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Biomedical subjects

E Foucar

Publications and source records attributed to E Foucar.

At least 19 recordsLinked to original sources

Childhood Ki-1 lymphoma: presentation as a buttock mass.

Ki-1 lymphoma is a rare, large-cell anaplastic non-Hodgkin's lymphoma that most commonly affects older children and young adults. Presentation usually occurs as a localized infiltration of the skin and lymph nodes. We report an unusual case of childhood Ki-1 lymphoma that presented as a buttock mass in an eight-year-old girl, Pathologic evaluation revealed the characteristic lymphoma cells expressing Ki-1 antigen (CD-30), HLA-DR, interleukin 2 (CD-25), T-cell gene rearrangement, and the cytogenetic karyotype t(2;5). The patient is in complete remission following treatment with combination chemotherapy. This report broadens the clinical spectrum associated with Ki-1 lymphomas and illustrates the importance of combining routine pathologic examination with other specialized diagnostic techniques in the evaluation of childhood soft-tissue masses.

Aneuploidy

The area of cancer in transurethral resection specimens as a prognostic indicator in carcinoma of the prostate: a computer-assisted morphometric study.

We used a computerized interactive morphometric method to determine total area of tissue and total area of cancer in routinely stained sections from each of 79 cases of prostatic adenocarcinoma, diagnosed by transurethral resection (TURP). The ability of two morphometrically determined parameters (area of cancer and percent area of cancer) to predict survival was compared with results obtained from two estimates of tumor area commonly used in clinical practice (number of chips and percent of chips involved by cancer). Total Gleason score was also determined. All patients were diagnosed prior to 1981, allowing follow-up of at least 5 years, or until death. Using the Cox proportional hazards regression analysis, our two morphometrically determined parameters, as well as the percentage of chips involved by cancer and total Gleason score, were significant predictors of survival. In contrast, the total number of chips involved by cancer did not reach statistical significance as a predictive factor for survival. By using our morphometrically determined area measurements as a bench mark for clinical utility of area estimates of cancer in TURP specimens, we concluded that calculating the percentage of involved chips yields prognostic information that closely approaches the "ideal" of these more time-consuming computer-assisted techniques.

Adult

Viralization due to Leydig cell tumor diagnosis by magnetic resonance imaging. Case management report.

A 3-year-old boy who presented with signs of virilization had a Leydig cell tumor of the left testis that could not be detected by physical examination or by high resolution ultrasonography. His very small tumor was demonstrated by magnetic resonance imaging. The implications of adding this sensitive method of imaging to the evaluation of prepubertal virilization of presumed testicular etiology are discussed.

Child, Preschool

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity.

This report reviews the clinical and pathologic features of 423 examples of sinus histiocytosis with massive lymphadenopathy (SHML) entered in a case registry, with special emphasis on extranodal manifestations of the disease. The most common sites of extranodal involvement in this population are skin, upper respiratory tract, and bone. However, SHML also can occur in a variety of other sites, including the genitourinary system, lower respiratory tract, oral cavity, and soft tissues. Involvement of kidney, lower respiratory tract, or liver was found to be a poor prognostic sign, and patients with associated immunologic disease often fared poorly. In general, prognosis has been found to correlate both with the number of nodal groups and with the number of extranodal systems involved by SHML. A complete bibliography of publications describing patients with SHML is included, and illustrations of the clinical, histopathologic, and radiographic features of the disease are provided.

Adolescent

The mononuclear phagocyte and immunoregulatory effector (M-PIRE) system: evolving concepts.

The phagocytic activity of macrophages and related cells has been recognized for over a century. Within the last decade a variety of other functions has been attributed to these same cell types. Examples include antigen processing and antigen presentation to B and T cells, and synthesis of a large number of immunoregulatory proteins. This recent explosion of information regarding the functions and cell types of this complex cell system has had little impact on the practice of pathology in that nomenclature, concepts of disease classification, and our diagnostic approach to the majority of "histiocytic" disorders have not been revised. As the mysteries of this complex cell system are unraveled, older names, such as the mononuclear phagocyte system that focus on only one function, have become obsolete. Because recent evidence shows that this cell system also plays a pivotal and multifaceted role in immunity, the designation mononuclear phagocyte and immunoregulatory effector (M-PIRE) system is recommended. Diverse cell types, including bone marrow precursor cells, histiocytes, various dendritic cells, and Langerhans cells, comprise the M-PIRE system, and these are located in tissues throughout the body. Although mature macrophages and dendritic cells are strikingly different in morphology and function, evidence from studies of both normal cells and proliferative disorders suggests a common cell of origin. There also is provocative evidence suggesting that transitions between cell types may occur, analogous to those observed in the lymphoid cell system. In this report, a conceptual overview of the M-PIRE system including origin, normal function, proposed transitional cell forms, and disease states is presented.

Animals

Malignant lymphoma arising in a large congenital neurofibroma of the head and neck. Report of a case.

A lymphoma developed in a large neurofibroma in a young woman who had no other stigmata of neurofibromatosis. The neurofibroma was congenital and extensively involved the soft tissue and bone of the face and neck. Despite multiple attempts to excise the neurofibroma, it persistently recurred. At the age of 30 the patient underwent another resection of the lesion, and a follicular, mixed, small and large cell lymphoma was present within the neurofibroma. Over 3 years the lymphoma transformed into a diffuse large cell type despite the fact that it remained localized to the neurofibroma. After the lymphoma had transformed, the patient received multiagent chemotherapy. She responded well, and a repeat biopsy showed no residual lymphoma. She is alive and well 25 months after completion of chemotherapy. To the best of our knowledge, this is the first report of a lymphoma arising in a soft tissue neoplasm.

