Malignant fibrous histiocytoma of bone. The experience at the Rizzoli Institute: report of 90 cases

Ninety cases of malignant fibrous histiocytoma (MFH) of bone are reported. An analysis is presented with regard to sex, age, site, symptoms, radiography, macroscopic, and microscopic features. The staging was assessed according to the following classification: I B: 3 cases; II A: 11 cases; II B: 66 cases; III: 10 cases. Among nonmetastatic patients, 68 had surgical treatment (adequate, 46; inadequate, 22); 8 had radiotherapy, and 3 refused any treatment. Among surgically treated patients, 20 had adjuvant chemotherapy, also. Follow-up information was obtained in all cases. Overall survival rates were 34% at 5 years, and 28% at 10 years. Surgery alone did not appear to be successful (5-year survival, 28%). Adjuvant chemotherapy improved the survival rate only in patients who underwent adequate surgery (5-year survival, 57%). The recurrence rate after surgical treatment was high (31%) with significant differences after inadequate (64%), wide (19%), or radical (6.5%) surgical treatment. Adjuvant chemotherapy was ineffective in preventing local recurrence. Radiation therapy effected a clinical cure in three cases; the authors believe that radiation therapy has to be used only in inoperable tumors.     

Re-excision after unplanned excision of soft tissue sarcomas: Long-term results

BackgroundUnplanned excisions of soft tissue sarcomas are still not infrequent events with patients presenting to referral Centers after having received an inadequate surgical treatment. In literature, both the wait-and-see policy and the “aggressive” management with a re-excision have been advocated. The purposes of this study were to analyze the incidence of detectable residual tumor in surgical specimens following a re-excision and to verify the long-term results of patients treated with a re-excision after previous unplanned excision.MethodsWe retrospectively evaluated 131 patients affected by localized soft tissue sarcoma (95 high grade; 36 low grade) of the limbs or the superficial trunk treated at our Institution, from 2000 to 2013, with a re-excision after a previous unplanned inadequate excision. Site, size, depth, histotype, grade, adjuvant therapies, time from unplanned surgery to re-excision were recorded and evaluated in association with clinical results. We specifically evaluated the disease-specific survival, local recurrence free survival, distant metastases free survival and the event free survival.ResultsMean follow-up for living patients was 10.9 years (median 11.2 years), with a follow-up ranging from 14 to 227 months. 34% of patients underwent a re-excision within the first 2 months after unplanned surgery, while 66% of patients at more than 2 months. Residual detectable tumor cells were found on histological examination in 54% of re-excisions. A wide margin was obtained in 123 cases, a persisting positive margin in 8 patients.Disease-specific survival was 93.5%, 91.6% and 89.6% at 5, 10 and 15 years for whole series and 90.9%, 88.2% and 85.7% for high grade tumors. Event-free survival in patients affected by high grade tumors rated 75.0% at 5 years, 72.4% at 10 years and 72.4% at 15 years. Local recurrence free survival in high grade tumors was 87.6%, 86.2% and 86.2% at 5, 10 and 15 years.The grade of the tumor (high grade) and the initial dimension of the tumor (≥5 cm) were associated with worst survival. High grade tumor impacted negatively also on local recurrence free survival and event free survival. Instead, the initial size of the tumor significantly affected the event free survival but not the local recurrence free survival. No significant differences of outcome were found analyzing tumor depth, time interval to re-excision, presence of residual tumor or margins.ConclusionBased on our results and literature findings, we believe that surgeons should offer a re-excision procedure in those patients presenting with an inadequate excision of a high grade soft-tissue tumor, in particular with tumors larger than 5 cm before excision. Indeed, if an adequate second treatment is performed with surgery ± radiotherapy, the long-term results of patients receiving a re-excision after unplanned excision of a high grade soft tissue sarcoma seem to be comparable to the results generally reported for wide primary excisions. More debatable is whether to perform a re-excision or not in patients with low-grade tumors. Perhaps, in this latter group a wait and see policy can eventually be offered as well as in high grade tumors when a re-excision procedure could involve major surgery or significantly affect postoperative function.     

