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PubMed1993

Adult rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study.

Twenty-seven cases of adult rhabdomyoma (ARM) of the head and neck are reported. The 20 male and seven female patients ranged in age from 33 to 80 years (median age, 60 years). Symptoms included airway obstruction and a mass within the mucosa or soft tissue. Median tumor size was 3.0 cm (range, 1.5 to 7.5 cm). Seven patients (26%) presented with multinodular tumors and one tumor was multicentric. Follow-up was available in 19 cases and ranged from 2 months to 18.5 years after diagnosis (median, 6.0 years). Lesions recurred locally in eight cases (42%) 2 to 11 years after diagnosis (median, 6 years). One recurrence was multicentric. Histologically, ARM was composed of closely packed, large polygonal cells having abundant, eosinophilic, granular, or vacuolated glycogen-rich cytoplasm with focal cross-striations. Immunohistochemical stains confirmed skeletal muscle differentiation; the majority of tumors stained for myoglobin (21 of 21 tumors), muscle-specific actin (21 of 21 tumors), and desmin (19 of 21 tumors). Focal or rare immunoreactivity for vimentin (six of 17 cases), alpha-smooth muscle actin (17 of 20 cases), S-100 protein (14 of 21 cases), and Leu-7 (10 of 20 cases) also was detected. Cytokeratin, epithelial membrane antigen, glial fibrillary acidic protein, and CD68 antigen (with KP1) were not found. The characteristic histology and immunophenotype distinguish ARM from other lesions with which it is frequently confused, including granular cell tumor, hibernoma, oncocytoma, and paraganglioma. The expression of alpha-smooth muscle actin has not been reported previously in ARM; its presence could reflect aberrant expression of smooth muscle actin in skeletal muscle or possibly be a recapitulation of early skeletal muscle embryogenesis.

Adult↗
PubMed1998

[Problems in diagnosis and therapy of carcinoma in stroma-abundant pleomorphic adenoma of the parotid gland].

The case is reported of a 27-year-old patient who developed multiple recurrences of a carcinoma arising from richly stromal pleomorphic adenoma of the parotid gland over 13 years. Originally the tumor was classified as a benign pleomorphic adenoma because there were no cytological characteristics of malignancy. Later on the diagnosis had to be modified to that of a carcinoma arising from a pleomorphic adenoma because of the infiltrating growth pattern of the tumor. A multicentric local recurrence and metastases on the left side of the neck supported the correct diagnosis. This case demonstrates that, beside the classic cytological criteria of malignancy invasion and penetration of the capsule can be decisive for the diagnosis of malign transformed pleomorphic adenoma. Treatment and prognosis of salivary gland tumors assume a clear determination of their diguity. The unusual course in this case of a pleomorphic adenoma originally diagnosed as benign demonstrates the importance of regular follow-up.

Adenoma, Pleomorphic↗
PubMed2005

[Risk factors for metastasis and recurrence of hepatocellular carcinoma at different stages].

OBJECTIVE: To analyze the risk factors for metastasis and recurrence of hepatocellular carcinoma (HCC) postoperatively. METHODS: Data of 270 cases of postoperative HCC were analyzed by SPSS software retrospectively. RESULTS: Out of the 270 cases, 162 got follow-up study and 136 showed metastasis and recurrence. Lots of risk factors induced the recurrence of HCC, such as AFP, tumor form, venous blood invasion, HBV infection, resection dimension and perioperative transfusion. There were different risk factors at different stages. CONCLUSION: The early recurrence of HCC may be mediated by macro- or micro-vessel blood invasion and metastasis, the late recurrence by multicentric carcinogenesis or introhepatic cacinoma de novo.

Adult↗
PubMed1996

Recolonization with selected alpha-streptococci for prophylaxis of recurrent streptococcal pharyngotonsillitis--a randomized placebo-controlled multicentre study.

