Infant survival after orthotopic liver transplantation

Of all head and neck neoplasms, 3% are malignant salivary neoplasms. Only 20% of them affect submandibular glands. These tumours vary histologically, which results from the complex embryogenesis of the glands. Malignant submandibular gland tumours are twice as frequent as parotid gland tumours. Simultaneous occurrence of quite different malignant tumours in the same salivary gland is extremely rare. The age range of patients affected with salivary gland neoplasms is wide. However, the occurrence of these neoplasms in children is exceptionally rare. The authors describe a case of a 13-year-old girl with acinose adenoid carcinoma and cystiscarcinoma coexisting in one submandibular salivary gland.     

Early and late results of surgical treatment in patients with primary mediastinal germ cell tumors]

A group of 37 patients--20 (54%) men and 17 (46%) women--aged 2-65, mean 31.6 years, was treated between 1978 and 1995 for different kinds of primary mediastinal germ cell tumours. In 14 (37.8%) of them a mature solid teratoma was diagnosed, in 13 (35.1%)--dermoid cyst, in 5 (13.5%)--malignant teratoma, in 1 (2.7%)--immature teratoma, in 4 (10.8%)--seminoma. Mediastinal tumour was removed completely in 30 (81.1%) patients, partially in 7 (18.9%). Out of 37 patients, 3 (8.1%) died during postoperative time. Out of 26 patients discharged after surgical removal of mature teratomas, all are alive and have been followed-up for 10 years (17 patients) or 5 years (6 patients). Out of 8 discharged after surgical removal of malignant neoplasm, 3 are alive and all of them had primary mediastinal seminoma. One of them has survived 17 years after complete resection, and two patients--7.5 years and 2 years after partial resection and radiotherapy. Mean survival time is 4 years and 2 months for all patients with malignant neoplasm.     

Malignant giant cell tumors of soft tissue

Clinco-morphological characteristics of malignant giant cell tumours of soft tissues in 31 patients are presented. Microscopically, the tumours consisted of histiocyte-like mononuclear cells possessing a phagocytic capacity, stretched out fibroblast-like cells forming collagen fibres, multinuclear giant cells of the type of osteoclasts. Three morphological variants were singled out: 1/ predominantly round-polygonal-cellular (histiocyte-like), 2/ predominantly spindle cell (fibroblast-like) and 3/ mixed. Clinically the tumours were divided into 2 groups: superficial (in 8 patients) and deep ones (in 23 patients) developed in skeletal musculature, deep fasciae and tendons, retroperitoneally, as well as in the mammary gland, mesentery of the small intestine, the stomach. The superficial tumours were characterized by relatively low malignancy. Prognosis in the majority of patients with deep tumours of soft tissues was unfavourable. The authors share the opinion of the investigators who consider these tumours to be a malignant variant of giant cell tumours of vagina tendinis and, bearing in mind the unestablished histogenesis of these tumours, think it expedient to retain the term "malignant giant cell tumours of soft tissues".     

Tumors in rheumatoid arthritis

Benign (bTu) and malignant tumours (mTu) were studied in a randomised autopsy material of 161 patients with rheumatoid arthritis (RA). The tissue specimens were fixed in 8% formaldehyde solution at pH 7.6 and embedded in paraffin. The tumours were diagnosed histologically and confirmed by immunohistochemical methods. Five benign (3.1%), and thirteen malignant tumours (8.1%) found observed in 18 (11.2%) of 161 cases. There was no significant difference between laboratory parameters of patients with malignant tumours and without tumours. One benign (0.62%) and seven malignant (4.35%) tumours led to death in 8 (4.97%) of 18 cases altogether. Neoplasms were detected clinically in 8 of 18 cases (44.4 rel%). None of tumorous patients received immunosuppressive treatment and only five had gold (Tauredon) therapy. Paraneoplastic syndromes with rheumatoid complaints may be excluded by the onset and duration of RA and tumours. Benign neurogenic tumours and malignant bronchioloalveolar carcinoma were frequently associated with RA not treated by immunosuppressive drugs. Our data do not support the assumption of a high risk of malignant lymphomas associated with RA treated with immunosuppressive therapy.     

Primary cardiac sarcoma treated by orthoptic cardiac transplantation. Apropos of a case

The authors report a new case of cardiac sarcoma treated by cardiac transplantation. This treatment has been proposed for these malignant tumours of poor prognosis when simple excision is impossible, with variable results. This patient is in good general condition 20 months after transplantation. Transplantation is a therapeutic procedure which should be considered in malignant tumours limited to the heart.     

