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Cystic neoplasms of the pancreas: benign to malignant epithelial neoplasms.

Clinical presentation and state-of-the-art imaging permit the differentiation of most cystic pancreatic neoplasms not only from other cystic pancreatic disorders but also from one another. The differentiation of serous cystic lesions from the mucinous neoplasms (cystadenoma or carcinoma and IPMT) is crucial because of the radically different biological characteristics of these two neoplasms. Although mucinous cystic neoplasms should be resected because of their premalignant or overtly malignant tendency, most patients with serous neoplasms require no operative intervention unless they are symptomatic. IPMT is best treated by a total pancreatectomy, although lesser subtotal resections should be strongly considered depending on patient age, medical comorbidity, and psychosocial situations.

Adenocarcinoma, Mucinous↗

Pancreatic tumors with cystic dilatation of the ducts: intraductal papillary mucinous neoplasms and intraductal oncocytic papillary neoplasms.

Intraductal papillary mucinous neoplasms (IPMNs) and intraductal oncocytic papillary neoplasms (IOPNs) are the 2 types of intraductal neoplasms of the pancreas that may appear cystic because of dilatation of the ducts. Both are characterized by intraductal proliferation of mucinous cells usually arranged in papillary patterns. This proliferation is often associated with intraluminal mucin accumulation, which produces cystic dilatation of the ducts, mimicking mucinous cystic neoplasms. Endoscopic and radiologic studies and careful macroscopic examination are crucial for the correct diagnosis of IPMNs and IOPNs by showing the origin within the native ducts. Microscopically, these tumors display a spectrum of cytoarchitectural atypia that ranges from adenoma to borderline and to carcinoma-in-situ. Although they are defined as "intraductal tumors," IPMNs and IOPNs are associated with invasive carcinoma in about a third of the cases. It, therefore, appears that, like mucinous cystic neoplasms or pancreatic intraepithelial neoplasia involving the smaller ducts associated with ordinary ductal adenocarcinomas, these tumors are precursors of invasive carcinoma. Invasive carcinomas associated with IPMNs are of either tubular or colloid (mucinous noncystic) types, whereas those associated with IOPNs may be oncocytic. Even in the presence of invasive carcinoma, these tumors may follow a more protracted clinical course than ordinary ductal adenocarcinoma. On the other hand, rare examples of IPMNs after an aggressive clinical course despite the lack of any identifiable invasive carcinoma are on record. Therefore, IPMNs and IOPNs should be examined carefully and sampled extensively, first, to confirm that the main pathology is an intraductal process and, more importantly, to rule out the presence of an invasive carcinoma.

Cystadenocarcinoma, Mucinous↗

Neoplasms with neural differentiation: a review. Part II: Malignant neoplasms.

Malignant peripheral nerve sheath tumors (MPNSTs) encompass a wide and unusual group of neoplasms with features of neural differentiation. They most commonly present as spindle cell neoplasms and it can be difficult to differentiate them from other spindle cell neoplasms such as leiomyosarcomas, fibrosarcomas and synovial sarcomas. Strict criteria need to be applied in order to make the diagnosis of MPNSTs. Helpful features include contiguity with a nerve or an association with von Recklinghausen disease. The use of immunohistochemical stains may also help to confirm the diagnosis. Markers such as S-100 protein, neurofilament, epithelial membrane antigen and Leu-7 (CD57) are frequently used to assess neural differentiation in these neoplasms. In addition to the spindle cell pattern, MPNSTs may also display an epithelioid pattern. Rarely, other elements may be seen including glands in the so-called glandular MPNST or muscle in triton tumors. In more unusual cases cartilage, adipose tissue and even bone are present. Also included in the group of MPNSTs are the peripheral neuroepithelial tumor, neurotropic or desmoplastic melanomas and malignant granular cell tumors. MPNSTs are highly aggressive tumors and should be treated accordingly.

Adult↗

Ultrastructural characterization of pulmonary neoplasms. I. The role of electron microscopy in characterization of the most common epithelial neoplasms.

