[Fibrocartilaginous lipoma: a clinicopathological analysis of six cases].

Objective: To investigate the clinicopathological characteristics, immunophenotype, molecular genetics and differential diagnoses of fibrocartilaginous lipomas which consist of adipose tissue, fibrocartilage and fibrous elements. Methods: The clinicopathological features, immunohistochemical profiles and molecular profiles in six cases of fibrocartilaginous lipomas diagnosed at Foshan Traditional Chinese Medicine Hospital, Fudan University Shanghai Cancer Center, the Fifth Affiliated Hospital of Zhengzhou University and the Fourth Affiliated Hospital of Harbin Medical University from January 2017 to February 2022 were included. The follow-up information, diagnosis and differential diagnoses were evaluated. Results: There were three males and three females with a median age of 53 years (range 36-69 years) at presentation. Tumors were located in the extremities, the head and neck region and trunk; and presented as painless masses that were located in the subcutaneous tissue or deep soft tissue. Grossly, three cases were well defined with thin capsule, one case was well circumscribed without capsule, two cases were surrounded by some skeletal muscle. The tumors were composed of fatty tissue with intermingled gray-white area. The tumors ranged from 1.50-5.50 cm (mean 2.92 cm). Microscopically, the hallmark of these lesions was the complex admixture of mature adipocytes, fibrocartilage and fibrous element in varying proportions; the fibrocartilage arranged in a nodular, sheet pattern with some adipocytes inside. Tumor cells had a bland appearance without mitotic activity. Immunohistochemical analysis using antibodies to SMA, desmin, S-100, SOX9, HMGA2, RB1, CD34, adipopholin was performed in six cases; the fibrocartilage was positive for S-100 and SOX9, adipocytes were positive for S-100, adipopholin and HMGA2; CD34 was expressed in the fibroblastic cells, while desmin and SMA were negative. Loss of nuclear RB1 expression was not observed. Other genetic abnormalities had not been found yet in four cases. Follow-up information was available in six cases; there was no recurrence in five, and one patient only underwent biopsy of the mass. Conclusions: Fibrocartilaginous lipoma is a benign lipomatous tumor with mature adipocytes, fibrocartilage and fibrous elements. By immunohistochemistry, they show the expression of fat and cartilage markers. No specific molecular genetics changes have been identified so far. Familiarity with its clinicopathological features helps the distinction from its morphologic mimics.

Ethyl Acetate Fraction from Trichilia catigua Confers Partial Neuroprotection in Components of the Enteric Innervation of the Jejunum in Diabetic Rats.

BACKGROUND/AIMS: Diabetes causes damage to the enteric nervous system. The enteric nervous system consists of neurons and enteric glial cells (EGCs). The present study evaluated the effects of an ethyl-acetate fraction (EAF) from Trichilia catigua (T. catigua; 200 mg/kg) on the total population of enteric neurons (HuC/D-immunoreactive [IR]) and EGCs (S100-IR and glial fibrillary acidic protein [GFAP]-IR) in the total preparation and jejunal mucosa in diabetic rats. METHODS: The animals were distributed into four groups: normoglycemic rats (N), diabetic rats (D), normoglycemic rats that received the EAF (NC), and diabetic rats that received the EAF (DC). The jejunum was processed for immunohistochemistry to evaluate HuC/D, S100, and GFAP immunoreactivity. The expression of S100 and GFAP proteins was also quantified by Western blot. RESULTS: The D group exhibited a decrease in the number of neurons and EGCs, an increase in the area of cell bodies, an increase in S100 protein expression, a decrease in GFAP protein expression, and a decrease in S100-IR and GFAP-IR EGCs in the jejunal mucosa. The DC group exhibited a decrease in the number of neurons and EGCs, a decrease in the area of cell bodies, a decrease in S100 and GFAP protein expression, and a decrease in S100-IR and GFAP-IR EGCs in the jejunal mucosa. The NC group exhibited maintenance of the number of neurons and EGCs, an increase in the area of cell bodies, and a decrease in S100 and GFAP protein expression. CONCLUSION: The EAF from T. catigua partially conferred protection against diabetic neuropathy in the enteric nervous system.

