Malignant Peritoneal Mesothelioma Arising in Young Adults With Long-standing Indwelling Intra-abdominal Shunt Catheters.

Only 50% to 70% of patients with mesothelioma report asbestos exposure. Other exposures (eg, radiation) play a role in some cases, but some patients have no obvious cause. We describe a series of patients with long-standing indwelling intra-abdominal shunt catheters who developed malignant peritoneal mesothelioma, suggesting a novel association. We identified 7 patients who had shunts and subsequently developed mesothelioma (5 women; median age: 31 y, range: 18 to 45 y). Clinical history and pathology materials were reviewed, and RNA sequencing was performed. Clinical presentations varied; 6 patients had hydrocephalus and a ventriculoperitoneal shunt, and 1 patient had portal hypertension and a portoatrial shunt. The median duration of shunt therapy in 5 cases was 29 years (range: 12 to 35 y); the remaining 2 patients also had shunts for many years, but specific details were unavailable. Two patients had radiotherapy for malignancies in childhood. One had an alleged exposure to asbestos and 1 had prior exposure to talc. The rest had no known risk factors. Histologically, all tumors were purely epithelioid. Treatments included surgical debulking, chemotherapy, and palliative care. All 7 died of disease (median survival: 7 mo, range: 1 to 18 mo). Molecular testing showed loss of NF2 and CDKN2A/B and a BAP1 mutation in 1 case, and no genomic alterations associated with mesothelioma in 2 cases. Peritoneal mesothelioma may represent a complication of long-standing indwelling shunt catheters. The mechanism is unknown, but chronic peritoneal irritation may play a role. Albeit rare, mesothelioma should be considered in patients with a shunt who present with new ascites.

Malignant Peritoneal Mesothelioma in the setting of a Ventriculo-Peritoneal Shunt: A novel clinical presentation.

We report a case of malignant peritoneal mesothelioma (MPM) in a 31-year-old male with history of cerebral palsy, hydrocephalus, and ventriculoperitoneal shunt (VPS) placed since infancy. He presented with fever, abdominal pain and distension. Computed tomography scan revealed a thick-walled rim-enhancing fluid collection, interpreted as pseudocyst. Intraoperatively, diffuse nodular peritoneal thickening with adhesions was demonstrated. The resection specimen consisted of multiple membranous fragments displaying firm nodules. Microscopic examination revealed a tumefactive malignant-appearing epithelioid proliferation involving the peritoneum, focally invading the underlying fat. Immunohistochemically, the tumor cells expressed keratin AE1/AE3, CK7, CK5/6, Calretinin, WT1 and D2-40, and were negative for CEA and MOC31. The findings were consistent with MPM, epithelioid type. The patient's condition continued to decline with increasing abdominal distension during the month following the original diagnosis. While atypical mesothelial hyperplasia has been described in association with long standing VPS, well-documented cases of MPM have not been previously reported in such context.

Debt Counselling for Depression in Primary Care: an adaptive randomised controlled pilot trial (DeCoDer study).

