Mesenchymal chondrosarcoma of the orbit: an unusual site for a rare tumour.

Mesenchymal chondrosarcoma is a rare tumour with orbital involvement being an exceptional occurrence. We present a case of a 22-year old man with such disease, together with details of his management. A brief literature review of this uncommon tumour was also enclosed.

Are two cues always better than one? The role of multiple intra-sensory cues compared to multi-cross-sensory cues in children's incidental category learning.

Simultaneous presentation of multisensory cues has been found to facilitate children's learning to a greater extent than unisensory cues (e.g., Broadbent, White, Mareschal, & Kirkham, 2017). Current research into children's multisensory learning, however, does not address whether these findings are due to having multiple cross-sensory cues that enhance stimuli perception or a matter of having multiple cues, regardless of modality, that are informative to category membership. The current study examined the role of multiple cross-sensory cues (e.g., audio-visual) compared to multiple intra-sensory cues (e.g., two visual cues) on children's incidental category learning. On a computerized incidental category learning task, children aged six to ten years (N = 454) were allocated to either a visual-only (V: unisensory), auditory-only (A: unisensory), audio-visual (AV: multisensory), Visual-Visual (VV: multi-cue) or Auditory-Auditory (AA: multi-cue) condition. In children over eight years of age, the availability of two informative cues, regardless of whether they had been presented across two different modalities or within the same modality, was found to be more beneficial to incidental learning than with unisensory cues. In six-year-olds, however, the presence of multiple auditory cues (AA) did not facilitate learning to the same extent as multiple visual cues (VV) or when cues were presented across two different modalities (AV). The findings suggest that multiple sensory cues presented across or within modalities may have differential effects on children's incidental learning across middle childhood, depending on the sensory domain in which they are presented. Implications for the use of multi-cross-sensory and multiple-intra-sensory cues for children's learning across this age range are discussed.

Incidental category learning and cognitive load in a multisensory environment across childhood.

Multisensory information has been shown to facilitate learning (Bahrick & Lickliter, 2000; Broadbent, White, Mareschal, & Kirkham, 2017; Jordan & Baker, 2011; Shams & Seitz, 2008). However, although research has examined the modulating effect of unisensory and multisensory distractors on multisensory processing, the extent to which a concurrent unisensory or multisensory cognitive load task would interfere with or support multisensory learning remains unclear. This study examined the role of concurrent task modality on incidental category learning in 6- to 10-year-olds. Participants were engaged in a multisensory learning task while also performing either a unisensory (visual or auditory only) or multisensory (audiovisual) concurrent task (CT). We found that engaging in an auditory CT led to poorer performance on incidental category learning compared with an audiovisual or visual CT, across groups. In 6-year-olds, category test performance was at chance in the auditory-only CT condition, suggesting auditory concurrent tasks may interfere with learning in younger children, but the addition of visual information may serve to focus attention. These findings provide novel insight into the use of multisensory concurrent information on incidental learning. Implications for the deployment of multisensory learning tasks within education across development and developmental changes in modality dominance and ability to switch flexibly across modalities are discussed. (PsycINFO Database Record

Symptoms of notalgia paresthetica may be explained by increased dermal innervation.

Notalgia paresthetica is a sensory neuropathy characterized by infrascapular pruritus, burning pain, hyperalgesia, or tenderness. To assess whether the symptoms may be caused by alterations in the cutaneous innervation, skin from the affected area of patients (n = 5) was compared with controls (n = 10) comprising the contralateral unaffected area from the same patients and site-matched biopsies of normals, using immunohistochemistry. Frozen sections were immunostained with antisera to the neuropeptides substance P, calcitonin gene-related peptide, vasoactive intestinal polypeptide, and neuropeptide with tyrosine, and to the general neural marker PGP 9.5 and the glial marker S-100 to show the overall innervation and glial cells, respectively. No discernible change in the distribution of neuropeptide-immunoreactive axons was found, but all of the specimens from the affected areas had a significant increase in the number of intradermal PGP 9.5-immunoreactive nerve fibers compared with unaffected areas from the same patients and normal controls. Epidermal dendritic cells immunoreactive for S-100, possibly Langerhans cells, were substantially increased. It is concluded that there is an increase in the sensory epidermal innervation in the affected skin areas in notalgia paresthetica, which could contribute to the symptoms, and that neural immunohistochemistry of skin biopsies could be helpful in the diagnosis of the disease.

