High frequency of germ-line BRCA2 mutations among Hungarian male breast cancer patients without family history.

To determine the contribution of BRCA1 and BRCA2 mutations to the pathogenesis of male breast cancer in Hungary, the country with the highest male breast cancer mortality rates in continental Europe, a series of 18 male breast cancer patients and three patients with gynecomastia was analyzed for germ-line mutations in both BRCA1 and BRCA2. Although no germ-line BRCA1 mutation was observed, 6 of the 18 male breast cancer cases (33%) carried truncating mutations in the BRCA2 gene. Unexpectedly, none of them reported a family history for breast/ovarian cancer. Four of six truncating mutations were novel, and two mutations were recurrent. Four patients (22%) had a family history of breast/ovarian cancer in at least one first- or second-degree relative; however, no BRCA2 mutation was identified among them. No mutation was identified in either of the genes in the gynecomastias. These results provide evidence for a strong genetic component of male breast cancer in Hungary.

Allelic imbalance and microsatellite instability in BRCA1 associated breast and ovarian tumors.

We have investigated the involvement of microsatellite instability (MSI) and allelic imbalance (AI) at chromosome 13q and 17 in 41 breast and 41 ovarian carcinomas and their association with BRCA1 and BRCA2 gene mutations. MSI was detected in 20% of ovarian and 7% of breast tumors. AI at the BRCA1 locus was detected in 59% and 32% of ovarian and breast tumors, respectively. At the BRCA2 locus, AI rates were 49% and 44% for ovarian and breast tumors, respectively. Germline BRCA1 mutations, identified in 5 (12%) ovarian tumors and in one (2%) breast tumor were not associated with MSI. In only 2/5 BRCA1 positive tumors loss of the wild-type allele was observed. We conclude that BRCA1 mutation status is not associated with MSI and that MSI found in a fraction of ovarian tumors may reflect possible mutations in one of the DNA mismatch repair genes.

Intrathoracic vagus nerve tumors: a report of two cases and review of the literature.

We have observed 741 mediastinal tumors during the past 25 years. Among these tumors, two intrathoracic vagal tumors were found; one bilateral neurofibrosarcoma and one neurilemmoma. To date, 49 intrathoracic vagal tumors have been reported in the literature, and the neurofibrosarcoma described here is the fourth malignant vagal tumor reported.

Surgery in Hungary.

After providing data on the geography and demographics of Hungary, the 1100 years of Hungarian history are briefly surveyed. The introduction, development, and present state of medical education and, in particular, surgical education, including specialty training, are discussed. Attention is devoted to the organization of the surgical community and of surgical manpower in the country. Various disease patterns and their influence on surgical practice are discussed. Attention is also devoted to health care provision and research activity. Finally, the future of surgery, especially general surgery, is outlined.

Clinical validity of new ultrasound methods in the differential diagnosis of breast diseases.

The clinical application of the extended-field-of-view (EFOV) technique, 3D ultrasonography (3D US) and a newly developed US method using a homogeneous tissue equivalent phantom, the so-called attenuation in phantom (AIPH) method, was evaluated in 200 patients with breast disease. These methods improved differentiation both in the preoperative diagnostic and in the postoperative follow-up. EFOV made possible to see large or multiple lesions and also their vasculature in a single image, allowing exact measurement, comparison and better assessment of traditional criteria. In addition, costs and examination time were also reduced. The possibility of viewing the spatial arrangement and the internal and external surfaces of the lesions improved preoperative decision making. Finally, the newly developed AIPH method allowed excellent evaluation of the posterior acoustic pattern of the lesions even when the mass was near the chest wall. For this reason, it was easier to distinguish malignant lesions from benign lesions of the breast.

Surgery of primary retroperitoneal tumours.

Upon detecting 112 retroperitoneal space-reducing processes the authors operated on 62 adults with primary retroperitoneal tumours (12 benign and 50 malignant). The diagnostics and complex therapy of these tumours are dealt with. It is established that although the CR, MRI and USG have resulted in a considerable development, the therapeutic results are still not much better. In the surgical management of benign lesions total tumour extirpation is recommended even with extensive multivisceral resections. For tumours of low grade malignancy the authors have the same standpoint. In case of PRTs of high grade malignancy--and a considerable number of patients belongs to this group--the ultraradical interventions are not associated with promising results and therefore are not recommended by the authors. In the complex management of primary retroperitoneal tumours the achievements of radio- and chemotherapy are nowadays rather only promising, rather than notable.

Ovarian metastases of colorectal tumours.

The authors, based on their own experience and review of the literature, discuss the incidence of ovarian metastases of patients with colorectal tumours. They take a stand for the prophylactic oophorectomy in the management of female patients over 40 years operated on for rectal cancer localized on the left colon side.

