Neonatal Severe Hyperparathyroidism Causing Life-Threatening Hypercalcemia Treated With Medical and Surgical Management.

A 3-day-old male presented to a peripheral remote hospital in New South Wales, Australia, with tachypnea. He was found to have hypercalcemia, with ionized calcium >2.5 mmol/L (>10 mg/dL) (0.97-1.5 mmol/L or 1.14-1.3 mg/dL) and serum calcium of 3.85 mmol/L (15.43 mg/dL) (2.2-2.8 mmol/L or 8.5-10.5 mg/dL). Peak serum calcium was 5.4 mmol/L (21.64 mg/dL). He was transferred to a tertiary pediatric intensive care unit. Medical management (including hyperhydration, diuretics, corticosteroids, bisphosphonates, cinacalcet, and calcitonin) failed to maintain normocalcemia; therefore, total parathyroidectomy was performed on day 16 of life. Hungry bones syndrome developed postoperatively, requiring high doses of calcium, calcitriol, and phosphate supplementation. Genetic testing identified compound heterozygosity for 2 likely pathogenic variants in the calcium-sensing receptor gene. He is now 3 years old and is growing and developing without any concerns. This case highlights the importance of aggressive initial management in addressing severe hypercalcemia through perioperative management principles as well as the prolonged nature of hungry bones syndrome.

The Utility of the Oncotype DX Test for Breast Cancer Patients in an Australian Multidisciplinary Setting.

Introduction: The Oncotype DX test is a genomic assay that generates a Recurrence Score (RS) predicting the 10-year risk of recurrence and response to adjuvant chemotherapy in ER+/HER2- breast cancer patients. The aims were to determine breast cancer distant recurrence and correlate with adjuvant chemoendocrine prescribing patterns based on the Oncotype DX recurrence score. Methods: We conducted a retrospective single-institution case series of 71 patients who had Oncotype DX assay testing after definitive surgery between 2012 and 2016. Both node-positive and node-negative patients were included. Patients were divided into Oncotype DX low risk (RS < 11) (n = 10, 14%), intermediate risk (RS 11-25) (n = 45, 63%), and high risk (RS > 25) (n = 16, 23%). Median follow-up was 6.1 years (range 4-8.9 years). Adjuvant treatment regimens and oncological outcomes were determined. Results. Mean age at diagnosis was 56 years (range, 33-77). Invasive ductal carcinoma (IDC) accounted for the majority (87%), with most tumors measuring between 10-20 mm (52%). 48% of the cohort were node positive. 15 of 16 high-risk patients (94%) received chemotherapy. 96% of intermediate-risk patients received endocrine therapy alone, one patient received chemoendocrine therapy (2%), and one declined systemic therapy (2%). In the low-risk group, 100% received endocrine therapy only. The high-risk group had the lowest mean ER% (P < 0.05), greatest mean mitotic rate (P < 0.05), and greatest proportion of Ki67% > 14. Five patients developed distant recurrence (7%): three from the intermediate-risk group (7%), one from the low-risk group (10%), and one from the high-risk group (6%). Conclusion: This is the first Australian study reporting the experience with medium-term recurrence outcomes of using the Oncotype DX assay in breast cancer. Chemotherapy was rarely given for patients with low-to-intermediate RS and always offered in high RS. This pattern of prescribing was associated with low rates of distant recurrence. National funding models should be considered.

Myoepithelioma-like tumour of the vulval region: A case report.

Myoepithelioma-like tumours of the vulvar region (MELTVR) are a newly described group of spindle cell neoplasm. Morphologically, they consist of epithelioid to spindled cells in a myxoid to collagenous stroma and can resemble epithelioid sarcomas, myoepithelial carcinomas or extraskeletal myxoid chondrosarcomas. However, they have a distinct pattern of immunohistochemical staining characterised by positivity for EMA, ER, PR and negativity for cytokeratin, GFAP and S100. Nuclear staining for INI-1 is lost. In addition, they lack the characteristic gene arrangements of these other lesions. MELTVR can recur locally when incompletely excised, however, do not appear to metastasize. We report a case of a MELTVR in a 49-year-old woman arising in the right mons pubis.

Correlating Ki67 and other prognostic markers with Oncotype DX recurrence score in early estrogen receptor-positive breast cancer.