In a randomized, placebo-controlled, double-blind, multicentre study, 130 patients with recurrence of group A beta-hemolytic streptococci (GAS) and clinical signs of pharyngotonsillitis were enrolled. The patients received antibiotic treatment for 10 days, followed by 10 days of alpha-streptococci-inhibitory to GAS-or placebo spray treatment. Patients taking antibiotic treatment for at least 9 days and using the spray for at least 5 days were included in the efficacy analysis. In addition, recurrence within the first 5 days of spray treatment was classified as 'early treatment failure'. The clinical recurrences (bacteriologically verified) in the alpha- (n = 51) and placebo-treated (n = 61) patient groups were 2% (n = 1) and 23% (n = 14) respectively, in patients given spray for at least 5 days (p = 0.004). The inclusion of 'early treatment failures' reduces this difference (p = 0.064). Both treatments were equally well tolerated. Thus, alpha-streptococci given as a spray and used for at least 5 days significantly prevented recurrence of GAS pharyngotonsillitis.

Adolescent↗
PubMed1999

Postoperative neuroimaging of high-grade gliomas: comparison of transcranial sonography, magnetic resonance imaging, and computed tomography.

BACKGROUND: A precise and comprehensive knowledge of tumor burden and its extent and growth pattern in the pre- and postsurgical states is required to optimize tumor therapy and to determine treatment success and failure. This prospective study compares the diagnostic potential of computed tomography (CT), magnetic resonance imaging (MRI), and transcranial sonography (TCS) in the postoperative follow-up of brain tumors. METHOD: Twenty-six patients with high-grade gliomas were included in the study. After tumor debulking, a total of 31 biopsy specimens were obtained from the resection margin in 21 patients and histological findings were compared with the findings of early postoperative TCS, CT, and MRI. Findings indicating residual tumor tissue were nonlinear contrast enhancement at the resection site revealed by CT or MRI or hyperechogenic lesions revealed by TCS. Follow-up examinations using all three imaging techniques were performed every 3 months. The end points of the follow-up were tumor recurrence as defined by CT and MRI, death, or severe clinical deterioration. RESULTS: On the basis of the above criteria, TCS identified residual tumor more often than did CT or MRI. In the group of 19 patients with histologically proven tumor remnants, residual tumor tissue was identified by TCS in all patients, whereas MRI and CT failed to show contrast enhancement in three and eight patients, respectively. However, the results of the TCS were false positive for one patient because of hemorrhage into the resection site. The average time to identification of tumor regrowth was 27 weeks using TCS, 29 weeks using CT, and 33 weeks using MRI. Only the differences between TCS and MRI reached statistical significance. For one patient, multicentric tumor recurrence was not detected using TCS. CONCLUSION: TCS may complement CT and MRI in the postoperative follow-up of patients with high-grade gliomas. Because none of these modalities alone is both sensitive and specific, an integrated analysis of imaging findings is recommended.

Adult↗
PubMed1998

A Case of Multiple Juvenile Papillomatosis of the Breast and Its Immunohistochemical Pathology.

Juvenile papillomatosis(JP)is a benign proliferative disease of the breast affecting young women. Some patients with JP develop breast carcinoma. We recentlytreated and followed a case of JP in a 16-year-old female, and studied its immunohistochemical characteristics. The patient noticed a small tumor, 5 mm in diameter, in the periareolar region on the medial side of the right breast. She underwent an excisional biopsy and pathology demonstrated JP. However, 6 months later, two new lesions were discovered by routine echogram examination, and were later excised. Immunohistochemistry demonstrated that the JP tumors were negative for estrogen receptor, but positive for progesterone receptor, epidermal growth factor receptor and erbB-2. In addition, tests for p53 were negative and Rb protein was normally expressed. Immunohistochemical analysis suggested that an abnormality in estrogen receptor is important in the pathogenesis of JP, and that the patient had a relatively high risk of developing breast carcinoma, since her JP is multicentric and recurrent. The present study also suggests that immunohistochemical pathology may be beneficial in assessing the malignant potential of JP.

Journal Article↗
PubMed1995

Patterns of p53, erbB-2, and EGF-r expression in premalignant lesions of the urinary bladder.

