CYSTOSARCOMA PHYLLODES: THE WESTERN AUSTRALIAN EXPERIENCE

Background : Cystosarcoma phyllodes is a rare breast tumour whose behaviour is not well understood by many clinicians. Methods : In 1998 a retrospective study was undertaken of women diagnosed with phyllodes tumour of the breast who had their initial surgery between 1983 and 1994 in Western Australian public hospitals. Results : Forty women were diagnosed and treated over this period; however, only 28 received ongoing follow-up (70%). Follow-up was obtained on 26 of these. The median age at diagnosis in this group was 46 years. Cases were predominantly Caucasian (85.5%). Postmenopausal women were affected in 26.9% of cases. Four patients had recurrences after surgery (one malignant and three benign). No patient had a recurrence with primary tumours with a diameter of ≤ 2 cm. Mean time to recurrence was 35.8 months. Conclusions : Breast-conserving surgery was used in almost all cases (96.2% of first operations).     

乳腺叶状囊肉瘤24例临床分析

目的探讨乳腺叶状囊肉瘤的临床特点、外科治疗方法及影响预后的因素。方法对24例乳腺叶状囊肉瘤患者采用局部切除11例,单侧乳房切除术10例,乳房切除并腋窝淋巴结清除3例,其中5例(20%)曾诊断为乳腺腺纤维瘤或巨纤维瘤因术后肿瘤复发而经历2~3次局部切除术。结果术后病检证实:低度恶性11例,中度恶性9例,高度恶性4例。随访1~10年(平均5.2年),局部复发率29.1%,5年生存率97%。结论乳腺叶状囊肉瘤发病率低,易误诊;手术切除是其首选的治疗方法,局部切除术后复发应行乳房切除;多数乳腺叶状囊肉瘤恶性程度较低,进展缓慢,预后较好;乳腺叶状囊肉瘤预后与肿瘤的病理类型、肿瘤大小和手术切除是否彻底有关。     

A Giant Juvenile Fibroadenoma in a 12-Year-Old Girl: A Case for Breast Conservation

Abstract: A 12-year-old girl presented to our service for evaluation of a rapidly enlarging 16 cm breast mass. The mass was removed by local excision and diagnosed to be a giant juvenile fibroadenoma. She had normal breast development over a 1-year postoperative follow-up period. We present this case to illustrate the diagnosis and management of large breast tumors in the adolescent age group, and to emphasize that these tumors are almost always benign and should be treated with breast-conserving surgery.     

Infiltrating ductal carcinoma developing within cystosarcoma phyllodes —A case report—

Malignancy in cystosarcoma phyllodes is uncommon and often confined to the stromal element. An extremely rare case of infiltrating ductal carcinoma developing within the stroma of cystosarcoma phyllodes is reported herein. A breast tumor with a diameter of 15 cm, which was diagnosed as cystosarcoma phyllodes, was excised from the right breast of a 47-year old woman. The histopathological examination revealed that hyperplastic ductal epithelial cells with dark cytoplasm and enlarged hyperchromatic nuclei were infiltrating the stroma. Thus, a diagnosis of ductal carcinoma within cystosarcoma was made. Subsequently, a standard radical mastectomy was performed. No recurrence or metastasis has been observed over the post-operative period of 5 years and 6 months.     

乳腺叶状囊肉瘤的诊断和治疗

目的 提高乳腺叶状囊肉瘤的诊治水平。方法 对我院1987—2001年间12例乳腺叶状囊肉瘤病例分别采用全乳切除术、全乳切除加腋淋巴结清扫、乳房大部切除术和乳房肿块切除术。其中1例发生纵隔转移再次手术切除。结果 12例均预后良好。结论 乳腺叶状囊肉瘤是一种罕见疾病，诊断主要靠病理组织学，病理可分为良性、恶性和临界型。主要的治疗方法是手术，良性肿瘤可选择包括肿瘤并距其边缘相当距离的健康组织在内的广泛局部切除术或全乳切除术，恶性选择全乳切除术更为合适。     

Malignant cystosarcoma phyllodes with lymph node metastasis —A case report—

A case of malignant cystosarcoma phyllodes with metastasis in the interpectoral lymph node (Rotter's) is presented in this paper. To the author's best knowledge, this is the first case in Japan on this disease, with lymph node metastasis. Although the surgical management of the disease has not yet been standardized, radical or modified radical mastectomy is thought to be the appropriate procedure.     

TUBERCULOSIS OF THE THYROID GLAND: A CLINICOPATHOLOGICAL PROFILE OF FOUR CASES AND REVIEW OF THE LITERATURE

Four cases of tuberculosis of the thyroid with different presentations including chronic sinus (following drainage of thyroid abscess), thyrotoxicosis, severe dysphagia clinically mimicking malignancy and euthyroid multinodular goitre are described. Except in the case presenting with chronic sinus (discharging acid-fast bacilli), the diagnosis was a pathological surprise (cytopathology in one and histopathology in two). Only in one case was there evidence of disease outside the cervical region. All cases showed multiple coalescing and caseating epitheloid cell granulomas along with giant cells, which are considered as diagnostic of tuberculous thyroiditis even if acid fast bacilli cannot be demonstrated. The literature is reviewed and the pathogenesis discussed.     

