Intracranial germ cell tumors: association with Klinefelter syndrome and sex chromosome aneuploidies

Intracranial germ cell tumors (ICGCTs) occur mainly in male children and adolescents. Polyploidy of the X chromosome and X hypomethylation have been suggested as mechanisms of malignant transformation independently of the histological tumor type. On the other hand, several reports associate these tumors with Klinefelter's syndrome (KS). Recent reports indicate that KS patients have an increased relative risk for development of malignant mediastinal germ cell tumors and also around 8% of male patients with primary mediastinal tumors have KS. In an attempt to explore the frequency of KS amongst patients with ICGCTs and to confirm the presence of X chromosome polyploidies in these tumors, we studied 13 young male patients with ICGCTs. Paraffin-embedded tumoral and normal tissues were studied by FISH. KS was found in 15% of the cases, demonstrating that this constitutive aneuploidy may be related to carcinogenesis. When tumor and non-tumor tissues were compared, statistically significant X and Y chromosome polyploidies in tumors were revealed. These results emphasize that aneuploidies are involved in ICGCT tumorigenesis.     

Osteogenic sarcoma of the mandible: a plea for radical initial surgery

A total of 24 cases of osteogenic sarcoma of the mandible have been reported to the SEER Program of the National Cancer Institute from 1973 to 1983. Out of 14 patients whose disease was staged as regional, only 9 were eligible for 5-year survival figures and only 2 are alive, for a 23 percent 5-year cure. For those patients staged as localized, only four of six eligible for 5-year cure survived, for a 66 percent 5-year survival. Considering all patients reported to SEER who were eligible for 5-year cure, the rate is 40 percent. We believe this reflects the practice of localized resection of these tumors. At Charity Hospital of Louisiana at New Orleans there have been 10 cases of osteogenic sarcoma of the mandible since 1948. There have been no 5-year survivors for those operated on by other services, usually by local resection. On the Tulane Plastic Surgery Service, a total of six cases of osteogenic sarcoma of the mandible were treated by radical surgery with 100 percent survival from 11 to 22 years.     

Results of combined therapy in patients with nonseminomatous testicular tumors

The results of multidrug therapy of 44 male patients with non-seminomatous testicular tumours are discussed. The diagnosed tumours were of all stages of the clinical advances and were treated with combined chemotherapy (surgery and PVP regimen). Thirty two patients (73%) are followed up between 7 and 103 months and survived, complete remission is noted in 29 patients (66%) including 6 patients who survived over 5 years. Symptoms of the tumour are found in 3 patients. Twelve patients (27%) died. The treatment did not produce life-threatening complications. The obtained results have been compared with those reported by other authors.     

The prognostic significance of the DNA distribution in squamous-cell carcinomas of the uterine cervix

To establish the prognostic parameters of DNA distributions of squamous-cell carcinomas of the uterine cervix, cervical smears from 30 patients were examined cytomorphometrically ten years after the diagnosis had been made. Most of the patients had stage II cervical carcinomas that had been treated with primary combined irradiation. In the DNA histograms, the position of the stemline proved to be the prognostically significant factor. All of the patients with diploid or euploid-polyploid stemlines were still alive after ten years while only 17.6% of those with aneuploid stemlines have survived. No correlation between the ten-year survival rate and the proliferation rate of the tumors could be established.     

Fine-needle biopsy in the early diagnosis of thyroid neoplasms

Among the patients directed to the Department of Endocrinology of the Institute of Internal Medicine for diagnostic examinations of the thyroid, 726 (647 women and 79 men, of age between 16 and 79 years) were qualified to surgery after scintigraphic detection of cold nodules. From this group, 474 patients had thin-needle biopsy specimens taken for cytologic examination. The results of the latter examinations have been compared with those of histologic examination performed on the tissue removed during surgery. The occurrence of a malignant tumor (in 99.1%--thyroid cancer) was found in 108 patients (22.8%) from the group having thin-needle biopsy performed. Comparison of the results of cytologic examination with those of postoperative histologic examination demonstrated low sensitivity of biopsy examination (50.0%), as opposed to high specificity (96.2%) and high accuracy (85, 3%). Analysis of the false-negative results (54/108) revealed that the main causes of errors are difficulties in differentiation of adenoma from follicular carcinoma. Widening of the indications to surgery by a group of patients with adenoma will increase sensitivity to 84.8% and will require including into the operated group of about 50% of patients with scintigraphically diagnosed cold nodules: The introduction of the new term: "urgent strumectomy", has been proposed; also a diagnostic algorithm, useful for early diagnosis of malignant tumors of the thyroid, has been discussed.     