Frequent recurrences and multicentricity of bladder cancer suggest that alterations of the urothelium distant from the tumor may be relevant to prognosis. In this study immunohistochemistry and fluorescence in situ hybridization (FISH) were used to examine expression of p53, erbB-2, and epidermal growth factor receptor (EGF-r), genomic aberrations, and tumor cell proliferation (Ki67 LI) in normal and dysplastic urothelium. Biopsy specimens examined included normal urothelium (n = 40), mild dysplasia (n = 34), moderate dysplasia (n = 18) and carcinoma in situ (CIS; n = 20). Several different oncogene expression patterns were found, only some of which were associated with dysplasia. EGF-r expression was equally frequent in normal and dysplastic urothelium and showed a strong association with Ki67 LI (P < .0001). A purely superficial erbB-2 positivity was present in both normal and dysplastic biopsies. However, diffuse erbB-2 positivity and p53 overexpression were both associated with advanced dysplasia (P < .0001 each). FISH analysis showed erbB-2 gene amplification and p53 deletions in selected CIS, as well as a marked chromosome 17 copy number heterogeneity in all six CIS examined. These findings indicate a considerable genomic instability in bladder CIS. They show that both erbB-2 and p53 are altered during malignant transformation. Detectable oncogene expression alone, however, is not diagnostic of malignancy in bladder urothelium.

Adolescent↗
PubMed1986

Posterior fossa hemangioblastomas.

The authors report 40 personal cases of infratentorial hemangioblastomas and review 36 series from the literature for a total number of 1023 patients. The clinical, pathological, and surgical features of these tumors are discussed, with particular consideration of localizations outside the nervous system, multicentric tumors, recurrences, and long-term results. Although hemangioblastomas are benign tumors, their high growth potential makes their long-term development unpredictable in some cases.

Adolescent↗
PubMed1992

Intraductal breast cancer: review of 183 consecutive cases.

The aim was to review a series of 183 intraductal breast cancer to define the diagnostic features and therapeutic outcome. Patients' average age was 54 years. Diagnostic procedures employed were clinical examination, mammography in 173 cases and fine-needle aspiration cytology in 98 cases. The sensitivity of clinical examination was 0.61, of mammography 0.74, of fine needle aspiration cytology 0.70. The sensitivity of clinical examination and mammography associated was 0.93. The surgical options adopted were: conservative surgery 80 cases, mastectomy 103 cases. Conservative surgery was followed by breast irradiation in 34 cases. Axillary dissection was performed in 122. 97 cases have been reviewed histologically. 60% of ductal carcinoma in situ (DCIS) were multifocal and 22% multicentric. Local recurrence, all infiltrating, occurred in the same breast after conservative surgery in 8 cases, 3 of which had received postoperative radiotherapy, and in 3 patients after mastectomy. Contralateral breast cancer was recorded in 13 cases, being synchronous in 4 (infiltrating in 3, DCIS in 1) and metachronous in 8 (all infiltrating). 3 patients died of breast cancer. The present series confirms the risk of ipsilateral cancer recurrence after conservative surgery but there are no significant differences relating to mammographic pattern, size, histological type, margin involvement and radiotherapy.

Adult↗
PubMed2006

Thoracic extradural paragangliomas: a case report and review of the literature.

STUDY DESIGN: Case report. OBJECTIVES: To report on a case of paraganglioma presenting in an uncommon extradural thoracic localization. SETTING: Department of Neurosurgery, Florence, Italy. CASE REPORT: A 43-year-old woman with a thoracic lesion extending into the extradural space along four levels, T(1)-T(4), presented with sudden spastic incomplete paraplegia and paresthesia at the lower limbs. RESULTS: The neoplasm was surgically resected 'en bloc' and histological findings corresponded to paraganglioma. One year after surgery, the patient was walking without assistance, a T(3)-T(4) hypoesthesia was still present and an magnetic resonance imaging (MRI) study showed no signs of focal recurrence. CONCLUSIONS: The imaging features of thoracic paragangliomas may be misleading and an advanced malignant lesion could be primarily suspected; thus, a histological study is always needed. Total resection is the gold standard therapy. Owing to the risk of recurrence or multicentric growth, follow-up must be prolonged and accurate.