BRAIN ABSCESSES: REVIEW OF 30 CASES TREATED WITH SURGERY

Thirty patients with brain abscesses who were treated at the Department of Neurosurgery, Tan Tock Seng Hospital, Singapore, during the period July 1989 to July 1993, were reviewed. There were seven females and 23 males with mean follow up duration of 19 months. The predisposing factors identified were otogenic infections in 10 cases (33%), paranasal sinus infection in 2 cases (7%) and congenital heart disease in 6 cases (20%). All cases underwent aspiration or excision of the abscess. Repeat operations were carried out if the abscess reaccumulated. A total of 49 aspirations and 16 excisions were performed. Twenty-five patients (84%) made a good recovery, three patients (10%) were moderately disabled, one patient (3%) was severely disabled and one patient (3%) died. There was no postoperative haemorrhage or wound infections. Two patients (7%) developed hydrocephalus and three (10%) had seizures postoperatively.     

MICROPAPILLARY BLADDER CARCINOMA: A CLINICOPATHOLOGICAL STUDY OF 20 CASES

PURPOSE: Micropapillary bladder carcinoma is rare, with only 18 cases reported to date. We report 20 additional cases with long-term followup. MATERIALS AND METHODS: A total of 680 patients with an initial diagnosis of bladder carcinoma in western Sweden in 1987 and 1989 were prospectively registered. The clinical records of all 816 patients with bladder cancer treated at Sahlgrenska University Hospital with external beam irradiation between 1962 and 1989 were reviewed. The histopathological material was reviewed and immuno-histochemical analyses were performed on 20 cases identified with micropapillary bladder carcinoma. RESULTS: The incidence of micropapillary bladder carcinoma was 0.7%. Mean patient age at diagnosis was 69 years (range 45 to 82) and the male-to-female ratio was 2.3:1. All but 5 patients had stage T3a disease or higher. There was no difference in stage or prognosis between the 5 prospectively identified patients and those treated with external beam irradiation. Only 2 patients had micropapillary bladder carcinoma as the only pattern, while 1 had 10% and the remainder had 20 to 95% micropapillary bladder carcinoma. Transitional cell carcinoma was noted in 17 patients and 5 had areas of gland forming adenocarcinoma. Carcinoma in situ was noted in 13 patients and 15 had lymphatic invasion. Only 5 patients survived 5 years, 1 of whom died of bladder cancer after 7 years. Radiation and chemotherapy did not seem to be effective. CONCLUSIONS: The light microscopic appearance, which is strikingly similar to ovarian papillary serous carcinoma, and immunohistochemical staining pattern lend some support to the theory that micropapillary bladder carcinoma is a variant of adenocarcinoma. Since even the focal presence of micropapillary bladder carcinoma is associated with a poor prognosis, recognition of this entity is important. Due to its rarity, the optimal treatment of micropapillary bladder carcinoma needs to be determined in a multicenter study.     

Malignant phyllodes tumor

The study aims to evaluate the survival and prognosis of patients with malignant phyllodes tumor. Between 1982 and 1998, 37 women with malignant phyllodes tumor were treated at the Regional Cancer Center, Trivandrum. Twelve patients were recurrent. Survival was estimated using the Kaplan-Meier method. Patient, disease, and treatment factors were compared using log-rank test. The Cox-proportional hazard model was employed to identify the prognostic factors. Thirty-six patients had surgery. Twenty-five patients received postoperative radiotherapy, and 2 received chemotherapy in addition. The median follow-up was 43 months (range 1–170 months). Eight patients failed locally, and 7 of these were successfully salvaged by surgery. The 5-year overall survival was 74.2% (95% CI, 0.44 to 0.89), whereas 5-year disease-free survival was 59.6% (95% CI, 0.39 to 0.7). The margin of surgical excision was found to be the only independent prognostic factor (p = 0.003). However, patients with tumor size more than 5 cm (hazard ratio 2.9) were found to have increased hazard, whereas those receiving adjuvant radiotherapy (hazard ratio 0.6), married women (hazard ratio 0.4), and those women over the age of 35 years (hazard ratio 0.7) showed a decreased hazards. Cystosarcoma phyllodes is a rare malignancy of the female breast. Surgery with adequate margins is the primary treatment. Adjuvant radiotherapy appears to improve the disease-free survival.     