We have attempted to summarize current concepts pertaining to the ultrastructural evaluation of epithelial pulmonary neoplasms, with an emphasis in potential clinicopathological correlations. Routine ultrastructural evaluation of the most commonly encountered pulmonary carcinomas seems to provide, in a significant number of cases, additional information that may be of help in patient management. Immunocytochemistry when employed as an adjunct to electron microscopic evaluation of pulmonary tumors also plays an important role in clarifying morphologic findings. With the advent of specific chemotherapy protocols and future development of monoclonal antibodies in the treatment of neoplasms, accurate tumor classification becomes crucial. The combined use of electron microscopy and immunocytochemistry seems to be extremely useful in providing accurate and reproducible criteria of great potential value in the overall assessment of pulmonary neoplasms. In part II of this study we will address the importance of the ultrastructural evaluation of less common epithelial and mesenchymal pulmonary neoplasms.

Adenocarcinoma↗

Intraductal papillary-mucinous neoplasms and mucinous cystic neoplasms of the pancreas differentiated by ovarian-type stroma.

BACKGROUND: Intraductal papillary-mucinous neoplasms (IPMN) and mucinous cystic neoplasms (MCN) of the pancreas have similar clinicopathologic findings. This study was intended to clarify clinicopathologic characteristics in IPMNs and MCNs differentiated by ovarian-type stroma. METHODS: Medical records for 77 patients with pancreatic cystic neoplasms with mucin secretion were reviewed. Patients were divided into IPMN (n = 70) or MCN (n = 7) according to the presence of ovarian-type stroma, and clinicopathologic parameters were compared between groups. RESULTS: IPMNs consisted of 32 adenomas, 12 borderline neoplasms, 13 adenocarcinomas in situ, and 13 invasive adenocarcinomas; MCNs included 6 adenomas and 1 invasive adenocarcinoma. The mean age of IPMN patients (66 years) was significantly older than that of MCN patients (55 years). The male:female ratio in IPMN (53/17) was significantly greater (P < .001) than in MCN (0/7). The location of the pancreatic mass differed, with 76% 0f IPMNs occurring in the head, while 86% of MCNs occurred in the body or tail. Mass mean size was significantly smaller (28 mm vs 78 mm, P < .001), and mean diameter of the main pancreatic duct was larger (6.8 mm vs 3.1 mm, P < .001) in IPMN than in MCN. Patulous papilla was present in 44% (31/70) of IPMNs, but none was present in MCNs. Communication between the cyst and main pancreatic duct was more frequent in IPMNs (67/70) than in MCNs (1/7). Overall 5-year survival rates were 84% (IPMN) and 100% (MCN). CONCLUSIONS: Clinicopathologic differences between IPMN and MCN are much clearer when differentiated by presence of ovarian-type stroma. Favorable prognosis for both neoplasms is offered by complete resection.

Adenocarcinoma↗

CD4+/CD56+ haematodermic neoplasm: a preculsor haematological neoplasm that frequently first presents in the skin.

CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK cell lymphoma, is an aggressive and rare preculsor hematologic neoplasm recently recognized by the WHO-EORTC classification consensus for cutaneous lymphomas. The neoplasm tends to affect elderly patients, who usually present with skin lesions but often have a disseminated disease, including bone marrow involvement. Although the lesions are composed of cells with a lymphoblast-like morphology and an NK-cell phenotype, exhibiting a CD4+, CD56+ positive immunophenotype, recent studies support a relationship to plasmacytoid dendritic cells. Because of the rarity of this disease, we describe two patients suffering a CD4+/CD56+ hematodermic neoplasm.

Aged↗

International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas.

Non-inflammatory cystic lesions of the pancreas are increasingly recognized. Two distinct entities have been defined, i.e., intraductal papillary mucinous neoplasm (IPMN) and mucinous cystic neoplasm (MCN). Ovarian-type stroma has been proposed as a requisite to distinguish MCN from IPMN. Some other distinct features to characterize IPMN and MCN have been identified, but there remain ambiguities between the two diseases. In view of the increasing frequency with which these neoplasms are being diagnosed worldwide, it would be helpful for physicians managing patients with cystic neoplasms of the pancreas to have guidelines for the diagnosis and treatment of IPMN and MCN. The proposed guidelines represent a consensus of the working group of the International Association of Pancreatology.

