Characterization of the apical membrane antigen-1 in Italian strains of Babesia bigemina.

Babesia bigemina is a parasite endemic in different parts of the world, including Europe and the Americas. One of the few genes characterized in this species codifies for the Apical Membrane Antigen 1 (AMA-1), a trans-membrane antigen recently identified. In this research, we characterized the ama-1 gene from three Italian B. bigemina strains, two B. bigemina strains obtained from Ragusa, Sicily (ITA1 and ITA3) and a third one obtained from Benevento, Campania (ITA2). Italian sequences were compared with those of the Australian strain obtained from the Sanger Institute web site and to strains from different parts of the world. The results obtained confirmed that this newly described ama-1 gene is highly conserved among Italian and foreign strains which has implications for vaccine development.

Prevention of self-mutilation in patients with Lesch-Nyhan syndrome: review of literature.

There have been no standard methods established to prevent the self-mutilation seen in LNS. Preventive treatment must be developed, therefore, for each patient based on clinical findings. The first method to be utilized should be a nonsurgical splint or mouth guard. If it proves to be unsuccessful then the benefits of surgical procedures should be considered. In the future, the use of medication, such as carbamazepine, may prove to restructure the current approach advocated by the literature today.

Reoperation for locally recurrent breast cancer in patients previously treated with conservative surgery.

BACKGROUND: This study aimed to analyse the possibility of surgical rescue of intrabreast tumour recurrence (IBTR) following conservative operation for breast cancer, i.e. quadrantectomy, axillary dissection and radiotherapy. METHODS: Of 2544 patients treated with this approach, 209 presented with an IBTR as the first and only sign of relapse. Some 197 patients were considered suitable for further surgery; 12 were inoperable. Six patients declined operation. RESULTS: Reoperative surgery was total mastectomy in 134 patients (70 per cent) and further local resection in 57 (30 per cent). Median follow-up after second surgery was 73 (range 1-192) months. The overall survival probability at 60 months was 70 per cent after mastectomy and 85 per cent following further local excision. There was no difference in disease-free survival between the two operative groups. Second IBTR was more common at 5 years in the re-excision group (19 versus 4 per cent). CONCLUSION: Since the type of surgery did not seem to affect survival, breast conservation can be considered in selected patients with IBTR.

Ten year results of a randomised trial comparing two conservative treatment strategies for small size breast cancer.

We report the 10-year results of a randomised clinical trial in which two different breast conservation treatment strategies were compared in women with small, non-metastatic primary breast cancer: quadrantectomy, axillary dissection and radiotherapy (QUART) versus tumorectomy and axillary dissection followed by external radiotherapy and a boost with 192Ir implantation (TART). No second surgery was given to women with affected surgical margins. Axillary node positive women received adjuvant medical therapy. From 1985-1987, this trial accrued 705 patients, 360 in the QUART and 345 in the TART arm. Crude cumulative incidence curves for intrabreast tumour recurrence (IBTR) and metastases as first events and mortality curves in each of the two treatment arms were computed. A crude cumulative incidence curve of IBTR as a second event (in women who had already had a local recurrence) was also computed. The two groups were compared in terms of hazard for IBTR, metastases or death occurrence by using Cox regression models, both with and without adjustment for patient age, tumour size, number of metastatic axillary nodes and histology. Possible interactions between the aforementioned prognostic factors and the type of surgery were also investigated. The two groups were well matched for baseline patient and tumour characteristics, the only exception being resection margins, which were more often positive in the TART group. At the Cox model, a significant difference between groups was detected for IBTR (P < 0.0001), but not for distant metastases and overall survival. In particular, 5- and 10-year estimates of crude cumulative incidence of IBTR were 4.7 and 7.4% in the QUART group and 11.6 and 18.6% in the TART group. The difference was not substantially affected by patient or disease characteristics. Likewise, the status of resection margins in women who underwent TART treatment did not significantly influence the risk of occurrence of IBTRs. Finally, the rate of second IBTR occurrence was relatively high, when compared with the rate of IBTR occurrence as first event. In summary, the results of this trial show that a better local control of the disease can be obtained with the more extensive surgical resection, i.e. QUART.

Efficacy, toxicity, and applicability of high-dose sequential chemotherapy as adjuvant treatment in operable breast cancer with 10 or more involved axillary nodes: five-year results.

