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Biomedical subjects

J C Manivel

Publications and source records attributed to J C Manivel.

At least 109 records · Page 6Linked to original sources

Angiomatoid malignant fibrous histiocytoma: cytologic, immunohistochemical, ultrastructural, and flow cytometric study of 20 cases.

Twenty examples of angiomatoid malignant fibrous histiocytoma (AMFH) were studied. Patients ranged in age from 3 mo to 42 yr, with a mean of 13.4 yr (median 10.5 yr). There were 12 males and eight females. Tumors occurred in the upper extremities in eight cases, lower extremities in 10 cases, and one case each in the buttock and neck. Five patients had local recurrence from 5 to 18 mo after initial surgery, and one patient developed regional lymph node metastases and eventually died of disease. Eight of nine patients with follow-up information were alive from 11 to 31 mo after the diagnosis. Preoperative fine needle aspiration cytology performed in two patients showed features that suggested the correct diagnosis. Ultrastructural and immunohistochemical studies demonstrated findings consistent with a fibroblastic-histiocytic nature of the neoplasm and failed to detect endothelial differentiation of the tumor cells. Flow cytometric analysis performed in six cases, including three recurrent tumors, revealed a DNA diploid pattern in all instances. AMFH appears to be a distinctive low grade sarcoma in the spectrum of malignant fibrous histiocytoma.

Adolescent↗

Mesenchymal chondrosarcoma. An immunohistochemical study.

Mesenchymal chondrosarcoma is an uncommon small-cell neoplasm of bone and soft tissue, the chondrogenic nature of which has been generally accepted. However, the phenotypic attributes of the small-cell population in this neoplasm have not been well characterized, and its relationship to "precartilage mesenchyme" remains unclear. In an attempt to address this issue, we performed an immunohistochemical analysis of nine cases, using antibodies to vimentin, S100 protein, Leu-7 antigen, neuron-specific enolase, synaptophysin, desmin, muscle-specific actin, cytokeratin, and epithelial membrane antigen, and the avidin-biotin-peroxidase complex (ABC) method. The small cells of mesenchymal chondrosarcoma failed to express S100 protein, whereas all components of the tumors (small cells, lacunar chondroblasts, and chondroid matrix) stained for Leu-7 antigen in six cases. Neuron-specific enolase was identified in the small cells of four cases and in the lacunar cells of seven. None contained desmin, actin, cytokeratin, epithelial membrane antigen, or synaptophysin. The immunophenotype of mesenchymal chondrosarcoma resembled that of embryonic cartilage and thus did not contradict the premise that this tumor was the neoplastic counterpart of fetal chondroid tissues. However, immunohistologic studies are not overly helpful in the differential diagnosis between mesenchymal chondrosarcoma and other small round cell lesions.

Adolescent↗

Intratubular germ cell neoplasia in testicular teratomas and epidermoid cysts. Correlation with prognosis and possible biologic significance.

The clinicopathologic features of 4 testicular teratomas in infants, 13 testicular epidermoid cysts in adults, and 8 pure teratomas in adults were compared. Intratubular germ cell neoplasia (ITGCN, carcinoma in situ) was observed in 88% of the teratomas in adults; 63% of the patients in this group had metastases and 37% died. No ITGCN was observed in infant testes with teratomas or adult testes with epidermoid cysts; all of the neoplasms in the latter two groups behaved as benign tumors. ITGCN is associated with malignant potential (P = 0.0004; Fisher's exact test). These tumors comprise a spectrum of clinicopathologic entities that probably reflect differences in pathogenesis.

Adult↗

Primary bronchopulmonary fibrosarcoma of childhood and adolescence: reassessment of a low-grade malignancy. Clinicopathologic study of five cases and review of the literature.

In children, primary tumors of the lung constitute a unique subset of quasineoplastic and unequivocally neoplastic lesions whose benign or malignant potential is not always predictable on the basis of morphologic findings. One such neoplasm in the latter category is the primary bronchopulmonary fibrosarcoma (PBPF). This clinicopathologic, ultrastructural, and immunohistochemical study documents our experience with five PBPFs in newborns and children up to 11 years of age at diagnosis. The tumors were either endobronchial or intraparenchymal in location. A uniform population of interlacing bundles and sheets of densely arranged spindle cells with variable mitotic activity was observed microscopically in each case. Ultrastructurally, the cells had the features of fibroblasts and vimentin was the only immunohistochemical marker identified. Despite the disturbing pathologic findings, the four children with more than 1 year of follow-up have survived well 4 to 9 years after surgical resection. Our results are compared with the 21 cases of PBPF reported in the literature and the differential diagnosis is discussed.

