Synovial sarcoma, a primary liver tumor--a case report.

An 18-year-old female presented with a mass involving the liver which was resected. Microscopically, it was diagnosed as monophasic synovial sarcoma. Cytogenetic study (FISH) revealed translocation t(X; 18), confirming the diagnosis.

Extrapulmonary small-cell carcinoma. A review of the literature with emphasis on therapy and outcome.

We report 18 patients with extrapulmonary small-cell carcinoma, which we regard as a distinct clinicopathologic entity. The natural history of this disease in some sites appears to differ from that of small-cell lung cancer. One patient with small-cell carcinoma of the esophagus was treated with combination chemotherapy and survived for 1 year. Another patient had ectopic adrenocorticotropic hormone production with small-cell carcinoma of the rectum. Two patients with small-cell bladder carcinoma are also reported in this series. Another patient with small-cell carcinoma in a cervical lymph node metastasis has survived more than 7 years after receiving radiation as the only modality of therapy. This case further substantiates the observations of others that extrapulmonary small-cell carcinoma may pursue an indolent course. Furthermore, in certain sites, local modalities of therapy may result in long-term survival or cure. This is particularly true for small-cell neoplasms of the head and neck region. An association between smoking and extrapulmonary small-cell carcinoma is postulated. The therapy and outcome of this disease is also discussed, with particular emphasis on the effectiveness of combined strategies of surgery, radiation, and chemotherapy.

Role of the spleen in the transdiaphragmatic spread of Hodgkin's disease.

Results of detailed staging laparotomy for Hodgkin's disease with supradiaphragmatic presentation in 78 patients showed 28 with subdiaphragmatic involvement. All 28 patients with intra-abdominal disease had splenic involvement. On the basis of findings of this study and published series on staging laparotomy, it is proposed that the spleen is the first site of intra-abdominal disease in this group of patients, and that Hodgkin's disease spreads to the intra-abdominal lymph nodes, liver, and bone marrow from the spleen.

Pseudo-Gaucher cell in IgMk plasmacytoid lymphoma.

Cells resembling those of Gaucher's disease were found on imprints of a lymph node from a patient with a plasmacytoid lymphoma. These cells contained nonspecific esterase, and electron-dense crystal-like structures. Associated plasma cells stained positively with anti-kappa and anti-mu sera but few macrophages were stained. The resemblance of these macrophages to Gaucher cells was due to the crystal-like inclusions that probably represented altered immunoglobulin.

Malignant nerve sheath tumor containing endocrine cells.

A malignant nerve sheath tumor occurred in the thigh of a 29-year-old man who had the stigmata of von Recklinghausen's neurofibromatosis. Chondroid foci, rhabdomyoblasts, and mucus-containing acini were identified in the tumor. Argyrophil cells and somatostatin-immunoreactive cells were present in acinar epithelium. Endocrine type granules were found in epithelial cells on ultrastructural examination. This is an example of a so-called "glandular schwannoma" and is unique in that it contained somatostatin-immunoreactive cells. A neural crest derivation is suggested for this complex tumor.

Prolongation of canine intestinal allograft survival with RS-61443, cyclosporine, and prednisone.

The efficacy of RS-61443 and cyclosporine utilized either alone or in combination was assessed in both a segmental heterotopic and total orthotopic canine intestinal transplant model. Twenty-eight dogs underwent segmental (150-cm) heterotopic intestinal transplants and five dogs total orthotopic transplants. Five heterotopic groups were compared: group 1, no immunosuppression; group 2, cyclosporine and prednisone; group 3, RS-61443, cyclosporine and prednisone; group 4, RS-61443 and prednisone; and group 5, RS-61443 and subtherapeutic cyclosporine. Group 3 animals achieved a median survival of 136 days, while the median survivals of groups 1, 2, 4, and 5 were < or = 10 days (P < .001). Twenty deaths in groups 1 through 5 were secondary to allograft rejection, two were due to infection, and two were unexplained. Four animals in group 3 were sacrificed, one at 83 days due to diarrhea and weight loss and three at the termination of the study. The median survival of five dogs undergoing total orthotopic transplantation (group 6) was 27.0 days and was significantly longer than groups 1, 4, and 5 (P < .01). No dog in this group had intestinal rejection--however, four were sacrificed due to weight loss and one died of malnutrition. The combination of RS-61443 and cyclosporine significantly prolonged intestinal allograft survival in both the heterotopic and orthotopic transplant models. Clinically, this combination may be promising for both combined liver-intestinal and isolated intestinal transplantation.

The predictive value of donor liver biopsies for the development of primary nonfunction after orthotopic liver transplantation.