BACKGROUND: Depression and debt are common in the UK. Debt Counselling for Depression in Primary Care: an adaptive randomised controlled pilot trial (DeCoDer) aimed to assess the clinical effectiveness and cost-effectiveness of the addition of a primary care debt counselling advice service to usual care for patients with depression and debt. However, the study was terminated early during the internal pilot trial phase because of recruitment delays. This report describes the rationale, methods and findings of the pilot study, and implications for future research. OBJECTIVES: The overarching aim of the internal pilot was to identify and resolve problems, thereby assessing the feasibility of the main trial. The specific objectives were to confirm methods for practice recruitment and the ability to recruit patients via the proposed approaches; to determine the acceptability of the study interventions and outcome measures; to assess contamination; to confirm the randomisation method for main trial and the level of participant attrition; and to check the robustness of data collection systems. DESIGN: An adaptive, parallel, two-group multicentre randomised controlled pilot trial with a nested mixed-methods process and economic evaluation. Both individual- and cluster (general practice)-level were was used in the pilot phase to assign participants to intervention or control groups. SETTING: General practices in England and Wales. PARTICIPANTS: Individuals were included who were aged ≥ 18 years, scored ≥ 14 on the Beck Depression Inventory II and self-identified as having debt worries. The main exclusion criteria were being actively suicidal or psychotic and/or severely depressed and unresponsive to treatment; having a severe addiction to alcohol/illicit drugs; being unable/unwilling to give written informed consent; currently participating in other research including follow-up phases; having received Citizens Advice Bureau (CAB) debt advice in the past year; and not wanting debt advice via a general practice. INTERVENTIONS: The participants in the intervention group were given debt advice provided by the CAB and shared biopsychosocial assessment, in addition to treatment as usual (TAU) and two debt advice leaflets. The participants in the control group were given advice leaflets provided by the general practitioner and TAU only. MAIN OUTCOME MEASURES: (1) Outcomes of the pilot trial - the proportion of eligible patients who consented, the number of participants recruited compared with target, assessment of contamination, and assessment of patient satisfaction with intervention and outcome measures. (2) Participant outcomes - primary - Beck Depression Inventory II; secondary - psychological well-being, health and social care utilisation, service satisfaction, substance misuse, record of priority/non-priority debts, life events and difficulties, and explanatory measures. Outcomes were assessed at baseline (pre-randomisation) and at 4 months post randomisation. Other data sources - qualitative interviews were conducted with participants, clinicians and CAB advisors. RESULTS: Of the 238 expressions of interest screened, 61 participants (26%) were recruited and randomised (32 in the intervention group and 29 in the control group). All participants provided baseline outcomes and 52 provided the primary outcome at 4 months' follow-up (14.7% dropout). Seventeen participants allocated to the intervention saw a CAB advisor. Descriptive statistics are reported for participants with complete outcomes at baseline and 4 months' follow-up. Our qualitative findings suggest that the relationship between debt and depression is complex, and the impact of each on the other is compounded by other psychological, social and contextual influences. CONCLUSIONS: As a result of low recruitment, this trial was terminated at the internal pilot phase and was too small for inferential statistical analysis. We recommend ways to reduce this risk when conducting complex trials among vulnerable populations recruited in community settings. These cover trial design, the design and delivery of interventions, recruitment strategies and support for sites. TRIAL REGISTRATION: Current Controlled Trials ISRCTN79705874. FUNDING: This project was funded by the National Institute for Health Research (NIHR) Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 21, No. 35. See the NIHR Journals Library website for further project information. Mark Gabbay and Adele Ring are part-funded by NIHR Collaborations for Leadership in Applied Health Research and Care (CLAHRC) North West Coast and Richard Byng and Rod S Taylor, Vashti Berry and Elizabeth Shaw part-funded by NIHR CLAHRC South West Peninsula.

Matrix-producing carcinoma: a rare variant of metaplastic breast carcinoma with heterologous elements.

Matrix-producing carcinoma (MPC) of the breast is a rare variant of the uncommon group of malignancies categorized as metaplastic breast carcinomas with heterologous elements. The major criterion for a diagnosis of MPC is the presence of invasive breast carcinoma with the direct transition to a cartilaginous or osseous stromal matrix without an intervening spindle cell component. The cellular origin of MPC remains unclear. It has been suggested that tumor cells in MPC have combined epithelial and mesenchymal features. Several reports have suggested that the tumor cells originate from myoepithelial cells. The prognosis of patients with MPC was originally described as similar to invasive mammary carcinomas of no special type (NST) of the same stage, but a more recent study has shown a worse prognosis than same-stage NSTs.

Chromobacterium violaceum cellulitis and sepsis following cutaneous marine trauma.

Chromobacterium violaceum is a gram-negative bacillary organism that characteristically produces the purple pigment violacein. Documented as the cause of clinically relevant human infections in only 35 cases in the United States, C violaceum is particularly seen in patients with a history of cutaneous injury or trauma. We report the case of an 18-year-old woman who was struck by a propeller in a boating accident and sustained multiple deep lacerations of her right lower extremity. Shortly after admission to the hospital, the patient became frebrile and developed leukocytosis. Bacterial cultures revealed C violaceum, which demonstrated a characteristic purple pigment production on blood agar. Antimicrobial therapy was initiated, but 2 days after admission, the skin and subcutaneous tissue surrounding the patient's wounds became necrotic, necessitating an above-the-knee amputation of the right lower extremity (transfemoral amputation). The patient's condition improved after continued antimicrobial therapy and she was subsequently discharged in good health. This case represents a successful outcome of a rare but frequently fatal infection due to a morphologically and geographically distinct human pathogen.

