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[Primary malignant liver tumors in children].

The review is based on the analysis of 17 national and 58 foreign publications dealing with the epidemiology, classification, age features, morphology, ultrastructure, histogenesis, complications and outcomes of primary malignant tumors of the liver in children. This analysis showed primary tumors of the liver in children to occupy the third place among neoplasia of the abdominal cavity and retroperitoneal space. There are no universally accepted terms for their designation and this prevents the development of a unique classification. Under 3 years, tumors develop predominantly from the embryonal tissue: hepatoblastomas of epithelial and mixed types frequently combined with congenital disorders and malformations of other organs. At more advanced age, hepatic carcinoma, a tumor of the adult type, is found. The review deals with the problems of histogenesis and classification of primary hepatic tumors in children. An opinion is given on the entodermo-mesodermal nature of embryonal hepatic tumors from the pluripotential blastema capable of malignization both into entodermal and mesodermal components. The prognosis of the disease is poor, 90% of children with hepatoblastoma and 80% with hepatocarcinoma die within one year after the diagnosis is established.,

Carcinoma, Hepatocellular↗

Type II collagen in mixed tumors of the skin. An immunohistochemical study and new speculations concerning histogenesis.

We studied 11 cases of mixed tumor of the skin with a polyclonal antibody against human type II collagen. Two of the cases were of the eccrine type, six were apocine, and three were malignant. In all of the benign cases of mixed tumor and in some of the malignant type, "chondroid" and mucinous portions stained. The clearly epithelial portions of the tumor stained as well, and staining was most intense in the eccrine types and least intense in the malignant types. The positive staining in mixed tumors may help elucidate the histogenesis of these unusual tumors as well as help differentiate among the various subtypes.

Adenoma, Pleomorphic↗

Congenital mesoblastic nephroma: an immunohistochemical and lectin study.

Eight cases of congenital mesoblastic nephroma (CMN) were examined. Three CMNs were of the classical (typical) variant, two were cellular (atypical), and three showed a mixed pattern. A panel of nephron segment-specific tubular epithelial markers (the lectins Tetragonolobus purpureas, Phaseolus vulgaris erythroagglutinin, and Arachis hypogaea and antibodies to epithelial membrane antigen, cytokeratin, and Tamm-Horsfall protein) were used to differentiate epithelial structures within the tumor. Antibodies against vimentin, desmin, and muscle-specific actin were used as mesenchymal markers. A monoclonal antibody to the long (embryonic) form of polysialic acid (PSA) on the neural cell adhesion molecule was used as a putative renal oncodevelopmental marker. An antibody to proliferating cell nuclear antigen also was applied, which revealed increased proliferative rate in cellular CMNs. In addition to clearly entrapped native renal tubules, CMNs contain tubular structures with immature, dysplastic epithelium and occasional epithelial cell clusters embedded deep within the tumor. These immature tubules and clusters express distal nephron, including collecting duct markers and, occasionally, vimentin and PSA. We propose that these primitive tubules and epithelial structures may originate from the ureteric bud. An epithelial differentiation of the tumor cells also is possible. In one pure cellular CMN and two mixed CMNs the cellular component showed diffuse staining for PSA. The PSA (neural cell adhesion molecule) expression of the cellular component suggests that CMN may originate from the uninduced nephrogenic mesenchyme.

Child↗

Mucinous tumors of the ovary. Ultrastructural studies of mucinous cystadenomas with histogenetic considerations.

On the basis of light microscopic and scanning and transmission ultrastructural observations, we have classified benign mucinous ovarian neoplasms into two types: a pure endocervical type and a mixed intestinal endocervical type. We have also reviewed the literature pertinent to the histogenetic origin of the ovarian mucinous cystoma and found convincing support for a mesodermal, coelomic epithelial, rather than a teratomatous, derivation.

Adult↗

Expression of MUC5AC apomucin in transitional cell carcinomas of the urinary bladder and its possible role in the development of mucus-secreting adenocarcinomas.

