Identifying poor adaptation to a new diagnosis of motor neuron disease: A pilot study into the value of an early patient-led interview.

This pilot study explored whether clinicians can identify patients who may not be coping with a diagnosis of MND early in the disease course by using patient-led interviews rather than psychological testing. Consecutive, newly diagnosed MND patients underwent a semi-structured interview six and 18 weeks after diagnosis, and completed SEIQoL-DW, ALSFRS-R, MND Coping Scale, MND Social Withdrawal Scale, SF 36 v2 and HAD. Three physicians independently used a list of factors from the literature associated with coping with a diagnosis of MND, and overall impression to assign patients from interview transcripts to groups of copers and non-copers. Ten of 13 recruited patients were categorized unanimously using the first interview alone. Four patients were categorized as non-copers. These showed significantly higher scores for depression, anxiety and social withdrawal and significantly lower scores for coping and mental composite scores on the SF 36 v2. Our pilot study suggests it is possible for experienced clinicians to identify patients who may struggle to cope with a diagnosis of MND early by patient-led interview alone. This provides some evidence for the therapeutic potential of the early follow-up appointment interview with a senior doctor soon after diagnosis, advocated by current British guidelines.

Renal failure and specialist palliative care: an assessment of current referral practice.

AIMS: This retrospective audit assessed the referral practice for patients with end-stage renal failure from the nephrology service to the specialist palliative care team in a large teaching hospital in the north-west of England. METHODS: Forty-nine referrals with 'renal' as a primary diagnosis over a two-year period were identified from referral data. General and palliative care notes were reviewed and a data collection tool was designed. RESULTS: Most common reasons for referral were for 'placement' (38.6%) and 'dying/distressed' patients (22.7%), although psychological support was also prevalent (15.9%). Renal teams discussed stopping dialysis in the majority of cases (89%), but documented preferred place of care less frequently (48.3%) and achieved discharge to these locations in less than half of cases (21.4%). CONCLUSION: There was well-established referral practice between the renal and the specialist palliative care team at the hospital examined. The renal team appropriately referred for symptom control and support in the dying phase of patients. There are issues surrounding placement and increased implementation of end-of-life care tools, including the Liverpool Care Pathway, Gold Standards Framework and Preferred Place of Care documentation for renal patients, which needs to be an ongoing priority.

Respiratory tract secretions in the dying patient: a comparison between glycopyrronium and hyoscine hydrobromide.

The evidence for the management of respiratory tract secretions (RTS) in dying patients with antimuscarinic drugs remains inconclusive. This study investigated the effectiveness of glycopyrronium versus hyoscine hydrobromide in controlling RTS using the Liverpool Care of the Dying Pathway (LCP) in 72 patients matched for age, diagnosis, and gender who died on a 30-bed specialist palliative care unit. All patients in the glycoypyrronium group had some response to glycopyrronium, whereas 22% of patients in the hyoscine group had no response to hyoscine hydrobromide. There was a significant difference in overall response between the two groups (p < 0.01). Twenty-eight percent of patients in the glycopyrronium and 42% of patients in the hyoscine group died with RTS present. There was no statistically significant difference in the levels of agitation following administration of either drug. This study provides further evidence that the LCP can be a useful tool in the evaluation of new drugs for symptom control in dying patients and suggests that glycopyrronium may be at least as effective in controlling RTS in dying patients as hyoscine hydrobromide.

Mutations of the BRAF gene in benign and malignant melanocytic lesions.

A single-point mutation in exon 15 of the BRAF gene has recently been reported in a high percentage in cultured melanoma cells and in 6 of 9 primary melanomas examined. To evaluate the impact of the T1796A BRAF mutation, we screened primary melanomas, various types of nevi and lesions where a melanoma developed in an underlying nevus. We could detect the mutation in 28 of 97 (29%) melanomas and in 39 of 187 (21%) nevi, including blue nevi (0/20) and Spitz nevi (0/69), which did not carry the mutation. In melanomas with an underlying nevus, either the mutation was present in both the laser-microdissected nevus cells and the laser-microdissected melanoma cells (3/14) or both lesions were negative for the BRAF mutation except one case. In conclusion, mutations in exon 15 of the BRAF gene are nonspecific for progression of a nevus to a melanoma. Other so far unknown cofactors seem to be of importance.