Endocrine Surgical Procedures↗

[The clinical study of tongue flaps repairing after resecting pharyngeal neoplasm and laryngeal neoplasm].

OBJECTIVE: To study the repair methods of the defect after resecting pharyngeal neoplasm and laryngeal neoplasm. METHODS: Four kinds of tongue flap, such as 1/3 tongue flap, 1/2 tongue base flap, tongue base flap and transverse tongue flap were devised and applied in 15 patients with pharyngeal neoplasm and laryngeal neoplasm. RESULTS: These tongue flaps were alive in all patients. The wound of 13 patients healed in I stage. Two patients dehiscenced because of infection and healed after treatment. All the patients deglutited well. One-year, 3-year, disease-free survive rate were 92.9% (13/14), 72.7% (8/11) respectively. CONCLUSION: Tongue flaps are obtained easily, with enough blood flow, adapted to pharyngeal environment, easily alive and low complication in repairing the pharyngeal defect. We think that these tongue flaps should be applied in clinic.

Adult↗

Neoplasms of the central nervous system in Norway. V. Meningioma and cancer of other sites. An analysis of the occurrence of multiple primary neoplasms in meningioma patients in Norway from 1955 through 1986.

The association between meningioma and a primary malignant neoplasm at another site was studied. The data from the population-based Norwegian Cancer Registry were analysed according to whether the meningioma occurred before or after the malignant neoplasm. Male patients with meningioma showed a raised risk for developing a subsequent renal cancer. A significant association was found between meningioma and subsequent breast cancer in females 50-64 years old at time of meningioma diagnosis and between breast cancer and subsequent occurrence of meningioma. Breast cancer patients with symptoms of an intracranial neoplasm may therefore have a potentially curable meningioma and female meningioma patients over 50 years should be considered for breast cancer screening programmes.

Adolescent↗

Neoplasms of the central nervous system in Norway. II. Descriptive epidemiology of intracranial neoplasms 1955-1984.

A population-based study of 8480 patients - 4508 (53%) males and 3972 females - with primary intracranial neoplasms reported to the Norwegian Cancer Registry during the period 1955-84, is presented. 81% of the cases were histologically verified. The peak age-specific incidence rate in the total series occurred in the age-group 55-64 years. Gliomas constituted the largest histological group with an age-adjusted incidence rate of 5.0 cases per 100,000 population per year for males and 3.5 for females. Case ascertainment of primary intracranial neoplasms is reduced above the age of 60 in Norway, mostly due to a a low autopsy rate. The major impact of the introduction of computer tomography (CT) in the case ascertainment of intracranial neoplasms has been a raised incidence, in patients over the age of 60, of neoplasms which are not histologically verified.

Age Factors↗

Photodynamic therapy for intracranial neoplasms: investigations of photosensitizer uptake and distribution using indium-111 Photofrin-II single photon emission computed tomography scans in humans with intracranial neoplasms.

Photodynamic therapy is being investigated as an adjuvant treatment for intracranial neoplasms. The efficacy of this therapy is based on the uptake of photosensitizer by neoplastic tissue, its clearance from surrounding brain tissue, and the timing and placement of photoactivating sources. Photofrin-II is the photosensitizer most actively being investigated. We labeled Photofrin-II with Indium-111 and studied the uptake and distribution of this agent in 20 patients with intracranial neoplasms, using single photon emission computed tomography (SPECT) with volume rendering in three dimensions. Of these patients, 16 had malignant glial tumors, 2 had metastatic deposits, 1 had a chordoma, and 1 had a meningioma. Anatomical-spatial data correlated well between the SPECT images and contrast-enhanced computed tomography or magnetic resonance images. Regions of focal uptake on SPECT images correlated with the surgical histopathological findings of the neoplasm. The kinetics of photosensitizer uptake varied according to the tumor's histological findings, the patient's use of steroids, and among patients with similar types of tumor histology. Peak ratios of target-to-nontarget tissue varied from 24 to 72 hours after injection. The study data show that, to be most effective, photodynamic therapy may need to be tailored for each patient by correlating SPECT images with anatomical data produced by computed tomography or magnetic resonance images. Photoactivating sources then can be placed, using computer-assisted stereotactics, to activate a prescribed volume of photosensitized tumor at the optimal time for treatment.