Phyllodes tumor of the breast: stromal overgrowth and histological classification are useful prognosis-predictive factors for local recurrence in patients with a positive surgical margin

BACKGROUND: The local recurrence rate of phyllodes tumors is high and ensuring a sufficient surgical margin is considered important for local control. However, the preoperative diagnosis rate of phyllodes tumors is low and we often encounter cases in which a sufficient surgical margin is not achieved, since in routine medical practice the lesion may not be diagnosed as phyllodes tumor until postoperative biopsy of a mammary mass. Furthermore, there are no established therapeutic guidelines for surgical stump-positive phyllodes tumors. We reviewed the outcomes of excision of phyllodes tumors to investigate factors involved in local recurrence and to determine the indication for re-excision in stump-positive cases. METHODS: The subjects were 45 patients treated for phyllodes tumors at our institution from January 1980 to July 2005. Age, tumor size, surgical method, stromal cellular atypia, mitotic activity, stromal overgrowth, histological classification and surgical stump status were analyzed. RESULTS: Median age was 45 years old (range 28-75) and tumor size was 1-17 cm (median 3.5 cm). Pathologic diagnoses were benign, borderline and malignant in 31, five and nine cases, respectively, and the surgical stump was negative in 27 lesions and positive in 15. Median follow-up was 101 months (range 1-273), with local recurrence in six cases and distant metastasis in one. The local recurrence-free rate was 88, 88 and 84% and the disease-free rate was 85, 85 and 81% after 5, 10 and 15 years, respectively. Overall 10-year survival was 97%. In univariate analysis, a positive surgical margin, stromal overgrowth and histological classification were predictive factors for local recurrence after breast-conservation surgery (P = 0.0034, 0.0003, 0.026). A positive surgical stump was the only independent predictor of local recurrence in multivariate analysis (RR 0.086; 95% CI 0.01-0.743, P = 0.012). Stromal overgrowth was a predictive factor for local recurrence in cases with a positive surgical margin (P = 0.0139). CONCLUSION: Wide excision is the preferred therapy for phyllodes tumor and preoperative diagnosis is important for good local control. Re-excision is recommended in cases with a positive surgical margin and stromal overgrowth and malignancy.     

Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities.

AIMS: Dermatofibrosarcoma protuberans is a rare condition which is frequently misdiagnosed at presentation, resulting in a high incidence of local recurrence due to inadequate resection. The archives of the Department of Orthopaedics at the University of Florida were analysed to investigate the natural history and results of treatment for this tumour. METHODS: Between 1975 and 1996, 35 cases of DFSP were treated at the University of Florida. Of these, one was treated primarily, five were treated for local recurrence, 17 had tumour bed excisions following inadequate primary excisions elsewhere and 12 had tumour bed excisions following inadequate resection of local recurrences elsewhere. The data were analysed to assess the impact of age, gender, duration of symptoms, tumour site and size, surgical margin, number of operations and adjuvant treatments on survival and local recurrence outcomes. RESULTS: Complete follow-up was available for 34 patients. Mean follow-up was 58 months (range 12-144 months). Thirty-three patients remain alive and disease-free. One patient died of unrelated causes. The margins obtained were wide in 28 patients, marginal in six and intralesional in one. Of the seven patients with inadequate surgical margins, four received adjuvant radiation therapy and remain disease-free. No patient with an adequate margin developed a local recurrence, but there were three local recurrences in the patients with an inadequate margin who did not receive adjuvant radiation therapy (local recurrence rate: 8%). No patient developed lymphatic or distant metastasis. Local recurrences were more likely to be classified Stage IB (17/17) than primary tumours (1/18) (P<0.001). Local recurrence was more likely where the surgical margin was less than 2.5 cm from the lesion. CONCLUSIONS: Dermatofibrosarcoma protuberans is a low-grade tumour that has a high potential for local recurrence unless it can be completely excised. The overall rate of local recurrence in referred patients in this series was 20/35 cases (57%). All occurred after inadequate margins at previous surgery in other institutions. Revision surgery in these patients showed a local recurrence rate of 8%. To avoid extensive surgery for recurrences, initial treatment should be by wide excision incorporating the underlying deep fascia and a cuff of 2.5-3 cm of normal skin tissue. Radiation therapy provides a useful adjunct where adequate margins cannot be obtained.     