Splenic marginal zone cell lymphoma.

We describe four female patients with primary splenic low-grade non-Hodgkin's B-cell lymphomas with the morphology and immunophenotype of splenic marginal zone lymphocytes. The patients presented with splenomegaly, anemia, and weight loss. The bone marrow was involved in all four cases. Liver involvement was found in one patient; and in another, a CT scan revealed lymphadenopathy in the chest and abdomen. The histology of the spleen was characterized by broad concentric strands of monomorphic medium-sized lymphocytes around lymphoid follicles in one case and infiltrating follicles in two cases. Selective replacement of follicles was seen in one case. Tumor in splenic hilar lymph nodes (four cases) and liver (one case) was similar. Three patients remain well 4, 9, and 12 months, respectively, after splenectomy without further treatment. One patient who received chemotherapy died 1 year after splenectomy.

The value of the polymerase chain reaction in the diagnosis of cutaneous T-cell infiltrates.

The distinction between reactive and neoplastic cutaneous T-cell infiltrates is difficult and requires good clinicopathologic correlation. Many cases manifest changes that are at the borderline between the two. The polymerase chain reaction (PCR) has been reported to detect monoclonality in 52-90% of cutaneous T-cell lymphomas and may be of use in the diagnosis of histologically borderline lesions. We have investigated the use of PCR in a series of borderline lesions including borderline biopsy samples from patients who subsequently developed cutaneous lymphoma. PCR amplification of T-cell receptor (TCR)-gamma chain gene was performed on formalin-fixed, paraffin-embedded tissue from 27 cases of clinically and histologically typical mycosis fungoides (MF), 22 borderline biopsy samples from 10 patients who subsequently developed MF (pre-MF), 32 clinically suspicious, histologically borderline lesions, and 31 cases of chronic dermatitis. Monoclonality was demonstrated in 16 of 27 (59%) cases of MF, 10 of 22 (50%) pre-MF biopsy samples (six of 10 patients), and six of 32 (19%) borderline biopsy samples. The same size monoclonal band was detected in pre-MF biopsy samples from six of seven patients in which a band was demonstrated in the diagnostic MF biopsy. Sequencing confirmed that the MF biopsy sample and the pre-MF biopsy sample contained the same clone. The 31 dermatitis cases gave rise to polyclonal PCR products. Monoclonality can be demonstrated using PCR in 59% of MF cases, which is comparable with other T-cell lymphomas and in up to 50% of borderline biopsy samples in patients who later develop lymphoma. Detection of T-cell monoclonality by PCR is strong evidence of an established or evolving cutaneous T-cell lymphoma.

Loss of wild-type p53 and C-erbb2 amplification correlates with high-grade breast carcinomas.

Breast cancer is the most common cancer in women in the western world. Two of the most frequently occuring chromosomal abnormalities in human breast carcinoma are the loss of p53 tumour suppressor gene function and the amplification of the c-erbB2 oncogene. Previous studies have demonstrated the role of p53 gene product in the maintenance of chromosomal stability and the correlation between c-erbB2 amplification and breast carcinogenesis. In this study we have examined the existence of a possible correlation between these genetic alterations in a panel of 83 malignant breast tumours (69 adeno and 14 lobular carcinomas). The status of a related gene, c-erbB3, was also examined. With the aid of microsattelite marker TP53CA loss of heterozygosity (LOH) was detected in the p53 locus in 49% of the tumours. Histochemical analysis of 64 of these tumours with the p53 antibody CM1 demonstrated staining, indicative of an elevated steady-state level of p53 protein in 23 rumours (36%). Amplification of the c-erbB2 gene was detected in 20 of 75 tumours analysed (27%). In the tumours with c-erbB2 amplification 12 also had p53 LOH. In at least another 2 tumours there was increased p53 protein level but no LOH. Therefore in 75% of the tumours with c-erbB2 amplification there was evidence of loss of normal p53 function. There was no evidence of c-erbB3 amplification in any of the 75 rumours analysed. The data presented demonstrates a strong correlation between the loss of p53 and tumour grade (p<0.00545), and a strong association between c-erbB2, but not c-erbB3, amplification and loss of p53 (p<0.0170).

