A 20-year review of haemangiopericytoma in Auckland, New Zealand

The records of all patients registered with a histological diagnosis of haemangiopericytoma in Auckland between 1970 and 1990 were reviewed retrospectively, with the aim of determining the natural history of the disease and the response to various treatment modalities. A total of 24 patients were identified, having a median age of 45 years.Twenty-one patients (87.5%) underwent surgery; the remaining three were deemed to be unfit for surgery. Seven patients (29%) were treated with surgery alone; nine (37.5%) received a radical course of radiotherapy and three (12.5%) received palliative radiation therapy for pain relief and/or dyspnoea. Five patients (21%) received chemotherapy during the course of their disease.Eight of the 24 patients (33%) were alive and disease free, 13 (54%) having died and three (13%) being lost to follow-up. Seven patients (29%) died as a result of metastatic disease. Three of the seven (43%) who were treated with surgery alone are known to be alive and disease free. The three patients who had received palliative radiotherapy, died within 2 months of completing the latter treatment. Five of the nine patients (56%) receiving a course of radical radiotherapy are alive and disease free at present. No local recurrence was noted following surgical excision and postoperative radical radiotherapy, whilst eight (67%) of those initially treated by excision alone developed recurrent disease. None of the patients treated with chemotherapy obtained significant palliation.Results suggest that adequate surgical excision followed by postoperative radiotherapy is effective in controlling haemangiopericytoma and that metastatic disease is at present invariably fatal. The role of chemotherapy needs further investigation.     

Squamous carcinoma arising in a pilonidal sinus

A patient with squamous cell carcinoma arising from an extensive pilonidal sinus was treated with wide local excision, resulting in an apparent cure over a 1-year follow-up period. Review of the literature revealed 32 previously reported cases of this entity. Forty-four percent of these patients developed recurrences or metastases, and 22% died of their disease. The largest historical treatment group with at least 1-year follow-up (19 patients with localized disease) underwent wide excision. Forty-two percent of these patients recurred or developed metastases. Ultimately, 89.5% of these patients were reported cured, but only five survivors were followed for as long as 5 years. Historical experience appears to identify pilonidal tract squamous carcinoma as an aggressive tumor. The reported success of wide local excision is based on a small number of patients followed for short periods of time. Continued experience with this disease should be reported to verify the adequacy of local excision or identify the need for adjunctive therapy.     

Uncontrolled local recurrence after treatment of breast cancer with breast conservation

Three hundred fifty-six patients with early (Stage I and II) breast cancer and 55 with advanced (Stage III and IV) breast cancer were treated between 1979 and 1985 with a consistent policy of breast conservation irrespective of tumor site, size, or histologic features. Only three patients underwent primary mastectomy (Stage III), and the remainder were treated either by wide local excision and postoperative radiotherapy (357 cases) or by needle biopsy and primary irradiation (51 cases). A total of seven of 356 (2%) Stage I and II patients have developed uncontrolled local or nodal recurrence at a median follow-up of 5 years, and nine of 55 (16%) of Stage III and IV patients. Of the 62 Stage I and II patients who have died, seven (11%) have died with uncontrolled locoregional disease. Of the 22 Stage III and IV patients who have died, eight (36%) have died with uncontrolled locoregional disease. Although the majority of local recurrences within the conserved breast could be salvaged by secondary surgery (37/38 Stage I and II patients), the development of chest wall or nodal recurrence was usually associated with the appearance of distant metastases and a poor prognosis. Data on uncontrolled local recurrence should be given in all studies of breast cancer treatment, since it represents an important end-point of therapy and a difficult clinical problem.     

