"Rhabdoid meningioma" lacking malignant features. Report of two cases.

We report 2 cases of meningiomas with rhabdoid morphology but lacking histological features of malignancy. Both occurred in adult women, one arising from the superior surface of the tentorium and the other in the Sylvian fissure. The tumors showed light microscopic, immunohistochemical and ultrastructural evidence of meningothelial differentiation together with diffuse or focal areas exhibiting rhabdoid morphology. The rhabdoid areas were characterized by cells with large cytoplasmic eosinophilic inclusions and large eccentric nuclei. Both cases showed areas with sheet-like growth and one had macronucleoli and brain invasion. The same case showed areas of necrosis that most likely related to pre-operative arterial embolization. Unlike most cases reported in the literature, these "rhabdoid meningiomas" lacked significant mitotic activity or other atypical features. The diagnostic and prognostic significance of this tumor entity is discussed along with a review of the literature.

Tactile corpuscle-like bodies in colonic mucosa.

During the routine examination of a segment of colon resected for adenocarcinoma, a diffuse proliferation of mucosal tactile corpuscle-like bodies was identified. The bodies showed a lamellar structure by light microscopy and were S-100 positive. Electron microscopy demonstrated parallel slender processes with prominent surface caveolae, arising from peripheral cell bodies. Similar structures sometimes occur in neurofibromas but they have not previously been reported in the gastrointestinal tract.

Microvillous inclusion disease: report of a case with atypical features.

Microvillous inclusion disease is a rare lethal disorder characterized by intractable, severe, watery diarrhea beginning in early infancy. The underlying defect is thought to be an autosomal recessive genetic abnormality resulting in defective brush-border assembly and differentiation. Normally, this diagnosis is easily established through the electron microscopic demonstration of characteristic microvilli-lined inclusions lying within the apical cytoplasm of surface enterocytes. In a small number of patients appearing to have microvillous inclusion disease it has not proven possible to demonstrate the typical inclusions. The existence of another entity, termed intestinal microvillous dystrophy, has been proposed to account for such occurrences. This assertion was founded in large part upon the observation that the few subjects studied all displayed a slightly atypical clinical presentation. The case now being presented exhibited the morphologic features ascribed to intestinal microvillous dystrophy but had a clinical presentation that was entirely typical of microvillous inclusion disease. It serves thus to conceptually unite intestinal microvillous dystrophy with microvillous inclusion disease.

Phaeochromocytoma in two coatimundi (Nasua nasua).

Two unrelated coatimundi (Nasua nasua) had bilaterally enlarged adrenal glands at necropsy, and sections of the glands from both animals had histopathological features consistent with neoplasia. They were differentiated from an adrenal cortical tumour on the basis of their light microscopical morphology, immunoperoxidase staining and electron microscopic studies and a final diagnosis of phaeochromocytoma was made. To the authors' knowledge, these are the first reported cases of phaeochromocytoma in coatimundi.

Hyalinising spindle cell tumour with giant rosettes: report of a case with unusual features including original histological and ultrastructural observations.

Hyalinising spindle cell tumour with giant rosettes (HSCTGR) is an uncommon, recently described low-grade sarcoma which shares many histological features with low-grade fibromyxoid sarcoma (LGFMS). We report a case of HSCTGR occurring in the deep soft tissues of the thigh of a 46-year-old woman, that presented as a slowly growing, painless mass. Microscopically the tumour was composed of spindled stromal cells amongst which were scattered so-called collagen rosettes. The distinctive feature of this case was the previously unreported finding of lymphoid cells of T-cell phenotype admixed with fibrohistioctyic cells in the cellular cuff surrounding the collagenous core of the rosettes. The case was further unusual in that it included focal areas of increased cellularity with a mitotic count of up to three per 10 high-power fields. While the latter feature has been associated with increased recurrences and metastases in LGFMS, it is not known whether the significance is similar in HSCTGR. The spindled stromal cells showed ultrastructural features of poorly differentiated fibroblasts, while those at the edges of the rosettes showed features of altered fibroblasts, some with a fibrohistiocytic appearance. These findings support the interpretation that HSCTGR forms part of the spectrum of sarcomas showing fibroblastic differentiation.

Microvillous inclusion disease: report of a case with atypical features.