Adult↗
PubMed2003

Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma.

Inhibin alpha subunit (inhibin A) expression in hemangioblastomas has not been previously reported in the literature. We analyzed the expression of inhibin A in 25 hemangioblastomas from 22 patients. Eleven cases were from 8 patients with von Hippel-Lindau disease, and these tumors were multicentric and/or recurrent. The remaining 14 cases from 14 patients were sporadic. The male-to-female ratio was 8:3, and the age at presentation ranged from 19 to 78 years (mean 35 years; median 45 years). Eighteen tumors were located in the cerebellum/posterior fossa, 1 in the medulla, 1 in the occipital lobe, and 5 in the spinal cord. Four metastatic renal cell carcinomas in brain, 10 renal cell carcinomas from 8 patients with von Hippel-Lindau disease, and 5 sporadic clear cell renal cell carcinomas were also included. Two patients with von Hippel-Lindau disease had both renal cell carcinoma and hemangioblastoma. The stromal cells of all 25 cases of hemangioblastoma expressed inhibin A. Strong, moderate, and weak cytoplasmic immunoreactivity was noted in 17, 5, and 3 cases, respectively. In contrast, none of the 19 renal cell carcinomas, primary as well as metastatic, expressed inhibin A. There was no difference in the inhibin A staining pattern between the sporadic hemangioblastoma and those associated with VHL. These findings demonstrate inhibin A to be a useful marker in distinguishing hemangioblastoma from metastatic clear cell renal cell carcinoma. While the diagnostic importance is evident, the pathophysiology of inhibin A expression by the stromal cells of hemangioblastoma remains unknown and further investigation is required.

Adenocarcinoma, Clear Cell↗
PubMed2005

Squamous papillomatosis of the bilateral nasal cavities.

The term "papillomatosis" indicates the tendency towards multicentricity and recurrence that these tumors exhibit (Snyder et al. 1972). A typical squamous papilloma arises from the nasal vestibule and is characterized by the epithelial proliferation growing an exophytic manner. We report a rare case of squamous papillomatosis of the bilateral nasal cavities. A 65-year-old man presented with a 2-year-history of bilateral nasal obstruction. Computed tomographic (CT) scans revealed a soft density mass in the bilateral nasal cavities and ethmoid sinuses. Because the tumors were limited to the nasal cavities and anterior ethmoid sinuses, total removal of the tumors was performed endoscopically. On the basis of the clinicopathological findings, the tumors were diagnosed as squamous papillomas. His post-operative course was uneventful, and he is currently free from disease 13 months after surgery. Nasal papillomas usually arise from the unilateral nasal cavity or paranasal sinus. While some cases of inverted (inverting) papillomas arising from the bilateral nasal cavities have been reported, bilateralism of the nasal squamous papillomas is quite rare. Diagnosis, clinical behavior and treatment of squamous papillomatosis of the bilateral nasal cavities are reviewed.

Aged↗
PubMed1995

Diagnosis of breast carcinoma with radiolabeled monoclonal antibodies (MoAbs) to carcinoembryonic antigen (CEA) and human milk fat globulin (HMFG).