The prevalence, key causes and management of insomnia in palliative care patients.

In a prospective audit, the prevalence, key causes and treatment of insomnia prior to admission were evaluated in a population of hospice patients using a questionnaire based on a review article of key features related to insomnia in the palliative care setting. Seventy-four patients completed the questionnaire. Fifty-two (70%) patients had insomnia symptoms. Uncontrolled physical symptoms, most often pain (15 patients), were the commonest cause of insomnia, cited by 31 (60%) sleep-disturbed patients. Thirteen (62%) of 21 patients who had been prescribed hypnotic medication reported an improvement with the prescribed medication. Twenty (38%) of the 52 patients with insomnia suggested that improved symptom control would improve their sleep, and only two (4%) suggested the need for more hypnotic medication. We conclude that insomnia is a common symptom in terminally ill patients and that improved symptom control should be a priority in the management of insomnia in this group of patients.

Percutaneous cervical cordotomy for the management of pain from cancer: a prospective review of 45 cases.

BACKGROUND: Percutaneous cervical cordotomy (PCC) is a well recognized but infrequently performed procedure for the relief of unilateral intractable pain from malignancy. There is a paucity of data regarding efficacy and safety of PCC. OBJECTIVES: The study's objectives were to demonstrate the efficacy and safety of PCC in cancer pain. DESIGN: The study was a prospective review of 45 cases undergoing PCC at a tertiary referral center over a three-year period. SETTINGS/SUBJECTS: All patients were suffering from severe, refractory unilateral pain secondary to malignancy with poor pain relief or intolerable side effects of conventional analgesics including opioids and adjuvants. MEASUREMENTS: Variables recorded preprocedure, at 2 days, and at 28 days postprocedure were numerical rating scale for maximum and average pain, oral morphine equivalent dose, and global impression of change. Adverse events and survival postprocedure were recorded. RESULTS: Prospective data was obtained in 45 patients. Survival postprocedure ranged from 7 days to 33 months. There was a significant reduction from baseline in pain scores at 2 days and at 28 days postprocedure. Thirty-two patients experienced significant pain relief--average numerical rating scale (NRS) of zero--on day 2. Improvement in pain scores was sustained at 28 days. There were no serious adverse events observed such as respiratory failure. CONCLUSIONS: PCC is a safe and highly effective procedure to treat intractable unilateral cancer pain. It offers significant advantages over other pain control methods. Patient selection and attention to detail is paramount for a successful outcome.

The evaluation of a peer-led question-writing task.

BACKGROUND: Novel studies have previously highlighted the educational benefits of peer-led learning and peer marking of examinations. Limited data exist about the educational value of students writing their own exam questions and sharing these with other students. AIM: To evaluate the potential for medical students to learn about palliative care through the process of writing examination questions. methods: Fourth-year medical students on a palliative medicine rotation were invited to write a short-answer exam question in a similar format to the official examination run by the medical school. The questions were checked for accuracy and applicability by the coordinator, and were then distributed to students. The answers to the student-generated questions were discussed in a feedback forum at the end of the rotation. RESULTS: Twenty students took part in the exercise. All agreed the exercise was beneficial to their learning. Eighteen (90%) students did not feel the task was too much additional work. Eight (40%) students felt more confident in passing the official medical school exams. Students enjoyed the exercise, with 19 (95%) indicating that they were keen to repeat this in the future. CONCLUSION: Peer-led examination question writing could complement the delivery of palliative care teaching for medical students.

[Fibrohistiocytic skin tumors]. / Fibrohistiozytäre Tumoren der Haut.