隆突性皮肤纤维肉瘤85例临床分析

目的：探讨隆突性皮肤纤维肉瘤的临床特点和治疗方法。方法：回顾性分析85例隆突性皮肤纤维肉瘤临床诊治过程。结果：85例隆突性皮肤纤维肉瘤行扩大切除术45例，扩大切除加植皮或转移皮瓣修补术34例，术前放疗2例，术后合并放疗10例，全组病例复发率为64．7％，5例死于肿瘤转移，结论：隆突性皮肤纤维肉瘤是皮肤低度恶性肿瘤，复发率高，首次治疗时彻底切除极为关键，外科手术是其主要的治疗方法，治疗作为辅助治疗有一定疗效。     

Primary Ewing's sarcoma of the ribs. A report from the intergroup Ewing's sarcoma study

Thirty-six patients with primary Ewing's sarcoma of the ribs have been reviewed. Of these, 21 had clinically localized disease at diagnosis and were entered on protocol IESS 7299, eight had regional and seven metastatic disease at diagnosis and were entered on protocol 7450. The 21 with localized disease were treated with surgical excision or biopsy, followed by local radiotherapy (in all but one patient) and randomization to one of three chemotherapy regimens. Eleven patients (52%) remain disease-free for periods ranging from 18 to 64 months, respectively. Seven of eight patients who underwent complete surgical excision of the primary lesion remain disease-free, compared with four of 12 (excluding one patient who died disease-free) who remain disease-free after partial excision or biopsy. However, analysis of size of tumor at diagnosis reveals that smaller primary tumors have a better prognosis irrespective of extent of surgery. Protocol IESS 7450, consisted of radiotherapy to all areas of known disease and four drug chemotherapy. Four (50%) with regional disease but none with metastatic disease have remained alive and continuously disease-free. It is concluded that an aggressive approach to Ewing's sarcoma of the ribs is justified by the results, as even regional disease may be curable. The apparent prognostic advantage for those patients undergoing surgical excision may be explained by patient selection.     

Osteosarcoma of the spine: experience of the Cooperative Osteosarcoma Study Group

BACKGROUND: Due to the low incidence rate, the optimal strategy for the treatment of patients with spinal osteosarcoma is unknown. METHODS: Twenty-two patients with osteosarcoma of the spine (15 with tumors of the sacrum and 7 with tumors at other sites) who received chemotherapy according to the Cooperative Osteosarcoma Study Group protocol were analyzed. Six patients presented with metastasis, and 16 patients had no evidence of metastasis at the time of entry into the protocol. Of 12 patients who underwent excision of their tumors, 2 patients underwent wide excision, 3 patients underwent marginal excision, and 7 patients underwent intralesional excision. Eight patients received irradiation: Six patients received conventional radiotherapy only, one patient received neutron beam therapy, and one patient received samarium-153-ethylene diamine tetramethylene phosphonate therapy. Follow-up ranged between 24 months and 105 months (median, 47 months). RESULTS: The median survival was 23 months, and three patients have survived without disease for > 6 years. Patients with primary metastases (P = 0.004), large tumors (P = 0.010), and sacral tumors (P = 0.048) had lower overall survival compared with patients who had no metastasis, small tumors, and nonsacral tumors, respectively. There was a significant difference in overall survival between 5 patients who underwent either wide or marginal surgery and 17 patients who underwent either intralesional surgery or no surgery (P = 0.033). Among 17 patients who underwent no surgery or intralesional surgery, overall survival tended to be better in 7 patients who received irradiation compared with the overall survival in 10 patients who did not receive irradiation (P = 0.059). CONCLUSIONS: Patients with metastases, a large tumors, and sacral tumors had a poor prognosis in the current study with small numbers of patients. Wide or marginal excision of the tumor improved survival. Patients with osteosarcoma of the spine should be treated with a combination of chemotherapy and at least marginal excision for those with surgically accessible tumors. Postoperative radiotherapy may be beneficial.     

Experience with the management of primary endodermal sinus tumor of the mediastinum

Ten male patients with extensive primary endodermal sinus tumor of the mediastinum were treated with chemotherapy (with or without surgical excision and radiation therapy) between 1977 and 1985. Three patients, treated with cyclophosphamide-vincristine-based chemotherapy, died 1.5, 2.5, and 6.0 months, respectively, after initial diagnosis. Of the seven patients treated with cisplatin-based chemotherapy, one patient died of septicemia at 2.5 months after diagnosis and was disease-free at autopsy examination. Three other patients died of progressive disease at 7.0, 13.0, and 14.0 months, respectively. The three survivors remain alive at 17.0, 31.0, and 40.0 months from diagnosis; all are without evidence of disease. Of the three patients who underwent excision of the residual mediastinal tumor after cisplatin-based chemotherapy, persistent local disease was found in two patients. One of these two patients died of recurrent disease. All surviving patients had surgical resection of the tumor either before or after cisplatin-based chemotherapy, with or without radiotherapy and the timing of therapeutic interventions was guided by changes in the serum alpha-fetoprotein concentrations after initial therapy and during follow-up. Our experience suggests that the optimal management of patients with primary mediastinal endodermal sinus tumor requires an aggressive multidisciplinary approach guided by the extent of the tumor and the serum tumor marker levels.     