Inhibitory effect of 660-nm LED on melanin synthesis in in vitro and in vivo.

BACKGROUND: Skin hyperpigmentary disorders including postinflammatory hyperpigmentation, melasma, solar lentigines, and conditions like freckles are common. The light-emitting diodes (LEDs) are the latest category of nonthermal and noninvasive phototherapy to be considered in skin pigmentation disorder treatment. PURPOSE: The purpose of this study was to investigate the effects of 660-nm LED on inhibition of melanogenesis. We investigated whether a 660-nm LED affected melanin synthesis in in vitro and in vivo models, and we explored the mechanisms involved. METHODS: The inhibitory effect of 660-nm LED on melanin synthesis was evaluated in B16F10 cells and HRM-2 melanin-possessing hairless mice were used to evaluate the antimelanogenic effects of 660-nm LED. RESULTS: Interestingly, 660-nm LED inhibited alpha-melanocyte-stimulating hormone-induced tyrosinase activity in B16F10 cells. We also found that 660-nm LED decreased MITF and tyrosinase expression and induced the activation of ERK. These findings suggest that the depigmenting effects of 660-nm LED result from downregulation of MITF and tyrosinase expression due to increased ERK activity. The 660-nm LED reduced UVB-induced melanogenesis in the skin of HRM-2 via downregulation of tyrosinase and MITF. CONCLUSION: These findings suggest 660-nm LED is a potentially depigmentation strategy.

Respuesta a valores de referencia de la proteína S100β en población pediátrica / Response to serum S100β protein reference values in a paediatric population

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Biomarkers of brain damage in preterm infants.

OBJECTIVE: There is growing evidence on the usefulness of biomarkers in the early detection of preterm infants at risk for brain damage. However, among different tools Activin A, S100B protein and adrenomedullin assessment offer the possibility to investigate brain/multiorgan function and development. This could be especially useful in perinatal medicine that requires even more non-invasive techniques in order to fulfill the minimal handling in diagnostic and therapeutic strategy performance. MATERIALS AND METHODS: The concept of Unconventional Biological Fluid (UBF: urine and saliva) is becoming even stronger and regards the assessment in non-invasive biological fluids of biochemical markers involved in the cascade of events leading to brain damage. RESULTS: Activin A, S100B protein and adrenomedullin in UBF were increased in preterm newborns developing brain damage and/or ominous outcome. CONCLUSIONS: The present manuscript offers an update on the usefulness of Activin A, S100B protein an adrenomedullin in UBF as brain damage markers. The findings open a new cue on the use of these markers in daily neonatal intensive care unit (NICU) activities.

Homeopathic treatment for peripheral nerve regeneration: an experimental study in a rat sciatic nerve transection model.

AIM: Effects of homeopathic treatment with Hypericum perforatum (Hypericum) on peripheral nerve regeneration was studied using a rat sciatic nerve transection model. METHODS: Fifty-four male healthy White Wistar rats were divided into three experimental groups (n = 18), randomly: Sham-operation (Sham), control: silicon tube (Sil) and treatment: silicon tube + Hypericum (Sil/Hypericum). In the Sham group after anesthesia left sciatic nerve was exposed through a gluteal muscle incision and after homeostasis muscle was sutured. In the Sil group the left sciatic nerve was exposed the same way and transected proximal to tibio-peroneal bifurcation leaving a 10-mm gap. Proximal and distal stumps were each inserted into a silicone tube. In the Sil/Hypericum group a silicone tube was implanted the same way and each animal received three oral drops of Hypericum 30c twice daily for 1 week. Each group was subdivided into three subgroups of six animals each studied 4, 8, 12 weeks after surgery. RESULTS: Data were analyzed statistically by factorial analysis of variance (ANOVA) and, the Bonferroni test for pair-wise comparisons. Functional study showed faster and better recovery of regenerated axons in Sil/Hypericum than in Sil group (P < 0.05). Gastrocnemius muscle mass in Sil/Hypericum was significantly greater than in Sil group. Morphometric indices of regenerated fibers showed number and diameter of the myelinated fibers in Sil/Hypericum were significantly higher than in control group. Immunohistochemistry, showed the location of reactions to S-100 in Sil/Hypericum was clearly more positive than in Sil group. CONCLUSION: Hypericum improves functional recovery of peripheral nerve regeneration in rats.