Thoracic surgery of testicular cancer patients.

Between 1 January 1980 and 31 December 1991 the authors treated 1450 patients with malignant testicular tumours. Out of them, in 42 patients aged 18-43 years with stage III non-seminoma germ cell testicular cancer, thoracic surgical interventions took place on 44 occasions since the combined cytostatic treatment did not result in a sufficient regression, or disease progression occurred. In one case the lesion was inoperable. In 43 cases successful resection was performed. In 27 cases unilateral, in two cases bilateral thoracotomy and in 15 cases median sternotomy took place. Solitary lesions were found in 26 and multiple ones in 18 cases, respectively. Two patients died in the direct postoperative phase. Forty patients were followed up for 4 to 130 months. Due to disease progression four and seven patients were lost within 12 and 12-24 months, respectively. Currently, 31 patients are alive 4 to 130 months following surgery (29 of them tumour-free, two with tumour). Based on adequate indications the thoracic surgery is justified both from diagnostic and therapeutic points of view. The metastasectomy might offer an advance in the management of these patients.

Comparative evaluation of manual and instrumental rectum resections.

The authors compared the perioperative results of 125 + 125 Dixon operations performed at two time intervals (1956-1979 and 1985-1990), the postoperative recurrences and the survival data. It is established that, from the anastomosis point of view, the instrumental resections--beside their rapidity and favourable sterility--are safer interventions associated with less complications. With respect to local recurrences the two methods do not differ significantly. A further advantage of the double stapling is that a larger number of patients can undergo a sphincter-saving resection, and a lower lying anastomosis can be performed than with hand-sewn sutures.

Oncogene patterns in breast and ovarian carcinomas.

Nineteen paraffin-embedded breast cancer tissue samples selected for long survival (more than 5 years) were analysed for detecting the amplification of the c-erbB-2 (Her-2/neu) oncogene by Polymerase Chain Reaction (PCR). Strong correlation was elucidated between c-erbB-2 amplification and survival; such correlation was also observed with histopathologic types and nuclear grading. Because of the similarity of the breast and ovarian cancer in the etiology of the diseases, amplification of c-erbB-2, c-myc and Ki-ras genes was examined in 32 ovarian carcinoma samples (stage I-IV). In ovarian carcinomas c-erbB-2 amplification occurred in 34% (11/32) of the fresh tumour samples, and correlation between amplification and clinical staging at P < 0.05 significance level was observed. Amplification of c-myc was detected in 9% (3/32) and none of the tumours showed amplification of Ki-ras.

Influence of hormonal status of patients with cystic disease on the composition of cyst fluid and breast cancer risk.

The relationship between the composition of breast cyst fluid (BCF), the menstrual status and in addition some endocrine events in the history of patients (n = 131) with gross cystic breast disease was investigated. The dehydroepiandrosterone (DHA) levels in type II (K+/Na+ < 1) cysts of the follicular group were significantly higher compared to the type II cysts of the luteal or postmenopausal groups. For testosterone a significant difference existed between the type I (K+/Na+ > or = 1) follicular and type I postmenopausal groups. Estrone levels were significantly higher in type I BCF of patients in the luteal phase compared to both the follicular and postmenopausal type I cysts. Progesterone levels were lowest in the postmenopausal subgroups (both in type I and II cyst). Significant correlations were found between the number of pregnancies and the levels of DHA-sulfate and also progesterone in BCF. DHA levels were correlated with the period of lactation. The K+/Na+ ratios were the lowest in women who lactated for the longest period. The estrone was lowest in BCF of current oral contraceptive (o.c.) users while the estradiol was lowest in patients who had never used o.c. A history of previous o.c. use was associated with a significantly high mean DHA level. A significantly higher DHA and lower testosterone level were demonstrated in BCF of patients who had some previous gynecological interventions. The composition of BCF and the "life of cysts" and thus the rate of breast cancer risk may depend on hormonal status during the menstrual cycles or postmenopause and also on endocrine history of patients.

Correlation of biochemical tumor markers with conventional pathological features in primary breast cancer.

In this prospective study the correlation of pathological with biological prognostic factors and serum tumor markers has been investigated in 574 patients with primary invasive breast cancer. The p53 protein and Bax level correlated positively with tumor size, lymph node status and histological grade. The serum levels of CEA, CA 15.3, TPA-M and TK correlated with tumor extent. There was a significant difference between pre- and postmenopausal breast cancer patients in serum levels of TPA-M and cytosol levels of Bax. Whether these correlations can help in predicting the prognosis of breast cancer by providing additional information with respect to the conventional factors, will have to be investigated by several years of careful clinical follow-up.

