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Biomedical subjects

F Gonzalez-Crussi

Publications and source records attributed to F Gonzalez-Crussi.

At least 19 recordsLinked to original sources
PubMed1992

The intriguing nature of gastric tumors in Carney's triad. Ultrastructural and immunohistochemical observations.

The authors describe clinical and pathologic features present in an adolescent girl who had a gastric tumor and mediastinal mass. The latter was shown to be a paraganglioma, and the gastric neoplasm was classified as malignant "leiomyoblastoma," with the use of current histologic criteria. This tumor had metastasized to the liver but not to the lungs. Although the histologic criteria for leiomyoblastoma were fulfilled, no definite evidence of smooth-muscle cell differentiation was present ultrastructurally or by immunostaining methods. Gastric tumors that form part of "Carney's triad" are known to differ clinically and pathologically in important ways from smooth-muscle cell malignant neoplasms that are not part of this syndrome. Some have been classified as gastrointestinal autonomic nerve tumors, but the current study did not confirm this contention. The nature of gastric leiomyoblastomas in Carney's multitumoral association remains undecided.

Adolescent↗
PubMed1992

Bulky naevocytoma of the perineum: a singular variant of congenital giant pigmented naevus.

We describe two instances of a previously unrecognized variant of congenital giant pigmented naevus (GPN), presenting as a bulky naevocytic tumour in the perineal region. In both cases the lesion was present at birth and attained massive dimensions. In addition to the characteristic histological patterns found in GPN, which included extensive areas with a neural appearance, these tumours presented an uncommon tendency to form pseudo-follicular structures lined by naevus cells. No features suggestive of malignant transformation were found. Because GPN may associate with an underlying malignancy, accurate diagnosis of this lesion is important in clinical practice.

Humans↗
PubMed1992

Acute megakaryoblastic leukemia in infants with t(1;22)(p13;q13) abnormality.

Six infants with acute megakaryoblastic leukemia and a translocation (1;22)(p13;q13) were studied. There were five female infants and one male infant, and the age at initial examination varied from 0.8 to 6.5 months (median, 2.3 months). All the patients had hepatosplenomegaly and anemia (6 to 8.3 g/dL), and four patients had thrombocytopenia (9,000 to 63,000/mm3). The bone marrow showed prominent fibrosis in five cases and reticulin fibrosis in one patient at presentation. Crush artifact often made the histologic sections difficult to interpret, but typical megakaryoblasts could be identified in the smears. Biopsy specimens of the liver and lymph node were suggestive of a nonhematopoietic malignant condition because of the cohesiveness of the tumor cells, stromal fibrosis, and the prominent sinusoidal and vascular pattern of infiltration. Immunophenotyping of peripheral blood mononuclear cells was helpful in identifying the blasts as belonging to the megakaryoblastic lineage. Using a panel of mononclonal antibodies, it was also possible to confirm the nature of the infiltration in paraffin sections and to differentiate it from other childhood small round cell tumors, especially neuroblastoma in paraffin sections (typical staining pattern: CD45-, CD43+, vW Factor, Ulex europeus I+, CD20-, CD45RO-, synaptophysin-, chromogranin-, cytokeratin-, desmin-). This special type of infantile acute leukemia can be recognized with confidence if one is aware of its clinical features, peculiar pathologic characteristics, the morphologic features and immunophenotype of the megakaryoblasts, and the unique cytogenetic abnormality.

Biopsy↗
PubMed1992

Fibroma of the meninges in a child: immunohistological and ultrastructural study. Case report.

A case of meningeal fibroma in a 5-year-old girl is described. The lesion presented as a benign intracranial tumor, eroding the frontal bone and protruding under the skin. It was composed of fibroblasts and collagen, embedded in a loose background with focal myxoid changes. The authors describe the patient's clinical presentation and the tumor's histological, immunohistochemical, and ultrastructural features, and discuss its differential diagnosis. It is concluded that fibromas of the meninges should be distinguished from fibroblastic meningiomas.

Child, Preschool↗
PubMed1992

Flow and image cytometric DNA analysis in Ewing's sarcoma.

Ewing's sarcoma is the second most common bone tumor in childhood, with an overall 5-yr survival of 40%. It is one of the poorly differentiated small spherical cell tumors frequently requiring distinction from rhabdomyosarcoma, neuroblastoma, osteosarcoma, primitive neuroectodermal tumor, and lymphoma. The majority of rhabdomyosarcomas, neuroblastomas, and osteosarcomas are aneuploid, whereas Ewing's sarcomas are usually diploid. To determine whether there is any correlation between DNA content, morphology, site, and survival in Ewing's sarcoma and extraosseous Ewing's sarcoma, 21 tumor samples were studied retrospectively (3 extraosseous Ewing's and 18 Ewing's sarcomas). The DNA analysis was performed on disaggregated paraffin-embedded tissue nuclei by flow (FCM) and image (IC) cytometry and correlated with the histology and clinical history. The DNA ploidy by FCM on 17 of 18 Ewing's sarcoma samples was 12 diploid, 1 aneuploid, and 4 tetraploid. By IC, the DNA ploidy on 16 samples was 13 diploid, 1 aneuploid, and 2 tetraploid. Three samples were nonevaluable (1 by FCM and 2 by IC). The agreement between FCM and IC was 12 of 16 (75%). The extraosseous Ewing's sarcoma tumors were 2 diploid and 1 aneuploid by IC. In this study there was no correlation between the DNA ploidy and either the histology, site, or survival.