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.     

Mediastinal needle biopsy. A 15-year experience with 139 cases

BACKGROUND: Radiologically guided needle biopsy and cytologic evaluation provide a reliable method of diagnosis for planning definitive therapy of patients with mediastinal lesions. MATERIALS AND METHODS: In this retrospective study of one of the largest series from a single institution, 141 consecutive mediastinal needle biopsies from 139 patients were reviewed during a 15-year period. RESULTS: Adequate material was obtained with a diagnosis achieved in 128 cases (92%). Of these, 33 cases (26%) had benign diagnoses; the remaining 95 (74%) had malignant diagnoses, including 81 carcinomas, 3 sarcomas, 8 lymphoproliferative lesions, 2 malignant germ cell tumors, and 1 malignant thymoma. All benign cases were diagnostically confirmed, and 94 of 95 malignant cases were classified correctly. The only discrepancy that occurred involved a malignant lymphoma diagnosed as a malignant germ cell tumor. Of the 13 inadequate samples, the major category included a nodular sclerosis variant of Hodgkin's disease (4 cases), 1 case of thymoma, 1 case of tuberculous lymphadenitis, and 7 cases for which no follow-up data were available. CONCLUSION: Needle biopsy is reaffirmed as a reliable and sensitive diagnostic tool for mediastinal lesions, with an overall cytologic diagnostic accuracy of 99% with adequate material. Sclerotic lesions may pose a limitation to this technique and require generous sampling before a more invasive diagnostic procedure is undertaken.     

Defective proximal tubule lysosomal acidification by Bence Jones proteins. An immunoelectron microscopy study

AIMS: To determine their significance, we examined the expression pattern of the four epidermal growth factor receptor (EGFR) family members as well as the phosphotyrosine kinase activity in breast tumour tissues. METHODS AND RESULTS: Fifty-three malignant breast tumours, four breast cancer cell lines, and 10 benign breast tumours were investigated. Fifty-three per cent (28/53) of the malignant tumours expressed EGFR protein, and the majority of these positive tumours were strongly positive. Eighty per cent (8/10) of the benign tumours also expressed EGFR protein, but all in a lower or moderate level. An association between EGFR expression and increasing malignancy grade was found in the group of infiltrating ductal carcinomas. Of the malignant tumours, 35.8% (19/53) expressed c-erbB-2 protein and 17% (9/53) c-erbB-3 protein, while no expression of c-erbB-2 and c-erbB-3 proteins was found in the benign tumours. Contrary to previous reports, we observed c-erbB-4 receptor protein to be less expressed in the malignant breast tumours. The 'normal' breast epithelial cells adjacent to the malignant tumours and the benign tumours demonstrated intensified membrane staining for c-erbB-4, while a number of the malignant tumours demonstrated a weak cytoplasmic staining or were negative. However, several malignant tumours with strong membrane staining for the c-erbB-4 protein were also found. No simple association between the expression of the four receptors and phosphotyrosine kinase activity was found. CONCLUSION: Our study has revealed a complex expression pattern of the EGFR family members in breast tumour cells. While the data about EGFR, c-erbB-2, c-erbB-3 and phosphotyrosine are largely in line with what has been reported, we found the c-erbB-4 protein expression to be decreased in the malignant tumours.     

Percutaneous needle aspiration of hilar and mediastinal masses

Needle aspiration biopsy of hilar and mediastinal masses was attempted in 100 patients and completed in 91. There were no major complications. In the group with completed biopsies, there were 83 patients with malignant neoplasms; a positive diagnosis of malignancy was established in 80 (96%). Two of the three false-negative results occurred in patients with Hodgkin disease. In all 8 patients with benign masses, the biopsy results were correct. Five of the nine incomplete biopsies occurred in patients with aortic aneurysms that simulated lung or mediastinal masses. In the other 4 patients, the needle biopsy was not completed for technical reasons. Needle biopsy can be performed in practically all areas of the mediastinum, does not require general anesthesia or hospitalization, and is well tolerated by the patient. In this series, the use of mediastinal needle biopsy made it possible to avoid surgery and mediastinoscopy in 72 patients with unresectable malignant neoplasms and 5 patients with innocuous benign mediastinal masses. Needle biopsy may be the preferred initial procedure to obtain a tissue diagnosis in patients with mediastinal masses.     

