Myxoid dermatofibrohistiocytoma: an indolent post-traumatic tumor composed of CD34+ epithelioid and dendritic cells and factor XIIIa+ dendrophages.

Factor XIIIa+ dendrophages and CD34+ "deep dermal dendrocytes" are distinct subsets of embryonic dermal dendritic stem cells that persist in interstitial and adventitial sites in adult dermis. We encountered a unique myxoid dermal tumor composed of these two cell types. It arose after trauma to the thumb of a 49-year-old man and was locally excised. The patient is without recurrence at 18 months. The disc-shaped tumor was lobulated, yellow, and mucoid and involved the margins. A fibrillar myxoid stroma contained mast cells, wispy collagen with medium-to-small vessels, and loosely deployed small eosinophilic tumor cells. The tumor cells were amitotic and had oval or bean-shaped, bland nuclei; some cells were binucleated. The cells were epithelioid or dendritic with bipolar, stellate, and racquet-shaped cytosomes whose tapering cell processes blended with fibrillar collagen. Vacuolated epithelioid cells focally formed vessel-like luminal structure. All cells strongly expressed vimentin. Thirty percent of the tumor cells were elongated, dendritic factor XIIIa+ cells whose dendritic processes enshrouded mast cells or FXIIIa-negative tumor cells. A subset of the FXIIIa+ cells also expressed MAC387 and lysozyme. The other 70% of the cells were CD34+. Many CD34+ cells were epithelioid with strong membrane and vacuolar decoration. Some CD34+ epithelioid cells had globular cytoplasmic inclusions. Other CD34+ cells were dendritic with multipolar fibrobroblast-like cytosomes and weaker CD34+ membrane decoration. Actin and S-100 were negative. Ki 67 was expressed in 1% of the tumor cells. Double stains for CD34 and Ki 67 showed that both CD34+ cells and FXIIIa+ dendrophages were Ki 67+, as were many papillary dermal vessel endothelial cells. The composition of the tumor by mast cells, FXIIIa+ dendrophages, and CD34+ primitive cells recapitulates the dermal microvascular unit. We propose the descriptive term myxoid dermatofibrohistiocytoma (MD) for this novel tumor. It appears to be an unusual response by dermal dendritic cells, perhaps due to continued stimulation by post-traumatic cytokines. Clarification of its biology and nosology awaits identification and study of more cases.

Disseminated tuberculosis in the acquired immunodeficiency syndrome era.

To assess the influence of human immunodeficiency virus type 1 (HIV)-induced immunodeficiency on the clinical, radiographic, and pathologic features of disseminated tuberculosis (TB), we studied 79 patients presenting in 1984 through 1987 with miliary or focal disseminated disease due to Mycobacterium tuberculosis, as well as 4 additional non-HIV patients diagnosed after 1987. Clinically defined acquired immunodeficiency syndrome (AIDS) or AIDS-related complex (ARC) was present in 51 (Group 1). A total of 20 had TB unrelated to HIV disease (Group 2). The remaining 12 were excluded because the role of HIV could not be determined. Clinical features were similar between groups aside from younger age; lower hemoglobin, total leukocyte, lymphocyte, and platelet counts; and more frequent tuberculin anergy (90 versus 40%) in AIDS/ARC patients (p less than or equal to 0.03). Chest radiographs showed a miliary pattern in about half of each group. Pleural effusion occurred only in AIDS/ARC patients (24%, p = 0.02), but intrathoracic lymphadenopathy was present in about a third of each group. Tissue biopsies (n = 70) usually revealed necrotizing granulomatous inflammation in each group, with a tendency to greater necrosis and more numerous acid-fast bacilli in Group 1. Granulomas were usually poorly formed in AIDS/ARC patients (59 versus 18%, p = 0.01). Autopsy of 9 AIDS/ARC patients with overwhelming miliary TB revealed a "nonreactive" histologic pattern with poorly organized or absent granulomas, extensive necrosis, and numerous bacilli. HIV-related disseminated TB causes a major constitutional illness with a high short-term mortality (25%).(ABSTRACT TRUNCATED AT 250 WORDS)

Definitive irradiation for localized non-Hodgkin's lymphoma of breast.

