Multiple colorectal carcinomas, polyposis coli, and neurofibromatosis.
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Adenomatous Polyposis Coli↗
Biomedical subjects
D M Parham
Publications and source records attributed to D M Parham.
Teratoid Wilms' tumor: the St Jude experience.
The term "teratoid" Wilms' tumor has been used recently to describe an unusual type of tumor in which, although classic nephroblastoma tissue is present, there is a significant diversity of cell types and tissues present. Examination of tissue samples from 290 patients treated at St Jude Children's Research Hospital from 1964 to 1987 disclosed that three children had teratoid Wilms' tumor. All three children had renal tumors and two of them presented with bilateral pyeloureteral obstruction, uremia, and hypertension. When compared with classic nephroblastoma, two of the children with teratoid elements responded poorly to chemotherapy and irradiation, although no metastatic disease was identified. One child died with sepsis and renal failure; the other two are surviving disease-free for 7 1/2 years and 26+ months since diagnosis. Because of the tendency for bilateral involvement, ureteral obstruction, and uremia, and their relative resistance to chemotherapy and irradiation, surgery is the principal form of therapy for patients with these tumors.
Child, Preschool↗
Laterally situated neck cysts derived from the embryological remnants of thyroid development.
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Adult↗
Morphometric analysis of breast carcinoma: association with survival.
In a retrospective study of 126 cases of infiltrating ductal adenocarcinoma of the breast the percentage area of sections of each neoplasm occupied by malignant cells was assessed by morphometry. The duration of postoperative survival was related to the area occupied by tumour cells within a neoplasm; the greater the tumour cell area to stroma the more prolonged the survival. This variable seemed to be independent of histological grade and was of greater prognostic importance than the diameter of the neoplasm. Furthermore, combination of percentage of tumour area with histological grade provided greater information on the survival characteristics of these patients. Extensive tumour necrosis was a particularly poor prognostic feature.
Adult↗
Quantitation of human milk fat globule (HMFG1) expression in breast carcinoma and its association with survival.
Expression of human milk fat globule (HMFG1) in immunohistochemically stained sections of breast carcinomas was assessed subjectively and objectively from 82 women (age range 41-96 years) to determine its prognostic importance. No correlation was observed between the degree of staining and prognosis even when the subcellular distribution of antigen expression was assessed. The total absence of staining with HMFG1 was possibly associated with a favourable outcome, although this did not quite achieve significance with the small numbers involved. The Quantimet 970 was used for objective semiautomated measurement of immunohistochemical reactions in paraffin wax sections and was found to produce better resolution and to eliminate subjective error.
Adenocarcinoma↗
Malignant rhabdoid tumor of the liver. Evidence for epithelial differentiation.
Malignant rhabdoid tumors most commonly occur in the kidney. Rarely does this tumor arise in extrarenal sites; a single documented primary hepatic tumor has been described. We describe two patients who had malignant tumors arising in the liver. These tumors demonstrated cytologic, histologic, and ultrastructural features typical of malignant rhabdoid tumor. By immunohistochemistry, the tumor cells from both patients expressed the epithelial membrane antigen, cytokeratin, and vimentin and cells from one patient contained alpha 1-antitrypsin. Neither patient had evidence of renal involvement at autopsy. Thus, we demonstrate that this rapidly fatal tumor contains cells showing features of epithelial differentiation, and that it may occur as a primary hepatic tumor.
Female↗
Symmetric gangrene of the extremities in late renal failure: a case report and review of the literature.
Medical calcification of arteries is common in chronic renal failure. We report on a patient with extensive calcification of the arterial media who developed symmetrical acral gangrene and severe cardiac failure shortly after cadaveric renal transplantation. At necropsy, the medial calcification was found to be accompanied by extensive intimal proliferation and multiple antemortem fractures, some healing by callus formation. Such medial calcification, which is similar to Monckeberg's sclerosis, affected all systemic arteries except the aorta, pulmonary artery and transplanted renal artery. Six years before this terminal illness he had undergone total parathyroidectomy for osteitis fibrosa associated with ruptured tendons. We review previous reports of patients with the syndrome of acral gangrene in azotaemic renal failure and discuss the histopathological features and pathogenesis in relation to the unusual features of our patient.
Adult↗
Characterization of cell lines derived from xenografts of childhood rhabdomyosarcoma.
Three human rhabdomyosarcoma cell lines (Rh10, Rh18, and Rh28) have been established from three independently derived xenografts. These lines have been characterized as mesenchymal in origin (reactivity to desmin and vimentin antibodies) and as expressing a human fetal muscle surface antigen recognized by monoclonal antibody 5.1 H11. Measurable levels of creatine phosphokinase have been detected in the cell lines. Rh10 and Rh28 exhibit the same chromosomal translocation and express an atypical lactate dehydrogenase isoenzyme which may be homologous to those previously reported in other tumor types. The karyotype analysis has confirmed that each cell line was derived from its respective tumor and thus provides a unique model for future investigations.