Adult↗

[Retrospective analysis of the incidence of midline supratentorial neoplasms in children and young patients: craniopharyngiomas, hypophyseal and pineal neoplasms].

The authors analyzed, in a retrospective study made at the Pathology Department of the Medical School of the University of São Paulo, 1632 cases of intracranial neoplasms in a period of 50 years (1931-1981). The investigation concentrated on 592 cases (36.2%) of patients with age ranging from 0 to 20 years. The overall number of craniopharyngiomas in this group was 21 (3.5%); 8 cases (1.35%) of hypophyseal neoplasms, and 4 cases (6%) of pineal neoplasms. Neoplastic infiltration of the hypophysis and pineal was present in 24 cases (4.0%). No metastases were observed. The description and importance of these findings, and their correlation with data from other authors are presented.

Adolescent↗

Neoplasms in skin and subcutis over the breast, simulating breast neoplasms: case reports and literature review.

Twenty cases of neoplasms in skin and subcutaneous tissue over the breast were reviewed. There were 17 women, from 15 to 70 years of age, and three men, from 25 to 66 years of age. Among the benign skin neoplasms, superficial leiomyoma, granular cell tumor, and eccrine acrospiroma were misdiagnosed clinically as primary breast carcinoma. Among the malignant neoplasms in subcutaneous tissue, there were three metastatic malignant melanomas, one metastatic epidermoid bronchogenic carcinoma, and two malignant lymphomas. It is interesting that four of these six patients had no prior history of malignant lesion, the subcutaneous nodule presenting as the first manifestation of an occult primary. It is concluded that histological diagnosis of such tumors may lead to avoidance of unnecessary radical surgery.

Adenoma, Sweat Gland↗

[Cutaneous mesenchymal neoplasms versus mesenchymal neoplasms of subcutaneous and deep soft tissue. Similarities and differences].

As in subcutaneous and deep soft tissues, cutaneous mesenchymal neoplasms are also relatively rare and characterized by a clinicopathological heterogeneity. There are striking differences in regard to the occurrence of different lines of differentiation of mesenchymal neoplasms in superficial and deep locations, and some distinct entities arise exclusively in the dermis. Superficially located mesenchymal neoplasms are characterized by a number of specific morphological features in comparison to deeper located lesions. Clinically important, dermal sarcomas of some lines of differentiation are associated with a favorable clinical prognosis, independent of the histological grading, which is important to know for the correct treatment of patients. In the following review similarities and distinguishing clinicopathological features of superficially and deeply located mesenchymal tumors are discussed.

Diagnosis, Differential↗

Ultrastructural morphometry distinguishes Burkitt's-like lymphomas from neuroendocrine neoplasms: useful criteria applied to the evaluation of a poorly differentiated neuroendocrine neoplasm of the nasal cavity masquerading as Burkitt's-like lymphoma.