Bleomycin, Lomustine, Cyclophosphamide, Vincristine, Procarbazine and Prednisone (BLEO-CCVPP) in Patients with Hodgkin's Disease who Relapsed after Radiotherapy Alone: a Long-Term Foliow-Up Study of the Eastern Cooperative Oncology Group (E3481)

Thirty-three evaluable patients with Hodgkin's disease who failed radiotherapy were treated on this phase II study with bleomycin, lomustine, cyclophosphamide, vincristine, procarbazine and prednisone given every 28 days for a minimum of eight courses. Twenty-five patients (76%; 95% CI=55.6-87.1%) achieved a complete remission, the median duration of which cannot yet be determined, but the probability of remaining in continuous complete remission at 10 years is 64. The median survival from entry on this study for all evaluable patients is 10 years, and 12 patients were alive at the time of this analysis with a median follow-up for them of 15.5 years. Of the 22 patients who died, 11 died of progressive or recurrent Hodgkin's disease and 11 died of other causes including 7 second primary neoplasms and at least one myocardial infarction. Both are now well known late complications of Hodgkin's disease treatment.     

Breast sarcoma. A clinicopathologic review of 25 cases

Sarcoma of the breast represents less than 1% of primary mammary malignancies; this study reports 25 such cases. The largest group had malignant fibrous histiocytoma (44%), followed by liposarcoma (24%) and fibrosarcoma (16%). Also represented were clear cell sarcoma, neurogenic sarcoma, leiomyosarcoma, and alveolar soft part sarcoma (4% each). Of 19 patients treated by wide local excision or simple mastectomy with or without adjuvant radiotherapy, 11 had local recurrence develop, of which one patient died and nine of the remaining ten had metastases develop. Of the remaining eight patients in this group with no local recurrence, only two had metastases develop. Of the six patients treated by radical or Patey mastectomy, none had local recurrence develop, but two died of metastases. No patient had metastases develop more than 5 years after diagnosis. Regional lymph node involvement with tumor was observed in only one patient (with malignant fibrous histiocytoma) despite regional lymphadenopathy in seven. The overall mortality at 5 years is 64% but does not increase thereafter. The authors' findings suggest that failure to establish local control is associated with a poor prognosis and that wide local excision or simple mastectomy does not provide sufficient clearance to be used as first-line treatment. Excision of the axillary lymphatics and adjuvant radiotherapy are unlikely to be beneficial.     

Radiotherapy for lacrimal gland tumours

Forty-two patients with lacrimal gland tumours were treated with radiotherapy at the Royal Marsden Hospital between 1950 and 1982. Thirty-three patients with malignant tumours had a median cause-specific survival of 10.4 years. The tumour control following radiotherapy was related to histology, extent of initial surgery, and radiotherapy dose. Those patients with adenocarcinoma, undifferentiated carcinoma and malignant mixed tumour, who had complete surgical excision and adjuvant radiotherapy all remained disease-free; after incomplete excision, radical radiotherapy achieved local control in only one of 13. In contrast, in patients with adenoid cystic carcinoma 7 or 13 (54%) with residual local disease remain disease-free after radical radiotherapy. The complication rate of radiotherapy was low; 79% of patients who remained disease-free with an intact eye retained good vision.     