The fibrohistiocytic tumors of the skin are a heterogeneous group of dermal/subcutaneous mesenchymal neoplasms which show fibroblastic, myofibroblastic and histiocytic (macrophage-like) differentiation, often one beside the other in the same tumor. "Fibrohistiocytic" means in this context the morphologic similarity of the cells with fibroblasts and histiocytes. The WHO classification of 2005 includes the following entities as fibrohistiocytic tumors of the skin: BENIGN: 1. Fibrous histiocytoma (FH)/(synonymous: Dermatofibroma. Variants of FH: 1a. cellular fibrous histiocytoma, 1b. atypical (pseudosarcomatous) fibrous histiocytoma, 1c. aneurysmatic fibrous histiocytoma, 1d. epithelioid fibrous histiocytoma; 2. dermatomyofibroma; 3. (juvenile) xanthogranuloma. INTERMEDIATE: 4. plexiform fibrohistiocytic tumor; 5. dermatofibrosarcoma protuberans; 6. atypical Fibroxanthoma. MALIGNANT: 7. malignant fibrous histiocytoma. All these entities are reviewed in this paper with particular attention devoted to differential diagnostic considerations.

How does current care practice influence the experience of a new diagnosis of motor neuron disease? A qualitative study of current guidelines-based practice.

Management during the diagnostic phase of MND is considered an important influence on the patient's further disease course. Guidelines for the management of MND have been developed, but little is known on how recommended practices influence patients' experience of a new diagnosis of MND. We undertook an interview-based study exploring issues surrounding the diagnosis of MND in a sample of consecutive patients recently diagnosed at a neurosciences centre. Data were analysed with Interpretative Phenomenological Analysis (IPA). Thirteen patients were recruited to the study. The major themes identified in descending order were 'Family/carers', 'Communication of the diagnosis', 'Reaction to the diagnosis', 'Physical difficulties', 'Time before diagnosis', 'Information', 'Future', 'Coping with the diagnosis' and 'Formal support'. Communication was perceived satisfactory if handled according to existing guidelines. Physical difficulties impacted considerably on patients' lives. Delays in the diagnostic process appeared in various interviews. Five patients were avoiding information following the diagnosis. Some patients commented on an increasing number of uncoordinated services becoming involved after their diagnosis. This study supports the current international guidelines for MND care around diagnosis from the patients' perspective and underlines the importance of a multi-disciplinary, coordinated approach for patients with MND from diagnosis onwards.

Unusual hyperplasia of anogenital mammary-like glands.

Anogenital mammary-like glands, although known to exist (as ectopic breast tissue) as early as 1872, have only recently been thoroughly studied and suggested to be the possible origin for various neoplastic (benign and malignant) and reactive conditions that show a striking homology with lesions in the breast. Reported herein is an unusual benign lesion involving anogenital mammary-like glands in the perineal area of a 41-year-old woman. The lesion is unique in that it does not fully fit into any of the previously recognized conditions affecting anogenital mammary-like glands nor has it an apparent well-defined mammary counterpart. Microscopically, at first glance the lesion appeared as a cluster of extended anogenital mammary-like glands of variable complexity and a lobular architecture, which probably resulted from a compact proliferation of closely situated ducts with small, round lumens (when cut transversally). When cut longitudinally, some of these tiny ducts appeared to bud from larger ductal structures, resembling so-called satellitosis in the breast and this gave a fibroadenomatous appearance to the areas. In addition, there were adenosis-like areas. This lesion extends the spectrum of changes seen in anogenital mammary-like glands.

Ductal carcinoma arising from a syringocystadenoma papilliferum in a nevus sebaceus of Jadassohn.