PURPOSE: To assess the efficacy, toxicity, and applicability of high-dose therapy administered as adjuvant initial treatment to women with breast cancer with extensive nodal involvement. PATIENTS AND METHODS: Sixty-seven patients with stage II to III breast cancer involving > or = 10 axillary nodes received a novel high-dose sequential (HDS) regimen, including the high-dose administration of three non-cross-resistant drugs (cyclophosphamide, methotrexate, and melphalan) given within the shortest interval of time as possible with hematologic and nonhematologic toxicity. RESULTS: Sixty-three patients completed the program as planned, one patient died of acute toxicity, and three patients were switched to standard-dose adjuvant therapy. After a median follow-up duration of 48.5 months and a lead follow-up of 78 months, actuarial relapse-free survival for all 67 registered patients is 57% and overall survival is 70%, respectively. Comparison with a historical control group of 58 consecutive patients showed a significantly superior rate of freedom from relapse for the HDS-treated group (57% v 41%, respectively), in particular when two subgroups of patients, more homogeneous for their number of involved nodes, were compared (65% v 42%). Overall, treatment was of short duration (median, 70 days), required a median of 32 days of hospital stay, and was associated with only a few severe side effects (the most distressing being oral mucositis after melphalan therapy). CONCLUSION: HDS therapy emerges as an effective and applicable regimen, whose major toxicity was occasional. Final assessment of its value in a randomized, multicenter trial is presently underway.

Malignant pheochromocytoma in multiple endocrine neoplasia type 2B syndrome. Case report and review of the literature.

A malignant behavior (i.e., distant metastatic spread) has been recorded in 3-4% pheochromocytomas occurring in the context of multiple endocrine neoplasia type 2A syndrome, but has never been documented in patients with the type 2B form. In this report we describe a case of malignant pheochromocytoma arising in the latter syndrome setting. The patient, a white young male, had the full-blown syndrome, including multicentric, bilateral medullary thyroid carcinoma metastatic to regional lymph nodes, mucosal neuromas, digestive ganglioneuromatosis, marfanoid habitus, and bumpy lips. Three and a half years after surgical resection of an apparently benign adrenal pheochromocytoma he developed widespread osseous metastases. The presence of hypertensive crises and high urinary catecholamine excretion rates, coupled to moderate hypercalcitoninemia, normal circulating carcinoembryonic antigen levels, negative whole-body 99mTc-(V) dimercaptosuccinic acid scan, and absence of neck or mediastinal disease by magnetic resonance imaging, proved that the metastases were from his previous adrenal and not thyroid tumor. Furthermore, since the bone metastases strongly accumulated 131I-metaiodobenzylguanidine, several courses of the radiocompound were given, which resulted in an objective, though partial, tumor regression.

Prognosis of breast cancer in males: an analysis of 170 cases.

A statistical analysis was performed on a series of 170 consecutive cases of operable (M0) breast cancer in males. All the patients underwent surgery. The end-points considered were: (i) overall mortality, (ii) all neoplastic events and deaths without evidence of breast disease (first event). Five- and ten-year overall mortalities were 26.9 and 54.3%, respectively. A multiple regression analysis showed that tumour size and nodal status (pT and pN) were statistically significant as prognostic factors. With regard to first events, 12 local recurrences (thoracic wall), one nodal relapse in the axilla and one contralateral tumour were observed. Primary tumours, other than breast cancer, occurred in 11 patients. The observed probability of surviving at 10 years from the treatment was definitely lower than that of the general population. For the follow-up periods of 0-5 and 6-10 years, the excess death rate per 100 man-years was 9.98 and 13.43, respectively. It appears from the analysis that prognosis of breast cancer is worse in men than in women.

"Occult" papillary carcinoma of the thyroid: a questionable entity.

A series of 72 cases of "occult" thyroid papillary cancer, i.e. tumours of less than 1.5 cm in diameter, was analysed. The patients--26 males and 46 females--were treated surgically, 25 by lobectomy and isthmusectomy and 47 by total thyroidectomy. In 51 cases nodal neck dissection was performed, bilateral in 2 cases. 9 thyroidectomised patients received radiometabolic therapy. Hormone therapy (T4) was continuously administered to 57 patients. The median duration of follow-up was 99 months (60-189). All the patients were alive (except one who died from other causes) and free of disease at last control. No relapses in the thyroid were observed in the conservatively treated patients. 2 patients of the 47 radically operated upon subsequently presented nodal metastasis and underwent neck dissection. The so-called "occult" thyroid papillary cancer does not differ from other papillary cancers with respect to morphological, clinical and prognostic factors--it differs only in size. Considering occult papillary tumours as an entity is questioned in this paper.

