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Biomedical subjects

M V Gresik

Publications and source records attributed to M V Gresik.

34 records · Page 2Linked to original sources
PubMed1988

Endovascular papillary angioendothelioma in the spleen.

Endovascular papillary angioendothelioma is a rare vascular neoplasm originally described as involving the skin and subcutaneous tissues. No case has been reported to involve the spleen, although structurally similar splenic neoplasms have been reported under different names. A 5-year-old boy presented with isolated splenomegaly and was found to have endovascular papillary angioendothelioma of the spleen. Studies supported the suggestion that the endovascular papillary angioendothelioma manifests focal "high" endothelial differentiation.

Child, Preschool↗
PubMed1987

Malignant rhabdoid tumor of the extremity.

Two cases of malignant rhabdoid tumor (MRT) involving the sciatic nerve are described. Despite the close affiliation of the tumor with neural tissue, staining characteristics in these patients do not suggest a neural origin of MRT. Survival in patients with renal and extrarenal MRT has been poor. Our patients were treated with an aggressive chemotherapy program using cisplatin, Adriamycin (doxorubicin), vincristine, cyclophosphamide, actinomycin D, and DTIC. One child died of progressive disease; the other is well 35 months after diagnosis.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1987

The Gardner-Silengo-Wachtel or genito-palato-cardiac syndrome: male pseudohermaphroditism with micrognathia, cleft palate, and conotruncal cardiac defect.

We report on two sib fetuses with similar abnormalities detected prenatally by ultrasound. The first fetus had micrognathia, was without cleft palate, and had low-set ears, double outlet right ventricle with a ventricular septal defect, and 46,XY gonadal dysgenesis. The second sib fetus was born with cleft lip and palate, micrognathia, transposition of the great vessels, ventricular septal defect, a right-sided aorta arch, and bilateral cystic kidneys with hypospadias. We were able to identify 11 additional cases in the literature with similar findings. We think this set of defects is a recognizable syndrome that appears to be inherited either as an autosomal recessive or as an X-linked recessive and may overlap with the Smith-Lemli-Opitz syndrome.

Abnormalities, Multiple↗
PubMed1986

The Perlman familial nephroblastomatosis syndrome.

In 1973, Perlman et al described a familial syndrome of bilateral renal hamartomas with or without nephroblastomatosis, macrosomia, islet cell hypertrophy, unusual facies, and early lethality. Two additional sibs were recently reported by Neri et al [1984]. We report on two sibs with polyhydramnios, fetal ascites, and abdominal muscular hypoplasia, visceromegaly, and subsequent development of Wilms tumor in one of them. Delineated features of this syndrome include visceromegaly, macrosomia, renal hamartomas, nephroblastomatosis, cryptorchidism in males, unusual facial appearance, polyhydramnios, fetal ascites, and Wilms tumor but do not include hemihypertrophy, omphalocele or umbilical abnormalities, aniridia, or other conditions known to be associated with Wilms tumor. This condition should be considered primarily in the differential diagnosis of fetal ascites without hydrops and possibly in the differential diagnosis of familial Wilms tumor, polyhydramnios, congenital hepatomegaly, or nephromegaly.

Adult↗
PubMed1986

Natural history of histiocytosis X: a Pediatric Oncology Group Study.

The best therapy for patients with histiocytosis X with disease involvement other than isolated bone lesions but without organ dysfunction is unclear. This retrospective study was undertaken to define the natural history of this group of patients. In 25 of the 92 studied patients, there was no progression of the disease after diagnosis. In 53 surviving patients, the disease either continuously progressed (40) or recurred intermittently (13). The onset of last disease activity was 24 months or less for 55% of these children. A fatal outcome occurred in 14 children. All of these children developed organ dysfunction and 11/14 died during or before the second year of disease. These three different outcomes could not be predicted from the parameters evaluated; however, the disease that never abated but was continuously active was associated with a suboptimal outcome, and the development of organ dysfunction was a grave prognostic sign.

Adolescent↗
PubMed1985

Intracardiac undifferentiated sarcoma in infancy.

A rare case of an intracardiac undifferentiated sarcoma in a 3 month old infant is described together with the clinical, angiographic, echocardiographic, surgical and histopathologic findings. The tumor was successfully removed surgically, and monthly echocardiographic follow-up is being performed.

Echocardiography↗
PubMed1981

Pneumonia in hairy-cell leukemia.