Adolescent↗
PubMed1991

Monomorphous histiocytoma in a child. Report of a case with ultrastructural features suggestive of dendritic cell differentiation.

The authors describe a rapidly growing soft tissue tumor of predominantly histiocytic composition in an 8-year-old child. The tumor cells were identified as elements of the mononuclear phagocyte system by histologic, histochemical, immunologic, and electron microscopic study. Despite the presence of a minor fibroblastic component, the tumor did not conform to established criteria for a diagnosis of malignant fibrous histiocytoma. Formation of frequent desmosome-like intercellular junctions raised the possibility of dendritic reticulum cell differentiation, since the latter cells seem to be the only elements of the mononuclear phagocyte system that display such specialized cell junctions. The results of immunostaining were discrepant with those reported for normal dendritic reticulum cells, but the currently available information makes it doubtful that the entire neoplastic spectrum of dendritic cell differentiation can currently be diagnosed in surgical pathology.

Child↗
PubMed1991

Urothelial leiomyomatous hamartoma of the kidney.

A 34-year-old woman presented with a renal pelvic mass. A nephroureterectomy including a cuff of bladder, was performed and pathologic examination revealed a hamartoma involving the renal parenchyma and pelvis. The pelvic portion of the tumor was composed of papillae lined by urothelium; the parenchymal portion of the tumor was composed of tubules within a fibromuscular stroma. The authors propose that this is a distinct entity not previously described in the literature.

Adult↗
PubMed1991

Congenital, infiltrating giant-cell angioblastoma. A new entity?

This report describes histopathologic, immunohistologic, and ultrastructural features of a locally aggressive soft-tissue tumor present since birth in an upper extremity of an infant. Because of extensive infiltration of local structures, the lesion had to be treated by amputation. The outstanding histologic feature consisted of nodular cell clusters resembling inflammatory granulomas, often with giant, multinucleated cells. On the basis of our findings, these cellular aggregates were interpreted as distorted attempts at formation of vessels. This interpretation was strengthened by the more obviously vascular structure of the tumor in many areas. A high content of stromal cells positive for factor XIIIa and histocompatability antigen (HLA)-DR was a characteristic that the tumor shared in common with angiomatoid malignant fibrous histiocytoma. However, there were also important differences that singularize the tumor described in the present study. We could find no closely comparable precedent for a lesion with these characteristics in current treatises on infantile soft-tissue tumors.

Female↗
PubMed1991

Cellular hemangiomas ("hemangioendotheliomas") in infants. Light microscopic, immunohistochemical, and ultrastructural observations.

Cellular hemangiomas of infancy, also known as "infantile hemangioendotheliomas," are benign tumors whose dense cellularity may lead to confusion with soft tissue sarcomas. Ultrastructural and immunohistologic study revealed considerable cellular heterogeneity in these lesions despite the monomorphous appearance revealed by routine histologic preparations. Pericytes and endothelial cells are predominant, but fibroblasts and mast cells are regularly present in these tumors. An interstitially located cell of uncertain identification, expressing factor XIIIa and a macrophage marker, not previously noted, comprises an important segment of the cell population. The cell composition reflects the dynamic potential of these lesions, whose natural tendency is to grow, then to become stable, and finally to involute.

Antibodies, Monoclonal↗
PubMed1990

Flow and image cytometric DNA analysis in rhabdomyosarcoma.

Rhabdomyosarcoma is the most common malignant soft-tissue tumor in childhood, with an overall 3-year disease-free survival of 73%. DNA content is known to correlate with prognosis and therapy response in many cancers. To determine the role of DNA content in rhabdomyosarcoma, 23 tumor samples were studied retrospectively: 18 primary tumors and 5 post-chemotherapy recurrences or specimens obtained at second-look surgeries. The DNA analysis was performed on disaggregated paraffin-embedded tissue nuclei by flow and image cytometry and correlated with the histology and clinical history. Of the primary tumors 4 were diploid, 4 polyploid, and 10 aneuploid (9 with a single aneuploid G0G1 peak and 1 multiploid) by flow cytometry. The concordance rate between flow and image cytometry was 19 of 23 (83%); one case did not have flow cytometry available. Most embryonal rhabdomyosarcomas were aneuploid (10 of 12; 83%), and they had a high incidence of recurrence in Stages III and IV (4 of 12; 33%). Although aneuploidy in pediatric cancers may predict a therapeutic response and good prognosis, this was not supported by our findings in rhabdomyosarcoma. The tumor DNA content correlated with the clinical stage but not with the patient's clinical course or tumor histopathological type. DNA content did not appear to be as important a prognostic tool as tumor stage.