Microvillous inclusion disease is a rare lethal disorder characterized by intractable, severe, watery diarrhea beginning in early infancy. The underlying defect is thought to be an autosomal recessive genetic abnormality resulting in defective brush-border assembly and differentiation. Normally, this diagnosis is easily established through the electron microscopic demonstration of characteristic microvilli-lined inclusions lying within the apical cytoplasm of surface enterocytes. In a small number of patients appearing to have microvillous inclusion disease it has not proven possible to demonstrate the typical inclusions. The existence of another entity, termed intestinal microvillous dystrophy, has been proposed to account for such occurrences. This assertion was founded in large part upon the observation that the few subjects studied all displayed a slightly atypical clinical presentation. The case now being presented exhibited the morphologic features ascribed to intestinal microvillous dystrophy but had a clinical presentation that was entirely typical of microvillous inclusion disease. It serves thus to conceptually unite intestinal microvillous dystrophy with microvillous inclusion disease.

Serum albumin and survival in CAPD patients: the implications of concentration trends over time.

HYPOTHESIS: Trends in serum albumin concentration over time provide a better prediction of clinical outcome in CAPD patients than a single mean value. METHODS: This was a retrospective review of outcome at 36 months in 225 adult CAPD patients. Mean serum albumin was determined for the first (SA1) and second (SA2) 6 months of treatment and patients grouped according to SA1 (group I, > 37; group II, 34-37; group III, < 34 g/l) and according to the change in serum albumin (delta SA) between the first and second 6 months (increased/static or decreased). Patient (PS) and technique (TS) survival were determined by Kaplan-Meier survival analysis. The effect of SA1 and delta SA on survival were determined in a multivariate Cox regression analysis model that included age and presence or absence of a systemic disease. RESULTS: By SA1 group, PS and TS survival at 36 months were 94 and 76% (group I), 64 and 53% (group II) and 70 and 52% (group III). If delta SA increased/remained static, then SA1 did not predict PS (group I, 100%; group II, 96%; group III, 74%; P = n.s.) or TS (group I, 72%; group II, 63%; group III, 65%; P = n.s.). If delta SA decreased, PS was worse in groups II and III, both as compared to group I (PS group I, 88%; group II, 52%; group III, 34%; P = 0.02) and as compared to the groups II and III when delta SA increased (PS group II, 74 vs 52%, P = 0.05; group III, 82 vs 34%, P = 0.005) The same trend was seen for TS. In the multivariate Cox regression model, age, direction of change in serum albumin, and presence of a multisystem disease were significant predictors of survival, whereas SA1 was not. CONCLUSION: Early hypoalbuminaemia in CAPD only predicts a worse patient and technique survival if mean serum albumin decreases further from the first to second 6 months of dialysis therapy. Change in serum albumin between the first and second 6 months of CAPD and the mean serum albumin over the first 6 months together offer better discrimination of outcome than either alone.

Generalized pustular psoriasis responsive to PUVA and oral cyclosporin therapy.

A patient with acute generalized pustular psoriasis was successfully treated with a combination of oral cyclosporin (6 mg/kg per day) and photochemotherapy (PUVA). Although early inpatient treatment with weak topical steroids and PUVA produced initial improvement, the patient's clinical condition fluctuated, with the subsequent development of erythroderma. The addition of oral cyclosporin produced dramatic improvement within 1 week of its commencement. The patient remained in remission 12 months following cessation of therapy.

Monomorphic (basal cell) salivary adenoma of ovary: report of a case.

A pregnant 26-year-old woman presented with an asymptomatic 10-cm left ovarian mass diagnosed at 19 weeks gestation. On removal, the tumor was shown to be a cribriform variant of basal cell salivary adenoma with no other teratomatous or germ cell tumor elements present. The pregnancy continued uneventfully to term and the patient remains symptom-free 10 months after diagnosis. Tissues of salivary type are occasionally observed within mature cystic teratomas of the ovaries and are presumed to have given rise to the present lesion, which is thought to be the first reported case of a neoplasm of salivary type arising in the ovaries.

Pleural mesothelioma with initial presentation as cervical lymphadenopathy.