Bronchial Neoplasms↗

Gross cystic disease fluid protein-15 as a marker for breast cancer: immunohistochemical analysis of 690 human neoplasms and comparison with alpha-lactalbumin.

The identification of metastatic carcinoma of the breast may be difficult in the absence of a previous history of breast cancer. Various immunophenotypic markers have been introduced to aid in this process. A monoclonal antibody directed at a 15-kilodalton (kd) gross cystic disease fluid protein (GCDFP-15) was applied immunohistochemically to paraffin sections of 105 breast cancers and 585 nonmammary malignancies in order to assess its value in this context. In addition, GCDFP-15 was compared with another putative mammary epithelial marker, alpha-lactalbumin (ALA), with respect to sensitivity and specificity for a diagnosis of breast carcinoma. Overall, the rates of specificity and sensitivity and the predictive value of a positive result for GCDFP-15 were 95%, 74%, and 74%, respectively. Corresponding statistical parameters for ALA were 50%, 50%, and 23%. A consistent congruency between the reactivity patterns of primary and metastatic breast cancers was noted for GCDFP-15 but not for ALA. Besides mammary carcinomas, the major tumor types that expressed GCDFP-15 were carcinomas of the salivary glands, sweat glands, and prostate. Since the latter three types of lesions are unlikely to be diagnosed as metastatic breast cancer, statistical indices were recalculated after exclusion of these three tumor types. Following this exclusion, the adjusted rate of specificity of GCDFP-15 and the predictive value of a positive result for a diagnosis of metastatic carcinoma of the breast were each 99%. In contrast, predictive parameters for ALA were not altered. These results show that GCDFP-15 is a specific marker for breast cancer and is superior to ALA in this respect.

Adenocarcinoma↗

Classical and cellular (atypical) congenital mesoblastic nephroma: a clinicopathologic, ultrastructural, immunohistochemical, and flow cytometric study.

Sixteen cases of congenital mesoblastic nephroma (CMN) were studied. The tumors showed variable patterns of growth, degrees of cellularity, and mitotic activity. Six tumors had the classical pattern of CMN, seven were of the cellular or atypical variant and three showed combined features. The mean ages at presentation were 16 days, 5.3 months, and 2.3 months, respectively. Average size and weight were 5.1 cm and 94 g for classical CMN, 9.1 cm and 620 g for cellular CMN and 10.5 cm and 150 g for combined tumors. Cyst formation, hemorrhage and necrosis were confined to cellular CMNs and to cellular areas of combined CMNs. Mitotic activity ranged from 0 to 1/10 high-power fields (HPFs) in classical tumors to 25 to 30/10 HPFs in cellular tumors. Clear cell sarcoma-like areas were observed in three neoplasms. In ten cases there was invasion of perirenal fat; in one case each, invasion of the psoas muscle, renal vein wall, and renal vein lumen was observed. Ultrastructural and immunohistochemical studies showed features consistent with myofibroblastic differentiation. Flow cytometric analysis revealed euploidy in one classic CMN, one cellular CMN and in classic areas of a combined CMN; cellular areas of the latter tumor were aneuploid. All patients with follow-up were alive without evidence of disease after a mean period of 5 years following nephrectomy alone. No correlation was observed between the pathologic features assessed and the biologic behavior of these neoplasms.

Aneuploidy↗

The outcome of renal transplantation in children with the prune belly syndrome.

Children with the prune belly syndrome are at high risk for renal failure. The effect of the prune belly syndrome on the outcome of renal transplantation was evaluated in a retrospective study in which 8 transplant recipients with this syndrome were randomly matched with 13 control, nondiabetic transplant patients. The parameters evaluated were patient and graft survival, renal function and interval until transplantation. The patients were matched by computer for age at transplantation, date of transplantation, immunosuppressive therapy used and type of donor. Data were analyzed by the Gehan test. There was no statistically significant difference in patient deaths (1 versus 3), graft survival (75 versus 69%) or graft function between patients with the prune belly syndrome and controls. Patients with the prune belly syndrome waited a shorter interval for transplantation than did controls (no statistically significant difference) because the distensible abdominal wall characteristic of the syndrome permits placement of an adult kidney in a young child. The prune belly syndrome did not adversely affect the outcome of renal transplantation in these patients.