Sweat-gland tumours: a clinical review of cases in one centre over 20 years

AimsSweat-gland tumours (SGTs) are uncommon, but malignant varieties are very rare. We have added our data on 30 new cases seen at the Royal Marsden NHS Foundation Trust to the published literature, particularly concentrating on clinical issues. We include a literature review.Materials and methodsThe Royal Marsden NHS Foundation Trust database was searched for cases of SGT from 1972. Data were collected on all cases, including patient demographics and tumour characteristics, treatment and outcome.ResultsThirty cases were confirmed histologically to be SGTs. Fourteen were malignant, 15 benign and the degree of malignancy in one was histologically indistinguishable. Mean age was 55 years (64 for malignant, 47 for benign tumours). The 15 patients with benign tumours were almost all treated with complete excision. Those with local relapse underwent successful re-excision. Their 5-year disease-free survival was 78% and cause-specific survival was 100%. Twelve of the 14 malignant tumours had localised disease at diagnosis, one had nodal disease and one had metastatic tumour nodules. All except one were treated with wide local excision. The patient with nodal involvement also had a lymph-node dissection. Two received adjuvant radiotherapy to the tumour bed. One received a melphalan limb perfusion. Eight of the 14 had no relapse. Six had locoregional relapse, and four of these also developed distant metastases. Visceral disease was always fatal. Radiotherapy and chemotherapy at relapse were unsuccessful. Five-year disease-free survival was 45%, and cause-specific survival was 57%.ConclusionThese rare tumours should be treated initially with complete wide local excision. In malignant tumours, lymph-node involvement is a poor prognostic sign. Wide local excision remains the primary treatment. Adjuvant radiotherapy may be useful in high-risk cases.     

上皮样肉瘤14例临床分析

背景与目的:上皮样肉瘤是一种组织起源尚不清楚的罕见软组织肉瘤.其生物学行为独特,容易发生局部复发、淋巴结扩散和/或远处转移.本研究旨在探讨上皮样肉瘤(Epithelioid sarcoma)的临床特点、诊断、治疗和预后.方法:对我院1990年1月至2005年12月间收治的14例上皮样肉瘤临床资料进行回顾性分析,并结合随访资料进行生存分析.全组14例均接受手术治疗.首次手术均在外院进行.再次手术11例在我院进行(9例行扩大切除术,2例行截肢术;4例同时行区域淋巴结清扫术),3例仍在外院行局部切除术或扩大切除术.术后9例接受辅助性治疗,其中单纯放疗6例,放疗 化疗3例.结果:全组在本院病理科复检确诊为上皮样肉瘤.12例(85.7%)出现肿瘤局部复发.4例(28.6%)发现区域淋巴结转移,9例术后3年内死亡.全组总的1、2、5、10年生存率为71.43%、55.56%、27.78%和13.89%.结论:上皮样肉瘤恶性程度并不很高,但局部复发率高,容易发生淋巴结和/或远处转移,预后不佳.广泛性切除或根治性切除合并预防性区域淋巴结清扫术是治疗上皮样肉瘤的有效手段.     

Treatment of breast cancer in elderly women: retrospective analysis of 111 wide lumpectomies performed in a day hospital regimen between 1982 and 1988.

Between 1982 and 1988, 111 elderly women with breast cancer but without clinical involvement of the axillary lymph nodes underwent wide lumpectomy in a Day Hospital regimen at the National Cancer Institute of Milan. The patients ranged in age from 70 to 92 years (median, 79). An adjuvant treatment was carried out in all but 9 cases: tamoxifen only in 84 cases, tamoxifen plus radiotherapy in 6 cases, radiotherapy alone in 12 cases. The median duration of follow-up was 44 months (range, 30-109 months). Four patients (3.6%) were lost to follow-up. In the remaining 107 patients, 10 local-regional relapses (9.1%) and 7 distant metastases (6.5%) occurred. Six patients died from the disease, 14 from unrelated conditions. This retrospective study showed that selected elderly patients with breast cancers can be treated successfully under local anesthesia on an outpatient basis. The treatment guarantees local control of the disease, meets the favor of elderly women and consequently improves their quality of life.     

Postcricoid carcinoma: a retrospective study of 13 patients

Thirteen consecutive patients presenting with squamous cell carcinoma in the postcricoid region (PCC) are studied and details of their age, sex, TNM status, treatment and results are analysed. Follow-up ranges from 33 months to 11 years. Three patients presented with cervical node involvement and 2 of these remain disease free without surgical intervention, at 33 months and 9 years. The principal treatment policy has been the combination of multi-agent chemotherapy with radical radiotherapy, reserving wide excision surgery for patients with residual or recurrent carcinoma. Only 4 patients underwent pharyngolaryngectomy, one radical neck dissection was performed and 7 of the 13 patients have remained free of disease for more than 3 years.     