Adolescent↗
PubMed1990

Congenital rhabdoid sarcoma with cutaneous metastases.

Rhabdoid sarcoma is an uncommon tumor that most frequently arises in the kidney. Congenital lesions have been documented in only a few cases. Primary or metastatic disease of the skin or subcutis is rare. We report the first case of congenital rhabdoid sarcoma that arose as a subcutaneous tumor in a neonate. Cutaneous metastases were present.

Diagnosis, Differential↗
PubMed1990

Intraabdominal desmoplastic small-cell tumors with divergent differentiation. Observations on three cases of childhood.

We studied three intraabdominal tumors that manifested in childhood and were attached to peritoneum, and in which the histologic pattern suggested metastatic tumor of epithelial nature but gave no evidence of a primary neoplasm in the major abdominal organs. Follow-up observation lasted from 1 to 6 years but never disclosed a primary site. Histologic, immunohistochemical, and electronmicroscopic observations indicated a primitive malignant neoplasm of uncertain histogenesis capable of simultaneously expressing epithelial, mesenchymal, and, less consistently, neural phenotypes. In childhood, the possibility of embryonic neoplasm, such as nephroblastoma occurring in atypical sites, is difficult to exclude. Despite the prevailing uncertainty about histogenesis, combined therapy achieved an apparent cure in one of our cases.

Abdominal Neoplasms↗
PubMed1990

Chondrosarcoma of the head in children.

Chondrosarcoma usually manifests in adult and elderly patients and involves primarily the long bones of the extremities and the osseous pelvis. Children and adolescents may be affected, however. In the young, these neoplasms tend to grow in atypical anatomic sites. A review of clinical experience at a pediatric institution disclosed three chondrosarcomas, including one of mesenchymal type, arising in tissues of the head in patients aged 15, 9, and 13 years. From a review of the pertinent literature as well as clinical experience, it appears that pediatric pathologists are apt to encounter chondrosarcomas rarely, and in those cases the clinical and pathologic features are at variance with those described for chondrosarcoma in adult patients. Low-grade lesions may pursue an indolent course, necessitating prolonged surveillance.

Adolescent↗
PubMed1990

Undifferentiated (embryonal) sarcoma of the liver: ultrastructure, immunohistochemistry, and DNA ploidy analysis of two cases.

We describe the histopathologic, immunohistochemical, electronmicroscopic features, and DNA flow cytometric analysis of 2 cases of undifferentiated (embryonal) sarcoma of the liver. The tumor cells were found to be uniformly nonreactive to antibodies that identify cells belonging to the macrophagic-monocytic system. Focal expression of cytokeratin was an unexpected finding. We conclude that the observations are compatible with origin of this sarcoma from primitive mesenchyme. Uncertainty still exists about histogenesis, but the morphologic features of this neoplasm are quite characteristic. Both tumors demonstrate aneuploid stem lines with high S phase. Whether these findings are prognostically significant remains to be seen.

Adolescent↗
PubMed1990

The role of the complement system in shock and tissue injury induced by tumour necrosis factor and endotoxin.

It has previously been shown that tumour necrosis factor-alpha (TNF), together with bacterial lipopolysaccharide (LPS), induces shock and bowel necrosis in the rat. Since the complement system plays an important role in inflammation and tissue injury, its role has been studied in a similar model in mice. In most of the present experiments, a low dose (0.2 micrograms/g) of TNF was used for priming, followed 30 min later by LPS (3 micrograms/g), and the experiment was terminated in 150 min. It is shown that: (i) TNF exerts no systemic effects by itself; LPS elicits only mild hypotension but causes no lethality; (ii) TNF-primed mice show exaggerated effects of shock, hypothermia, haemoconcentration and bowel injury after LPS; the majority of these mice died within 150 min; (iii) administration of LPS alone mildly activates the complement system in vivo, while TNF alone has no effect; (iv) the effects of TNF and LPS on complement activation are synergistic; (v) the acute development of shock and bowel injury in response to TNF-LPS is dependent on an intact complement system, more specifically C5, since C5-deficient mice were protected from TNF-LPS-induced shock and tissue injury; C5-deficient mice also showed less hypotension, hypothermia, haemoconcentration and better intestinal perfusion compared with C5-sufficient animals; (vi) however, when the priming dose of TNF was raised to 0.5 micrograms/g, most of the C5-deficient mice developed marked hypothermia, hypotension, haemoconcentration, bowel injury and died. Thus, it is concluded that TNF and LPS act synergistically in activating the complement system, which plays an important role in mediating the tissue injury and lethality induced by these agents.

Animals↗