Salivary gland tumors of the oral cavity

Between 1961 and 1985, 62 patients with malignant salivary gland tumors of the oral cavity underwent surgery with curative intent at the University of California, Los Angeles (UCLA) Medical center. All patients had a minimum follow-up of 2 years. Fifty of 62 (81%) patients presented with T1-2 primary tumors. The tumors arose from the palate in 41/62 (66%) patients. The most common histologic type was adenoid cystic carcinoma comprising 34 of 62 (55%) cases. Radical resection was performed in 46 cases and wide local excision in 16 patients. Postoperative radiation therapy was used in 24 cases due to advanced stage and/or positive surgical margins. Results of treatment were analyzed by stage of disease, modes of treatment, histology, and surgical extent. Local control of small lesions reached 100% at 10 years with either radical resection alone or local excision. With residual tumor at the surgical margins, the incidence of local recurrence was 4/14 (29%) for those who received adjuvant radiation therapy and 5/10 (50%) for those who did not. The vast majority of mucoepidermoid carcinomas were early stage and low grade. These lesions had an excellent prognosis with a control rate of 100%. In contrast, there was a 29% (10/34) failure rate for adenoid cystic carcinoma. The 5-, 10-, and 15-year actuarial survivals for the whole group were 94%, 84%, and 73%, respectively. Our results indicate that for early stage disease, wide local excision may offer patients the chance to avoid the cosmetically and functionally debilitating effects of radical surgery without compromising treatment outcome. Adjuvant radiation therapy appears to reduce the local recurrence for those with residual tumor at the surgical margins.     

Extraskeletal myxoid chondrosarcoma: a Multi-Institutional Study of 42 Cases in Japan

BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant neoplasm. Despite a consensus for the distinct clinicopathologic entity of EMC, its clinical features remain controversial. In addition, most studies have contained a small number of patients who underwent definitive surgical treatment. METHODS: Forty-two cases of EMC, which had been identified from files of eight affiliated hospitals and confirmed for histologic diagnosis at the Pathology Center, were analyzed for histologic grade, demographics, treatments, outcomes, and prognostic factors. The average follow-up period was 7.4 years. RESULTS: Included in the study were 20 men and 22 women with a mean age at diagnosis of 52.1 years. The tumors were located mainly in the lower extremities (69%). Thirty-three tumors (79%) were classified as Grade 1 and nine as Grade 2 according to the modified French System. Overall survival was 100% at 5 years and 88% at 10 years. Disease-free survival was 45% at 5 years and 36% at 10 years. Inadequate initial surgery was defined as a significant risk factor for local recurrence by univariate analysis of all 42 patients but not by the analysis of those 30 patients who had undergone wide tumor excision or amputation. Wide excision led to the recurrence rate of 14%. CONCLUSIONS: These findings supported the role of wide excision in the local control of EMC, irrespective of the previous excision procedure or recurrence. The protracted clinical course of the tumors and the presence of patients who had distant metastasis develop after definitive surgery of the primary tumor represented EMC as intermediate malignancy.     

Desmoplastic neurotropic melanoma: a clinicopathologic analysis of 128 cases

BACKGROUND: Several studies have suggested that desmoplastic neurotropic melanoma (DNM) is associated with higher local recurrence rates than other types of melanoma. The authors investigated the local recurrence rates for patients with DNM after surgery alone or surgery followed by radiotherapy (RT). METHODS: One hundred twenty-eight patients with DNM were treated at the Sydney Melanoma Unit and the Sydney Cancer Center from 1996 to 2007. All patients underwent local excision, 27 patients also received RT. For both groups, clinical and pathologic features, treatment details, and local recurrence data were analyzed. RESULTS: The median age at diagnosis was 65.5 years. The ratio of men to women was 2.7:1. The head and neck was the most common location (51%). The median Breslow thickness was 4 mm, and 99% of patients had Clark Level IV or V primary tumors. Patients who received adjuvant RT had thicker tumors (P = .003), deeper Clark level invasion (P < .001), and narrower excision margins (P < .001). There were 8 local recurrences, including 6 (6%) in the surgery only group and 2 (7%) in the adjuvant RT group. A positive margin (P < .001) and head and neck location (P = .03) were significant predictors of local recurrence. CONCLUSIONS: The local recurrence rate in this series was lower than the rates reported in historic control groups and in the authors' previous temporal cohort. The results indicated that clear surgical margins are of paramount importance in minimizing local recurrence; when margins are compromised, the addition of RT may reduce local recurrence rates compared with historic controls. A prospective randomized trial is needed to quantify the risk reduction with adjuvant RT.     

Primary malignant retroperitoneal tumors: analysis of a single institutional experience.