Our experience with surgical treatment of primary cancer of the gallbladder

Witkin 1980-1988, 42 patients (4 males and 38 females) were operated for the gall-bladder cancer; it makes 2.9% of the patients who underwent cholecystectomies. Average age of female patients was 65 and males--71 years. No typical complaints were revealed making possible an early diagnosing of the gall-bladder cancer. The far advanced stages of this cancer were found in 41 cases. In i case no macroscopic features of the cancer were found. Cancer was diagnosed by means of histopathological examination. One patient (woman) survived more than 3 years after operation; 3 women survived more than 1 year. Average survival is 4 months.     

Epidemiological analysis of lung and mediastinal neuroendocrine neoplasms in Japan based on the national database

BackgroundNeuroendocrine neoplasms (NENs) are rare and can originate from any body part. However, there are only few epidemiological studies, especially on lung and mediastinal NENs. This study investigated the epidemiological trends and differences between lung and mediastinal NENs in Japan.MethodsPatients with lung and mediastinal NENs were identified in a national hospital-based cancer registry between 2009 and 2015 in Japan. NENs were subclassified into neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs). NECs were further subdivided into large neuroendocrine carcinomas (LCNECs) and small cell carcinomas (SCCs). We examined the patient characteristics: sex, age, histology, year of diagnosis, diagnostic opportunity, and initial treatment.ResultsWe identified 48,433 patients with 47,888 lung (98.9%) and 545 mediastinal (1.1%) NENs. The commonest subtype of lung NENs was SCCs (87%), followed by LCNECs (10%) and NETs (3%). In the mediastinum, SCCs were also the commonest (48%), followed by NETs (38%) and LCNECs (14%). The number of lung NEN annually increased; however, that of mediastinal NENs did not change over time. The mean age of patients with lung NETs was lower than that of patients with lung LCNECs and SCCs (NETs, 62 ± 14 years; LCNECs, 70 ± 9 years; SCCs, 71 ± 9 years; p < .001). The lung and mediastinal NENs were mainly detected based on symptoms, except for lung NETs. Surgical intervention, including multimodal therapy, was performed for 89.3% of lung NETs (surgery alone: 83.6%), while only 15.6% of lung NECs were treated with surgery. For the mediastinum, 75.9% of NETs were treated with surgery, with 27.1% of cases treated with surgery plus multimodal therapy. Surgery was performed more frequently for mediastinal NECs (37%) than for lung NECs (15.6%).ConclusionsThis study highlights differences in trends of lung and mediastinal NENs. This study’s findings support the importance of epidemiological evaluations based on the primary sites and histological subtypes.     

Klatskin tumor--results of surgical therapy

Between January 1st 1990 and December 31st 1999, 24 patients affected by Klatskin tumor underwent operation in our department of surgery. According to Bismuth's classification, there were 0 (0%) type I, 5 (21%) type II, 6 (25%) type IIIa, 4 (17%) type IIIb and 9 (37%) type IV tumors. Five patients (21%) were treated by curative resection (group I) while in 14 patients (58%) palliative surgical procedure was performed (group II). In 5 cases (21%) the extension of malignancy did not allowed any procedure (group III). Curative resection for malignant tumors of the hepatic duct bifurcation included wide tumor excision and bile duct resection at the liver hilum (with wedge hepatic resection in one patient) and creation of biliary-enteric anastomosis. Palliative surgical procedure included stent insertion. Jaundice was completely relieved in all patients undergoing resection, since 3 patients (21%) after stenting hadn't satisfactory biliary drainage. There was 1 (20%) perioperative death in the group 1, while in group 2, 5 patients (36%) died postoperatively. In this series, the mean postoperative survival of all patients was 16 months. The mean postoperative survival of patients undergoing localized tumor resection with curative intent was 38 months, in contrast to 10 months for those undergoing operative stent insertion. in addition, only 1 patient from group III, in whom only exploratory surgery were performed survived 7 months, while other 4 patients died in the hospital. This retrospective review suggests that aggressive surgical treatment could improve survival and quality of life in patients suffering from Klatskin tumor.     