Determination of the biological variation of S100ß and lactate dehydrogenase in disease-free patients with malignant melanoma.

BACKGROUND: Biological variation is important for determining analytical goals and for establishing the magnitude of change between two consecutive measurements. The aim of this study was to determine the biological variation for S100ß and lactate dehydrogenase in patients diagnosed with malignant melanoma but without evidence of disease recurrence. METHODS: The biological variation of S100ß and lactate dehydrogenase was estimated from a mean of four consecutive measurements in 32 patients diagnosed with malignant melanoma but without evidence of disease recurrence, 3 months after tumor resection or 4 months after finishing adjuvant treatment. The mean sampling interval was 3 months. RESULTS: Mean concentrations of S100ß and lactate dehydrogenase were 0.0557 µg/L and 6.3 µkat/L, respectively. Between-run analytical variation was 3.5% at 0.181 µg/L for S100ß and 3.5% at 2.83 µkat/L for lactate dehydrogenase. Biological variations obtained for S100ß and lactate dehydrogenase were 14.2% and 8.2%, respectively. The analytical goals (defined as 50% of biological variation) were 7.1% for S100ß and 4.1% for lactate dehydrogenase. CONCLUSIONS: The estimation of biological variation allows us to calculate analytical goals and reference change values. These are necessary tools for the correct interpretation of serial measurements in patient follow-up.

Neurological biomarkers in the perioperative period.

The rapid detection and evaluation of patients presenting with perioperative neurological dysfunction is of great clinical relevance. Biomarkers have been defined as biological molecules that can be used as an indicator of new onset or progression of a biological process or effect of treatment. Biomarkers have become increasingly important in this setting to supplement other modalities of diagnosis such as EEG, sensory- or motor-evoked potential, transcranial Doppler, near-infrared spectroscopy, or imaging methods. A number of neuro-proteins have been identified and are currently under investigation for potential to provide insights into injury severity, outcome, and the ability to monitor cellular damage and molecular events that occur during neurological injury. S100B is a protein released by glial cells and is considered a marker of blood-brain barrier dysfunction. Clinical studies in patients undergoing cardiac and non-cardiac surgery indicate that serum levels of S100B are increased intraoperatively and after operation. The neurone-specific enolase has also been extensively investigated as a potential marker of neuronal injury in the context of cardiac and non-cardiac surgery. A third biomarker of interest is the Tau protein, which has been linked to neurodegenerative disorders. Tau appears to be more specific than the previous two biomarkers since it is only found in the central nervous system. The metalloproteinase and ubiquitin C terminal hydroxylase-L1 (UCH-L1) are the most recently researched markers; however, their usefulness is still unclear. This review presents a comprehensive overview of S100B, neuronal-specific enolase, metalloproteinases, and UCH-L1 in the perioperative period.

In vitro S100B secretion is reduced by apomorphine: effects of antipsychotics and antioxidants.

Astrocytes express dopamine receptors and respond to dopamine stimulation. However, the role of astrocytes in psychiatric disorders and the effects of antipsychotics on astroglial cells have only been investigated recently. S100B is a glial-derived protein, commonly used as a marker of astroglial activation in psychiatric disorders, particularly schizophrenia. We investigated S100B secretion in three different rat brain preparations (fresh hippocampal slices, C6 glioma cells and primary astrocyte cultures) exposed to apomorphine and antipsychotics (haloperidol and risperidone), aiming to evaluate, ex vivo and in vitro, whether dopamine activation and dopaminergic antagonists modulate astroglial activation, as measured by changes in the extracellular levels of S100B. The serum S100B elevation observed in schizophrenic patients is not reflected by the in vitro decrease of S100B secretion that we observed in hippocampal slices, cortical astrocytes and C6 glioma cells treated with apomorphine, which mimics dopaminergic hyperactivation. This decrease in S100B secretion can be explained by a stimulation of D2 receptors negatively coupled to adenyl cyclase. Antipsychotic medications and antioxidant supplementation were able to prevent the decline in S100B secretion. Findings reinforce the benefits of antioxidant therapy in psychiatric disorders. Based on our results, in hippocampal slices exposed to apomorphine, it may be suggested that antipsychotics could help to normalize S100B secretion by astrocytes.