Possibilities of bronchoscopic diagnosis in bronchogenic carcinoma (author''s transl)

36 malignant tumours of the eyelids (basaliomas, basal-cell carcinomas and squamous-cell carcinomas) have been treated by intrapalpebral injections of a 5% to 25% solution of urea as recommended by E.D. Danopoulos. 1.5 to 3.0 ml of the solutions were injected around the tumours twice per week. Six malignant tumours disappeared completely after a treatment of eleven months; their largest initial diameter averaged 0.53 cm. Considering the protracted treatment, the uncertainty of success, without histological examination of the excised scar tissue, and finally because of the low rate of verified success, injections of dissolved urea around malignant tumours of the eye lids cannot replace radical excision.     

Incidence of iron-deficiency anaemia and depleted iron stores among nine-month-old infants in Vancouver, Canada

The authors analysed a group of 80 patients who were operated in 1985-1990 on account of non-small cell carcinoma of the lungs in stage IIIA with affection of the ipsilateral mediastinal nodes. The patients were not treated by neoadjuvant chemotherapy and systematic dissection of the mediastinal nodes was not performed. The results of five-year survival in the group of patients with affection of the mediastinal nodes (N2) were compared with those in the group of patients without affection of the mediastinal and hilar nodes (NO). Patients with affected N2 nodes who survived five years were significantly fewer than patients with negative mediastinal and hilar nodes. The probability of five-year survival in N2 was 15.8%, in patients with NO 28.0%. From data in the literature it is known that neoadjuvant chemotherapy and subsequent complete resection of the lung with the tumour combined with dissection of the mediastinal nodes may improve long-term survival after surgery. The authors assume that introduction of the described methods in their department will improve postoperative results.     

Incidence of primary bone tumours and tumour like lesions in and around Dakshina Kannada district of Karnataka

A total of 523 cases of primary bone tumours and tumour like lesions in and around Dakshina Kannada district of Karnataka were diagnosed over a period of 36 years. About 39% of these tumours were malignant and the remaining benign. Among the malignant tumours the highest incidence was of osteosarcoma (45.7%) followed by Ewing's sarcoma (19.4%). Osteochondroma was the most frequent in the benign tumour category (30.3%). Peak incidence of tumour was in the 2nd and 3rd decade of life with a male preponderance. The most commonly affected bones were femur, tibia and humerus in that order. Results indicate a significantly higher incidence of primary bone tumours in this part of India.     

Isolated angiomyolipomas of the kidney. Diagnostic difficulties

The authors report 4 cases angiomyolipomas in which the preoperative diagnosis was that of a malignant tumour. They stress the difficulties of arteriographic and even histological diagnosis of these rare tumours of the renal parenchyma.     

Reduced expression of retinoblastoma gene product (pRB) and high expression of p53 are associated with poor prognosis in ovarian cancer

Paraffin sections (n = 168, 27 benign, 16 low malignant potential [LMP] and 125 malignant tumours) from epithelial ovarian tumours were evaluated immunohistochemically for expression of retinoblastoma gene product (pRB) and p53 protein, and the relationship among pRB, p53 and cyclin-dependent kinase inhibitor 2 (CDKN2) gene product p16INK4A (p16) was analysed, following our previous study of p16. Forty-one percent of the benign, 50% of the LMP and most (71%) of the malignant tumours showed high pRB expression. High expression of pRB (>50% pRB-positive cells) significantly correlated with non-mucinous histological subtypes. Reduced pRB expression, substage and residual disease were significant predictors for poor prognosis in stage I patients. All the benign and most of the LMP (81%) tumours were in either the p53-negative or low p53-positive category, but nearly half of the malignant tumours had high p53 expression. High p53 accumulation was found in non-mucinous, high grade and late stage tumours. For well-differentiated carcinomas, high p53 expression was a predictor of poor prognosis. However, even though high p53 expression was not associated with histological subtype, stage or the presence of residual disease, high p53 expression was not an independent predictor when all clinical parameters were combined. For all ovarian cancers, a close correlation was found between high p53 and high p16 expression. The relationship between the expression of pRB and p16 depended on tumour stage. In stage I tumours, high pRB was associated with low p16 reactivity. On the other hand, most advanced tumours showed both high pRB and high p16 reactivity.     