The present study describes the potential usefulness of ultrastructural morphometry in diagnosis. Ultrastructural morphometric criteria were applied to the evaluation of a poorly differentiated neuroendocrine neoplasm of the nasal cavity that was initially thought to be a Burkitt's-like lymphoma (BLL). Although the nasal lesion in question failed to stain with over 50 cell lineage-relevant antibodies, it did stain for vimentin (an intermediate filament protein) and Ki-67 (a nuclear antigen associated with cell proliferation). Routine electron microscopy revealed a primitive neoplasm with abundant cytoplasmic lipid droplets and sparse dense granules with no intercellular junctions. Treatment options, which included extensive facial surgery, prompted more study. Tissue processed for the uranaffin reaction revealed sparse uranaffin-positive granules indicating the presence of true neurosecretory granules. An ultrastructural morphometric analysis of the neoplastic nuclei of this patient placed the tumor outside the morphometric domains for BLLs (18 cases) and neuroblastomas (11 cases) and within the morphometric domain of neuroendocrine carcinomas (9 cases). A greater mean standard deviation (P less than 0.05) and mean coefficient of variation (P less than 0.02) of nuclear perimeter in the neuroendocrine (NE) group related to the BLL group indicated greater nuclear pleomorphism within the NE group as illustrated in bivariate graphic displays. The possible origin of the neoplasm within the nasal mucosa is discussed.

Burkitt Lymphoma↗

Malignant small bowel neoplasm of enteric plexus derivation (plexosarcoma). Light and electron microscopic study confirming the origin of the neoplasm.

An unusual small intestinal tumor was found in a 61-year-old male who complained of intermittent right lower quadrant burning pain, tenesmus, and bloody diarrhea. On gross examination, the tumor was located intramurally in the distal jejunum with no involvement of the overlying mucosa. Histologic examination revealed two elements: a spindle cell component with characteristics reminiscent of a Schwann cell neoplasm, and nests of larger epithelioid-like cells almost undistinguishable from the Zellballen of nonchromaffin paragangliomas. The neoplasm originated within the intestinal wall without apparent mucosal origin. Ultrastructural studies revealed features recapitulating the normal enteric plexus structure and quite unlike those described in normal or neoplastic smooth muscle, Schwann or paraganglion cells. On this basis, this neoplasm is considered as a malignant enteric plexus tumor or a plexosarcoma.

Humans↗

International Collaborative Ovarian Neoplasm trial 1 and Adjuvant ChemoTherapy In Ovarian Neoplasm trial: two parallel randomized phase III trials of adjuvant chemotherapy in patients with early-stage ovarian carcinoma.

BACKGROUND: Adjuvant chemotherapy has been suggested as a possible strategy to improve survival in women with early-stage ovarian cancer; however, all randomized studies to date have been too small to answer this question reliably. METHODS: We performed a preplanned combined analysis of two parallel randomized clinical trials (International Collaborative Ovarian Neoplasm 1 [ICON1] and Adjuvant ChemoTherapy In Ovarian Neoplasm [ACTION]) in early-stage ovarian cancer that compared platinum-based adjuvant chemotherapy with observation following surgery. Between November 1990 and January 2000, 925 patients (477 in ICON1 and 448 in ACTION) who had surgery for early-stage ovarian cancer were randomly assigned to receive platinum-based adjuvant chemotherapy (n = 465) or observation (n = 460) until chemotherapy was indicated. Kaplan-Meier analysis was used to compare overall and recurrence-free survival by treatment allocation. In subgroup analyses of pretreatment age, tumor stage, histologic cell type, and differentiation grade, the differences in relative size of effect were tested using a chi-square test for interaction or a chi-square test for trend. All tests of statistical significance were two-sided. RESULTS: After a median follow-up of over 4 years, 245 patients had died or had a recurrence (ICON1: 133, ACTION: 112). Overall survival at 5 years was 82% in the chemotherapy arm and 74% in the observation arm (difference = 8% [95% confidence interval (CI) = 2% to 12%]; hazard ratio [HR] = 0.67, 95% CI = 0.50 to 0.90; P =.008). Recurrence-free survival at 5 years was also better in the adjuvant chemotherapy arm than it was in the observation arm (76% versus 65%, difference = 11% [95% CI = 5% to 16%]; HR = 0.64, 95% CI = 0.50 to 0.82; P =.001). Subgroup analyses provided no evidence of a difference in the size of effect of chemotherapy on survival in any pretreatment subcategory. CONCLUSIONS: Platinum-based adjuvant chemotherapy improved overall survival and recurrence-free survival at 5 years in this combined group of patients with early-stage ovarian cancer defined by the inclusion criteria of the ICON1 and ACTION trials.

Aged↗