AIMS: In order to achieve complete resection in the surgical management of retroperitoneal tumors, it is crucial to know the tumor's anatomical location relative to neighboring organs. METHODS: Forty-nine patients with primary malignant retroperitoneal tumors were divided by tumor location into two groups [upper abdomen (group 1) or lower abdomen (group 2)], and clinicopathological features, tumor recurrence, and patient survival were assessed. RESULTS: No significant differences in preoperative clinical characteristics existed between two groups, and liposarcoma was the most frequently observed tumor type. The difference in the rates of complete resection between the two groups was not statistically significant (75.9% for group 1 and 85% for group 2). En-bloc combined resection was performed in 52% and 30% of patients in groups 1 and 2, respectively. The local recurrence rate in group 2 (31.3%) was higher than that in group 1 (9.5%), despite the fact that the differences in rates of complete resection and distant recurrence rates (14.3% in group 1 and 12.5% in group 2) between the two groups were not statistically significant. The overall 5-year survival rates were 67.9% for group 1 and 43.2% for group 2 (p=0.038). The 5-year survival rate of patients with tumors smaller than 10 cm was 78.4%, while that of patients with tumors larger than 10 cm was 38.1% (p=0.017). The 5-year survival rate after complete excision was 61%, whereas that after incomplete resection or biopsy only was 40.0% (p<0.0001). CONCLUSIONS: An upper abdominal tumor location is a positive prognostic factor even if small tumor size (<10 cm) and complete resection of the tumor are still more important factors to improve outcome in patients with malignant primary retroperitoneal tumors. Because complete resection was shown to be the most important prognostic factor, an aggressive and careful surgical approach is recommended for the treatment of such tumors.     

Extent of excision margin width required in breast conserving surgery for ductal carcinoma in situ

BACKGROUND: Breast conserving surgery (BCS) is common practice for unifocal ductal carcinoma in situ (DCIS) less than 4 cm in size, but the extent of tumor free margin width around DCIS necessary to minimize recurrence is unclear. METHODS: Clinical and pathologic details were recorded from all patients with pure DCIS < 4 cm in size, treated with BCS between 1978 and 1997. Histologic margins were measured by using an ocular micrometer. Patients with clear margins (> 1 mm) were divided up into 3 groups for analysis based on margin of normal tissue excised: 1.1-5 mm, 5.1-10 mm, and 10.1-40 mm. RESULTS: There were 66 patients with close margins (< or = 1 mm), of which 25 cases (37.9%) recurred. The recurrence rates for the 3 clear margin groups ranged from 4.5-7.1%. Median followup was 47 months (range 12-197 mos). Risk of recurrence in the group with close margins was greater than the subgroups with clear margins (P < 0.001); no differences in recurrence was seen between the individual subgroups with clear margins. Nuclear Grade 3 was predictive of recurrence (P = 0.03). Following excision alone, the recurrence rate was 18.6%, compared with 11.1% when radiotherapy was given as adjuvant therapy. Women with clear margins following excision had a recurrence rate of only 8.1%. CONCLUSION: After BCS for DCIS, close margins were associated with a high risk of local recurrence. Radiotherapy did not compensate for inadequate surgical clearance.     

Results of surgery for malignant fibrous histiocytomas of soft tissue

Background: To address the prognosis and the role of surgery in the management of patients with malignant fibrous histiocytoma (MFH), strict definition and accurate evaluation of local recurrence is mandated, together with adequate gross and microscopic evaluation of the achieved surgical margins. Methods: One hundred and twenty-three patients with MFH were retrospectively analyzed regarding prognostic factors of the disease, which included the following: (1) primary or recurrent, (2) histological grade, (3) adjuvant chemotherapy, (4) size, (5) local recurrence after primary surgery done at the authors' institute, (6) adjuvant radiotherapy, and (7) surgical margin. Initially, all patients were metastasis-free (M0N0). Results: Local recurrence after primary surgery done at the authors' institute was the most significant prognostic factor, where 20 of 123 patients developed local recurrence (P < 0.0001). The cause of local recurrence were insufficient surgical margin in 16 patients, while in 4, local recurrences were related to lymph node metastasis, skip metastasis, and tumor embolism. The local recurrence rate for each surgical procedure was 75% for intralesional, 44.4% for marginal, 30.8% for inadequate wide, 12.3% for adequate wide, and 5% for curative procedures. In patients with a history of recurrent tumor or infiltrative pattern, local recurrence was not observed after a curative procedure, but occurred even after an adequate wide procedure. Conclusion: To improve the prognosis of MFH, surgical prevention of local recurrence is essential. An adequate wide procedure for primary tumors and a curative procedure for recurrent tumors and tumors with an infiltrative pattern provide safe surgical margins. Received: April 19, 2002 / Accepted: December 18, 2002 Correspondence to:S. Matsumoto     