手术切除胆囊囊壁附肝组织6例临床病理分析

目的 探讨手术切除胆囊囊壁附肝组织的临床病理意义及其并发症防治。方法 对6例手术切除胆囊囊壁附肝组织进行大体检查,取胆囊系膜面底、体、颈作常规石蜡切片,HE染色,光镜观察。结果 6例手术切除胆囊组织,病理巨检其胆囊壁均未发现有明确的肝组织。而光镜检查发现胆囊体系膜面均有薄层肝组织附着,肝细胞有不同程度的变性,小胆管周围可见慢性炎细胞浸润。结论 手术切除胆囊系膜面囊壁附肝组织表明有胆囊床肝损伤,对于胆囊炎症较重,胆囊周围广泛粘连的病例中,手术中应仔细处理胆囊床,以避免损伤肝组织,减少术后并发症的发生。     

QUALITY OF LIFE AFTER BREAST CONSERVATION OR MASTECTOMY: A SYSTEMATIC REVIEW

Background : For early breast cancer, survival after breast conservation is similar to that after mastectomy. Some women may not have a clear preference and wish to have further information about quality of life experienced after the alternative treatments. This paper describes a systematic review of randomized trials on mastectomy versus breast conservation for which there are data on quality of life or psyehosocial outcomes. Methods : Literature was reviewed to find all randomized controlled trials comparing breast conservation to mastectomy, with quality of life or psychological effects as an outcome. Studies were then critically appraised by two reviewers independently and any disagreements about their quality and results resolved by discussion. Results : A total of six randomized trials met our inclusion criteria. In general, they are of poor quality. Women who had breast conservation had a more favourable body image of themselves than those who had mastectomy in all five studies in which it was examined. The evidence was statistically inconclusive for all the other dimensions measured, namely perceptions of psychological health, sexual health, physical health, fear of the future and global quality of life. Radiotherapy may be a determinant of poorer psychological health and body image. Conclusions : Apart from body image, it is unclear whether breast conservation or mastectomy results in better psychosocial outcomes. Moreover, the studies were done before evidence was available to inform women about the equivalence of survival with these alternative treatments. Therefore there is inadequate information available to help many women decide about their choice of treatment in the future. Preference trials should be conducted, using standardized quality-of-life measures, in which women who are uncertain about which treatment to choose are randomized to breast conservation or mastectomy.     

PROGNOSTIC FEATURES OF TERATOMAS WITH MALIGNANT TRANSFORMATION: A CLINICOPATHOLOGICAL STUDY OF 21 CASES

Purpose

Teratomas with malignant transformation comprise up to 6% of metastatic teratomas. The prognosis of patients with these tumors can vary considerably. We delineate factors that may be related to prognosis in a cohort of men with teratoma with malignant transformation.

Materials and Methods

We analyzed pathological features, treatment, response, recurrence, time to recurrence, subsequent followup and survival for 21 patients (median age 28 years) diagnosed with teratoma with malignant transformation during a 7-year period at our institution.

Results

Malignant nongerm cell elements were present in the primary tumor in 11 cases (52%). Of 18 patients with testicular primaries 17 (94%) presented with metastatic disease. Despite aggressive treatment with surgery and chemotherapy 17 of 21 cases (81%) recurred (median time 6 months). Overall, 5 patients (24%) died of disease (median survival 23 months), 5 (24%) are alive with metastases (median followup 41 months) and 11 (52%) have no evidence of disease (median followup 50 months). Progression/recurrence was substantially greater for 2 of 2 cases with a mediastinal origin, 3 of 4 with rhabdomyosarcomatous differentiation and 5 of 6 with neural differentiation compared with the remainder of the cohort (p <0.05).

Conclusions

Teratomas with malignant transformation are usually metastatic at presentation, have a high recurrence rate and are more aggressive than teratomas without malignant transformation. Prognosis is especially poor for mediastinal teratomas with malignant transformation and for those with neural or rhabdomyosarcomatous differentiation. Complete surgical resection of residual or recurrent disease appears to offer the best chance for prolonged survival.     

ACHALASIA CARDIA: A REVIEW OF 100 CASES

Achalasia cardia is the commonest benign obstructive lesion of the oesophagus in India. We have reviewed 100 cases over a 20-year period. This is the largest series that has been reported from India. Some unusual features were observed. Males were more often affected than females in a ratio of 2.3:1. Children below the age of 10 years are not often affected, but we had 10 subjects in this age group. In our series dysphagia for solids has been the main presenting feature. Barium swallow X-ray studies of the oesophagus were diagnostic. The treatment of choice has been surgical, and a modified Heller's operation has been the chosen procedure. Our preference is for the thoracic approach. Postoperative complications were few. Long-term follow-up of 65 patients over a 19-year period revealed excellent results in SO patients (76.9%). good results in 10 (15.4%), and fair and poor results in two (3.1%) and three (4.6%)patient's respectively. The fair and poor results occurred in patients with recurrence of symptoms or stricture formation due to reflux oesophagitis. The excellent results, even on long-term follow-up, we believe are due to the adequate myotomy that was performed.     

INTUSSUSCEPTION IN CHILDHOOD: A REVIEW OF 139 CASES

A retrospective survey of 128 patients, representing 139 instances of proven intussusception is reviewed. An initial hydrostatic enema reduction attempt was performed in 75% of cases, with 50% of these being successful. A high recurrence rate of 18.4% followed the hydrostatically reduced cases, compared with 2.7% for the surgically treated. Hydrostatic reduction was particularly unsuccesful in infants less than six months of age, when the duration of symptoms exceeded 12 hours and when the presentation is painless. Earlier surgery is recommended for these children.