Congenital fibrous fusion of the jaws (synechiae) with cleft palate: A case report.

BACKGROUND: Congenital maxillomandibular fusion is a rare disorder. Maxillomandibular fusion is usually discovered immediately after birth because the newborn is unable to open mouth or feed normally. The fusion defects can present with a wide range of severity, ranging from mucosal band (synechiae) to complete bony fusion (syngnathia). CASE DESCRIPTION: We report a case of congenital alveolar synechiae with posterior cleft palate in a 3-day-old male neonate. The newborn was managed with the help of a multi-disciplinary approach. CONCLUSION: Fusion of the gums is a very rare congenital anomaly. Early division of fibrous bands not only allows effective feeding but also prevents the development of facial deformities. However, anesthesia for this procedure can be challenging.

Chemoradiotherapy for squamous cell anal carcinoma: a review of prognostic factors.

AIM: Previous literature has sought prognostic factors for the survival of anal cancer patients. The present study aimed to determine prognostic factors for local disease recurrence, distant metastasis and survival for patients treated with radical chemoradiotherapy (CRT) at the Rosemere Cancer Centre, Preston, UK. METHOD: Patients treated with CRT for nonmetastatic squamous cell anal cancer between September 2000 and January 2013 were studied. Kaplan-Meier and Cox regression analysis assessed the prognostic value of age, sex, tumour size, the proportion of the anal canal circumference involved (ACCI), nodal disease, tumour location and pretreatment haemoglobin. RESULTS: One hundred and 48 patients with a mean age of 63 years were studied, of whom 15% suffered local disease recurrence and 10% developed distant metastasis. The 5-year overall and cancer-specific survival rates were 84% and 86%, respectively. Predictors of local recurrence were tumour size >5 cm and over two-thirds ACCI (P < 0.01). Predictors of distant metastasis and poor survival were tumour size >5 cm (P < 0.01), node positive disease on imaging (P < 0.05), over two-thirds ACCI (P < 0.01) and a pretreatment haemoglobin level below 130 g/l (P < 0.05). Multivariate analysis found large tumour size to be the most significant factor for local recurrence (P = 0.002) and survival (P = 0.02) whilst over two-thirds ACCI was most predictive of distant metastasis (P < 0.001). Age, gender, palpable lymph nodes and tumour location were not of prognostic value for local disease recurrence, distant metastasis or survival. CONCLUSION: Tumour size, nodal disease, over two-thirds ACCI and low pretreatment haemoglobin confer poorer prognostic and survival outcomes. Use of intensity-modulated radiation therapy may allow greater radiation doses to be given for locally advanced tumours, thus improving local control and survival and reducing morbidity.

An exploratory randomized controlled trial comparing telephone and hospital follow-up after treatment for colorectal cancer.

AIM: Following treatment for colorectal cancer it is common practice for patients to attend hospital clinics at regular intervals for routine monitoring, although debate persists on the benefits of this approach. Nurse-led telephone follow-up is effective in meeting information and psycho-social needs in other patient groups. We explored the potential benefits of nurse-led telephone follow-up for colorectal cancer patients. METHOD: Sixty-five patients were randomized to either telephone or hospital follow-up in an exploratory randomized trial. RESULTS: The telephone intervention was deliverable in clinical practice and acceptable to patients and health professionals. Seventy-five per cent of eligible patients agreed to randomization. High levels of satisfaction were evident in both study groups. Appointments in the hospital group were shorter (median 14.0 min, range 2.3-58.0) than appointments in the telephone group (median 28.9 min, range 6.1-48.3). Patients in the telephone arm were more likely to raise concerns during consultations. CONCLUSION: Historical approaches to follow-up unsupported by evidence of effectiveness and efficiency are not sustainable. Telephone follow-up by specialist nurses may be a feasible option. A main trial comparing hospital and telephone follow-up is justified, although consideration needs to be given to trial design and practical issues related to the availability of specialist nurses at study locations.

Metachronous bilateral adrenal metastases following curative treatment for colorectal carcinoma.

A delayed, metachronous presentation of bilateral adrenal metastases following colorectal cancer has never previously been reported. We describe the case of a 68-year-old man who underwent curative surgery and adjuvant chemotherapy for a locally invasive sigmoid adenocarcinoma, only to be diagnosed with metachronous bilateral adrenal metastasis necessitating further resection and chemotherapy. We discuss the literature surrounding this pathology and highlight the importance of continual, vigilant radiological surveillance of the adrenal glands after curative treatment of colorectal carcinoma with or without subsequent adrenal metastasis.