The outcome of patients with mediastinal tumors detected by mass screening versus the outcome of those initially presentating with symptoms

Forty seven patients with mediastinal tumors, who were diagnosed between 1976 and 1996 at Tsukuba University Hospital, were evaluated according to the circumstances surrounding their initial consultations. The outcome of 19 patients (40.4%) whose tumors were detected by mass screening was compared with the outcome of 20 patients (42.6%) whose tumors were detected after presenting with symptoms. In the mass screening group, only 21% of the patients had a malignant neoplasm, whereas 75% of patients in the symptomatic group had a malignant neoplasm (p = 0.0008). Patients in the screening group had a shorter hospital stay than those in the symptomatic group (60.5 vs. 118. 1days; p = 0.0022). In patients with thymomas, in the mass screening all patients group were at stage I or II. Only 20% of the lesions in symptomatic patients were at stage I or II (p < 0.0001). Based on the results of this study, we believe that the early detection of mediastinal tumors via mass screening might have clinical significance.     

Cystic tumours of the breast

A retrospective survey of cystic tumours of the breast seen at the North Middlesex and St Bartholomew's Hospitals, London, has been undertaken to determine their incidence and clinical features. During the years 1967-76 4530 histological examinations were carried out on breast tissue and 1277 breast cancers diagnosed. Intracystic papillary tumours, both benign and malignant, were the least uncommon of cyst tumours, but the malignant type accounted for 0.55% of all the breast cancers. Aspects suggesting the possible presence of an intracystic tumour are discussed.     

CT imaging in adults with neurofibromatosis-1: frequent asymptomatic plexiform lesions

OBJECTIVE: The authors examined the incidence and radiologic characteristics of plexiform neurofibromas in neurofibromatosis-1 (NF-1) to define a cohort at greatest risk for malignant nerve-sheath tumors. BACKGROUND: Plexiform neurofibromas are a frequent complication of NF-1. They can impair function, produce disfigurement, and be the site for the development of malignant nerve-sheath tumors. The incidence and natural history of plexiform neurofibromas is unknown. METHODS: CT imaging of the chest, abdomen, and pelvis was performed in 91 of 125 consecutive adults (age, > or = 16 years) with NF-1. RESULTS: Twenty percent of patients had plexiform neurofibromas of the chest in the paraspinal, mediastinal, or supraclavicular area. Approximately 40% of patients had abnormal abdominal/pelvic scans. The paraspinal, sacral plexus, sciatic notch, and perirectal regions were the most common sites. Most plexiform neurofibromas were asymptomatic. Imaging also revealed a number of tumors, including malignant nerve-sheath tumors, adrenal tumors, carcinoids, and schwannomas. CONCLUSIONS: The frequency of plexiform lesions and other tumors in NF-1 indicates that clinicians should monitor young adults carefully; however, imaging characteristics alone cannot reliably distinguish benign from malignant lesions.     

Gastric carcinoma with ovarian metastases in an adolescent

Gastric carcinoma represents only 0.05% of malignant paediatric gastrointestinal tumours. This condition is associated with a poor prognosis because its rarity and non-specificity of the symptoms usually delay its diagnosis. The authors present a 16-year-old girl with a poorly differentiated gastric carcinoma (signet-ring-cell type) with bilateral ovarian metastases who died of peritoneal carcinomatosis despite extensive surgery and chemotherapy. The epidemiological, clinical, and pathological features of gastric carcinoma in childhood are discussed.     

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.     

Benign and malignant teratomas in children: analysis of 85 patients

Teratomas were observed in 85 infants and children. Fifty-eight (68%) were girls and 27 (32%) boys. Site of origin was sacrococcygeal in 55 patients (64.8%), mediastinal in ten (11.7%), gonadal in ten (11.7%), presacral in four (4.8%), retroperitoneal in three (3.5%), and cervical in three (3.5%). Sixty-seven (78.8%) teratomas were benign and 18 (21.1%) malignant. Malignant tumors were noted in 11 of 55 sacrococcygeal (20%), two of ten mediastinal (20%), three of eight ovarian (37.5%), and both testicular lesions. Cervical, retroperitoneal, and presacral tumors were benign. Age at diagnosis (greater than 1 month), presence of symptoms (urinary and colonic obstruction), and serum positive for alpha fetoprotein were indicators of malignancy in sacrococcygeal cases. Age was not a factor for teratomas at other sites. Tumor size, presence of calcification, and gross appearance (cystic or solid) did not relate to the tumor's benign or malignant nature. Three of four presacral tumors were associated with anorectal anomalies. Operative resection is the treatment of choice. Four deaths were related to operative hemorrhage. Four of five survivors with malignant teratoma received radiation and chemotherapy. A more aggressive role for combined adjunctive measures is suggested in cases of malignancy.