Radiofrequency hyperthermia as adjuvant therapy following surgical resection of an experimental malignant neoplasm

Local recurrence after radical surgery is a major problem with many primary solid cancers. The use of radiofrequency hyperthermia (RFHT) as adjuvant therapy to surgery was explored in the Fischer bladder carcinoma (FBCa)/F344 rat tumor system. After subcutaneous innoculation of 34 rats with 10(6) FBCa cells in suspension, RFHT was administered to 17 animals on days 1, 5, 8, and 12. The development of palpable tumors was delayed but not prevented, and tumor growth was retarded in RFHT-treated animals. In another experiment 40 rats were innoculated by subcutaneous trocar injection with a 1 mm3 piece of FBCa. After tumor excision on day 17, adjuvant therapy (untreated control, mitomycin C, RFHT, or RFHT plus mitomycin C) was started on day 20 (10 rats/treatment). The 20 RFHT-treated rats had only 1 incisional recurrence as compared to 9 recurrences in sham-heated rats (P less than 0.005). The authors conclude that RFHT has considerable value as adjuvant therapy to surgery in these tumors. Additional studies of RFHT as adjuvant treatment after surgical excision of tumors are planned.     

软组织肉瘤补充广泛切除术的疗效分析

目的探讨补充广泛切除术在软组织肉瘤外科治疗中的意义。方法回顾性分析1998年1月至2005年10月收治的外院误诊为良性肿瘤而行局部切除的软组织肉瘤患者58例，均行补充广泛切除手术治疗。结果术后病理标本切除缘评价5cm者14例；3cm者32例；3cm以下者12例。首次术后肿瘤残存26例，占45％。补充广泛切除术后有28例需要行软组织修复重建。补充广泛切除手术后随诊10～94个月，8例复发，12例转移，死亡9例。均为高度恶性软组织肉瘤。Kaplan—Meier法计算5年生存率为84．5％，局部复发率为14％。切除缘的5年生存率3cm以上组和3cm以下组之间无显著性差异。局部复发率3cm以上组和3cm以下组有显著性差异（P〈0．05）。结论补充广泛切除术是误诊软组织肉瘤最主要的治疗手段，补充广泛切除的标准切除缘的制定是今后的研究课题。缩小切除缘，可以改善预后，减少组织缺损所造成的修复重建。     

Dermatofibrosarcoma protuberans: review of 20-years experience

Introduction Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm with low-intermediate grade of malignancy. It is a locally aggressive tumour with a high recurrence rate. Surgical excision with adequate margins is the main treatment. Materials and methods We describe the clinicopathological features of 21 cases of DFSP. The mean size of the lesions was 5.6 cm, mostly located in trunk. 61.9% of the cases underwent sugical excision without previous biopsy. 52.4% of the patients presented positive margins, that required surgical extension. Results The recurrence rate was 28.6% (6 cases), five of them local recurrences treated with new surgical excision with wide margins. Median period free of illness was 52.5 months. Medium follow-up period was 55.25 months. Conclusions DFSP has a locally high recurrence rate, that has been associated to inadequate surgical margins. The histological knowledge preoperatively would permit surgical excision with adequate margins, and probably this could reduce the recurrence rate. Radiotherapy could avoid the surgical extension of margins in these cases with positive ones. Chemotherapy could be indicated in metastasic cases. Most recurrences appear in the first 3 years, but it is important a long-term follow-up of these patients.     