Colorectal cancer follow-up: patient satisfaction and amenability to telephone after care.

PURPOSE: To explore patient satisfaction on different aspects of follow-up service provision following treatment for colorectal cancer and amenability to an alternative strategy for follow-up care. METHODS AND SAMPLE: A postal survey was administered to 297 eligible patients who had been treated for colorectal cancer at a large hospital in the North West of England. Patients were asked to indicate responses to questions comprising likert scales, including views on organisation of care, information and advice, personal experience of care, satisfaction with information and care, views on specialist nursing services and amenability to telephone follow-up. KEY RESULTS: One hundred and eighty-seven completed surveys were returned (62.97% response rate). Analysis of scale data indicated high levels of satisfaction on all outcome measures but sub-optimal rates of satisfaction on some items. Respondents indicated high levels of satisfaction with information related to disease and treatment but lower levels of satisfaction for items related to genetic risk, sexual attractiveness and self care. Colorectal nurse specialists were highly rated, especially in terms of information provision and personal experience of care. Patients were generally amenable to telephone follow-up, although male patients indicated higher levels of willingness to accept this approach than females. CONCLUSIONS: Satisfaction with traditional medical based follow-up is generally high in this patient cohort but there is room for improvement in terms of service delivery. High levels of satisfaction with the care delivered by colorectal nurse specialists and patient acceptance of telephone follow-up suggests nurse-led telephone follow-up is a viable alternative to traditional hospital based follow-up.

Economic evaluation of a randomized clinical trial of hospital versus telephone follow-up after treatment for breast cancer.

BACKGROUND: This was an economic evaluation of hospital versus telephone follow-up by specialist nurses after treatment for breast cancer. METHODS: A cost minimization analysis was carried out from a National Health Service (NHS) perspective using data from a trial in which 374 women were randomized to telephone or hospital follow-up. Primary analysis compared NHS resource use for routine follow-up over a mean of 24 months. Secondary analyses included patient and carer travel and productivity costs, and NHS and personal social services costs of care in patients with recurrent breast cancer. RESULTS: Patients who had telephone follow-up had approximately 20 per cent more consultations (634 versus 524). The longer duration of telephone consultations and the frequent use of junior medical staff in hospital clinics resulted in higher routine costs for telephone follow-up (mean difference pound 55 (bias-corrected 95 per cent confidence interval (b.c.i.) pound 29 to pound 77)). There were no significant differences in the costs of treating recurrence, but patients who had hospital-based follow-up had significantly higher travel and productivity costs (mean difference pound 47 (95 per cent b.c.i. pound 40 to pound 55)). CONCLUSION: Telephone follow-up for breast cancer may reduce the burden on busy hospital clinics but will not necessarily lead to cost or salary savings.

Preoperative downstaging chemoradiation with concurrent irinotecan and capecitabine in MRI-defined locally advanced rectal cancer: a phase I trial (NWCOG-2).

BACKGROUND: The aim of this study was to investigate the safety of neoadjuvant chemoradiation using radiotherapy (RT) combined with concurrent capecitabine and irinotecan for locally advanced rectal cancer before surgery. METHODS: Forty-six patients were recruited and treated on the basis that MRI scanning had shown poor-risk tumours with threatening (< or =1 mm) or involvement of the mesorectal fascia. Conformal RT was given using 3 or 4 fields at daily fractions of 1.8 Gy on 5 days per week to a total dose of 45 Gy. Concurrently oral capecitabine was given twice daily throughout radiotherapy continuously from days 1 to 35 and intravenous irinotecan was given once per week during weeks 1 to 4 of RT. Dose levels were gradually escalated as follows. Dose level 1: capecitabine 650 mg m(-2) b.i.d. and irinotecan 50 mg m(-2); Dose level 2: capecitabine 650 mg m(-2) b.i.d. and irinotecan 60 mg m(-2); Dose level 3: capecitabine 825 mg m(-2) b.i.d. and irinotecan 60 mg m(2); Dose level 4: capecitabine 825 mg m(-2) b.i.d. and irinotecan 70 mg m(-2). RESULTS: Diarrhoea (grade 3, no grade 4) was the main serious acute toxicity with lesser degrees of fatigue, neutropenia, anorexia and palmar-plantar erythrodysesthesia. The recommended dose for future study was dose level 2 at which 3 of 14 patients (21%) developed grade 3 diarrhoea. Postoperative complications included seven pelvic or wound infections and two anastomotic and two perineal wound dehiscences. There were no deaths in the first 30 days postoperatively. Of 41 resected specimens, 11 (27%) showed a pathological complete response (pCR) and five (12%) showed an involved circumferential resection margin (defined as < or =1 mm). The 3-year disease-free survival (intent-to-treat) was 53.2%. CONCLUSION: In patients with poor-risk MRI-defined locally advanced rectal cancer threatening or involving the mesorectal fascia, preoperative chemoradiation based on RT at 45 Gy in 25 daily fractions over 5 weeks with continuous daily oral capecitabine at 650 mg m(-2) b.i.d. days 1-35 and weekly IV irinotecan at 60 mg m(-2) weeks 1-4, provides acceptable acute toxicity and postoperative morbidity with encouraging response and curative resection rates.