Knowledge-based computer system to aid in the histopathological diagnosis of breast disease.

A knowledge-based computer system, designed to assist pathologists in the histological diagnosis of breast disease, is described. This system represents knowledge in the form of "disease profiles" and uses a novel inference model based on the mathematical technique of hypergraphs. Its design overcomes many of the limitations of existing expert system technologies when applied to breast disease. In particular, the system can quickly focus on a differential problem and thus reduce the amount of data necessary to reach a conclusion. The system was tested on two sets of samples, consisting of 14 retrospective cases and five hypothetical cases of breast disease. Its recommendations were judged "correct" by the evaluating pathologist in 15 cases. This study shows the feasibility of providing "decision support" in histopathology.

Breslow thickness of cutaneous malignant melanoma in paraffin wax and frozen sections.

Breslow tumour thickness was measured in frozen and paraffin wax sections from 21 excision biopsies of cutaneous malignant melanomas by two observers. There was no consistent variation between frozen and paraffin wax sections, with recorded differences ranging from +0.3 mm to -0.2 mm. Interobserver differences ranged from +0.4 mm to -0.2 mm. The interobserver variations exceeded the intraobserver variations, but neither were significant. These findings show conclusively that, when using high quality frozen sections, there is no significant difference between Breslow thickness measured in frozen or paraffin wax sections and therefore that frozen sections can be used to microstage melanoma. Interobserver variations seem to be a more likely source of erroneous measurements of tumour thickness.

Computer assisted diagnosis of fine needle aspirate of the breast.

The development of the National Breast Screening Programme has created a demand for the widespread availability of fine needle aspiration cytology services. To meet this demand there must be a rapid increase in the number of pathologists and laboratories able to offer this service. In turn there is a need for improved training methods. The technique of fine needle aspiration cytology is not inherently complicated. The number of possible conclusions is essentially limited to four: unsatisfactory, benign, suspicious and malignant. A computer based expert system, designed to assist pathologists in the diagnosis of fine needle aspirates of the breast, has been developed. The system prompts pathologists to categorize a number of variables in the aspirate including nuclear and cytoplasmic features, and the degree of cellular cohesion, and uses these data to reason about possible conclusions. The final diagnosis is displayed with a detailed explanation listing the factors supporting it. Initial trials with this system have been encouraging and it is envisaged that this system will be of value both in training and as an aid to routine diagnosis.

Merkel cell carcinoma: a malignant neuroendocrine tumour of the eyelid.

Merkel cell carcinoma is a recently described tumour which may occur on the face of the elderly. We report such a lesion which presented as a cyst on the eyelid of an 88-year-old woman. This tumour differs from other tumours of the eyelids in its propensity for local and distant spread.

Seasonality and breast cancer.

We have analysed the data of biopsy, tumour size, size, side and axillary lymph node status in a group of 866 patients presenting with breast cancer in Southampton from September 1975 to August 1981 and a group of 1424 women presenting with benign disease in the same period. The whole group showed a seasonal variation, with a peak presentation in June. The pre-menopausal age group account for almost all of this seasonality. The cause of the variation is not established and requires further investigation. The control group of benign breast disease cases did not show a seasonal variation.

A computer-based decision support system for diagnostic histopathology of the breast.

Accurate histological diagnosis of breast lesions is essential for the appropriate management of the patient. However, the technique of histological typing is problematic due to the large number of histological patterns, often of a complex and variable nature, which occur in breast disease. The introduction of the Breast Screening Programme has increased the burden on pathologists, and emphasised the need for training. Problems arise because mammographic screening detects a greater proportion of special histological types, with their attendant difficulties of identification, when compared to clinically palpable lesions. A computer-based decision support tool has been developed to assist pathologists in the histological diagnosis of breast disease. Unlike conventional expert systems, which seek to recreate the problem-solving processes of an expert, this system has been designed to act as an intelligent assistant to the pathologist. The system represents knowledge in the form of 'disease profiles', and utilises a novel inference model based upon the mathematical technique of hypergraphs. Initial trials with this system have demonstrated that a high level of diagnostic accuracy can be achieved.