The current study attempted to assess the potential proficiency of radioimmunodetection (RAID) of primary, residual, multicentric, and recurrent breast carcinoma using two radiolabeled murine monoclonal antibodies (MoAbs), anti-human milk fat globulin (HMFG1) labeled with iodine (123I) and anti-carcinoembryonic antigen (CEA) labeled with technetium (99Tc). Thirteen patients with suspicious clinical and/or mammographic primary or recurrent breast carcinoma were studied in a phase I-II prospective, consecutive, nonrandomized, noncontrolled study. Five patients received intravenous infusion with 0.5-2.0 mg anti-CEA MoAb type CYT 380 labeled with 99Tc [13-22 millicurie (mCI)] and 8 patients received intravenous infusion with 0.25-1.0 mg anti-HMFG1 MoAb (Unipath, U.K.) labeled with 123I (4-17 mCI). Both MoAbs used in this study demonstrated ability to bind specifically to breast cancer lesions, resulting in successful RAID in 10 of 12 of studied patients (5 of 5 patients in the anti-CEA-99Tc and 5 of 7 in the anti-HMFG-123I group--accuracy 83.3%). One patient was excluded due to protocol violation. Seven patients had true-positive scans when correlated with surgery (sensitivity 87.5%). The MoAb scans accurately diagnosed lesions in 3 of the 4 primary invasive breast carcinomas confirmed histologically. Presence of residual carcinoma following wide excision was established in 1 of 2 patients and presence of soft tissue metastases in 3 patients. Three patients had true-negative scan (specificity 75%): 2 patients presented with suspicious mammographic recurrence postlumpectomy and 1 patient had questionable soft tissue recurrence. One patient with primary breast carcinoma had a false-negative scan and another had a false-positive scan in the presence of fibrosis following lumpectomy and radiation therapy. No adverse reactions were noted in the patients studied. RAID findings were confirmed by immunohistochemistry in 6 of 9 cases studied. Our data suggest that radiolabeled MoAbs used in this study are potentially useful diagnostic agents for evaluation of primary or recurrent breast carcinoma, particularly in the areas where conventional methodology is limited.

Adult↗
PubMed1992

Origin heterogeneity of Hb Lepore-Boston gene in Italy.

Forty-three hybrid delta-beta-globin genes were characterized by DNA sequence analysis and associated RFLP haplotypes in 40 families from Abruzzo and Campania, which are on the east and west coast of Italy, respectively. All the genes had the delta-globin sequence up to the exon 2 codon 87 and had the beta-globin sequence from IVS-2-8; between these two ends, they had 58 bp in common with the delta- and beta-globin genes. Thus, they were all of the Lepore-Boston type. A chromosomal background heterogeneity was present among the mutant genes. In fact, they were all associated with (+ - - - -) 5' subhaplotype, but 23/31 from Campania were associated with (+ +) 3' subhaplotype, whereas 12/12 genes from Abruzzo and 8/31 from Campania were associated with (+ -). DNA sequencing of homozygous subjects showed that (+ +) 3' subhaplotype was associated, at IVS-2-74, with G, while (+ -) was associated with T; that is they were associated with the beta-globin gene sequence of frameworks 1 and 2, respectively. The molecular characteristics of this heterogeneity, as well as its geographical patterns in the eastern and western regions of Italy, represent strong evidence for the recurrent and multicentric origins of the mutation.

Base Sequence↗
PubMed1988

Pheochromocytoma.

Although pheochromocytoma is an uncommon cause of secondary hypertension, it is also a curable form of hypertension. With proper treatment, the outlook for patients with pheochromocytoma is excellent. If undiagnosed or untreated, pheochromocytoma causes serious complications and death. The key to reaching the diagnosis is a high index of suspicion coupled with careful clinical evaluation and laboratory testing. Our ability to diagnose and localize pheochromocytoma has improved vastly, owing largely to the modern biochemical and radiologic techniques. Despite this, a number of pheochromocytomas remain undiagnosed only to be uncovered after a serious complication or at postmortem. The diverse manifestations and problems presented by patients with pheochromocytoma can be managed only with combined medical and surgical expertise, with assistance from our colleagues from the radiology and anesthesiology disciplines. Although sporadic pheochromocytoma is the most common form of presentation, the overall clinical picture may depend on the subset of affected individual--childhood, pregnancy, associated MEN, and neurocutaneous syndromes. Fortunately, more than 90 per cent of all pheochromocytomas originate in the adrenal gland, but the tumor can occur at any site in the sympathetic chain. The spectrum of clinical manifestations is so wide that a pheochromocytoma may mimic a variety of common disorders. It is as challenging to diagnose what is not a pheochromocytoma as it is to confirm its diagnosis. Surgical removal of pheochromocytoma is the treatment of choice. With proper preoperative medical management aimed at blocking the effects of catecholamines, operative mortality should be close to zero. For patients with inoperable, malignant, recurrent, or multicentric pheochromocytomas, chronic medical therapy is indicated in the form of alpha- and/or beta-blockade or inhibition of catecholamine synthesis with alpha-methyl-para-tyrosine. An understanding of the pathophysiology and natural course is vital to the rational management of patients with pheochromocytoma.