Adolescent↗

Alveolar variant of infiltrating lobular carcinoma of the breast with stromal osteoclast-like giant cells.

An alveolar variant of infiltrating lobular carcinoma of the breast in which a prominent feature was the presence of numerous osteoclast-like giant cells in a richly vascularized stroma is described. A similar stromal reaction has previously been reported in 46 breast carcinomas of various histologic types, but only once in a pure infiltrating lobular carcinoma. The fine needle aspiration cytological and histological appearances and the immunohistochemical and ultrastructural features of this tumor are reported. Our study confirms the histiocytic nature of the multinucleated giant cells and their similarity to normal osteoclasts.

Adult↗

Immunohistochemistry in the differential diagnosis in the second-look operation for ovarian carcinomas.

The second-look laparotomy is the procedure of choice to obtain peritoneal and nodal biopsies from patients who have received a full course of chemotherapy for an ovarian malignancy. If there is pathologic evidence of persistent tumor, chemotherapy is continued. In most cases, the histopathologic interpretation is straightforward in terms of positive or negative results. However, atypical mesothelial reactions or glandular inclusions, or both, in lymph nodes are potential and real difficulties in differential diagnosis. This study evaluated a panel of immunohistochemical markers, namely carcinoembryonic antigen (CEA), Leu M1, human milk fat globule protein, cytokeratin, epithelial membrane antigen, and LN2 antibody for their usefulness in differentiating benign epithelium and mesothelium from carcinoma. Only CEA and Leu M1 were specific and sensitive, respectively, for malignant tumors in this study.

Antigens, Neoplasm↗

Prune belly syndrome: clinicopathologic study of 29 cases.

The clinical course and the pathologic features of 29 patients with the prune belly syndrome (PBS) are reviewed. There were 26 males and 3 females. In addition to the classical triad of deficient abdominal musculature, urinary tract abnormalities, and cryptorchidism, a broader spectrum of other defects was found including musculoskeletal (58%) and gastrointestinal (31%) abnormalities. Genital anomalies were present in all three female patients. Many of these defects may be inapparent at birth, but are the cause of morbidity and mortality later in life. Severe urinary tract maldevelopment and pulmonary hypoplasia as part of the oligohydramnios syndrome was the most common cause of perinatal deaths. In these patients, major portions of the renal parenchyma were dysplastic, but in survivors, renal dysplasia, when present, was minor by comparison, and affected less than 1/3 of the parenchyma. Although several questions remain unanswered, we believe that the PBS results from the effect of one or more teratogenic agents on the somatic mesoderm, producing inappropriate mesenchymal development and inadequate mesenchymal-epithelial interactions that lead to abnormal development and dilatation of some of its derivatives (abdominal muscles, ureter, bladder, prostate, urethra, and gubernaculum). Although abnormalities in derivatives of the intermediate mesoderm (kidney) may also be produced by the injurious agent(s), they are more likely a result of urinary obstruction. Abnormalities in other organs and systems are the consequence of oligohydramnios.

Abnormalities, Multiple↗

Central nervous system lymphomas. Immunohistochemical and clinicopathologic study of 26 autopsy cases.