We present an example of ductal carcinoma connected to a syringocystadenoma papilliferum situated in a nevus sebaceus of Jadassohn on the scalp of a 22-year-old woman. The ductal carcinoma involved the entire thickness of the dermis and extended to the subcutaneous fat. Because syringocystadenoma papilliferum is considered a hamartoma with apocrine differentiation, the ductal carcinoma here described was interpreted as an apocrine ductal carcinoma. Syringocystadenocarcinoma papilliferum is an exceedingly rare neoplasm, most examples of which seem to have arisen in its benign counterpart, syringocystadenoma papilliferum. From a histopathologic point of view, syringocystadenocarcinoma papilliferum usually shows a papillary configuration similar to that of syringocystadenoma papilliferum. In contrast, the case here described a ductal carcinoma superficially connected to a syringocystadenoma papilliferum, but mostly composed of small ductal structures embedded in a desmoplastic stroma and involving the full thickness of the dermis. We review the literature about the malignant neoplasms arising in the nevus sebaceus of Jadassohn.

CD34-positive glomus tumor: clinicopathologic and immunohistochemical analysis of six cases with myxoid stromal changes.

BACKGROUND: Glomus tumors are benign, mainly superficially located perivascular neoplasms, composed of cytologically characteristic neoplastic cells staining immunohistochemically positive for vimentin and muscle actin, closely associated with often branching blood vessels. METHODS: Six cases of glomus tumor were analysed histologically and immunohistochemically. RESULTS: We report six cases of glomus tumor (three solid glomus tumors, two glomangiomas, one glomangiomyoma) arising on the fingers of adult patients (five female and one male patient; age range 35-65 years) that showed prominent myxoid stromal changes and immunohistochemically a coexpression of alpha-smooth muscle actin and CD34 by neoplastic cells. CONCLUSIONS: Neoplastic cells in glomus tumor may show a coexpression of alpha-smooth muscle actin and CD34, an important finding regarding the differential diagnosis of these lesions and the relationship of perivascular neoplasms.

Anetoderma: another facet of Lyme disease?

Lyme disease has been suspected to be one cause of secondary anetoderma. We present a 25-year-old male patient with multiple lesions of anetoderma with a diameter of up to 2 cm that developed within the last 4 weeks without subjective symptoms. The histopathologic pattern was similar to the inflammatory stage of acrodermatitis chronica atrophicans. Polymerase chain reaction analysis out of the paraffin-embedded tissue, confirmed by sequencing of the obtained nucleotide product, revealed a part of the 23 S ribosomal RNA gene of Borrelia burgdorferi sensu lato. Enzyme-linked immunosorbent assay showed an increased serum IgG titer against B burgdorferi corroborated by Western blot analysis. After a treatment with oral doxycycline (100 mg twice a day) for 30 days the anti-B burgdorferi titer decreased significantly and no new lesions appeared. Some cases of anetoderma might be caused by Borrelia and patients with anetoderma should be examined for borreliosis including serology and polymerase chain reaction of lesional skin. In cases of Borrelia-induced anetoderma, early antibiotic treatment could prevent further progression of the disfiguring skin lesions and the underlying disease.

The T1796A mutation of the BRAF gene is absent in Spitz nevi.

BACKGROUND: BRAF, a serine/threonine kinase, is a component of the retrovirus-associated sequence (RAS)-RAF-extracellular-regulated protein kinase (ERK)-MAP kinase signal transduction pathway mediating signals from RAS to ERK. The T1796A single point mutation in exon 15 of the BRAF gene has recently been reported in a high percentage of malignant melanomas and benign melanocytic lesions such as congenital nevi, compound nevi, intradermal nevi and dysplastic nevi. The T1796A mutation has been shown to promote cell proliferation. METHODS: We screened 21 Spitz nevi and six spitzoid malignant melanomas for the presence of the T1796A BRAF mutation. RESULTS: The T1796A BRAF mutation could not be detected in any of the 21 Spitz nevi but was present in two of the six spitzoid malignant melanomas. CONCLUSIONS: Our results, in conjunction with data from a previous investigation, suggest that the melanocytic proliferation of Spitz nevi might be induced by components of the RAS-RAF-ERK-MAP kinase pathway different from BRAF, possibly combined with other genetic aberrations. The lack of the T1796A BRAF mutation might be of practical importance in distinguishing Spitz nevi from other melanocytic lesions simulating Spitz nevi as a part of a future complex diagnostic assay.