were observed in 25%, 65% and 10% of the patients, respectively. Symptomatic relief was obtained in 75% of patients. Median time to response was 4 months. Overall CB was 86%. Adverse events G≥2 according to National Cancer Institute Common Toxicity Criteria were recorded in ten patients.DiscussionPresent series provides evidence to make toremifene an option in patients with DF, even after failure on different hormonal agents. A prospective trial is ongoing to confirm these results.     

腹膜后纤维瘤病1例报告并文献复习

目的:探讨腹膜后纤维瘤病临床特点。方法:报道1例腹膜后纤维瘤病侵袭输尿管致严重血尿的诊治,并复习有关文献。结果:术中发现腹膜后右侧输尿管末端有一直径6cm实性肿瘤,固定于盆壁,与髂外动静脉致密粘连,包裹右侧输尿管末端,上段输尿管扩张。行肿瘤大部切除术+输尿管再植术,切除肿瘤大部分为肌瘤样组织,中间坏死形成囊腔,与输尿管相通;术中快速冰冻片报告梭形细胞肿瘤,良恶性待定;术后病理报告为腹膜后纤维瘤病,免疫组化:CD34（-）、CD117（-）、Dog-1（-）、SMAS-100（-）、Vimentin（+）、Desmin（-）、NSE（-）。结论:腹膜后纤维瘤病是一种十分罕见的软组织肿瘤,可以因发生位置不同而以不同临床症状就诊,提高对这一肿瘤的认识可以减少误诊。     

Development and rapid growth of a desmoid tumor in the surgical corridor after suboccipital craniotomy for recurrent low-grade astrocytoma

Desmoid tumors are histologically benign but locally invasive tumors that can occur in the head and neck. We present the rare case of a desmoid tumor that occurred in the surgical corridor after suboccipital craniotomy for recurrent low-grade astrocytoma. A 30-year-old woman underwent a repeat suboccipital craniotomy for recurrent low-grade astrocytoma. A gross total resection was achieved. Nine months later, a firm, palpable mass was noted near the surgical scar. No new neurologic deficits were noted on exam. A magnetic resonance imaging scan demonstrated a homogeneously enhancing lesion superficial to the dural graft. Surgical resection of the lesion was performed through the original midline incision. The mass was resected en bloc. Histology demonstrated clear surgical margins and a tumor of low cellularity consistent with a desmoid tumor. Desmoid tumors should be considered in the differential diagnosis for superficial masses occurring in the surgical bed after posterior cervical surgery.     

Wnt targets genes are not differentially expressed in desmoid tumors bearing different activating β-catenin mutations

IntroductionSporadic desmoid-type fibromatosis (DTF) is a rare soft tissue tumor of mesenchymal origin. It is characterized by local invasive growth and unpredictable growth behavior. Three distinct mutations involving the CTNNB1 (β-catenin) gene have been identified in the vast majority of DTF tumors, which cause activation of the Wnt signaling pathway and impact prognosis. This study examines whether the different CTNNB1 mutants (T41A, S45F) occurring in DTF tumors differentially affect Wnt signaling activity, which might explain the different disease course between DTF patients harboring different CTNNB1 mutations.Materials and methodsReal-time polymerase chain reaction (RT-PCR) on 61 formalin fixed paraffin embedded DTF samples with known CTNNB1 status was used to measure the relative mRNA expression level of Wnt target genes AXIN2, DKK1 and CCND1. Additionally, publicly available mRNA expression data retrieved from the Gene Expression Omnibus of 128 DTF samples were used for an unsupervised cluster analyses based on the expression of a selection of Wnt targets.ResultsNo statistically significant difference in relative expression levels of Wnt target genes AXIN2, DKK1 and CCND1 was identified between either CTNNB1 wild-type, S45F or T41A mutated DTF samples. Moreover, the hierarchical cluster analyses using selected Wnt targets did not discriminate between different CTNNB1 mutation types.ConclusionsNo differences in the expression levels of Wnt target genes were observed between the different CTNNB1 mutation types in DTF tumors. Further studies are needed to decipher the mechanism accounting for the diverse disease courses between DTF patients with different CTNNB1 variants.