Methods of accurately assessing the suitability of donor livers prior to transplantation are required if the incidence of primary nonfunction (PNF) is to be reduced. This study evaluated the ability of donor liver biopsies to predict the development of primary nonfunction after transplantation. From June 1987 until May 1990, 170 liver transplants were performed in 147 patients. A total of 124 donor liver biopsies were obtained and divided into three groups. Group 1 biopsies (n = 77) were obtained after revascularization of the liver, group 2 biopsies (n = 19) were obtained prior to donor hepatectomy but examined only after implantation, and group 3 biopsies (n = 28) were obtained as in group 2, but were examined prior to implantation. Three of 89 (3.4%) livers interpreted as having normal histology developed primary nonfunction, while one of 26 (3.8%) biopsies interpreted as having a minimal or moderate amount of fatty infiltration developed primary nonfunction. PNF occurred in 7 of 8 livers with severe fatty infiltration (3), hydropic degeneration (3), and centrilobular necrosis (1). A fourth liver with hydropic degeneration and poor function ultimately failed requiring retransplantation 8 weeks later. Analysis of liver function revealed progressive elevation in aspartate-aminotransferase, alanine-aminotransferase, lactate dehydrogenase, and serum ammonia (NH3) with increasing degrees of fatty infiltration. Donor age and weight was also found to be significantly higher in livers with fatty infiltration. This study suggests that donor liver biopsies demonstrating normal histology or minimal-to-moderate fatty infiltration function adequately, but that donor livers with severe fatty infiltration or hydropic degeneration function poorly and should not be transplanted.

[131I]iodocholesterol scintiscan and a rare "functional" black adenoma of the adrenal cortex.

A rare functional black adenoma (FBA) of the adrenal cortex was found to be the cause of hypertension and cushingold features in a 34-yr-old white female. Preoperative studies included [131I]iodocholesterol scanning (ICS) of the adrenal glands, which demonstrated the increased release of cortisol from the affected adrenal gland, with the failure of the opposite adrenal gland to record. This is evidence that cortisol was suppressing adrenocorticotropin (ACTH) output by the pituitary gland. This case documents the clinical utility of "functional" imaging techniques in this clinical setting.

Identification of the prostate cancer micro-foci with chromosome 8p deletion at the tumor interface area by histopathological-FISH parallel examination.

We used a histopathological-fluorescence in situ hybridization (histo-FISH) parallel examination technique to identify the micro-foci of prostate cancer with chromosome 8p deletion at the interface area adjacent to the tumor. The archival paraffin embedded prostate tissue sections from 27 prostate cancer patients were evaluated. Seventy-eight percent of the patients showed chromosome 8p deletion in the tumor sections. Eight of the 27 patients (30%) had been found positive for tumor micro-foci with chromosome 8p deletion at the tumor interface area. There is a strong trend for patients with late stage tumors to have positive findings for tumor micro-foci at the interface area (p<0.002). Our data suggests that the formation of tumor micro-foci with chromosome 8p deletion and ploidy change at the interface area is a significant development in the advanced prostate cancer, and identification of these micro-foci may serve as a prognostic parameter in the clinical assessment of prostate cancer patients.

Primary orbital Ewing sarcoma in a middle-aged woman.

A 43-year-old woman had unilateral exophthalmos caused by primary orbital Ewing sarcoma. Specialized immunohistochemical stains, primarily MIC-2 (CD99), aided in the diagnosis of Ewing sarcoma. Twenty-two months after radiotherapy and multiagent chemotherapy, the patient remained tumor free. To our knowledge, this is the first reported case of orbital Ewing sarcoma to present in an adult beyond the fourth decade of life.

Myxoid variant of epithelioid smooth muscle tumor.

The light-microscopic and ultrastructural features of a paravaginal epithelioid smooth muscle tumor that contained prominent myxoid stroma are reported. Because of its prominent myxoid component and the soft tissue location, this tumor was difficult to classify by histologic features alone. The ultrastructural studies demonstrated a constellation of features that have been accepted to be indicative of smooth muscle origin. The histogenesis and differential diagnosis are discussed. The elucidation of the biologic behavior of this peculiar variant of smooth muscle tumor awaits accumulation of further cases.

Liver transplantation for alcoholic liver disease.

BACKGROUND: Alcoholism is the leading cause of end-stage liver failure in the United States, but the application of liver transplantation to the treatment of alcoholic liver disease remains controversial because of medical and ethical concerns. Information about the outcome of patients who undergo transplantation for alcoholic cirrhosis would help to resolve these concerns. METHODS: The results of 41 patients (Group 1) with alcoholic liver disease were compared with those of patients who underwent liver transplantation for other medical problems (group 2) at this center. Thirty of the 32 survivors from group 1 and 30 matched subjects from group 2 were interviewed to assess substance dependence, recidivism, and activity level. RESULTS: Compared with control subjects, patients with alcoholic liver disease had equivalent patient and graft survival rates and achieved an equal level of postoperative health. These results were achieved even though patients with alcoholic liver disease had significantly worse liver failure and more morbidity before surgery, and one third of the patients in this group were not abstinent before transplantation. CONCLUSIONS: We conclude that patients with alcoholic liver disease merit equal consideration for liver transplantation compared with other causes of liver failure. Treatment of the addictive disorder should be included before and after surgery.

Delayed hemorrhage of a persistent ectopic pregnancy following laparoscopic salpingostomy and methotrexate therapy.