Surgery for local recurrences following deficient radical mastectomy for breast cancer: a selected series of 39 cases.

Thirty-nine patients with locally recurrent cancer, previously treated elsewhere by mastectomy, were considered. At clinical examination, doubts arose as to the efficiency of previously performed mastectomies. Recurrent lesions, in the absence of distant metastases, were nodular, cutaneous or subcutaneous, in the area of previous 'radical mastectomy'. Second surgery consisted of a wide excision together with a surgical revision of axilla. No radiotherapy was administered to the thoracic wall after surgery. Adjuvant chemotherapy (CMF) was given to 26 node-positive women. Median follow-up was 48 months. Pathological reports showed that portions of mammary gland and axillary lymph nodes had been left behind by primary surgery in 29 and 34 cases, respectively. In 26 cases lymph nodes were metastatically involved. Local control has been maintained in 32 patients, 21 of whom are alive and free of disease.

Malignant schwannoma of the breast.

A case of rare, malignant schwannoma of the breast has been analysed with regard to its history and diagnostic problems, such as the mammographic pattern and the role of cytology in the preoperative mesenchymal suggestion.

Surgical treatment of phyllodes tumors of the breast.

Eighty-one female patients with phyllodes tumors of the breast, surgically treated from 1974 to 1983, were studied. Their age ranged from 9 to 88 years. According to histology, the series was divided into three groups, of 28 (34.5%) benign tumors, 32 (39.5%) border-line tumors, and 21 (25.9%) malignant tumors. Because ten patients were lost to follow-up, only 71 women could be evaluated. All the patients had received surgical treatment: 51 women had been treated conservatively (11 enucleations, 40 wide resections), and 20 had undergone radical operations (13 underwent total and five underwent subcutaneous mastectomies, whereas one underwent modified and one underwent radical mastectomy). The mean follow-up, for the three groups, was 106 months for benign, 84 months for borderline, and 82 months for malignant tumors; in no case was radical surgery followed by local recurrence: of 51 women conservatively treated, 14 experienced local relapse, i.e., one of 24 women with benign, ten of 22 with borderline, and three of 8 with malignant lesions. Only two of 47 patients (4.2%) with borderline or malignant tumors developed distant metastasis and died from disease. No relationship between tumor size and risk of local recurrence could be demonstrated, and no difference could be identified between borderline and malignant lesions, in terms both of local and distant relapse. Local recurrences do not appear to affect survival: as a consequence, wide resection should be the primary treatment. Enucleation is to be proscribed. Total mastectomy has been indicated for very large tumors and for local recurrences of borderline and malignant lesions. Axillary dissection is not worthwhile.

Complications in surgery of the right colon.

One hundred and fifteen cases of right hemicolectomy for cancer or others pathological conditions, operated on over a 5-year period, were evaluated. The overall incidence of complications was high (47%). A more thorough analysis of data showed that infective complications, such as bronchopneumonia (27.8%) and urinary infections (10.4%) markedly affected this incidence. In contrast, there was a much lower incidence of surgical complications mainly wound infection (14.8%) and anastomotic fistulas (4%), the incidence of which was lower than that reported in the literature. Furthermore, the difference between stapled and hand-made sutures, in terms of fistulas, was only 2%, in favour of the former.

Surgical treatment of 43 retroperitoneal sarcomas.