[Markers of brain injury in non-invasive biological fluids]. / Markers of brain injury in non-invasive biological fluids.

Hypoxia-ischemia (H-I) constitutes the main phenomenon responsible for brain-blood barrier permeability modifications leading to cerebral vascular autoregulation loss in newborns. Hypotension, cerebral ischemia, and reperfusion are the main events involved in vascular auto-regulation loss leading to cell death and tissue damage. Reperfusion could be critical since organ damage, particularly of the brain, may be amplified during this period. An exaggerated activation of vasoactive agents, of calcium mediated effects could be responsible for reperfusion injury (R-I), which, in turns, leads to cerebral hemorrhage and damage. These phenomena represent a common repertoire in newborns complicated by perinatal acute or chronic hypoxia treated by risky procedures such as mechanical ventilation, nitric oxide supplementation, brain cooling, and extracorporeal membrane oxygenation (ECMO). Despite accurate monitoring, the post-insult period is crucial, as clinical symptoms and standard monitoring parameters may be silent at a time when brain damage is already occurring and the therapeutic window for pharmacological intervention is limited. Therefore, the measurement of circulating biochemical markers of brain damage, such as vasoactive agents and nervous tissue peptides is eagerly awaited in clinical practice to detect high risk newborns. The present article is aimed at investigating the role of dosage biochemical markers in non-invasive biological fluids such as S100B, a calcium binding protein, activin A, a protein expressed in Central nervous System (CNS).

The effect of long-term lactobacilli (lactic acid bacteria) enteral treatment on the central nervous system of growing rats.

The aim of this study was to explore the relationship between consumption of large doses of lactic acid bacteria (LAB) and the behaviour and brain morphobiochemistry of normal growing rats. Four groups of rats were treated with LAB cultures twice daily for 6 months. The control group received 1 ml of saline per treatment, while two experimental groups received 1 ml of living bacteria (Lactobacillus plantarum and Lactobacillus fermentum, respectively) and the remaining group received a heat-treated (inactivated) L. fermentum culture. After 2 and 6 months of treatment, respectively, eight animals from each group were sacrificed, and specimens were taken for further analyses. The behaviour of the rats was evaluated five times in an open-field test at monthly intervals throughout the study. Lactobacilli treatment for 2 months induced changes in the motoric behaviour of the rats. The concentration of the astrocytesoluble and filament glial fibrillary acidic protein (GFAP) decreased in the posterior part of the hemispheres, including the thalamus, hippocampus and cortex of the rats treated with L. fermentum. A greater decrease in filament GFAP (up to 50%) was shown in the group receiving the live form of L. fermentum. In contrast, the GFAP in the live L. plantarum-treated group increased, showing elevated levels of the soluble and filament forms of GFAP in the posterior part of the hemispheres. A 60-66% decrease in the amount of the astrocyte-specific Ca-binding protein S-100b was shown in the posterior parts of the hemispheres and in the hindbrain of rats given LAB for 2 months. Prolonged feeding with LAB for 4 months up to full adulthood led to a further decrease in astrocyte reaction, reflected as an additional decrease in the amount of soluble GFAP and locomotor activity in all experimental groups. The changes in filament GFAP and S-100b appeared to disappear after prolonged feeding (total of 6 months) with LAB. In summary, LAB dietary treatment affected the ontogenetic development of the astrocytes, with the highest intensity observed in the early stages of rat development. It can be postulated that LAB treatment may play a preventive role in neurological diseases by decreasing astrocyte reaction and, consequently, lowering locomotor activity.

Successful elimination of non-neural cells and unachievable elimination of glial cells by means of commonly used cell culture manipulations during differentiation of GFAP and SOX2 positive neural progenitors (NHA) to neuronal cells.