Humans↗
PubMedDate not supplied

Hyperthermia as an adjuvant to radiation therapy of recurrent or metastatic malignant melanoma. A multicentre randomized trial by the European Society for Hyperthermic Oncology.

The ESHO protocol 3-85 is a multicentre randomized trial investigating the value of hyperthermia as an adjuvant to radiotherapy in treatment of malignant melanoma. A total of 134 metastatic of recurrent malignant melanoma lesions in 70 patients were randomized to receive radiotherapy alone (3 fractions in 8 days) or each fraction followed by hyperthermia (aimed for 43 degrees C for 60 min). Radiation was given with high voltage photons or electrons. Tumours were stratified according to institution and size (above or below 4 cm) and randomly assigned to a total radiation dose of either 24 or 27 Gy to be given with or without hyperthermia. The endpoint was persistent complete response in the treated area. A number of 128 tumours in 68 patients were evaluable, with an observation time between 3 and 72 months. Sixty-five tumours were randomized to radiation alone and 63 to radiation + heat. Sixty received 24 Gy and 68 tumours received 27 Gy, respectively. Size was < or = 4 cm in 81 and > 4 cm in 47 tumours. Overall the 2-year actuarial local tumour control was 37%. Univariate analysis showed prognostic influence of hyperthermia (rad alone 28% versus rad + heat 46%, p = 0.008) and radiation dose (24 Gy 25% versus 27 Gy 56%, p = 0.02), but not of tumour size (small 42% versus large 29%, p = 0.21). A Cox multivariate regression analysis showed the most important prognostic parameters to be: hyperthermia (odds ratio: 1.73 (1.07-2.78), p = 0.02), tumour size (odds ratio: 0.91 (0.85-0.99), p = 0.05) and radiation dose (odds ratio: 1.17 (1.01-1.36), p = 0.05). Analysis of the heating quality showed a significant relationship between the extent of heating and local tumour response. Addition of heat did not significantly increase the acute or late radiation reactions. The overall 5-year survival rate of the patients was 19%, but 38% in patients if all known disease was controlled, compared to 8% in the patients with persistent active disease.

Adult↗
PubMed1996

Analysis of the cellular origin of hepatocellular carcinoma by p53 genotype.

The polymerase chain reaction and HaeIII enzymatic digestion were used to study the seventh exon of the p53 gene in 29 primary and recurrent hepatomas in paraffin-embedded samples from 11 patients. The mutation rate of the p53 gene and its genotypes in samples of primary and recurrent tumours and multiple nodules were investigated. The cellular origins of hepatocellular carcinoma were analysed by p53 genotype. p53 mutation rates were found to be 69.0% (20/29) in the primary and recurrent tumours, 58.8% (10/17) in tumours with a single nodule and 83.3% (10/12) in tumours with multiple nodules. The p53 genotypes were found to be different in 6 pairs of primary and recurrent tumours, and another 5 pairs had the same p53 genotypes. The samples with multiple nodules in the same patients had the same p53 genotypes. Seven recurrences were of multicentric origin and four were of unicentric origin. It is suggested that the recurrent lesions developed from both unicentric and multicentric origins.

Adult↗
PubMed1994

Recurrent intracranial epithelioid hemangioendothelioma associated with multicentric disease of liver and heart: case report.

Epithelioid hemangioendothelioma is an unusual vascular neoplasm with prominent cytoplasmic vacuolization representing primitive lumen formation. A case is presented of this unique vascular neoplasm in a woman with a seizure disorder who had cardiac, hepatic, and recurrent nervous system lesions. To our knowledge, this is the third known case of intracranial epithelioid hemangioendothelioma. Emphasis is placed on the indolent course of this rare neoplasm, with a recommendation for aggressive surgical treatment and diligent follow-up.

Adult↗