Adenocarcinoid tumor of the ampulla of Vater.

We report a rare case of adenocarcinoid tumor of the ampulla of Vater. The tumor contained an intermixture of adenocarcinoma and carcinoid tumor and was removed successfully by pancreaticoduodenectomy. The characteristics of these rare tumors are reviewed.

Extramedullary hematopoiesis during therapy with granulocyte colony-stimulating factor.

Granulocyte colony-stimulating factor is a glycoprotein that promotes the proliferation and differentiation of neutrophils. It also results in an increase in circulating hematopoietic progenitor cells. We describe two cases of extramedullary hematopoiesis in patients receiving granulocyte colony-stimulating factor with chemotherapy for metastatic breast cancer.

Primitive neuroectodermal tumor of the chest wall in a patient with Jeune's syndrome and renal transplant.

Jeune's syndrome is a rare autosomal disorder characterized by osseous dysplasia, fetal respiratory distress, and renal failure in later life. We describe a 27-year-old man with Jeune's syndrome who underwent renal transplantation and 6 years later developed a sarcoma (primitive neuroectodermal tumor [PNET]) in the soft tissue of the chest wall, a principal site of dysplasia in this disorder.

Immunophenotypic markers in renal cell carcinoma.

UNLABELLED: We studied immunophenotypic and tumor cell markers in renal cell carcinoma (RCC) to determine if there are patterns of expression which may correlate with biologic behavior and response to therapy. Fourteen RCCs from 13 patients were stained by the immunoperoxidase technique using primary antibodies to Leu 4, Leu 14, Leu 2a, Leu 3a and b, lysozyme, dendritic reticulum cell (DRC), S-100, HLA-DR, epithelial membrane antigen (EMA) and beta-2-microglobulin (B2-MG). Staining was correlated with tumor stage, nuclear grade, histologic patterns, degree of cellular infiltrate, and clinical followup. Four RCCs were stage T1, four T2, five T3, and one T4. Most tumors were clear or granular cell type, with a solid or tubular growth pattern. The number of infiltrating lymphocytes and monocytes correlated with tumor grade and stage. Tumor-infiltrating lymphocytes (TILs) were predominantly Leu 3-positive (T-helper phenotype). B-cell markers were negative. Dendritic cells were rare. HLA-DR was present on endothelial cells in 11 tumors and on tumor cells in ten. HLA-DR expression increased with tumor grade. Tumor cells expressed EMA in 12 cases; B2-MG in four cases. Two patients, stages 3 and 4, died at 2 and 6 mo. CONCLUSIONS: (a) T-helper cells and monocytes infiltrate RCCs. Their numbers increase with tumor grade and stage. (b) HLA-DR expression by tumor cells tends to correlate with increasing stage and grade. (c) Dendritic cells are infrequent in RCCs.

Gastric small lymphocytic proliferation with immunoglobulin gene rearrangement in pseudolymphoma versus lymphoma.

The nature of gastric infiltrates consisting primarily of benign-appearing small lymphocytes is at present a controversial issue. Earlier reports of gastric lymphoma developing in gastric pseudolymphoma and more recent immunohistochemical studies demonstrating monoclonal B-cell populations in pseudolymphoma suggest that at least some cases represent low-grade lymphomas or clonal precursor lesions that may develop into lymphoma. Observations of a small lymphocytic infiltrate arising in the region of a gastric ulcer that lacked definitive morphologic evidence of malignancy (lymphoma) but was clearly a monoclonal B-cell proliferation by immunohistochemical and gene rearrangement studies support the notion that some gastric lymphoproliferative lesions that histologically have been called pseudolymphomas may include one or more clonal lymphoid expansions. A histopathologic/molecular model suggesting a potential pathway for the development of morphologically recognizable lymphoma from benign-appearing small lymphocytic infiltrates is presented, and the concept that for a variety of lymphoid proliferations clonality and malignancy may not be synonymous is discussed.

Malignant mixed Müllerian tumors of the uterus. An immunohistochemical study.