Early developments in the ability to understand the relation between stimulus and reward.

Delayed nonmatching to sample (DNMS) is used to test the recognition memory function dependent on the medial temporal lobe. Children cannot succeed on this task until about 21 months. Because robust recognition is present well before then, the late emergence of another ability must account for the late success on DNMS. Evidence is presented here that the critical late-maturing competence is the ability to grasp the relation between stimulus and reward--that is, to understand that the stimulus is a symbol or marker for the reward. Infants of 9 and 12 months were tested on 3 conditions of DNMS. A sample object was presented. After a delay, the sample and a novel object appeared; choice of the novel object was rewarded. In the standard task, the reward was in a well beneath the stimulus. In the verbal-reward condition the reward was not a separate object but was praise and applause. In the Velcro condition, the reward, although a separate and separable object, was attached to the base of the stimulus. Most infants at both ages succeeded in the verbal-reward and Velcro conditions but not in the standard condition.

Rule selection versus rule execution in preschoolers: an error-detection approach.

In 2 experiments, an error-detection approach was used to determine whether 3-year-olds' perseverative errors on the postswitch phase of the Dimensional Change Card Sort (DCCS) are due to lack of response control or representational inflexibility. In Experiment 1, 3-, 4-, and 5-year-olds watched a puppet sort perseveratively on the postswitch phase and evaluated its responses. Most 4- and 5-year-olds detected the puppet's perseverative errors, whereas most 3-year-olds failed to do so despite detecting errors on a simpler card sort. Experiment 2 revealed that 3-year-olds who failed to correctly evaluate the puppet's behavior tended to fail their own DCCS. Results imply that perseveration on the DCCS cannot be attributed to difficulty inhibiting prepotent motor responses. Instead, changes in rule use between 3 and 5 years of age are interpreted in terms of the development of representational flexibility.

Noonan's syndrome and cyanotic congenital heart disease. A report of 4 cases and review of the literature.

The clinical features of four adult patients with Noonan's syndrome and cyanotic congenital heart disease are presented. These findings, together with those of previously reported cases, illustrate the wide spectrum of cardiac anomalies that may be associated with the multiple somatic abnormalities of Noonan's syndrome.

Object-oriented design and programming in medical decision support.

The concept of object-oriented design and programming has recently received a great deal of attention from the software engineering community. This paper highlights the realisable benefits of using the object-oriented approach in the design and development of clinical decision support systems. These systems seek to build a computational model of some problem domain and therefore tend to be exploratory in nature. Conventional procedural design techniques do not support either the process of model building or rapid prototyping. The central concepts of the object-oriented paradigm are introduced, namely encapsulation, inheritance and polymorphism, and their use illustrated in a case study, taken from the domain of breast histopathology. In particular, the dual roles of inheritance in object-oriented programming are examined, i.e., inheritance as a conceptual modelling tool and inheritance as a code reuse mechanism. It is argued that the use of the former is not entirely intuitive and may be difficult to incorporate into the design process. However, inheritance as a means of optimising code reuse offers substantial technical benefits.

Neurofibromas of the paranasal sinuses.

Two cases of neurofibroma of the paranasal sinuses are presented. The tumours presented as space-occupying lesions and were treated by local excision. Histologically one was a typical neurofibroma, whilst the other showed some features of a schwannoma. The patients show no evidence of tumour recurrence at 18 and 6 months respectively.

Malignant parotid salivary gland peripheral nerve sheath tumour in a twelve-year-old girl.

A case of a malignant parotid salivary gland nerve sheath tumour is reported in a 12-year-old girl who developed a right parotid mass. Initial incisional biopsy showed a tumour with a mesenchymal spindle cell appearance. Immunohistochemical studies showed positive staining of tumour cells for vimentin and focally for S-100 protein. These features together with ultrastructural evidence of basal lamina material suggested that the tumour was of nerve sheath origin. After subtotal parotidectomy the tumour metastasised to cervical lymph node and lung. There was evidence of a partial response to chemotherapy. A detailed illustrated histopathological description of the tumour is given.