Adolescent

Nonleukemic granulocytic sarcoma of the heart: a report of a fatal case.

We report the clinical and pathological features of a cardiac fatality caused by granulocytic sarcoma in a young adult man with no evidence of leukemia involving bone marrow or peripheral blood. At autopsy, walls of the four cardiac chambers were massively infiltrated by tumor, resulting in a cardiac weight of almost three times normal. Routine cytochemical stains plus immunoperoxidase staining for OKM1 confirmed the diagnosis. Although subclinical cardiac infiltrates are commonly detected at autopsy in patients with acute nonlymphoblastic leukemia (ANLL), massive cardiac granulocytic sarcoma in the absence of bone marrow disease has not been previously described. Cytochemical and immunological methods of diagnosing granulocytic sarcoma in fixed tissues are reviewed.

Adult

The New Mexico Melanoma Registry. A model of a statewide cooperative program.

The prevalence of cutaneous melanoma is high among Anglo residents of New Mexico. In order to achieve consistency in diagnosis and pathologic staging, a melanoma registry was established in 1980 in conjunction with the University of New Mexico Cancer Center. The registry functions through pathology panel review of newly diagnosed melanomas combined with collection of clinical data. Reports are submitted gratis to contributing pathologists and dermatologists. The melanoma registry works closely with the population-based New Mexico Tumor Registry. Acceptance of the melanoma registry has been excellent. We believe that in excess of 90% of new melanoma cases in the state are sent spontaneously to the registry. During 1986 approximately 150 melanomas were reviewed together with 450 atypical, benign pigmented lesions such as dysplastic nevi. In addition to pathology consultations, the registry serves an educational function and has potential for a variety of epidemiologic studies.

Data Display

Urticating histiocytosis: a mast cell-rich variant of histiocytosis X.

Histiocytosis X and mastocytosis are proliferative processes that may have similar cutaneous manifestations. However, a positive Darier's sign (urtication on stroking of the lesion) is thought to reliably distinguish between these two diseases. We recently studied a 13-year-old girl with a 2-year history of extensive skin lesions and a positive Darier's sign. Routine histopathologic studies revealed a polymorphous cutaneous infiltrate composed of histiocytes, mast cells, eosinophils, and lymphoid cells. Electron microscopic studies demonstrated Langerhans granules in some of the histiocytes, and immunologic studies of frozen tissue showed that a significant subpopulation of the histiocytes marked as Langerhans cells. Giemsa staining of specimens from eight other cases of cutaneous histiocytosis X from our files revealed mast cells in all of the lesions, although none showed the abundance of mast cells present in the case with urtication. Our studies emphasize the often polymorphous nature of the cell population in cutaneous histiocytosis X and demonstrate that confusing clinical findings can result when the mast cell population in histiocytosis X produces urtication.

Adolescent

Lymphangioma circumscriptum following mastectomy and radiation therapy.

We describe a woman who developed clear, tense vesicles in an area of chronic lymphedema 8 years following mastectomy and radiation therapy. The vesicles showed the clinical and pathologic features of lymphangioma circumscriptum, which can develop as a late sequelae of the lymphatic damage inherent in some forms of cancer therapy.

Breast Neoplasms

Intrauterine herpes simplex infection resembling mechanobullous disease in a newborn infant.

We present a case of transplacentally acquired intrauterine herpes simplex virus infection in a newborn delivered at 36 weeks' gestation by cesarean section because of intrauterine growth retardation and maternal preeclampsia. The mother experienced a single episode of serotype 2 herpes progenitalis at 14 weeks' gestation. At birth the infant manifested clinical findings of herpes simplex virus infection, which resembled epidermolysis bullosa and aplasia cutis congenita. Preexisting cutaneous lesions and intact fetal membranes at delivery strongly support a transplacentally acquired intrauterine herpes simplex virus infection. Repeated Tzanck smears, viral cultures, and immunohistochemical studies of the skin were required to confirm the diagnosis. Intrauterine herpes simplex virus infection is associated with significant morbidity and mortality but responds to antiviral therapy. Therefore this diagnosis must be considered in the neonate born with bullous or eroded skin lesions.

Adult

Malignant melanoma in childhood and adolescence.

A retrospective chart review of 25 patients under the age of 20 years with malignant melanoma, diagnosed between the years 1938 and 1984 at the University of Iowa Hospitals and Clinics, was conducted. Nine of the patients were under the age of 14 years, and 18 patients were females. There appeared to be a frequent delay in diagnosis of the primary lesion, occasionally incorrect initial diagnosis, and under-treatment in these young patients. Primary treatment varied from limited excision of the lesion to wide excision and regional lymph node dissection. Females appeared to have a better prognosis than males, and wide local excision with or without regional lymph node dissection was associated with the most favorable outcome. Overall 10-year survival in this group of patients was 56 per cent, a rate that compares well to the overall survival in adult patients with malignant melanoma.

Adolescent

Epidermotropic metastatic squamous cell carcinoma. Report of two cases showing histologic continuity between epidermis and metastasis.

Two unusual cases of squamous cell carcinoma (SCCa) metastatic to skin from distant sites occurred. In both metastatic sites, the malignant squamous epithelium fused with benign surface epithelium, and the resulting transition simulated that typically seen in primary cutaneous SCCa. We believe that this previously unreported phenomenon is an expression of the natural tendency of squamous epithelium to "heal" denuded connective tissue surfaces by proliferation followed by epithelial fusion. Because epithelial continuity can be established between benign keratinocytes and metastatic SCCa, one cannot rely on this single morphologic finding to separate primary from metastatic disease.

Aged