Animals↗
Development and characterization of human ependymoma xenograft HxBr5.
We describe the successful heterotransplantation of a human ependymoma in CBA/CaJ mice immune deprived by infant thymectomy and whole-body irradiation. The xenograft, HxBr5, was established from a fourth ventricular ependymoma, locally recurrent in an 11-yr-old girl who had been treated with radiation therapy to the posterior fossa. HxBr5 retains histological and ultrastructural fidelity to the tumor from which it was derived as does the DNA content, as confirmed by flow cytometric analysis. The karyotype of the xenograft, which is pseudodiploid and exhibits trisomy 1q and deletion of 1p, is the first human ependymoma banded karyotype to be reported. Growth rates of the xenograft tumors are similar to the primary tumor as clinically observed with a doubling time of approximately 42 days. Cell kinetic parameters indicate that this slow-growing tumor has a relatively high growth fraction of 70.8% with a high cell loss of approximately 91%. We anticipate that HxBr5 may be useful as one component of a more complex model for studying the biology and differentiation of human ependymoma.
Animals↗
Challenges in the treatment of childhood fibromatosis.
Between 1968 and 1985, we treated 20 children for fibromatosis (also called desmoid tumor and aggressive fibromatosis). The primary sites included head and neck (seven patients), extremity (seven patients), and trunk (six patients). Lesions ranged from 3 to 18 cm in diameter. The tumors were smaller than 5 cm in 13 patients, and in seven patients they were larger than 5 cm. A total resection was not feasible in any of the patients with lesions larger than 5 cm. Ten of the 11 patients treated with wide local resection, in whom the margins were clearly negative or close, remained free of disease for six to 16 years. Nine patients required additional treatment with radiotherapy (nine patients) and chemotherapy (five patients). Two died of local disease progression. In the remaining seven children, the disease was controlled. We describe our strategies for managing this disease in a pediatric population.
Adolescent↗
The impact of delayed surgery on radiotherapy dose and local control of rhabdomyosarcoma.
To determine if delayed surgery permits the modification of radiotherapy dose while maintaining local control in children with localized, unresectable rhabdomyosarcoma, a prospective study was launched in 1981 to test this objective. Treatment consisted of 16 weeks of preoperative chemotherapy, with or without delayed surgery, and radiotherapy using 35 to 40 Gy (3500 to 4000 rad) for microscopic and 50 to 55 Gy (5000 to 5500 rad) for gross residual tumor, plus 14 months of chemotherapy. Among 22 patients treated, surgery was feasible in 11 of 14 patients with residual tumor after chemotherapy and was performed in eight (avoiding radical surgery in three), leaving microscopic (seven patients) or gross residual (one patient) tumor. Progressive disease or amputation precluded radiotherapy in two patients. After radiotherapy local control was sustained in 12 of 14 patients with microscopic lesions vs none of six patients with gross tumor. Delayed surgery may permit the use of lower-dose radiotherapy and should be considered in the treatment plan for this subset of patients.
Adolescent↗
Infectious mononucleosis. The spectrum of morphologic changes simulating lymphoma in lymph nodes and tonsils.
Lymph-node and tonsillar biopsies occasionally are obtained from patients with the infectious mononucleosis syndrome secondary to Epstein-Barr viral infection, particularly if the clinical presentation is atypical and a viral etiology is not suspected. The presence of Reed-Sternberg-like cells in infectious mononucleosis resulting in confusion with Hodgkin's disease is well-known; however, similar difficulty in excluding a non-Hodgkin's lymphoma can be encountered. Eleven cases of reactive lymphoid hyperplasia with the morphologic features of infectious mononucleosis are reported, nine of which had documented Epstein-Barr viral infection. The spectrum of morphologic changes associated with Epstein-Barr viral infection is discussed, with emphasis on the features that permit their distinction from non-Hodgkin's lymphoma. Morphologic features mimicking lymphoma included extensive immunoblastic proliferations in sheets and nodules and marked cytologic atypia. Hodgkin's disease was simulated by the tendency in some cases for the atypical Reed-Sternberg-like cells to cluster about necrotic foci and to show pronounced cytologic atypia. Features permitting the distinction from non-Hodgkin's lymphoma included persistent reactive foci with the classic features of infectious mononucleosis, a polymorphous background of transformed lymphocytes rather than irregular or twisted lymphoid cells as seen in non-Hodgkin's lymphoma, and preservation of underlying reticulin architecture rather than destruction, even in cases with extensive immunoblastic proliferation. Hodgkin's disease was excluded by requiring strict criteria for Reed-Sternberg cells and noting the reactive background as inconsistent with Hodgkin's disease. Immunoperoxidase staining of seven of the cases with anti-Leu-M1 failed to demonstrate immunoreactivity of the Reed-Sternberg-like cells with this monoclonal antibody.