BACKGROUND: Although extensive research has been performed to control differentiation of neural stem cells - still, the response of those cells to diverse cell culture conditions often appears to be random and difficult to predict. To this end, we strived to obtain stabilized protocol of NHA cells differentiation - allowing for an increase in percentage yield of neuronal cells. RESULTS: Uncommitted GFAP and SOX2 positive neural progenitors - so-called, Normal Human Astrocytes (NHA) were differentiated in different environmental conditions to: only neural cells consisted of neuronal [MAP2+, GFAP-] and glial [GFAP+, MAP2-] population, non-neural cells [CD44+, VIMENTIN+, FIBRONECTIN+, MAP2-, GFAP-, S100beta-, SOX2-], or mixture of neural and non-neural cells.In spite of successfully increasing the percentage yield of glial and neuronal vs. non-neural cells by means of environmental changes, we were not able to increase significantly the percentage of neuronal (GABA-ergic and catecholaminergic) over glial cells under several different cell culture testing conditions. Supplementing serum-free medium with several growth factors (SHH, bFGF, GDNF) did not radically change the ratio between neuronal and glial cells--i.e., 1,1:1 in medium without growth factors and 1,4:1 in medium with GDNF, respectively. CONCLUSION: We suggest that biotechnologists attempting to enrich in vitro neural cell cultures in one type of cells - such as that required for transplantology purposes, should consider the strong limiting influence of intrinsic factors upon extracellular factors commonly tested in cell culture conditions.

S100B Protein concentration in milk-formulas for preterm and term infants. Correlation with industrial preparation procedures.

Human milk S100B protein possesses important neurotrophic properties. However, in some conditions human milk is substituted by milk formulas. The aims of the present study were: to assess S100B concentrations in milk formulas, to verify any differences in S100B levels between preterm and term infant formulas and to evaluate the impact of industrial preparation at predetermined phases on S100B content. Two different set of samples were tested: (i) commercial preterm (n = 36) and term (n = 36) infant milk formulas; ii) milk preterm (n = 10) and term infant (n = 10) formulas sampled at the following predetermined industrial preparation time points: skimmed cow milk (Time 0); after protein sources supplementation (Time 1); after pasteurization (Time 2); after spray-drying (Time 3). Our results showed that S100B concentration in preterm formulas were higher than in term ones (p < 0.01). In addition, S100B concentrations during industrial preparation showed a significant increase (p < 0.001) at Time 1 followed by a slight decrease (p > 0.05) at Time 2, whereas a significant (p < 0.001) dip was observed at Time 3. In conclusion, S100B showed a sufficient thermostability to resist pasteurization but not spry-drying. New feeding strategies in preterm and term infants are therefore warranted in order to preserve S100B protein during industrial preparation.

Molecular staging for patients with malignant melanoma.

Molecular staging of cancers hold the promise of being more accurate compared with routine histology, particularly with regard to determining regional-nodal status. With newer reverse transcriptase-PCR (RT-PCR)-based assays, sensitivities reported are as high as identifying one cancer cell in a background of a million normal cells. Although this sensitivity is 100-times what the human eye can differentiate under the microscope, the new challenge becomes determining the relevance of this low-volume disease in the regional basin, in particular, the sentinel lymph node (SLN). Patients with melanomas greater than 0.75 mm in tumor thickness participated in a research study that examined their SLNs with routine histology, immunhistochemical staining and a RT-PCR assay based on the tyrosinase probe. A total of 311 patients were involved in the study and patients whose SLN were negative from all three assays for metastatic disease had a good survival, with a 92% disease-free survival (DFS) and a 97% overall survival (OS) regardless of the tumor thickness or the ulceration status of the primary melanoma. Patients upstaged with the RT-PCR assay had a significantly decreased DFS and OS compared with patients who were SLN negative. Patients who had enough tumor burden in the SLN that allowed their metastatic disease to be identified with routine histology had a 48% recurrence rate at 5 years. A recently published meta-analysis confirmed that molecular staging of the SLN in melanoma contains important prognostic information. Micrometastatic disease missed by routine histology in the SLN in melanoma patients is clinically relevant disease. Molecular staging has the potential of providing a more accurate staging in the SLN, for prognostication and directing adjuvant therapies.