Analysis of phyllodes tumor recurrence according to the histologic grade

Local recurrence of phyllodes tumor (PT) of the breast is an adverse outcome that can result in sarcomatous degeneration. The aim of this study was to investigate the histologic and surgical factors associated with local recurrence. A total of 193 PT cases were studied: 145 (75.1 %) benign cases, 33 (17.1 %) borderline cases, and 15 (7.8 %) malignant cases. Stratifying our analysis according to histologic grade, we investigated the relationship between disease-free survival (DFS) and both histologic and surgical factors, including histologic grade, stromal cellularity, stromal atypia, stromal mitosis, stromal overgrowth, tumor margin, type of surgical procedure (local excision, wide excision, and mastectomy), surgical margin status, and radiation therapy. In the case of benign PT, all patients with local recurrences (3.4 %) had been treated with local excision, and all recurrent tumors were also benign. The local recurrence rate for locally excised benign PTs was not associated with surgical margin status or radiation therapy. In the case of borderline PT, local excision was associated with an increased local recurrence rate (P = 0.046). In malignant PT, small tumor size (≤4.0 cm) was associated with an increased local recurrence rate (P = 0.041). Univariate analyses indicated that surgical procedure (mastectomy < local excision < wide excision; P < 0.001) was significantly associated with shorter DFS in borderline PT. A positive surgical resection margin (P < 0.001) was associated with DFS in malignant PT. The factors associated with local recurrence differed with the histologic grade of PT, as did the features of local recurrence itself. In particular, benign PT had very low rate of local recurrence regardless of surgical margin status or radiation therapy, even when treated with local excision. In the case of benign PT, no recurrent tumors had worse histologic grades than the initial tumors.     

Radiation therapy for high grade soft tissue sarcomas of the extremities treated with limb-preserving surgery.

AIM: It is common to use either pre- or post-operative radiation for high grade sarcomas undergoing limb-conserving surgery. Since 1977, we have adopted a selective policy of post-operative radiation, given only in patients with inadequate surgical margins. METHODS: A retrospective review of 114 patients (1977-1995) with high grade adult soft tissue sarcomas of the extremities was carried out. Amputation was required in 10 (9%). Patients with a minimum surgical margin <2 cm (n=33) received adjuvant radiation (29%). RESULTS: No complications occurred in 81/114. Wound complications were infection (14%), seroma (6%), haematoma (4%), dehiscence (4%) and skin edge necrosis (2%). Remedial operations were required in four (3%). Overall, the local recurrence rate was 23/114. Patients with surgery only (n=57) or surgery plus systemic chemotherapy (n=24) manifested local recurrence in 15/81 (19%) and those with surgery plus radiation (n=21) or surgery and radiation and chemotherapy (n=12) suffered local recurrence in 8/33. The local recurrence rate for tumours < or =5 cm was 6/32 and for those >5 cm 17/82, P=1.0. The 5 year survival rate was 60% for tumours < or =5 cm (n=32) and 46% for tumours > or =5 cm (n=82) (P=0.009). CONCLUSIONS: (1) Limb preservation was feasible in 91% of patients. (2) When the local treatment modality was surgery alone ('wide' margins) the local recurrence rate was 19%, and when it was surgery plus radiation (narrow margins) it was 24%. (3) Selective use of radiation (in patients with narrow margins) and reliance on surgery alone in cases amenable to wide resection may be a legitimate alternative to universal application of radiation with conservative resection.     

Microscopie-controlled excision of cutaneous tumors: chemosurgery, fresh tissue technique.

A modification of Mohs' chemosurgery which eliminates the zinc chloride paste but follows the careful piece by piece excision, marking, and mapping has been developed for the treatment of cutaneous tumors. The technique is called Microscopic Controlled Exicision (MCE). This 8-year retrospective study reports a 97.2% cure rate for 532 determinant lesions. On hundred eighty-five (35%) of these cases followed for 5 years or longer had a 96% cure rate. The modified technique eliminates the pain of zinc chloride fixative paste, shortens the time required to perform the surgery, removes less uninvolved, normal tissues, and the final defect is a fresh surgical wound which can be repaired immediately. These advantages become highly significant since a high 5-year cure rate can be obtained. It is recommended that this technique be used for all tumors which recur after routine treatment by excision, radiation therapy, curettage and desiccation, or cryosurgery, for tumors with poorly defined clinical borders, for sclerosing basal cell epitheliomas, and for primary cutaneous carcinomas in areas which have a predilection for recurrence.     

Cystosarcoma phyllodes. Diagnosis and management

The diagnosis and management of cystosarcoma phyllodes is analyzed by reviewing the literature and presenting cases from our institution. This rare neoplasm of the female breast represents less than 1% of all breast tumors. Most patients present in the fourth and fifth decades of life. The predominant complaint is a palpable mass in the breast. Systemic manifestations occur after metastases have developed. Treatment is surgical excision. There is some controversy, however, over which type of surgical procedure should be performed. We recommend total mastectomy. Local recurrence and metastases do occur and have been related to inadequate surgery and various histological characteristics (mitotic activity, tumor margin, and stromal cellular atypia).     