The histogenesis of primary nonurachal mucus-producing adenocarcinomas of the urinary bladder including signet ring cell carcinomas remains to be elucidated, since the normal bladder contains neither columnar nor mucus-secreting glandular epithelium. Based upon the assumption that adenocarcinomas may develop secondarily from pre-existent transitional cell carcinomas (TCC) by a metaplastic process, it was the purpose of the current immunohistochemical study to analyze whether urothelial carcinomas are capable of secreting MUC5AC apomucin, using the monoclonal antibody 45MI. This antibody has been initially demonstrated to strongly react with the mucus-producing columnar cells of the surface gastric epithelium, recognizing a specific epitope located on the peptide core of glycoproteins as major components of mucins. Nine of 64 uniformly differentiated papillary (14.1%) and 5 of 66 nonpapillary (solid) TCC with a uniform urothelial differentiation (7.6%) expressed the MUC5AC antigen, yielding an overall incidence of 10.8%. Transitional cell carcinomas with a focally altered cellular and structural differentiation (squamous cell, pseudoglandular, true glandular and mixed differentiation) stained positively in a substantially higher percentage of 43.8% (21 of 48 cases). A positive immunoreactivity was also observed in 3 of 19 mixed transitional cell and nonurothelial carcinomas. The tumor-associated resurgence of normally cryptic MUC5AC antigenic determinants in transitional cell carcinomas is considered as a re-expression of oncofetal antigenicity, probably as a result of the embryologic origin of the urinary bladder from the pluripotent tissues of the cloacal endoderm and the mesodermal wolffian ducts. Our findings may help to better understand the histogenetic development of mucus-secreting vesical adenocarcinomas from pre-existent urothelial carcinomas.

Adenocarcinoma, Mucinous↗

[Sarcomas of the uterus: immunohistochemical characterization and diagnosis].

Pathologic findings and immunohistochemical characterizations of 18 cases of uterine sarcomas were studied. In endometrial stromal sarcoma (ESS), 5 out of 10 cases had ovarian sex cord-like pattern and 4 out of 10 cases had smooth muscle differentiation. Immunohistochemical findings showed vimentin, desmin and cytokeratin positive in 9/10, 6/10, 2/10 cases respectively which reflects that ESS may differentiate into both epithelium and muscle components morphologically. In malignant mixed Mullerian tumors (MMT), its carcinomatous structure may be positive about vimentin, and its sarcomatous structure may be positive to the epithelium markers, which indicates that both the sarcoma and carcinoma structures have possibly a common origin. It is considered to be of value for the diagnosis of MMT, if the tumor has differentiated both epithelium and mesoderm components or to be positive to myoglobin, NSE* in immunoreaction, accompanying with the morphologic characterizations of the tumor.

Desmin↗

Surface epithelial tumours of the ovary.

Surface epithelial tumours (SET) constituted 65.7% of all the ovarian tumours. Benign tumours were 182 (71.9%), of low malignant potential (LMP) 11 (4.4%) and frank malignant 60 (23.7%). Maximum number of cases, 102 (40.3%) belonged to 3rd decade. Mean age for serous cystoma was 31.5% years as compared to 30.8 years for mucinous cystoma. The commonest presenting feature was the abdominal lump observed in 182 cases (71.9%) and pain in abdomen in 120 (47.4%). Serous cystomas were t he most frequent tumours and comprised of 32.21% of all the ovarian tumours or 46.01% of all the SET or 65.56% of all the cystic SET. Seventeen (11.7%) of serous tumours were bilateral. Mucinous cystomas constituted 14.55% of all the ovarian tumours or 30.8% of all the SET. These tumours were bulky (78.6%; 15 cm diameter) and multilocular (83.9%). Mucinous cystadenocarcinoma was the commonest malignant epithelial tum our (36.6%). Endometroid carcinoma comprised 3.65% of all the SET or 8.4% of all the ovarian malignancy. Squamous metaplasia was seen in one case whereas 2 cases were of mesodermal mixed tumour with heterologous element as rhabdomyosarcoma. Clear cell carcinoma, Brenner tumour and unclassified group constituted 0.79%, 1.18% and 1.58% of all SET respectively.

Adenocarcinoma, Clear Cell↗

Epithelial-mesenchymal transformation of a newly established cell line from ovarian adenosarcoma by transforming growth factor-beta1.