Expression of S100A2 and S100A4 predicts for disease progression and patient survival in bladder cancer.

OBJECTIVES: To determine the expression patterns and prognostic value of S100A2 and S100A4 in surgical specimens from radical cystectomy for transitional cell carcinoma of the urinary bladder. METHODS: Immunohistochemical staining for S100A2 and S100A4 was performed in 92 archived radical cystectomy and 38 normal specimens. The immunoreactivity of these proteins was stratified on a 0 to 6 scale and then correlated with the pathologic features and clinical outcome. RESULTS: S100A2 expression was significantly decreased in the bladder cancer specimens compared with the controls (P <0.0001), and S100A4 expression was significantly greater in the bladder cancer specimens (P = 0.03). The loss of expression of S100A2 and increased expression of S100A4 were associated with muscle invasion (P <0.05). These alterations in expression were also associated with a greater risk of disease progression and a decreased chance of cancer-specific survival at a median follow-up of 25.3 months (P <0.0001 for both). After adjusting for the effects of the pathologic findings, S100A4 expression remained a significant predictor of disease progression (P <0.0001) and cancer-specific survival (P <0.0001). CONCLUSIONS: S100A4 appeared to be an independent predictor for the treatment outcome in bladder cancer. The expression patterns of S100A2 and S100A4 correlated well with the pathologic stage, disease progression, and cancer-specific mortality. This finding could aid in identifying more biologically aggressive cancers and thus patients who might benefit from more intensive adjuvant therapy.

A Comparison of 3 tumor markers (MIA, TA90IC, S100B) in stage III melanoma patients.

PURPOSE: There is no consensus regarding the optimal tumor markers for melanoma. We compared 3 tumor markers, TA90-immune complex (TA90IC), melanoma-inhibiting activity (MIA) protein, and S100B protein in Stage III melanoma patients undergoing adjuvant vaccine immunotherapy. EXPERIMENTAL DESIGN: The serum of 75 patients representing 3 prognostic cohorts was assayed for the tumor markers prior to initiating immunotherapy and at 6 follow-up time points. Upper limits of normal for TA90IC, MIA and S100B were set at OD 0.41, 8.5 ng/ml, and 2.5 microg/l, respectively. RESULTS: At least 1 marker became elevated prior to 41 (80 percent) of 51 recurrences. TA90IC was the earliest elevated marker in 29 (57 percent), MIA in 11 (22 percent), and S100B in 4 (8 percent). Multivariate regression analysis revealed that TA90IC was an independent predictor of survival when elevation occurred between 2 weeks and 3 months, whereas MIA was an independent predictor at 4-6 months. In the poor prognostic cohort, mean values for MIA and S100B increased progressively, whereas TA90IC exhibited a parabolic curve. CONCLUSION: In this patient population, TA90IC and MIA were complementary; elevation of the immune complex preceded elevation of the tumor antigen in patients who developed recurrence. Additional studies in populations not receiving vaccine will further clarify the clinical utility of these assays.

An unusual case of adult disseminated cutaneous Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) represents a group of rare histiocytic syndromes characterized by tissue infiltration with dendritic cells. The management of LCH is difficult because these disorders respond inconsistently to immunosuppressive and chemotherapeutic strategies. We describe a refractory and relapsing case of skin and nail limited LCH in a 27-year-old man. He presented with a 7-year history of an erythematous papular eruption of the scalp, ears, face, trunk, axillae, groins, fingernails, feet, and toenails. Diagnosis of LCH was made based on skin histopathology and immunohistochemical staining. Histological studies of biopsy specimens revealed a dense infiltrate of histiocytic mononuclear cells beneath the epidermis; these cells reacted strongly with anti-S-100 antibodies. In addition, CD1a was positive in most of the infiltrating cells. Extensive investigations failed to detect systemic involvement. The patient's cutaneous eruption did not respond to various therapeutic interventions, including phototherapy with oral psoralen with long-wave UV radiation in the A range (PUVA) and cyclosporine. Marked but temporary clinical improvement was achieved with thalidomide, etoposide with systemic steroid, and total body electron beam radiotherapy. Now the patient is on maintenance therapy with thalidomide and is under acceptable control.