Malignant mixed müllerian tumors (MMMT) of the uterus have been subdivided into two types: those with heterologous sarcomatous elements (e.g., rhabdomyosarcoma, and chondrosarcoma) and those with only homologous elements (e.g., stromal sarcoma). The distinction, which may have prognostic significance, was based on the identification by light microscopy of cells that exhibited definite cross-striations, cartilage, or osteoid production. We studied 32 cases of uterine MMMT to assess the value of immunohistochemical markers in delineating the sarcomatous and epithelial components. Of 32 cases, 11 showed heterologous sarcoma (6, rhabdomyosarcoma, and 5, chondrosarcoma), and the remaining 21 were homologous MMMT. Six antigens--desmin, myoglobin, S-100, alpha 1-antichymotrypsin (ACT), epithelial membrane antigen (EMA), and monoclonal cytokeratin (AE1 and AE3), (to test for possible myogenic, chondroid, fibrohistiocytic, and carcinomatous differentiation)--were analyzed by the avidin-biotin-peroxidase method. EMA was found in neoplastic cells in all cases; 31 of 32 cases showed keratin. Desmin reactivity was detected in 14 of 32 cases, whereas myoglobin was present in 10 of 32. Three cases exhibited S-100 positivity (two in areas of chondrosarcoma, and one in some stromal sarcoma cells). Twenty-two cases (69%) exhibited ACT reactivity. Several cases displayed a malignant fibrous histiocytoma pattern. These demonstrated ACT positivity in both the neoplastic spindle and giant cells. We conclude that immunohistochemical staining for the above mentioned antigens is a useful diagnostic aid in delineating the sarcomatous and carcinomatous elements in MMMT.

Secondary Ph-positive CML with a minority monosomy 7 clone.

We report a case of Ph-positive chronic myelogenous leukemia (CML) secondary to previous treatment for a lymphoma. At the time of original diagnosis of lymphoblastic lymphoma, chromosome studies of blood and bone marrow were normal. Following therapy and a clinical remission complicated by CNS relapse, the patient presented 16 months after treatment was discontinued with a WBC of 110,000 mm-3, consistent with CML. Blood and marrow cytogenetic studies at this time showed a Ph chromosome, t(9;22)(q34;q11) translocation, without other karyotypic alteration. A separate small clone with the karyotype 45,XY, -7 was found in the blood. His disease followed an aggressive course and he died 3 months later. The autopsy findings indicated CML in blast crisis. Molecular studies performed on cells replacing a lymph node revealed a rearrangement of the breakpoint cluster region (bcr) of chromosome #22 and of the immunoglobulin heavy chain locus. Taken together, it seems most likely that the patient's CML developed as a second neoplasm following successful elimination of his lymphoblastic lymphoma by therapy.

Alveolar soft part sarcoma. A clinicopathologic and immunohistochemical study.

The histogenesis of alveolar soft part sarcoma (ASPS) has been investigated since its description. Twenty ASPS cases were analyzed for immunohistochemical content, with emphasis directed toward the paraganglial, Schwann cell, and muscle theories of histogenesis. In addition, the cases were examined for possible prognostic clinical features. The clinical characteristics of the patients were similar to those reported previously concerning average age (23 years); male:female ratio (1:1); and predominant primary site (lower extremity, nine cases). Despite a local recurrence rate of 20% and a metastatic rate of 68% (including four at presentation), the natural history was often indolent and relapse commonly occurred very late. The average follow-up period was 10.1 years. While the overall 5-year survival was 67%, only seven of 18 patients were alive without disease at last follow-up (1.7-32 years), and one patient died of tumor after a 28-year disease-free interval. Neither tumor size nor site appeared to affect prognosis. The tumors were analyzed immunohistochemically for neurofilament, S-100 protein, met-enkephalin, leu-enkephalin, acetylcholinesterase, alpha 1-antichymotrypsin, Factor VIII-related antigen, serotonin, lysozyme, neuron-specific enolase, myoglobin, cytokeratins, desmin, and vimentin. Except for weak vimentin immunoreactivity, no other antigenic expression was detected despite multiple repeated experiments with several antibodies. S-100 protein which is present in virtually all granular cell tumors was absent in the cases of ASPS. The lack of detectable expression of neurofilament, met-enkephalin and leu-enkephalin, and neuron-specific enolase is interpreted as evidence against the paraganglial theory of histogenesis. Similarly, the repeated absence of the muscle proteins, desmin and myoglobin, in contrast to a previous report, is interpreted as evidence against a myogenic origin.