Preoperative neo-adjuvant therapy for curable rectal cancer--reaching a consensus 2008.

OBJECTIVE: Our aim was to determine the range of neo-adjuvant therapy the multidisciplinary team (MDT) currently offers patients with curable (M(0)) rectal cancer. METHOD: A senior oncologist from each of the four oncology centres in north Wales and the north-west of England (approximate target population 8 million - Glan Clwyd, Clatterbridge, Christie and Preston) reviewed his/her understanding of the current evidence of neo-adjuvant therapy in rectal cancer. Then a representative from each centre was asked to identify which of three neo-adjuvant options (no neo-adjuvant therapy, short-course radiotherapy 25 Gy over five fractions and long-course chemoradiotherapy) he/she would use for a rectal cancer in the upper, middle or lower third of the rectum staged by magnetic resonance imaging as being T(2)-T(4) and/or N(0)-N(2). RESULTS: In all cases of locally advanced rectal cancer (T(3a) N(1)-T(4)), oncologists from the four oncology centres recommended long-course chemoradiotherapy before rectal resection. This consensus was maintained for cases of lower third T(3a) N(0) cancers. Thereafter, the majority of patients with rectal cancer are offered adjuvant short-course radiotherapy. CONCLUSION: Neo-adjuvant therapy is less likely to be offered if the tumour is early (T(2), N(0)) and/or situated in the upper third of the rectum.

Irinotecan+5-fluorouracil with concomitant pre-operative radiotherapy in locally advanced non-resectable rectal cancer: a phase I/II study.

In the UK, 10% of patients diagnosed with rectal cancer have inoperable disease at presentation. This study ascertained whether the resectability rate of inoperable locally advanced rectal cancer was improved by administration of intravenous irinotecan, 5-fluorouracil (5-FU) and pelvic radiotherapy. During phase I of the trial (n=12), the dose of irinotecan was escalated in three-patient cohorts from 50 mg m(-2) to 60 mg m(-2) to 70 mg m(-2) to identify the maximum tolerated dose (60 mg m(-2)). In phase II, 31 patients with non-resectable disease received 45 Gy radiotherapy and 5-FU infusions (200 mg m(-2) per day) for 5 weeks. Irinotecan (60 mg m(-2)) was given on days 1, 8, 15 and 22. After treatment, patients were operated on if possible. Thirty patients completed the protocol, 28 underwent surgery. Before surgery, MRI restaging of 24 patients showed that 19 (79%) had a reduction in tumour stage after treatment (seven complete clinical response and 12 partial). Of 27 patients followed up after surgery, 22 (81%) had clear circumferential resection margins. Disease-free and overall survival estimates at 3 years were 65 and 90%, respectively. The regimen was well tolerated. Irinotecan, 5-FU and radiotherapy results in tumour downgrading, allowing resection of previously inoperable tumour with acceptable toxicity.

Extramedullary plasmacytoma of the head and neck region: clinicopathological correlation in 25 cases.