Forty-three cases of primary retroperitoneal sarcomas, observed and treated from 1970 to 1983 at this Institute, were analysed. The series consisted of 16 liposarcomas (37%), 10 leiomyosarcomas (23%), 7 rhabdomyosarcomas (16%), 5 fibrosarcomas (12%), 2 malignant histiocytomas (5%), 2 sarcomas NOS (5%) and 1 mesenchymoma (2%). All the patients underwent surgery. In order to evaluate the results of surgery, the patients were divided into three groups, according to the type of operation performed, that is open biopsy, resection and excision. Survival of the patients in the first group never exceeded 24 months. The symptom-free period for the patients treated by incomplete removal of the tumour (second group) lasted 3-24 months. Further surgery, in three cases for this group, did not result in a useful control of the disease. As regards radically treated cases, local recurrence was observed in 3 of the 7 liposarcomas, 2 of the 5 leiomyosarcomas, 1 of the 2 rhabdomyosarcomas and 1 fibrosarcoma. Out of 16 cases of the third group, regularly followed up, only 3 patients (liposarcomas) were alive and free of disease at 5 years from first operation. Overall 5-year survival for this group was 31.9%; disease-free survival was 18.7%. For the whole series of 43 cases, overall 5-year survival was 11.4%. As far as histology was concerned, liposarcomas showed the highest operability rate: out of 16, 7 were resected and 9 radically excised. There is a lack of convincing evidence for the utility of post-operative chemotherapy. On the contrary, post-operative radiotherapy seems to be worthwhile for liposarcomas, especially after non-radical operations.

The surgeon's attitude to malignant lymphomas of the breast. Analysis of eight cases.

Eight cases of malignant lymphomas of the breast were considered. The age of the patients ranged from 26 to 57 years. All the cases were staged according to the Ann Arbor classification system; the distribution was 2 Ie, 2 IIe, 1 IIIe and 3 IVe. Surgery was limited to excisional biopsy in all patients, one of whom had been operated on elsewhere. Six patients had radiotherapy following limited surgery. All of them received chemotherapy. The treatment resulted in complete local control of the disease in all cases and only 2 patients died from disseminated disease. Six patients were alive and free of disease from 34 to 84 months after the diagnosis. Our experience and analysis of the literature allow us to conclude that the role of surgery in the diagnosis and treatment of malignant lymphomas with breast involvement should be limited to removal of the nodule, when possible, or to incisional biopsy in the case of massive involvement of the breast. In effect, aggressive surgery with a radical aim is not presently justified; in contrast, systemic chemotherapy should always be administered.

Clinical considerations on 8 cases of abdominal carcinoids.

Eight cases of abdominal carcinoids are reported, 5 males and 3 females, whose age ranged from 21 to 66 years. The site of the carcinoids was appendix in 3 cases, ileum in 3, cecum in 1 and the ovary in 1. Synchronous metastases were present at regional lymph nodes and liver in 5 of the 8 cases. Radical surgery was performed in 3 patients, whereas in 3 cases nonradical surgery, due to the presence of nodal or hepatic metastases, was followed by chemotherapy (2 cases) and radiotherapy (1 case). The patients radically operated on are alive and free of disease at 22, 27 and 27 months. Surgery should be resorted to, when possible, even in the presence of liver metastases, since the life expectancy for these patients is longer than that of patients with liver secondaries from tumors other than carcinoids. Chemotherapy and radiotherapy seem to play a complementary role, if any.

Malignant pheochromocytomas: report of 4 cases.

Four cases of pheochromocytomas are reported: 3 cases of malignant pheochromocytomas of the adrenal glands and 1 case of pheochromocytoma, diagnosed on the basis of clinical and biologic signs, whose site and malignant character were not demonstrated. A rare association of pheochromocytoma and stenosis of the renal artery, recorded for one of the cases, is discussed.

Mesothelioma of the pericardium: report of a case.

One case of cardiac tamponade from malignant mesothelioma of the pericardium is reported. The tumor was diagnosed by cytology of the fluid drained by pericardial centesis. Necroscopy and histology confirmed the diagnosis.

Abdominal and extra-abdominal desmoid tumors. Report of 21 cases.

Desmoid tumors are easily diagnosed when located within the muscles of the abdominal wall. On the contrary, extra-abdominal desmoids, because of their various sites and lack of pathognomonic signs, can be very difficult to diagnose. A review of 21 cases of the Istituto Nazionale Tumori of Milan confirms that these tumors primarily affect young, multiparous women and that even extra-abdominal desmoids are prevalent in women. All patients were treated by radical surgery consisting of wide exicision in 18 cases, hemimandibulectomy in 1 case, and amputation of the lower limb in 2 cases. Radical surgery resulted in no recurrences in all cases but one. Our results are in contrast with the relatively high recurrence rates reported in the literature.