A diagnosis of primary non-Hodgkin's lymphoma (NHL) confined to the breast or breast and ipsilateral lymph nodes was biopsy proven in 19 patients who presented to our institution between 1970 and 1984. Four patients were treated with a modified radical mastectomy, and one with chemotherapy only. The remaining 14 patients comprise the basis of this study. All patients were clinically staged. There were 12 patients with 1A-E disease and two with IIA-E disease. All patients were treated with breast radiation. The axillary nodes were included in seven of the patients and two, in addition, received "mantle" irradiation. The majority of the patients received between 3500-4400 cGy, but four received lower doses. One patient, with Stage IIA-E disease, received 6 months of systemic chemotherapy, followed by 3500 cGy to the breast and axilla. Three of the 14 patients relapsed within the treated area, with a local control rate of 78%. One was salvaged with a mastectomy and remains alive and well 15 years after diagnosis. Seven of the 14 patients relapsed distantly, including two with local failures, but only three have died of disease. An additional two patients have died of other causes without evidence of disease, for a survival of 66% at 48 months (Kaplan-Meier Calculations).

Immunocytochemical assay of progesterone receptors in paraffin-embedded specimens of endometrial carcinoma and hyperplasia: a preliminary evaluation.

An immunohistochemical method for the detection of progesterone receptors (PgR) using the monoclonal anti-PgR antibody KD 68 was utilized to study paraffin-embedded tissue sections from women with endometrial carcinoma and hyperplasia. Stromal as well as myometrial nuclear PgR were nearly always apparent. In carcinoma, 11/24 (46%) of cases showed epithelial positivity, whereas in hyperplasia 8/9 (89%) were PgR-positive (P less than 0.05). Initial biochemical PgR assays by the dextran-coated charcoal method were compared with results of PgR-immunocytochemical assays (ICA) in the paraffin-embedded tissue and were in concordance in 92%. In the one discordant specimen, PgR-ICA-negative tumor cells were seen infiltrating PgR-ICA-positive myometrium, and the biochemical assay was thus felt to be falsely positive. Twelve additional cases of endometrial carcinoma were also studied for estrogen receptor (ER) by immunocytochemistry. Two were positive for both ER and PgR, while five were negative for both receptors. The immunocytochemical methods described allow for analysis of routinely fixed, paraffin-embedded specimens, thus permitting analysis of very small specimens and archival material.

Uterine sarcoma with liposarcomatous differentiation: report of a case and review of the literature.

Heterologous sarcomas of the uterus are uncommon neoplasms. A liposarcoma in combination with a leiomyosarcoma of the uterus has been reported only once prior to this case report. We report a case of uterine sarcoma demonstrating two distinct variants of liposarcoma. This has not been seen to date.

Malignant lymphoma of the breast. A study of 53 patients.

Fifty-three patients with non-Hodgkin's lymphoma of the breast were reviewed and classified using four current classifications of lymphoma. All patients were female with a mean age of 57 years. The majority of patients had histiocytic or large-cell lesions and presented as clinical Stage I. The tumors were described clinically as primary in the breast, and mammary parenchyma was found in 79% of the diagnostic biopsy specimens. The other specimens showed lymphoma in mammary adipose tissue. Survival was not influenced by the presence or absence of breast parenchyma in the biopsy. Statistically significant survival differences were found to be related to stage at presentation as well as to tumor grade, using Kiel and Working Formulation categories. Patients with Stage I disease and those with low-grade lesions had a more favorable prognosis. No discernible factors, including stage or histologic findings, appeared to affect the recurrence rate.

Localized non-Hodgkin's lymphoma of the breast.

Chart review identified 18 patients seen at Memorial Hospital from 1970 to 1984 with primary non-Hodgkin's lymphoma localized to the breast. Sixteen involved the breast alone (Stage IE), whereas two also involved ipsilateral axillary lymph nodes (Stage IIE). None had B symptoms. Histologic subtypes included 11 diffuse histiocytic, 4 diffuse poorly differentiated, 2 diffuse mixed and one nodular mixed. Thirteen patients (72%) are alive with 55 months median follow-up (11 months-14 years). Three patients died with recurrent disease at less than 2 years, one recurred at 4 years and died 6 years after diagnosis, and one died at 30 months without clinical evidence of disease. Seven (39%) have remained continuously disease-free (11 months-11 years; median, 54 months). Of the clinical Stage I patients, 1 of 3 treated with mastectomy alone recurred in the scar, whereas 1 of 12 treated with irradiation alone recurred locally. Thus, radiation therapy alone achieved good local control, although distant relapses remain a problem in these clinically staged patients. Overall, the outcome of clinically localized non-Hodgkin's lymphoma of the breast is similar to that for localized lymphoma in other sites.