Medulloblastoma: time–dose relationship based on a 30-year review

Purpose: Time–dose relationships have proven important in many cancer sites. This study evaluates the time factors involved in the successful postoperative radiotherapy of medulloblastoma, based on a 30-year experience in a single institution.Methods and Materials: Fifty-three patients with medulloblastoma received postoperative craniospinal radiotherapy with curative intent between 1963 and 1993. Seven patients (13%) underwent biopsy alone, 28 patients (53%) had subtotal excision, and 18 patients (34%) had gross total excision. Eleven patients received adjuvant chemotherapy. The mean posterior fossa dose was 53.1 Gy; most patients received 54.0 Gy (range, 34.3 to 69.6 Gy). For 41 patients receiving once-a-day therapy, the mean dose was 50.6 Gy (range, 34.3 to 56.0 Gy). For 12 patients receiving twice-a-day therapy, the mean dose was 61.8 Gy (range, 52.6 to 69.6 Gy). Minimum follow-up was 2 years, and median follow-up was 10.7 years. Survival, freedom from relapse, and disease control in the posterior fossa were calculated using the Kaplan-Meier method, and multivariate analysis was performed for prognostic factors. Variables related to radiotherapy were examined, including dose to the craniospinal axis, dose to the posterior fossa, fractionation (once-a-day vs. twice-a-day), use of adjuvant chemotherapy, risk group [high (≥T3b or ≥M1) or low (≤T3a and M0-MX)], interval between surgery and radiotherapy (excluding patients receiving chemotherapy before radiotherapy), and duration of radiotherapy.Results: At 5 and 10 years, overall survival rates were 68 and 64%, respectively, and freedom-from-relapse rates were 61 and 52%, respectively. Rates of disease control in the posterior fossa at 5 and 10 years were 79 and 68%, respectively. At 5 years, absolute survival rates after biopsy alone, subtotal excision, and gross total excision were 43, 67, and 78%, respectively (p = 0.04), and posterior fossa control rates were 27, 89, and 83%, respectively (p = 0.004). Duration of the treatment course was the only radiotherapy-related variable with a significant impact on freedom from relapse and posterior fossa control. For patients whose radiation treatment duration was ≤45 days, posterior fossa control was 89% at 5 years, compared with 68% for those treated for >45 days (p = 0.01). Duration of treatment also affected freedom from relapse at 5 years: ≤45 days (76%) compared with >45 days (43%), p = 0.004.Conclusion: Our study demonstrates that if adequate doses are used, then radiotherapy treatment duration will significantly affect the outcome in terms of control of disease in the posterior fossa and freedom from relapse. Fractions of at least 1.75 Gy given once a day, or a twice-a-day regimen should yield optimal local control results.     

Cystosarcoma phyllodes: Our institutional experience

Twenty‐six patients of Cystosarcoma phyllodes, treated between July 1994 and July 2001, were analysed retrospectively. Median age at presentation was 38 years (range 13?61 years). Mean size of the lesion was 6 cm. There were 77% left‐sided lesions and 23% right‐sided lesions. Histologically, 58% lesions were benign, 11% borderline and 31% malignant lesions. All patients underwent definitive surgical procedure in the form of wide local excision or mastectomy. Four patients received postoperative radiotherapy. Median follow‐up period was 35 months. Six patients showed recurrence, and four of these were malignant. Median disease‐free survival period was 34 months.     

The rationale for planned reoperation after unplanned total excision of soft-tissue sarcomas