腹部恶性纤维组织细胞瘤47例临床和预后分析

目的探讨腹部恶性纤维组织细胞瘤(MHF)的临床病理特点、治疗策略及预后相关因素。方法回顾性分析1970年1月至2000年1月间天津医科大学附属肿瘤医院47例腹部恶性纤维组织细胞瘤患者手术切除治疗情况。所有患者均经手术病理证实,病变单一。结果术后随访中共有32例患者出现复发、转移,术后单纯局部复发13例(29.5%),出现肺及胸膜转移7例(15.9%),肝转移4例(9.0%),骨转移6例(13.6%),肾脏转移2例(4.5%),复发 转移5例(11.4%)。术后1,3,5年无瘤生存率分别为65.9%、40.9%和29.5%,累计生存率分别为70.5%、52.3%和31.8%,中位生存期为18个月。影响预后的主要因素为肿瘤发生的部位、治疗方式、病理类型、肿瘤的局部复发等。以手术为主的综合治疗较单纯手术及放化疗预后好,术后辅以放疗可降低局部复发率,尤其对那些切端阳性患者。结论腹部MHF侵袭性强,复发率高,病理分级差,以手术切除为主辅以放疗有助于提高患者的远期生存率。     

Outcomes and factors impacting local recurrence of ductal carcinoma in situ

BACKGROUND: The optimal management of ductal carcinoma in situ (DCIS) remains controversial. Investigators have focused on identifying patients who are eligible for treatment by excision alone. A retrospective analysis of patients with DCIS treated by various modalities was conducted to compare outcomes and determine factors significant for local recurrence (LR). METHODS: Between 1985-1992, 88 consecutive diagnoses of DCIS were identified in 85 patients. Seventy-four percent were detected mammographically. The most common histologic subtypes were comedo (54%) and cribriform (23%). Tumor sizes were < 2.5 cm (49%), > 2.5-5 cm (26%), > 5 cm (23%), and unknown (2%). Final resection margins were tumor free (75%), close/positive (23%), and unknown (2%). Treatment methods included mastectomy (30%), localized surgery and radiation therapy (LSR) (43%), or wide localized surgery alone (LS) (27%). Radiation therapy (RT) was comprised of 50 grays to the breast, and 53% of treated patients received local "boost" irradiation. RESULTS: The median follow up was 8.3 years. The overall recurrence rate was 13. 6%, whereas the median time to LR was 27.8 months. Recurrence rates according to treatment modality were: LS: 25%; LSR: 13%; and mastectomy: 4%. However, if surgical margins were tumor free, LSR had a LR rate of 3.4%. After RT, no LR occurred prior to 15 months, and 4 of 5 tumors were noninvasive. Nine patients treated by excision alone conformed to the criteria of Lagios et al. criteria and LR occurred in three of nine tumors. Of the factors analyzed, margin status was found to be the best predictor for LR (P = 0.05). CONCLUSIONS: If surgical margins are tumor free, the LSR regimen is equivalent to mastectomy for local tumor control. Annual mammograms may be adequate for the follow-up of patients with irradiated breasts, but biannual studies still are recommended for patients treated with excision alone.     

Margins in extra-abdominal desmoid tumors: a comparative analysis

BACKGROUND AND OBJECTIVES: The main treatment of extra-abdominal desmoid tumors remains surgery, but recurrence rates up to 80% are reported. The impact of microscopic surgical margin status according to the Enneking classification system is discussed controversially. METHODS: Therefore, the authors screened the published literature for reliable data on the importance of a wide or radical excision of extra-abdominal desmoid tumors. All studies with more than ten patients, a surgical treatment only, and margin status stated were included. RESULTS: Only 12 out of 49 identified studies fulfilled the inclusion criteria. One hundred fifty-two primary tumors were excised with wide or radical microscopic surgical margins, while in 260 cases a marginal or intralesional excision was performed. In the first group 41 patients (27%) and in the second one 187 patients (72%) developed a recurrence. Therefore, microscopic surgical margin status according to the Enneking classification system is a significant prognostic factor (P < 0.001). CONCLUSIONS: The data of this review underline the strategy of a wide or radical local excision as the treatment of choice. Furthermore, as a large number of studies had to be excluded from this analysis, exact microscopic surgical margin status should be provided in future studies in order to allow comparability. .