Elevated expression of isopeptide bond cross-links contributes to fibrosis in scleroderma and the healing wounds of tight skin mice.

Scleroderma is a chronic disease characterized by excessive tissue fibrosis. Recent studies indicate that cultured dermal fibroblasts isolated from patients produce excessive amounts of collagen and other extracellular matrix components. In this study, we investigated the mechanism(s) of abnormal extracellular matrix accumulation in the scleroderma biopsies and the healing wounds of Tsk1/+ mice. Full-thickness excisional wounds were made in Tsk1/+ and wild-type mice and were subsequently harvested at days 7, 10, and 14 postinjury. The levels of pro-fibrotic cytokine, transforming growth factor were elevated in the wounds of Tsk1/+ mice. Interestingly, the levels of matrix metalloproteinase were significantly reduced in the granulation tissue of Tsk1/+ mice in comparison with wild-type. Furthermore, immunohistochemical analysis of the wounds indicated that the levels of gamma-glutamyl-epsilon-lysine cross-links were elevated in the granulation tissue of Tsk1/+ mice as well as the fibrotic lesions of scleroderma specimens. Collectively, these findings indicate that elevated collagen synthesis and decreased matrix metalloproteinase levels, in combination with increased isopeptide bond cross-links, contribute to abnormal collagen synthesis and assembly in granulation tissue of Tsk1/+ mice and the fibrotic lesions of scleroderma patients.

Fatal cerebral phaeohyphomycosis due to Curvularia lunata in an immunocompetent patient.

Curvularia infections in humans are relatively uncommon despite the ubiquitous presence of this soil-dwelling dematiaceous fungus in the environment. Originally thought to be solely a pathogen of plants, Curvularia has been described as a pathogen of humans and animals in the last half-century, causing respiratory tract, cutaneous, and corneal infections. Only three previous cases of central nervous system involvement by Curvularia have been documented in the medical literature. We report a fatal case of cerebral Curvularia infection in which there was no known history of immunocompromise or prior respiratory tract or sinus infection in the patient.

A model for consolidation of clinical microbiology laboratory services within a multihospital health-care system.

To determine the cost-effectiveness of consolidating clinical microbiology services in a three-hospital health-care network while maintaining high-quality laboratory services, a retrospective review of the total costs of maintaining separate clinical microbiology laboratories within our health-care system was compared to the cost of providing these services after consolidation. Turnaround times before and after consolidation were compared to assess efficiency of the consolidated services. Input of clinicians was also solicited to ensure that quality of services and customer satisfaction remained high. The results of the consolidation project show that the net fiscal saving because of consolidation of clinical microbiology services within our health-care system will be approximately 100,000 dollars per fiscal year. This value includes increased courier charges as well as personnel savings. Although fiscal savings are an integral part of any laboratory consolidation plan, the financial considerations must be balanced by quality of service. The response to consolidation from clinicians was decidedly mixed before implementation of the plan because of fear of increased turnaround times and limited access to laboratory information. The consolidation process, however, was smooth with few physician complaints. The consolidation of our clinical microbiology services illustrates that significant financial savings can be achieved without compromise of efficiency or quality of service.

Infiltrating syringomatous adenoma of the nipple: a case report and 20-year retrospective review.

Infiltrating syringomatous adenomas are rare lesions of the nipple that were first described in 1983. The exact origin of these lesions is uncertain, although derivation from eccrine structures of the nipple has been postulated because the lesions are microscopically reminiscent of other tumors of eccrine origin, such as syringomatous carcinoma. The lesions are usually infiltrative, showing an expansile pattern of proliferation into adjacent tissues of the nipple and underlying breast. Involvement of the epidermis, however, has not been described. The lesions behave in a benign fashion, with no evidence of regional or distant metastasis in any of the reported cases. Complete local excision appears to be sufficient therapy, with only incompletely excised cases showing recurrence. We report an additional case of infiltrating syringomatous adenoma of the nipple and review the medical literature related to this lesion published in the 20 years since its initial description.