A 41-year-old woman presented with a left neck mass. Light microscopy suggested metastatic large cell carcinoma, but subsequent electron microscopy demonstrated typical epithelial mesothelioma. Initial clinical investigations were negative and the primary pleural tumor remained inapparent for a further 8 months. The woman ultimately died from the effects of superior vena caval obstruction. Mesotheliomas frequently metastasize late in the course of the disease, but it is exceptional for them to present as a metastatic tumor of unknown primary origin.

Ultrastructural studies in Langerhans cell histiocytosis: a search for evidence of viral etiology.

The ultrastructure of lesional cells in biopsy material from 50 cases of Langerhans cell histiocytosis (LCH) was studied for the effects of virus and/or cytokines. Viral "footprints," which actually represent ultrastructural signs of the effects of cytokines on cells, were found in 76% of the cases. These were detected in lesional Langerhans cells, endothelial cells, and lymphocytes and consisted of tubuloreticular structures, cylindrical confronting cisternae, and curvilinear membranous formations. No virus particles or virus-specific cell products were found. These studies suggest that LCH cells are subject to cytokine stimulation but provide no evidence to implicate a virus in the disease process.

Australia's first case of AIDS? Pneumocystis carinii pneumonia and HIV in 1981.

OBJECTIVE: To present the earliest Australian case of the acquired immunodeficiency syndrome (AIDS) reported to date. CLINICAL FEATURES: A 72-year-old man developed a prolonged illness, beginning in February 1981, characterised by anorexia, malaise, weight loss and an episode of herpes zoster. In July he noted the insidious onset of dyspnoea with a productive cough. He was admitted to hospital in August, where Pneumocystis carinii pneumonia was diagnosed from a transbronchial lung biopsy. Splenomegaly and generalised lymphadenopathy were noted but a scalene lymph node biopsy examined at that time failed to establish an underlying diagnosis. The patient was single and lived alone in an inner suburb of Sydney. He had never left Australia and had never received a blood transfusion. His sexual history is not recorded, nor is there any documented history of intravenous drug use. OUTCOME: The patient died in September 1981. Recent re-examination of the preserved lymph node specimen by means of an in-situ hybridisation method detected human immunodeficiency virus (HIV). Preserved prostatic tissue from a resection performed in January 1980 on the same patient was also found to be HIV positive. CONCLUSION: AIDS existed in Australia as early as July 1981, around the time of the publication of the first American case reports. Whether this represents an isolated case in a man who progressed rapidly because of his relatively advanced age, or whether HIV was present earlier in Australia than previously thought, remains unanswered.

Electron microscopy what Izzits revisited: an ultrapath VI quiz.

Thirteen cases of various natures, selected from a collection shown and discussed at Ultrapath VI, are presented in quiz format for recognition or diagnosis.

Characterization of a new human bladder cancer cell line, UCRU-BL-28.

A new human bladder cancer cell line, UCRU-BL-28 has been established and characterized from a relapsed, cisplatin resistant, grade II, stage T4 tumor. This line is tumorigenic in nude mice and reflects the pathology of the original tumor. The morphology, the expression of tumor-associated antigens and EGF receptors, and the ability to grow both in an anchorage independent manner and in the absence of serum is explored. The BL-28 line has 71-74XXY chromosomes, with del 5q, der(9) and i(19q). Further studies on the molecular basis of bladder cancer, chemosensitivity to cisplatin, growth factor production and tissue invasion are under way.

Peritoneal desmoplastic small round cell tumors with divergent differentiation: a review.

Peritoneal desmoplastic small round cell tumors with divergent differentiation are recently described highly aggressive neoplasms with characteristic clinical, morphologic, and immunohistochemical features. This review covers 38 cases that have been reported in the literature. The average age of patients is 18.4 years, and males are affected twice as frequently as females. Tumors generally present as multiple peritoneal nodules without obvious organ involvement. Histology shows islands of small cells set in dense desmoplastic stroma. Immunohistochemical stains are usually positive for cytokeratins, epithelial membrane antigen, desmin, and vimentin. Many cases also stain for neuron-specific enolase but rarely for other neuroepithelial markers. Ultrastructural appearances range from undifferentiated small cells to larger epithelial elements. Paranuclear aggregates of intermediate filaments are characteristic. Dense-core granules and other neuroendocrine features have been described in a minority of cases. Some tumors respond to chemotherapy, but most patients die within months to a few years. The histogenesis of these tumors is uncertain.