Gamma-chain heavy-chain disease with primary thyroid plasmacytoma.

A case of gamma-chain heavy-chain disease was associated with primary thyroid plasmacytoma. The patient has been free of complaints for two years since the removal of the tumorous thyroid. The heavy-chain fragment (molecular weight, 32,000 daltons) has disappeared from the serum and urine, while the previously low levels of normal immunoglobulins have increased. The immunoglobulin anomaly may be correlated with the chronic antigen stimulus.

[Gigantomastia in pregnancy]. / Terhességi gigantomastia.

A 26 year old women presented with huge enlargement of breasts from 10th week of her first pregnancy. Both breast became very swollen and painful, more than tripled in size. The skin became oedematous, red, and subsequently ulceration and bleeding occurred. Interruption performed at the 12th week, it didn't stop the process. 3 months later bilateral mastectomy with free transplantation of the nipples and the areoles to the abdominal wall was performed. The pathologic diagnosis was hyperplasia of the connective tissue elements with fibroadenomas. 6 and 8 years later she delivered healthy babies. The authors review the literature of gigantomastia during pregnancy with discussion of the treatment.

[Diagnosis and therapy of retroperitoneal tumors]. / A retroperitonealis daganatok diagnosztikája és therapiája.

Upon detecting 112 retroperitoneal space reducing processes the authors operated on 62 adults with primary retroperitoneal tumours. Of them there were 12 benign and 50 malignant tumours. The diagnostics and complex therapy of these tumours are dealt with. It is established that although the CR, MR, and USG have resulted in a considerable development the therapeutic results are still not far better. In the surgical management of benign lesions total tumour exstirpation is recommended even with extensive multivisceral resections. For tumours of low grade malignancy the authors have the same standpoint. In case of retroperitoneal tumours of high grade malignancy--and a considerable number of patients belongs to this group--the ultraradical interventions are not associated with promising results therefore they are not recommended by the authors. In the complex management of primary retroperitoneal tumours the achievements of radio- and chemotherapy are nowadays rather only promising than notable.

[Cellular DNA determination and its significance in the identification of tumor recurrences, metastases and second primary tumors]. / A sejtenkénti DNS tartalom meghatározásának jelentösége daganat recidívák, metastasisok, illetve második primer tumorok azonosításában.

The DNA content of 30 malignant tumors and their metastases were compared. The DNA indices of tumors (expressed by the mean DNA content of G0.1-phase tumor cells and leukocytes) differ from each other, but in metastases they are almost the same with the values of primary tumors. It can also be observed, if a tumor consisted of more than one subpopulation of tumor cells, the metastatic potential of clones can be different. However, there is no significant relationship between the degree of ploidy and the occurrence of metastasis. The stability of DNA index in tumors and their metastases were used to distinguish two primary tumors even if their histological patterns were very similar. We present an example of the above mentioned phenomena in which the results of DNA analysis is supported by the results of mucin histochemistry and the follow up data.

[Pseudomyxoma peritonei]. / Pseudomyxoma peritonei.

With reference to the clinical courses of three patients treated at the Department of Surgery, National Institute of Oncology, Budapest, the literature on the diagnosis and treatment of peritoneal pseudomyxoma has been reviewed. The disease is considered to be a rare low grade malignancy. Abdominal ultrasonography, computer tomography and laparoscopy are of significance in the diagnosis of this disease which is yet confirmed usually during surgery. The treatment is principally surgical, the results of chemotherapy are modest. The disease has a slow course and is susceptible to recurrence. Despite of early diagnosis and radical surgery cure can be reached rarely.

[Giant lipoma of the ligamentum teres hepatic]. / A ligamentum teres hepatis nagy kiterjedésü lipomája.

The authors succeeded in removing a lipoma of 20 x 18 x 15 cm in size. It originated from the ligamentum teres hepatis of a 66-year old man. This is the first case described in the Hungarian and second in the world literature. Based on their observations the anatomy, physiology and pathology of the ligamentum teres hepatis are discussed.

[Retroperitoneal Castleman tumor]. / Retroperitonealis Castleman-tumor.

Authors removed a 4 cm diameter tumour from below and behind of the hepatic hilum of a 40 y. o. woman. Histology revealed Castleman disease. 40 retroperitoneal Castleman tumour can be red in the literature, without any Hungarian one. The pathological and clinical characteristics of Castleman tumour are discussed, literature is surveyed.

[Malignant schwannoma of the breast]. / Az emlö malignus schwannomájáról.

Authors describe a malignant solitary schwannoma observed in the left breast of a 67-year old woman. Eight months after tumour removal the patient is symptom and complaint-free. The present case of breast schwannoma is the first clinical observation in the national and the 14th one in the world literature.