Twenty-six malignant lymphomas involving the central nervous system were studied. Eleven were primary (P) and 15 were systemic (S). Eight cases (3 P, 5 S) occurred in immunocompromised patients. Age at presentation in immunocompromised patients was typically younger than in the nonimmunocompromised patients. Presenting complaints of central nervous system involvement included headache, seizures, personality changes, memory lapses, ataxia, cranial nerve symptoms, and impaired consciousness. Cerebrospinal fluid involvement was seen only in 3 S cases. In 8 of the P cases, the diagnosis was first established at autopsy; in 6 of the S cases, central nervous system involvement was first documented at autopsy. Survival was longer in treated patients than in those who received no therapy (5 months in P cases and 9.3 months in S cases; 2.3 months without therapy). Regardless of therapy, the average survival of immunocompromised patients was 2.4 months. The majority of cases were multifocal. Of the P cases, 1 was of low histologic grade, 9 were of intermediate grade, and 1 was of high grade. Of the S cases, 5 were of low grade, 9 were of intermediate grade, and 1 was of high grade. Immunophenotypic studies were performed on formalin-fixed, paraffin-embedded tissue with antisera against common leukocyte antigen (all reactive), B-cell markers (L26, MB2, LN1, and LN2), T-cell markers (UCHL1 and MT1), Leu-M1, Leu-7, and HLA-DR (LN3). Two S cases were of T-cell phenotype; all others were of B-cell derivation. Eleven cases were HLA-DR positive (all of B-cell phenotype). One T-cell lymphoma was reactive for Leu-7. All cases were nonreactive for Leu-M1. All cases in immunosuppressed patients and all P cases were of B-cell phenotype.

Adolescent↗

Postirradiation sarcomas of the head and neck.

We discuss four cases of postirradiation sarcomas of the head and neck. Two cases were metachronous sarcomas that appeared after operation and irradiation for primary sarcomas, 1 case was a mandibular malignant fibrous histiocytoma that developed on the opposite side of the jaw from a malignant histiocytic neoplasm that was irradiated 8 years previously, and 1 case was a laryngeal tumor that appeared 5 years after combined operative and radiation therapy for a laryngeal squamous carcinoma. Immunohistochemical studies more precisely defined and classified these tumors, and assisted in determining a therapeutic protocol. The therapy for postirradiation sarcomas includes extirpative operation when possible, but the role of chemotherapy is uncertain. The aggressive behavior of these neoplasms was attested to by the death of three patients within 18 months of their operations.

Adult↗

Pulmonary artery trunk sarcoma: a clinicopathologic, ultrastructural, and immunohistochemical study of four cases.

Pulmonary artery sarcomas are rare tumors that arise in the region of the bulbus cordis, the embryologic structure that gives rise to the pulmonary trunk. Nearly 100 cases have been reported in the literature, yet considerable debate exists regarding the histogenesis and biologic properties of these neoplasms. We report four additional cases in which ultrastructural and immunohistochemical studies demonstrated that these tumors contain cellular constituents with features of myofibroblastic, cartilaginous, and osteogenic differentiation. Polyphenotypic expression of several mesenchymal lineages suggests that the progenitor cell has pluripotential properties. Our findings and a review of the literature appear to confirm that pulmonary artery sarcomas are histopathologically heterogeneous, possibly reflecting the indeterminate character of the mesenchymal cell(s) of origin. The poor prognosis is attributable to the critical anatomic location of the neoplasm rather than its metastatic potential, which is low.

Actins↗

Lesions of the breast in children exclusive of typical fibroadenoma and gynecomastia. A clinicopathologic study of 113 cases.

This large series of cases demonstrates that the breast of the child or adolescent is the potential site for a number of interesting lesions exclusive of fibroadenoma and gynecomastia. Adenoma of the nipple, juvenile papillomatosis, and juvenile or cellular fibroadenoma should be correctly diagnosed to insure conservative surgical management and appropriate clinical follow-up. The results of the Juvenile Papillomatosis Registry suggest that this lesion is a morphological marker of potentially more serious breast disease in the future. Recognition of a cellular fibroepithelial neoplasm, especially in young black females, as a cellular fibroadenoma will hopefully eliminate the need for the diagnosis of "benign" cytosarcoma phyllodes. On occasion, the equivocal case may be labeled a "phyllodes tumor fo indeterminate biologic behavior." Those neoplasms with stromal atypia and mitotic activity, overgrowth of stroma with epithelial dissociation, and sarcomatous elements are appropriately designated as cytosarcoma phyllodes. In contrast to vascular tumors of the breast in adults, the majority of vascular lesions in the child's breast are bening and represent either a capillary or a histiocytoid hemangioma. Our experience and reports in the literature support the conclusion that most malignancies of the breast in children are metastatic. Rhabdomyosarcoma was the most common example in our study, but secondary involvement by acute leukemia or malignant lymphoma also occurs.

Adenofibroma↗