Adolescent↗
Comparison of modified Minitek system with Staph-Ident system for species identification of coagulase-negative staphylococci.
A modified Minitek system (BBL Microbiology Systems, Cockeysville, Md.) for species identification of coagulase-negative staphylococci was compared with the Staph-Ident system (Analytab Products, Plainview, N.Y.). The conventional method of Kloos and Schleifer (J. Clin. Microbiol. 1:82-88, 1975) was used as the reference method for identification. When tested with 78 coagulase-negative staphylococcal isolates from 63 children with cancer. Minitek concurred with the conventional identification in 67 (86%) isolates and Staph-Ident concurred in 61 (78%) isolates. Identification of species other than Staphylococcus epidermidis was better with Staph-Ident (19 of 23 isolates) than with Minitek (14 of 23 isolates). Concordance of results was likewise high, 88% (7 of 8) for Minitek and 100% (8 of 8) for Staph-Ident in studies with eight coagulase-negative staphylococcal strains obtained from the American Type Culture Collection (Rockville, Md.). We conclude that the modified Minitek system is a practical and reliable test for identifying coagulase-negative staphylococci in clinical microbiology laboratories. Its versatility and larger repertoire of biochemical tests may offer advantages over the Staph-Ident system.
Coagulase↗
Childhood multifocal osteosarcoma. Clinicopathologic and radiologic correlates.
The authors reviewed the clinical charts and pathologic and radiographic materials of nine pediatric patients (mean age, 11.0 years) presenting with multiple skeletal foci of osteosarcoma. Four patients presented with pulmonary metastases, and five had osseous disease only. All had densely sclerotic, predominantly osteoblastic tumors, usually in metaphyseal locations. Evidence of aggressive tumor cell behavior--such as vascular, articular, epiphyseal, or soft-tissue invasion--was usually present in surgical specimens. Despite intensive chemotherapy, all patients died from 6 to 37 months after diagnosis (median survival, 12 months). Comparison of clinical and pathologic features for patients presenting with or without pulmonary metastases failed to disclose any notable differences between the two groups, indicating a common pathogenesis and evolution for their multifocal disease. The authors conclude that this variant of osteosarcoma is a highly aggressive form of the disease that may be accompanied by pulmonary metastases.
Adolescent↗
Disseminated herpes simplex infection complicating Darier's disease: successful treatment with oral acyclovir.
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Acyclovir↗
The significance of calcified regional lymph nodes at the time of diagnosis of osteosarcoma.
The presence of lymph node metastases from osteosarcoma was rare at diagnosis, occurring in only four of 176 patients (2.3%) at St. Jude Children's Research Hospital. An additional patient presented with calcified lymph nodes, which were demonstrated to contain no evidence of metastatic osteosarcoma. These patients did not differ in race, sex or age at diagnosis from other osteosarcoma patients. Each of the four patients with lymph node metastases of osteosarcoma had tumors composed predominantly of the osteoblastic type. Regional nodal metastases, whether alone or in conjunction with other metastatic disease were a poor prognostic factor. Despite aggressive therapy, median survival was 8 1/2 months from diagnosis, which did not differ significantly from the survival achieved by other osteosarcoma patients who had other hematogenous metastases.
Adolescent↗
Malignant peripheral nerve sheath tumors arising from ganglioneuromas.
Two typical malignant peripheral nerve sheath tumors (PNST) arising in preexisting ganglioneuromas are described. To the best of our knowledge, this association of tumors has not been reported in detail previously. Neither patient had the stigmata nor family history of Von Recklinghausen's neurofibromatosis. In both cases, the ganglioneuromas evolved from more primitive neuroectodermal tumors (one neuroblastoma, one ganglioneuroblastoma) and both patients developed their malignant PNST at previously irradiated sites. Both patients died within 2 years of the diagnosis of their malignant PNST. The origin of these malignant PNSTs from Schwann cells is supported by an ultrastructural analysis of 27 neuroblastomas, ganglioneuroblastomas, and ganglioneuromas.
Adolescent↗
Pseudallescheria boydii in a patient with acute lymphoblastic leukemia.
Pseudallescheria boydii, a common soil organism, is best known as the causative agent of mycetoma. Isolation of the organism from sites other than the extremities is relatively uncommon. However, the use of corticosteroids and cancer chemotherapy has been associated with an increasing frequency of localized superinfection as well as disseminated disease. Recently, an increased number of disseminated P boydii infections, primarily associated with immunosuppressed patients, has been reported in the literature. We diagnosed an additional case of P boydii in a 16-year-old boy who had acute lymphoblastic leukemia and manifested a disseminated infection during reinduction chemotherapy. The unusual features of this case were the extent of organ involvement, which was more wide-spread than previously noted in the literature, and the presence of conidia in sections of an endocardial thrombus.
Adolescent↗