Intracerebral schwannoma clinically and radiologically mimicking meningioma.

A case of intracerebral schwannoma (ICS) occurring in a 33-year-old woman is presented. The patient's history of headache, numbness, tingling and the recent development of weakness of the right upper extremity with right facial droop began during pregnancy. Magnetic resonance imaging (MRI) showed a 4 x 2 x 2 cm heterogeneous, gadolinium-enhanced mass at the left frontoparietal junction, with peritumoral edema and a dural-based attachment. During her pregnancy, the mass increased in size. The surgically resected specimen consisted of lobulated, somewhat gelatinous soft tissue. Microscopically, the tumor demonstrated classic biphasic Antoni type A and B patterns, admixed with degenerative changes. Immunohistochemically, the neoplastic cells were positive for S-100 protein (diffuse and strong), CD34 (primarily in Antoni B areas), glial fibrillary acidic protein (GFAP; weak and diffuse) and calretinin (mainly in Antoni A areas), while none was positive for CD31, estrogen and progesterone receptors, bcl-2, or epithelial membrane antigen (EMA). Ultrastructurally, basal laminae and Luse bodies were identified. The differential diagnosis includes fibrous meningioma, solitary fibrous tumor, and ICS. Twenty-seven cases of ICS were reviewed in which the histological diagnosis was confirmed immunohistochemically or ultrastructually, and the cases were summarized (including the present case). A combined use of immunostains (S-100 protein, EMA, CD34, and maybe calretinin) is of great help in distinguishing ICS from its histological mimickers.

Isolation and characterization of human repetin, a member of the fused gene family of the epidermal differentiation complex.

The human repetin gene is a member of the "fused" gene family and localized in the epidermal differentiation complex on chromosome 1q21. The "fused" gene family comprises profilaggrin, trichohyalin, repetin, hornerin, the profilaggrin-related protein and a protein encoded by c1orf10. Functionally, these proteins are associated with keratin intermediate filaments and partially crosslinked to the cell envelope (CE). Here, we report the isolation and characterization of the human repetin gene and of its protein product. The repetin protein of 784 amino acids contains EF (a structure resembling the E helix-calcium-binding loop-F helix domain of parvalbumin) hands of the S100 type and internal tandem repeats typical for CE precursor proteins, a combination which is characteristic for "fused" proteins. Repetin expression is scattered in the normal epidermis but strong in the acrosyringium, the inner hair root sheat and in the filiform papilli of the tongue. Ultrastructurally, repetin is a component of cytoplasmic non-membrane "keratohyalin" F-granules in the stratum granulosum of normal epidermis, similar to profilaggrin. Finally, we show that EF hands are functional and reversibly bind Ca(2+). Our results indicate that repetin is indeed a member of the fused gene family similar to the prototypical members profilaggrin and trichohyalin.

Primary sweat gland carcinosarcoma of the scrotal skin.

BACKGROUND: Carcinosarcoma is a biphasic tumor composed of malignant epithelial and mesenchymal elements. Although the tumors have been reported in different locations, they rarely occur in the skin and have not been reported in the skin of external genitalia. CASE REPORT: We present such a case in a 71-year-old Taiwanese man. He had had a long-term indolent nodule on scrotal skin, but it enlarged rapidly in 2 weeks. Wide excision of the tumor was performed. RESULTS AND DISCUSSION: The pathologic examination revealed a sweat gland carcinosarcoma consisting of admixed mucin-producing adenocarcinoma and solid spindle cell sarcoma. The two components exhibited contrasting immunohistochemical profiles with the epithelial component diffusely positive for epithelial markers and S-100 protein, while the sarcomatous component positive for vimentin and smooth muscle actin but negative for epithelial markers and S-100 protein. The immunoreactivity for S-100 protein in the epithelial component supports sweat gland origin. The tumor behaved aggressively. Local recurrence and distant metastases to lungs and brain occurred 6 months and 18 months later. The patient died of the disease 20 months after the initial diagnosis.