Extramedullary plasmacytomas (EMP) of head and neck are rare tumours. Between 1972 and 1993, 25 cases of EMP of head and neck were seen at our institute. The clinical and pathological features and response to treatment are presented. At initial presentation, 23 (92%) patients presented with disease confined to a single extramedullary site only and two patients had in addition clinical involvement of cervical lymph nodes. All except these two patients received radiotherapy to the primary site only as initial treatment. Initial primary control of local disease was obtained in 16 of 24 (67%) patients treated with radical intent. With salvage treatment of further radiotherapy and/or chemotherapy, local disease control was achieved in 21 of 24 (88%) patients. One patient was treated with palliative intent. Conversion to multiple myeloma was seen in two patients (8%). Pathologically, the tumours were classified into low, intermediate and high grade, which correlated closely with outcome. This classification has been used for the first time in extramedullary plasmacytomas and is based on the multiple myeloma grading criteria devised by Bartl et al (1987). Fifteen of eighteen (83%) low-grade tumours and only one of six (17%) intermediate- and high-grade tumours were locally controlled after primary radiotherapy. This is statistically significant for local control (P= 0.0019) but not for overall survival (P= 0.12). The median survival and 5-year overall survival is 68 months and 58.9% respectively. We recommend consideration of adjuvant chemotherapy in patients with higher grade disease.

Osteosarcoma: experience of the Tata Memorial Hospital, Bombay, India.

This is a report on the management of 210 patients with biopsy-proven OS seen at the Tata Memorial Hospital, Bombay, India from January 1985 to December 1988. The treatment administered to these patients reflects the constraints experienced in cancer management by developing nations. The small number of patients who received neoadjuvant chemoradiotherapy showed the highest survival figures of 30% at 5 years. In the context of the developing countries, limitations of affordability of optimum chemotherapy and the lack of adequate monitoring and support facilities warrant modifications in the currently recommended therapy schedule to suit indigenous needs.

Endobronchial brachytherapy: a preliminary experience.

Fourteen patients with malignant airway obstruction were treated with a placement of a flexible nylon catheter for low dose rate manual afterloading Iridium 192 endobronchial brachytherapy using a flexible fibreoptic bronchoscope. Eight patients had obstructive pneumonitis at initial presentation, while 6 cases were recurrences after previous external irradiation. Six evaluable patients of the former group had complete or partial reinflation of lung and were followed by external radiotherapy. Of the latter group, 3 were evaluable and had moderate to good palliation of their symptoms. No complication was observed. The technique is simple and safe with good patient compliance. Further evaluation is indicated to assess its role in the locoregional management of lung cancer.

Dysgerminoma of the ovary: review of 27 cases.

Twenty-seven patients with pure dysgerminoma were seen at the Tata Memorial Hospital, Bombay, between January 1980 and December 1984. Of the 10 patients in stage I, 2 patients underwent a unilateral salpingoophorectomy (USO) and were kept under observation without any adjuvant therapy, while the remaining patients received adjuvant treatment following surgery. All of the 6 patients in stage II and 4 of 7 in stage III had total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) followed by postoperative radiotherapy. One patient presented in stage IV and 3 patients presented with recurrence after previous definitive treatment undertaken at other institutions. The disease free, as well as the overall survival, at 108 months, for the 24 cases primarily treated at this institution, is 81% and 88% respectively, and, for patients in stages I and II, 100% (Kaplan-Meier estimation). The need for controlled clinical trials to devise optimal therapy in the early clinical stages and use of chemotherapy for advanced stages of this highly curable entity are stressed.

Primary lymphoma of bone: experience of 39 cases at the Tata Memorial Hospital, India.

Primary lymphoma of bone (PLB) is an uncommon clinical entity and a rare presentation of non-Hodgkin's lymphoma. At the Tata Memorial Hospital, over a period of 10 years from 1976 to 1985, 39 cases with a diagnosis of PLB were seen. Twenty-seven cases completed the prescribed treatment and were evaluable for treatment response. Eight patients (21%) presented in clinical stage I (E), four patients (10%) in stage II (E), and 27 patients (69%) in stage IV (E). All of the evaluable patients except two were treated with combination chemotherapy, which consisted of cyclophosphamide, vincristine, and prednisolone in 18 patients, and seven patients received Adriamycin in addition. The majority of patients received six courses of chemotherapy extending over 8 to 12 months. External radiotherapy was given to all except one patient, who had surgery as local treatment. Five patients had generalised relapse, one of which had in addition a local relapse. Five were resistant to treatment. Overall and disease-free survival by Kaplan-Meier method at 60 months are 66% and 56%, respectively.