DNA cytophotometric analysis of breast cancer. Follow-up for 10 years.

Forty-four cases of newly diagnosed invasive ductal breast cancer were studied by static cytometry at the time of diagnosis and were followed for 10 years to determine if the survival rate correlated with the nuclear DNA measurements. In 22 cases the stem line was in the 2c range, in 15 cases in the 3c range and in 7 cases in the 4c range. Thirty-four percent (n = 15) of the patients died of metastases during the first five years, 11% (n = 5) died of metastases between 5 and 10 years, and 55% (n = 24) survived for > 10 years without metastases. No correlation between increased tumor cell proliferation and recurrence of tumors could be found. In this study all patients with diploid tumors, tumor size T1 and negative lymph node status survived for > 10 years. In the presence of node metastasis (T1N1M0) all patients (n = 4) having tumors with the stem line in the 2c range survived for 5 years but only 2/4 for > 10 years. The percentage of patients surviving for 10 years showed no significant differences between the groups: in the 2c range, 59% (13/22); in the 3c range, 53% (8/15); and in the 4c range, 43% (3/7). The small number of cases does not allow definitive statistical conclusions, although the logistic regression analyses showed that stem line, pT and pN were the important prognostic factors for five-year survival. However, only pT and especially pN were of prognostic value for 10-year survival.     

Adult T-cell leukemia-lymphoma with mediastinal involvement and an asymptomatic chronic phase.

A black, West Indian woman with adult T-cell leukemia-lymphoma (ATLL), hypercalcemia, peripheral and retroperitoneal lymphadenopathy, and serum antibodies to human T-lymphotropic virus (HTLV) was found to have massive mediastinal adenopathy, a feature not previously reported in patients with ATLL. In addition, she had had asymptomatic leukocytosis with marked lymphocytosis for at least 6 years before presenting with full-blown ATLL. These findings broaden the clinical picture of ATLL. Cell surface-marker studies and close follow-up are recommended for patients with apparent chronic lymphocytic leukemia, especially if they have pleomorphic lymphocytosis, are younger than usual or are from the Caribbean or Japan.     

Abdominal resection of rectal cancer]

Preservation of anal sphincters in the surgical treatment of rectal cancer is often controversial as far as an extent of surgery, incidence of complications and an increase in mortality rate in perioperative period are concerned. The study was aimed at results of the retrospective analysis of patients, who undergone abdominal excision of the rectum for malignant tumours. Totally 136 patients were operated. Perioperative mortality rate was 5.14%. Totally 26.4% of patients survived for at least 5 years, including 80% of patients operated in A stage of cancer, according to Duke, and 43.3% of patients with tumour in B stage. An emphasis is on the necessity to leave a 2-centimeter margin of healthy tissues around the tumour, provided, that the result of intraoperative histological examination was negative. Such a margin is sufficient for anastomosis. Low anastomoses do not produce early complications and recurrences.     

Consolidative mediastinal radiotherapy for advanced-stage classical Hodgkin lymphoma with bulky disease in patients who achieve complete response after chemotherapy in PET-CT era