Hairy-cell leukemia (HCL) is an unusual malignant hematologic disorder involving splenomegaly, pancytopenia, and circulating mononuclear cells with prominent cytoplasmic projections. As in most forms of leukemia, the risk of pulmonary infection by normal pathogens and opportunistic invaders alike is high. HCL may be associated with granulomatous infections of the lung, especially mycobacterioses. Of the authors' series of 33 patients, 9 had a fungal or mycobacterial infection, including 5 atypical mycobacterial species. Five of the 6 patients with mycobacterioses and 1 of the 3 with fungal pneumonia survived the infection with appropriate therapy. Granulomatous infections, particularly mycobacterioses, should be seriously considered in the differential diagnosis of pulmonary parenchymal disease in a patient with HCl.

Adult↗
PubMed1980

Clinical and immunologic spectrum of plasmacytoid lymphocytic lymphoma without serum monoclonal IgM.

As first suggested by Lukes & Collins (1975), and recently confirmed in the literature, the pathological lesion associated with Waldenström's macroglobulinaemia can be found in patients with serum IgG or IgA monoclonal proteins. To expand upon the clinical and immunologic spectrum of this lesion, we reviewed the records of 12 patients with identical tumour morphology, though lacking an IgM paraprotein. The median age of the patients was 62 years. In five patients the tumour secreted monoclonal IgG, while one secreted monoclonal IgA. One patient had normal serum protein electrophoresis, although monoclonal light chains were demonstrated on urinary protein electrophoresis. The remaining patients had no detectable monoclonal protein. Marker studies demonstrated the B cell origin of tumour, these findings were consistent with the monoclonal protein secreted. Clinically, these cases varied, from peripheral blood involvement resembling chronic lymphocytic leukaemia, to typical lymphomatous processes, to presentations resembling multiple myeloma, reflecting the similarity of all of these conditions as varying expressions of neoplasia of the B cell system.

Adult↗
PubMedDate not supplied

Neutrophil-rich anaplastic large cell lymphoma of the skull presenting after head trauma.

The presentation of anaplastic large cell lymphoma in bone is uncommon. We report a case of anaplastic large cell lymphoma of the skull that was diagnosed after head trauma. Biopsy revealed significant destruction of the outer table of the frontal bone. Histopathologically, the initial evaluation suggested osteomyelitis because of a mixed inflammatory infiltrate with large numbers of neutrophils. However, several clusters and individual mononuclear cells were atypical. The tumor cells had large, pleomorphic nuclei; these cells stained positively with antibodies to Ki-1 (CD 30), ALK-1, and EMA. Fluorescence in situ hybridization (FISH) showed rearrangement of the ALK gene, which usually results from the t(2;5) translocation, present in most anaplastic large cell lymphomas. There was no evidence of systemic disease. The patient has tolerated chemotherapy and is free of disease 12 months later.

Activin Receptors↗
PubMedDate not supplied

Life-threatening congestive heart failure as the presentation of centronuclear myopathy.

Myocardiopathy is associated infrequently with centronuclear myopathy. We present biopsy studies of a 15 1/2-year-old black male who presented with profound acute congestive heart failure and diffuse muscular atrophy. Cardiac symptoms had been present for 6 months; limb weakness had been unassociated with either infantile hypotonia or developmental delay. Cardiac catheterization demonstrated a dilated myocardiopathy and poor left ventricular contractility. Biopsies of both ventricles revealed striking hydropic degeneration and fibrosis. Right triceps biopsy disclosed centronuclear myopathy. Because the spectrum of disease expression in centronuclear myopathy is extensive, an association with cardiac disease always should be considered in these patients. In addition, we recommend that patients who present with idiopathic myocardiopathy should be evaluated for this and other skeletal muscle diseases.

Adolescent↗
PubMedDate not supplied

Multifocal osteosarcoma: an unusual presentation.

PURPOSE: Report the unusual presentation, clinical course, and cytogenetic abnormalities in a child with multifocal osteosarcoma. PATIENTS AND METHODS: A 10-year-old boy had multifocal osteosarcoma involving the entire skeleton, pleura, bone marrow, and lungs. He had marked anemia, thrombocytopenia, and severe hypocalcemia at diagnosis. RESULTS: Despite aggressive chemotherapy, he died from progressive disease 1 month after diagnosis. Cytogenetic analysis of tumor cells within the pleural fluid showed multiple chromosomal abnormalities with amplification of the c-myc oncogene. CONCLUSION: Multifocal osteosarcoma should be considered in the differential diagnosis of a child with pancytopenia and multiple bone lesions. Amplification of the c-myc oncogene may have had a significant role in the pathogenesis, etiology, and rapid progression of this patient's multifocal disease. Additional studies will be needed to determine the biologic significance of c-myc amplification in multifocal osteosarcoma.

Child↗