This report describes the first known case of delayed hemorrhage of a persistent ectopic pregnancy following surgical and medical therapy. A 2.5-cm ampullary pregnancy without cardiac activity was removed by laparoscopic salpingostomy. The serum beta-hCG level before laparoscopy was 1542 mIU/mL, increasing to 3163 mIU/mL by the eighth postoperative day. Administration of methotrexate and citrovorum factor caused a 48% decline in the level of serum beta-hCG over 72 hours, but did not prevent delayed hemorrhage by the tubal gestation. This case emphasizes that close surveillance during the treatment of persistent ectopic pregnancy should continue until serum beta-hCG is undetectable.

The development of polymyositis in a patient with toxoplasmosis: clinical and pathologic findings and review of literature.

A 58 year old female was evaluated for fever, rash, myalgias, muscle weakness and cervical lymphadenopathy. She was found to have myositis on muscle biopsy, toxoplasmic lymphadenitis on lymph node biopsy, and markedly elevated IgM and IgG antibody titers to Toxoplasma gondii. The patient was treated with prednisone and a 2 month course of sulfadiazine and pyrimethamine. The patient improved over the next six months and has been followed for approximately a five year period. During this time, antibody levels to the toxoplasma antigen have significantly decreased but the patient has developed a chronic myositis indistinguishable from polymyositis.

Significance of apparent intratympanic meningiomas.

Meningioma is the most common tumor of the central nervous system, but it has only been reported in 79 patients to involve the temporal bone. The 4 cases presented here show striking clinical similarity to a subgroup of 20 meningiomas reported to be entirely intratympanic; however, in each instance the extent, origin, and potential of the disease was not initially evident. Precise histopathologic diagnosis may be aided by electron microscopy. Current concepts of embryology lend credence to the possible role of arachnoid endothelial cells in the pathogenesis of intratympanic meningioma. An advanced intracranial meningioma may be overshadowed by the hearing loss, tinnitus, and otalgia calling attention to an intratympanic component. The clinician should remain suspicious of intracranial disease even after excision of an apparently well circumscribed intratemporal lesion. Progressive sensorineural hearing loss and persistent otalgia portend recurrent or intracranial disease. Follow-up for at least 10 years with judicious use of CT scans is suggested.

Retroperitoneal masses, adenopathy, and adrenal glands.

The diagnosis of primary retroperitoneal masses is approached best by CT scan-guided transretroperitoneal core biopsy or by open biopsy. Excisional or wedge biopsy is the preferred method for undiagnosed lymphadenopathy. Resection without biopsy is indicated for large or enlarging nonfunctional primary adrenal masses.

Coexistence of a gonadoblastoma and mixed germ cell-sex cord stroma tumor.

A 46 XY woman with a dysgerminoma and gonadoblastoma is described. Both dysgerminoma-gonadoblastoma and mixed germ cell-sex cord stromal tissue was present upon microscopic examination. This case exemplifies the transition that can exist between these two distinctly separate tumors.

Infiltrating ductal carcinoma of the vulva.

The eleventh case of primary infiltrating ductal carcinoma of the vulva is reported with a review of the literature. The infiltrating tumor is associated with an intraductal component as well as noninvolved mammary-like glandular tissue (ectopic breast tissue) and metastases to inguinal lymph nodes. Estrogen receptor and progesterone receptor immunohistochemical staining is negative utilizing the HSCORE method. The treatment regimen is patterned after approach to node-positive breast carcinoma.

Emergence of acute non-lymphocytic leukemia in breast cancer patients.

The appearance of acute non-lymphocytic leukemia (ANLL) in patients with other hematologic and non-hematologic malignancies is now a well recognized syndrome. These patients have an unusual form of leukemia with distinct clinical and morphological features. With improved therapy many cancer patients have experienced prolonged survival and cure. Some of these patients will develop ANLL as a consequence of their previous therapy, or for reasons associated with their primary tumor (host factors or common oncogenic virus). It is also possible that a fraction of these patients will develop the usual form of leukemia (de novo ANLL) that is unrelated to the underlying malignancy. Indeed, a statistical analysis assuming ANLL incidence rates to be unaffected by breast cancer or cancer therapy, predicted the occurrence of three new ANLL cases with a history of breast cancer in Wisconsin per year. Eleven patients with ANLL and a history of breast cancer were seen at the University Hospitals over a ten-year span (1969-1978). Clinical, morphological and cytogenetic features were examined and it was found that five of the eleven patients had features atypical for therapy related leukemia. Indeed, two of the patients had not received breast cancer therapy. The distinction between de novo and therapy related leukemia is important because remission and prolonged survival are more likely in the former and morbidity and mortality associated with therapy are more likely in the latter. We suggest that if features of de novo leukemia exist, despite previous therapy for underlying malignancy, intensive antileukemia therapy should be considered.

Hepatic lipogranulomatosis simulating veno-occlusive disease of the liver.

Two cases of unsuspected veno-occlusive disease of the liver were discovered at autopsy. Nearly all central veins and sublobular venules were occluded by lipogranulomas consisting of lipid-laden macrophages, epithelioid histiocytes, giant cells, fibroblasts, lymphocytes, and deposits of collagen. The process extended into the sublobular hepatic venules. Large hepatic veins were uninvolved. Known causes of veno-occlusive disease were ruled out, and the cause of the present cases is unknown.