Multimodality management of soft-tissue sarcomas of the extremity is often based on the presence or absence of residual primary disease. Reoperation is warranted or radiotherapy doses altered if the physician is aware that the tumor was incompletely excised. Most patients with soft-tissue masses undergo an initial excision before definitive therapy. These initial unplanned total excisions are usually excisional biopsies for presumably benign disease. Ninety patients were reviewed to evaluate the adequacy of unplanned total excision. All patients underwent unplanned supposed total excisions. Most patients were then treated with preoperative intraarterial Adriamycin (Adria Laboratories, Columbus, Ohio) and radiation therapy, followed by wide reexcision of the prior operative field. Forty-six patients (51.1%) had no gross residual tumor in the reoperative specimen. In two patients, there was microscopic but not macroscopic disease. Forty-four patients (48.9%) had identifiable macroscopic residual disease in the reoperative specimen. When comparing these 44 patients with visible (macroscopic) residual tumor to the remaining 46, no differences were seen in age, sex, stage, histologic type, time from excision to reoperation, or size of initial lesion. This previously unrecognized high incidence of gross residual disease must be considered when planning definitive therapy. Unplanned total excisions are inadequate to remove local disease and, despite multimodality therapy, may result in local failure. Reoperation should be a planned part of definitive management for patients with soft-tissue sarcoma of the extremity whenever the initial surgical procedure was done without a histologic diagnosis or was not planned to be a wide excision. If reoperation cannot be performed, radiotherapy doses to treat gross residual disease should be used.     

Prognosis of level V malignant melanoma

Level V melanomas have been reported to have a poor prognosis, but in-depth analyses of prognostic factors and treatment have not been reported. From 1952 through 1982, 41 patients presented with primary Clark's Level V melanomas. There were 23 patients who presented with clinical Stage I disease and 18 with Stage II. Among Stage I patients, 9 were treated by wide excision alone and 13 underwent wide excision plus prophylactic regional lymph node dissection (RLND); 8 of 13 patients had histologically positive nodes. Twelve Stage II patients were treated by wide excision and RLND (including three hemipelvectomies), and four refused surgery. The 5-year survival was 52%. For Stage I patients, survival was 62% and disease-free survival (DFS) 28% at 5 years; 6 of 10 recurrences were local or regional only. Prophylactic RLND reduced the incidence of recurrence but did not appear to influence survival rates. Among 14 evaluable Stage II patients, overall survival was 60% and DFS 42% at 3 years; of 4 patients who subsequently had a recurrence, 3 had distant metastases. All seven patients with distant metastases at the time of first recurrence died of disease within 14 months (median, 4 months) of detection of metastatic disease. Primary melanomas of the foot (11 patients) and trunk (4 patients) appeared to have a worse prognosis than other sites. Ulceration (seen in 21 patients) did not appear to significantly influence outcome. These data suggest that most patients with Level V melanoma present with clinically localized disease. Prophylactic RLND did not significantly affect overall survival. The invasiveness of these deep tumors appears to reduce the influence of other factors, including primary site, sex, race, and ulceration. The prognosis of patients with Level V melanoma, even with clinically or histologically positive lymph nodes, is not hopeless, and these patients should be treated aggressively.     

A comparison of wide local excision with abdominoperineal resection in anorectal melanoma

Anorectal melanoma is a rare condition and its surgical management is controversial. Seventeen large case series from over the past 10 years were reviewed. The survival of patients treated by either abdominoperineal resection (APR) or wide local excision (WLE) was analysed according to the stage of the disease. Comparison of the survival of patients who underwent APR with those who underwent WLE showed no statistically significant advantage for either procedure in patients at all disease stages. APR should therefore only be performed when local excision is not possible or for palliative purposes.     

Conservative Surgery and Radiotherapy as Primary Treatment for Early Breast Cancer: Results of a 10 Year Experience

A 10 year period of treatment at the Radiotherapy Department of the Royal Prince Alfred Hospital was analysed retrospectively in order to assess the efficacy of conservative management of early breast cancer. From May 1968 to May 1978 a total of 1,785 patients with breast cancer were treated. Of these, 30 underwent conservative surgery for Stage I or II disease, followed by a course of “radical” irradiation. Patient follow-up ranged from 0.5 to 11.1 years (median 6.5). The 10 year actuarial probability of local tumour control for the entire group was 91% and the 5 and 10 year actuarial survivals were 82 and 53% respectively. Only three patients have died from their primary disease, and three have had local recurrences, all readily controlled with mastectomy. Sixteen patients remain alive and well, but six have died from inter-current illness. No significant radiation complications have occurred although eight patients (28%) have developed a second primary malignancy over the ten year period. Local excision plus radiotherapy appears to be an effective alternative approach to the local treatment of primary breast cancer in selected patients. Recommendations are made concerning axillary node sampling, interstitial brachytherapy and adjuvant chemotherapy for patients treated by this technique.