We report on 2 cell lines (designated OVAK-I and OVAK-II) established from an adenosarcoma, a rare variant of a malignant Mullerian mixed tumor, of the ovary. OVAK-I was not tumorigenic in nude mice and showed no responsiveness to transforming growth factor (TGF)-beta1, whereas OVAK-II was tumorigenic and had various biological properties. Light- and electron-microscopic studies showed that the morphology of the cells was converted from an epithelial to a mesenchymal form by TGF-betaI. Furthermore, the protein and mRNA expressions of the epithelial marker carcinoembryonic antigen and the mesenchymal marker vimentin were down- and up-regulated, respectively, by TGF-beta1. These findings suggested that TGF-beta1 plays a role in epithelial-mesenchymal transformation and support the single-stem-cell theory which explains the origin of malignant Mullerian mixed tumors: that the epithelial and mesenchymal components of these tumors are of common stem-cell origin.

Adenosarcoma↗

Ultrastructure of chondroid syringoma: role of the myoepithelial cell in the development of the mixed tumor of the skin and soft tissues.

The ultrastructure of a chondroid syringoma is described. The tumor nests are formed by clumps, solid cords and tubular structures distributed diffusely throughout a stroma of varying density. Two different types of cells form this tumor: dark cells forming the inner layer of the tubuloalveolar and ductal structures and having epithelial features, and light cells which form the outer layer of the tubular structures and show myoepithelial differentiation. The myoepithelial cells seem to be responsible for the production of the chondroid areas and thus the mixed appearance of the tumor. The epithelial cells forming the solid nests of the tumor show intracytoplasmic luminal formation and other features that favor the origin of chondroid syringoma from the eccrine sweat gland duct.

Epithelium↗

Malignant müllerian tumours of the uterus.

Thirty-two cases of malignant Müllerian tumours of the uterus of the mixed mesodermal type were investigated. Histological examinations were performed on tissue obtained during curettage or biopsy and/or surgical removal of the uterus; in 4 cases postmortem examination was also carried out. The biphasic tumours of the uterus were classified into four groups and comprised 44% of alle uterine sarcomas. In 10 of the 28 cases in which uterine curettage or cervical biopsy was carried out, the final diagnosis was clarified when the tumorous uterus was removed. The prognosis was poorer for heterologous than for homologous tumours. In the heterologous group, the prognosis was better for the form with chondrosarcomatous features (2 patients are still alive after more than 5 years) than for the form containing striated muscle tissue (all 5 patients died within 1 year). Attention is drawn to the fact that the adenosarcoma should be considered a separate type; its prognosis was relatively good (2 such patients are still alive after more than 5 years).

Adenocarcinoma↗

Immunolocalization of extracellular matrix components in mixed tumors of the skin.

Mixed tumors of the skin are characterized by a proliferation of epithelial cells embedded in a mesenchymal matrix with a wide spectrum of histologic appearances. The characterization of the extracellular matrix components of mixed tumors of the skin has so far received little attention. We performed an immunohistochemical study of type IV collagen, laminin, fibronectin, and tenascin distribution in a series of 10 mixed tumors of the skin. Laminin localized at the basement membrane around solid epithelial nests and tubulo-alveolar structures, whereas type IV collagen, fibronectin, and tenascin were also expressed in the myxoid stroma. Tenascin and fibronectin localized in the chondroid matrix. The extracellular matrix components were prominently expressed in mixed tumors of the skin, suggesting that they could play an important role in the formation and organization of myxoid and chondroid matrices and in the epithelial-mesenchymal interactions of these tumors.

Adenoma, Pleomorphic↗

[A classification of uterine tumors based on embryogenesis].

On the basis of their results of studies of human embryos and foetuses, the authors believe that the myometrium is derived from the primitive mesenchyme, while the endometrium is derived from the coelomic mesothelium. The myometrium is the site of non-specific tumours common to all soft tissues (of other organs): leiomyoma, lipoleiomyoma, leiomyoblastoma, leiomyosarcoma; vascular tumours: angioma, haemangiopericytoma. The endometrium is the site of tumours derived either from the epithelial contingent (of glandular tubules): adenoma, adenocarcinoma; or from the epithelio-connective tissue contingents: mullerian dysplasia, carcinosarcoma (homologous mixed mesodermal tumour). The authors classify heterologous mixed mesodermal tumours in a separate group; they believe that they constitute true mixed mesodermal tumours derived from two cell clones: one of mullerian origin and the other derived from the primitive mesenchyme.