BackgroundThe role of consolidation mediastinal radiotherapy (RT) for mediastinal bulky disease in advanced-stage classical Hodgkin lymphoma (cHL) is controversial in the positron emission tomography/computed tomography (PET-CT) era.Materials and methodsWe reviewed the medical charts of patients with advanced-stage (clinical stage IIX–IVX) cHL and mediastinal bulky that achieved a complete response after first line chemotherapy treatment between August 2010 and December 2020 and compared the results of those who received with those who did not receive consolidation mediastinal RT. Inclusion criteria required PET-CT imaging for staging and response assessment.ResultsWe included 115 patients; 91 received mediastinal RT and 24 did not. Patient’s characteristics were balanced between the two groups. The median age in patients that received and did not receive mediastinal RT was 28 years and 24.5 years, respectively. Median International Prognostic Score among patients that received and did not receive mediastinal RT was 2 and 2.5, respectively. Disease free survival (DFS) was statistically better in patients that received mediastinal RT (p = 0.013). Two-year DFS for patients that received and did not receive mediastinal RT was 95.2% [95% confidence interval (95% CI): 87.6–98.2%] and 76.4% (95% CI: 52.2–89.4%), respectively. Overall survival (OS) was not different between the two groups (p = 0.617). In multivariate analysis, not receiving mediastinal radiotherapy and only achieving partial response (vs. complete response) after 2 cycles of chemotherapy were factors predictive of lower DFS.ConclusionDFS, but not OS, was superior in patients that received mediastinal RT.     

Primary mediastinal malignancies: findings in 219 patients

The purpose of this study was to determine the demographics, histology, methods of treatment, and survival in primary mediastinal malignancies. We did a retrospective review of the statewide New Mexico Tumor Registry for all malignant tumors treated between January 1, 1973 and December 31, 1995. Benign tumors and cysts of the mediastinum were excluded. Two hundred nineteen patients were identified from a total of 110,284 patients with primary malignancies: 55% of tumors were lymphomas, 16% malignant germ cell tumors, 14% malignant thymomas, 5% sarcomas, 3% malignant neurogenic tumors, and 7% other tumors. There were significant differences in gender between histologies (P < 0.001). Ninety-four percent of germ cell tumors occurred in males, 66% of neurogenic tumors were in females; other tumors occurred in males in 58% of cases. There were also significant differences in ages by histology (P < 0.001). Neurogenic tumors were most common in the first decade, lymphomas and germ cell tumors in the second to fourth decades, and lymphomas and thymomas in patients in their fifth decades and beyond. Stage at presentation (P = 0.001) and treatment (P < 0.001) also differed significantly between histologic groups. Five-year survival was 54% for lymphomas, 51% for malignant germ cell tumors, 49% for malignant thymomas, 33% for sarcomas, 56% for neurogenic tumors, and 51% overall. These survival rates were not statistically different (P > 0.50). Lymphomas, malignant germ cell tumors, and thymomas were the most frequently encountered malignant primary mediastinal neoplasms in this contemporary series of patients. Demographics, stage at presentation, and treatment modality varied significantly by histology. Despite these differences, overall five-year survival was not statistically different.     

Cytogenetics of extragonadal tumors.

Tissue from six human extragonadal teratomas was obtained for cytogenetic study. These included two gluteal lesions in infant females, and one each of thyroid, mediastinal, sacral and gastric teratomas in males. Tissue culture characteristics indicated tumor growth. All tumors, except the mediastinal lesion showed the presumed karyotype of its host, XX or XY. The mediastinal mass was tetraploid XXYY and contained an X chromatin body and double Y bodies. Our data support the concept that extragonadal teratomas in males and females arise mitotically from diploid cells and are more similar to identical twins. No heterosexual (XX) tumors were seen in males which would be expected in parthenogenetic tumors. The sex chromatin positive tumors seen in earlier studies may have resulted from polyploidy. Thus, male and female extragonadal teratomas have a different origin from the postmeiotic ovarian teratomas.     

Efficiency of radiation or chemoradiation therapy for diffusely growing brainstem tumors in children

Radiation or chemoradiation therapy was performed in 97 children with diffusely growing brainstem tumors. A reduction in the severity of neurological disorders was achieved in 92 (94.8%) patients. The course of the disease was traced in 79 patients. Control studies recorded no complete tumor regression. Following 4-8 months, there was growth resumption of a tumor with its lysis and cyst formation in most children. The principal cause of death was local tumor progression. Six-month survivors were 64 (81%) patients; one-, two-, and three-survivors were 21 (26.5%), 8 (10.1%), and 3 (7.65) patients, respectively; one (2.8%) patient survived 5 years. A combination of radiotherapy and monochemotherapy with temodal, vincristine, oncofer, and theraloc failed to improve immediate and long-term results     