Varied clinical presentations of Vibrio vulnificus infections: a report of four unusual cases and review of the literature.

Vibrio vulnificus is a Gram-negative, motile, curved bacillus of the family Vibrionaceae that is a rare cause of gastroenteritis, septicemia, and wound infections in humans. V. vulnificus is halophilic, flourishes in warm temperatures, and is part of the bacterial flora of the marine environment. The location of our health care setting, on the Gulf of Mexico, has given us the opportunity to observe a wide variety of clinical presentations of infections caused by this organism. In the first case, a 27-year-old man struck by lightning while windsurfing was found pulseless in the water and was resuscitated. The patient subsequently developed cardiac arrhythmias, respiratory failure, and necrotizing fasciitis, blood cultures yielded V. vulnificus. After antibiotic therapy and several fasciotomies, the patient recovered. The second case was that of a 43-year-old Asian man employed as an oyster shucker who presented with complaints of redness, tearing, and photophobia of the right eye. The diagnosis of corneal ulcer secondary to V. vulnificus was made after culture of the right eye revealed the organism. The third case involved a 46-year-old man who presented with complaints of abdominal pain, nausea, chills, and bullous lesions on the lower extremities. He developed disseminated intravascular coagulation, and cultures of the lesions on his lower extremities showed V. vulnificus. Initially, the patient denied any exposure to raw seafood or seawater, but he eventually remembered eating raw oysters 3 days before his illness. The fourth case is that of a 32-year-old, human immunodeficiency virus-positive, hepatitis C-positive woman with cirrhosis who presented with productive cough, chills, fever, and red spots on her extremities and buttocks. Blood cultures revealed V. vulnificus and the patient was treated with antibiotics and improved clinically. These four cases illustrate the wide range of clinical presentations associated with this organism.

Lymphoepithelial cysts of the thyroid gland. A case report and review of the literature.

We report a case of multiple lymphoepithelial cysts of the thyroid gland in a patient with severe chronic lymphocytic thyroiditis. Lymphoepithelial cysts of the thyroid gland are rare lesions that are histologically similar to branchial cleft cysts found in the lateral neck. The cysts have an epithelial lining that is usually stratified squamous epithelium but may be focally respiratory-type epithelium. Abundant lymphoid tissue is present beneath the epithelium, and lymphoid follicles with reactive germinal centers are common features in the walls of the cysts. Because of their similarity to branchial cleft cysts and the presence of intrathyroidal branchiae-derived structures such as thymus and parathyroid gland tissue in the vicinity of some cysts, lymphoepithelial cysts of the thyroid have been postulated to arise from remnants of branchial derivatives; origination from solid cell nest remnants of the ultimobranchial bodies has been raised as a possibility. A definitive origin, however, has not been established. An association with chronic thyroiditis has been noted in 8 of the previously reported 16 cases occurring in a background of Hashimoto or chronic lymphocytic thyroiditis.

Reference test review by pathology house staff: a cost-containment strategy for the clinical laboratory.

In this era of fiscal responsibility in health care, new strategies are evolving to decrease costs in the clinical laboratory. At our institution, one such strategy implemented in 1994 consisted of the review by pathology housestaff of all send-out tests that cost more than $75 to ensure that an appropriate diagnostic test hierarchy was being used and that clerical misorders were corrected. Throughout the first fiscal year this system was implemented, the annual institutional reference laboratory costs decreased by 10% ($24,784), and the number of send-out tests decreased by 23% (2,707 tests). Over the next 2 fiscal years, the number of sendout tests maintained a level approximately 25% lower than the original pre-review level and the reference laboratory costs remained 4% to 13% below the pre-review level. A major factor preventing a more extensive cost reduction over this time period was the increased frequency of orders for expensive molecular studies. Even with the increased use of these relatively expensive tests, however, our annual reference laboratory budget and number of send-out tests have remained consistently below the levels of 1994. Implementation of similar test review systems in other health-care laboratories could provide useful budgetary reductions.