Endometrium↗

Peripheral odontogenic fibroma: a clinicopathologic study.

The clinicopathologic features of 30 cases of peripheral odontogenic fibroma are reviewed. The age distribution is wide (11 to 76 years), and there is a slight predilection for males. The majority of the lesions (93%) occurred in blacks on the attached gingiva, and with equal frequency in the maxilla and mandible. Size varied between 1 and 3 cm in diameter. One case recurred after 14 months. Histologically, the lesions are nonencapsulated and poorly delineated. The amount of odontogenic epithelium varies considerably and consists usually of small islands or strands, although larger follicles are sometimes present. In one case the epithelial cells had a clear cytoplasm, whereas in another it was granular and eosinophilic. Origin from the surface oral mucosa can sometimes be seen. The connective tissue component is usually cellular, but collagenous, myxomatous, and mixed forms occur. Calcifications were present in 22 cases and consisted of tissue interpreted as either dentinoid, cementum, bone, or dystrophic calcific material.

Adolescent↗

The epidemiology of sarcomas of the uterus.

Data from nine population-based cancer registries (1973-81) were analyzed to examine the demographic characteristics that are related to the occurrence of sarcomas of the uterus. Leiomyosarcoma and mixed mesodermal sarcoma accounted for 86% of 1,452 uterine sarcomas reported, and both were more common in black women than in white women. The incidence of mixed mesodermal sarcoma was low through middle age, after which it rose sharply. The incidence of uterine leiomyosarcoma, however, was highest in middle-aged women and declined thereafter. The age-incidence pattern of uterine leiomyosarcoma resembles that of carcinomas of the female reproductive tract, unlike the pattern of other uterine sarcomas or sarcomas occurring elsewhere. This suggests that one or more factors that play a role in the etiology of carcinomas of the reproductive tract may be involved in the etiology of this tumor as well.

Adult↗

Malignant mixed osteogenic tumours of the breast. An ultrastructural study of two cases.

Two cases of malignant mesodermic tumours of the human mammary gland with osteogenic and chondrogenic structures were analysed by optical and electronic microscopical means. One of them was associated with an adenocarcinoma of the gland. The histological pattern was similar to that of those cases previously examined under the optical microscope in the mammary gland and in extraskeletal osteogenic sarcomas of soft tissues. When investigated under the electron microscope, the chondroblasts possessed a highly developed RER in active synthesis with an amorphous material which contributed to the building up of the ground substance matrix of the tumorous cartilage. Osteoid fields with scattered osteoblasts appear throughout the tumorous stroma and were associated with calcium deposits. They were continuous with fibroblasts and mesenchymal undifferentiated cells of a very immature character. Giant cells of osteoclastic type were included within the mononucleated mesenchymal cells mimicking bone osteoclastoma. The presence of all these cell types suggests the existence of a common malignant origin, the stem cell being differentiated into epithelial carcinomatous and mesenchymal sarcomatous chondral and osteogenic tissues.

Adenocarcinoma↗

Tumors of the epididymis.

Tumors of the epididymis, both primary and secondary, whether benign or malignant are very rare. The elements of the spermatic duct system, whether inside the testis or outside it in the epididymis and spermatic cord, exhibit only 1/20th of that of the germinal epithelium. In accordance with the mesodermal, or mesothelial origin of the whole spermatic ducr system, tumors of the epididymis and spermatic cord are - with few exceptions - mesenchymal tumors, the most frequent of these being the so-called adenomatoid tumor, a benign slowly growing tumor. With regard to the relative frequency of the various extratesticular tumors, a British panel has shown that one quarter of its cases are adenomatoid tumors, a second quarter sarcomas of children and juveniles (rhabdomyosarcomas and embryonic sarcomas), a third quarter sarcomas of adults and aged, while the last quarter includes simple benign mesenchymal tumors, mixed tumors, metastatic tumors, and others. There is only one extremely rare epithelial tumor of the epididymis, the papillary cystadenoma, which seems to be a partial menifestation or forme fuste of Hippel-Lindau's disease. So far only 40 cases have been described in the world literature. Therapy of intrascrotal but extratesticular tumors always ought to be surgical: if the tumor proves to be benign, simple excision suffices. In cases of malignancy, immediate inguinal lymphadenectomy and - as with testicular tumors - lymphography followed by retroperitoneal lymphadenectomy. Whether one should, in principle, then continue treatment either chemotherapeutically or radiotherapeutically, or do so only when there is evidence of metastasis, is a question whiich still remains open.