Laparoscopic colon surgery: our results

Formerly, the laparoscopic surgery was accepted as a method of choice for benign diseases, and for palliative operations in progressive stages of malignant diseases of the colon. Today, the laparoscopic surgery of the colon has been also adopted in treating malignant diseases. The first laparoscopic colon resection was performed in our Clinic on December 12, 2002, and 114 patients have been successfully operated until June 1, 2007. Among those 114 patients 56 were men and 57 were women with the average age 65 (ranging from 28-86) years. A series of various laparoscopic operations have been performed for malignant disease mainly (almost 80%). The pre-surgical treatment, preparation of patients and the types of the operations were identical to those applied in patients treated by open surgery. Patients with colon carcinoma have been operated on with the principles of oncologic radicality. In post-operative period we encountered eight complications (four minor and four major) with only one fatal outcome. According to our experience and the facts found in literature, the results of laparoscopic colon surgery are comparable with open surgery.     

Prognostic value of histochemical evaluation of steroid binding sites in breast tumors

Frozen tumor sections and/or cytological smears and aspirates from 614 patients were studied utilizing the fluorescent estrogen binding technique of Lee. Survival analyses of the results indicated that women less than 55 years of age and with positive tumors had a statistically better disease-free interval and survived significantly longer than older patients. The method is thus an independent predictor of survival and disease-free interval in this subgroup of breast cancer patients.     

Orthotopic liver transplantation: the first 60 patients.

Between May 1968 and 31 December 1976 60 orthotopic liver allografts were transplanted by the Cambridge-King''s College Hospital team. During this period there were changes in the selection of patients and their management. Initially some patients were operated on when they were too ill, often because of a prolonged wait for a donor. There was a high incidence of fatal complications of biliary drainage in patients who survived longer than the first week after operation. Improved results have been due to the development of a simple method of preserving the liver, thereby increasing the pool of potential donors, and by a new technique of biliary drainage, which allows well-vascularised anastomoses without tension, retains the sphincter of Oddi, and leaves access for radiological examination of the biliary tree. Uncontrollable rejection of the liver has occurred in less than 10% of cases. This contrasts strongly with the incidence of rejection among kidney transplants. Six patients have lived for over a year, the longest surviving for more than five years, and 13 patients were still alive at the beginning of 1977.     

Mortality rate in subsequent years following myocardial infarction]

Eight hundred out of 997 patients with myocardial infarction hospitalized at cardiological ward in Kielce in 1977-1985 survived. Seven hundred seventy one patients were followed up for 2-10 years. Basing on the obtained results, tables of the mortality rates and probability of survival depending on sex, age, and classification into appropriate prognosis group have been prepared. The highest mortality rate (9.6%) was seen in the first year following myocardial infarction. Mean annual mortality rate in this group was 6% during 10 years of the follow up. Mortality rate in women with myocardial infarction was significantly lower than in men during 10 years of the follow up.     

Net survival in recurrence-free colon cancer patients

PurposeConditional net survival in recurrence-free patients (CNS-RF) provides relevant clinical information and has never been assessed yet in a non-selected colon cancer population. We aimed to estimate conditional 5-year net survival in recurrence-free patients with colon cancer in the population-based Digestive Cancer Registry of Burgundy (France).MethodsCNS-RF was estimated in the 3736 patients resected for cure for primary colon cancer between 1976 and 2006, using a flexible parametric model of net survival for every additional year survived at diagnosis and from 1 to 5 years thereafter.ResultsThe net probability of surviving 5 more years increased from 72% at diagnosis to 92% for recurrence-free patients who survived 5 years after diagnosis. CNS-RF was over 90% 3 years after diagnosis in patients aged 75 and below. CNS-RF was over 95% in patients diagnosed after 2000 who were recurrence-free 3, 4 or 5 years after diagnosis. CNS-RF was similar between patients with stage I and II disease from 2 years after diagnosis and patients with stage III disease from 5 years after diagnosis. The initial differences in net survival related to gross features, clinical presentation, number of harvested nodes in stage II, and number of involved nodes in stage III disappeared after 2 years.ConclusionsCNS-RF is a relevant measure of prognosis in patients who have already achieved a period of remission. Providing an updated estimation of prognosis in the years following diagnosis may improve the survivors’ quality of life and access to credit or insurance.