Adult↗

Giant cavernous sinus teratoma: a clinical example of a rare entity: case report.

OBJECTIVE AND IMPORTANCE: Teratomas represent 0.5% of all intracranial tumors. These benign tumors have tissue representative of the three germinal layers: ectoderm, mesoderm, and endoderm. Most teratomas are midline tumors located predominantly in the sellar and pineal regions. Cavernous sinus location is very rare; only two purely intracavernous teratomas have been reported. CLINICAL PRESENTATION: A 14-year-old boy presented with a history of progressive right eye proptosis and visual acuity impairment, headaches, and a neuralgia-like facial pain in the right V1 distribution. A head computed tomographic scan and magnetic resonance imaging disclosed a large tumor filling the right cavernous sinus and extending into the ipsilateral middle fossa. These scans also demonstrated mixed signals derived from different tissues conforming to the tumor (fat, cartilage, muscle strands, bone, and a primordial tooth). Heterogeneous enhancement was seen after infusion of contrast medium. Significant bone erosion and remodeling was evident in the middle fossa floor and right orbit, with secondary proptosis. A presumptive diagnosis of mature teratoma was made. INTERVENTION: With the use of a right frontotemporal interfascial approach, a combined extra- and intradural dissection of the tumor was performed. The lesion entirely occupied the cavernous sinus, laterally displacing the Gasserian ganglion and trigeminal branches (predominantly V1 and V2). The internal carotid artery and Cranial Nerve VI were medially displaced by the tumor mass. The lesion was composed of different tissues, including hair, fat, cartilage, muscle, nerve-like tissue, bone, and a primordial tooth. The tumor was removed completely, and the pathological report confirmed the diagnosis of a mature teratoma. There was no evidence of recurrence at the 8-month follow-up examination. CONCLUSION: Because of the lesion's location in the lateral wall of the cavernous sinus, a total removal was achieved with the use of standard microsurgical techniques. Knowledge of the microanatomy is essential in treating intracavernous pathology. We present the third reported case of a giant mature teratoma of the cavernous sinus.

Adolescent↗

Uterine sarcoma in the south of Israel: study of 36 cases.

BACKGROUND: Uterine sarcomas are rare, charaterized by rapid clinical progression and poor prognosis, and their management has been a challenge. The purpose of this study was to investigate the clinical and histologic findings, treatment, and outcome of patients with uterine sarcoma in the south of Israel. METHODS: Data from the files of 36 patients with uterine sarcoma who were managed at the Soroka Medical Center between January 1961 and December 1994 were evaluated. RESULTS: The 5-year survival rate was 32% overall; 63% for 9 patients with endometrial stromal sarcoma (ESS). 30% for 14 patients with mixed mesodermal sarcoma (MMS) and 18% for 13 patients with leiomyosarcoma (LMS): 41% for 22 patients with Stage I and 19% for 14 patients with Stages II, III, and IV. Only the difference in the 5-year survival rate between ESS and LMS was statistically significant (P < 0.05). Eleven patients (30.6%) were treated with surgery alone, 4 (11.1%) with surgery followed by pelvic radiotherapy, 11 (30.6%) with surgery followed by chemotherapy, 8 (22.2%) with surgery followed by pelvic radiotherapy and chemotherapy, one (2.8%) with chemotherapy alone, and one (2.8%) had no treatment. CONCLUSIONS: Uterine sarcomas are aggressive tumors with a poor prognosis. The treatment is surgery generally followed by adjuvant pelvic radiotherapy and/or systemic chemotherapy.

Adult↗