AIM: Decisions regarding adjuvant chemotherapy for early breast cancer are complex. Ki67 is increasingly used, in conjunction with conventional prognostic markers, to help decide the use of adjuvant chemotherapy for early breast cancer. Ki67 has been proposed as an economical alternative to Oncotype DX recurrence score (RS), which is a validated prognostic marker for disease recurrence and predictive marker for benefit from chemotherapy. This study aimed to determine in patients where conventional prognostic markers did not provide a clear recommendation for adjuvant chemotherapy, whether Ki67 could be a substitute for RS. METHODS: We reviewed all cases of luminal-type node-negative early breast cancer (T1-2, N0-1mi, M0, estrogen receptor positive, HER2 negative) referred for Oncotype DX testing by the multidisciplinary team at an Australian tertiary private hospital from 14th December 2006 to 31st December 2013, when conventional prognostic markers did not provide a clear recommendation for adjuvant chemotherapy. RS was correlated with Ki67, along with other conventional prognostic markers including tumor size, grade, mitotic rate and lymphovascular invasion. Spearman's rank order correlation coefficient and Pearson product-moment correlation coefficient (r) were used for ordinal and continuous variables, respectively. RESULTS: A total of 58 patients were analyzed, median Ki67 was 15% (range 2-50%) and the median RS was 16 (range 3-65). There was no positive correlation between Ki67 and RS (r = 0.01, P = 0.93). No single conventional prognostic marker was shown to significantly correlate with RS, including tumor size (r = -0.02, P = 0.88), grade (r = 0.10, P = 0.44), mitotic rate (r = -0.07, P = 0.69) and lymphovascular invasion (r = -0.12, P = 0.39). CONCLUSION: Ki67 and conventional prognostic markers do not correlate with Oncotype DX RS. In the setting where conventional prognostic markers do not show a clear indication for or against adjuvant chemotherapy as determined by consensus in a multidisciplinary team, Ki67 is not a substitute for Oncotype DX testing. RS may provide additional information to aid decision making for adjuvant chemotherapy.

Neoadjuvant chemoradiation (modified Eilber protocol) versus adjuvant radiotherapy in the treatment of extremity soft tissue sarcoma.

BACKGROUND: Local control for extremity soft tissue sarcomas (STS) requires surgery combined with radiotherapy, usually given pre-operatively or post-operatively. The modified Eilber protocol, a neoadjuvant chemoradiation regimen, has been reported with excellent local control rates. This retrospective single-centre study compared outcomes for patients treated with the modified Eilber protocol with those treated with standard adjuvant radiotherapy. METHODS: Patients with a diagnosis of extremity STS were retrospectively reviewed from the Prince of Wales Hospital Sarcoma Database from 1995 to 2012. Sixty-three patients underwent curative surgery with either neoadjuvant Eilber chemoradiotherapy (Eilber) or adjuvant radiotherapy (Adjuvant). RESULTS: Twenty-nine patients were treated with modified Eilber protocol. Thirty-four patients received adjuvant radiotherapy. Three patients (10%) in the Eilber group and five patients (15%) in the Adjuvant group developed local recurrence (P = 0.87). Major acute wound complications were noted in four patients in each group (P = 0.55). One patient (3.4%) in the Eilber group developed Grade 3 or 4 late toxicities after 1 year compared with nine patients (27%) in the Adjuvant group (P = 0.02). CONCLUSION: Neoadjuvant chemoradiation (Eilber protocol) provided similar rates of local control when compared with adjuvant radiotherapy. Acute wound complication rates were similar but there was less severe late toxicity in the Eilber group.

Wound infusion with local anaesthesia after laparotomy: a randomized controlled trial.

BACKGROUND: The use of a continuous local anaesthesia infusion after laparotomy may reduce opioid requirements and facilitate earlier return of bowel function, independent mobilization and hospital discharge. METHODS: We performed a double-blinded, randomized controlled trial on 55 patients who underwent laparotomy. Patients were randomly allocated to receive a continuous infusion of either 0.2% ropivacaine or normal saline into their midline abdominal wound at the fascial level. The end points of the study were: total opioid requirements at 24 and 48 h; time to first flatus, bowel movement and independent ambulation; length of hospital stay; complications; and daily mean patient-reported pain scores at rest and movement. RESULTS: The two treatment groups were well controlled for factors that influence analgesia requirements, including age, weight, length of wound incision and type of operation. Patients allocated to ropivacaine infusion used, on average, 32 mg less morphine at 48 h (95% confidence interval 7, 57; P= 0.01). This was highly statistically significant after adjusting for age, gender and type of operation (P= 0.0006). Ropivacaine infusion was associated with a significantly decreased time to independent mobilization (P= 0.02), time to first flatus (P= 0.02) and reduced post-operative ileus (2/28 versus 9/27, χ(2) = 5.89, P= 0.02). There was no significant effect of ropivacaine infusion on time to first bowel movement (P= 0.94) nor length of hospital stay (P= 0.77). CONCLUSIONS: Local anaesthesia infusion at the fascial plane provides effective analgesia. This improves patient recovery through earlier return to bowel function and mobilization.

Outcomes of appendicectomy in an acute care surgery model.

OBJECTIVE: To assess the outcomes of appendicectomy in an acute care surgery (ACS) model compared with a traditional on-call (Trad) model. DESIGN: Retrospective historical control study comparing appendicectomy outcomes in the Trad period (April 2004 to March 2005) with outcomes in the ACS period (April 2006 to March 2007). SETTING: The Prince of Wales Public Hospital, a metropolitan tertiary referral centre in Sydney. PATIENTS: All adult patients undergoing appendicectomy during 1-year periods before and after the introduction of the ACS model. INTERVENTION: The introduction of an ACS model for managing all emergency general surgical presentations. MAIN OUTCOME MEASURE: Complication rate. RESULTS: A total of 402 appendicectomies were performed, 176 during the Trad period and 226 during the ACS period. There was no perioperative mortality. The complication rate was lower in the ACS period than the Trad period (9.3% v 17.0%; P = 0.02). After the intervention, there was no significant change in the time from presentation to arrival in theatre or in length of stay, but the proportion of operations performed at night (24:00-08:00) was reduced from 26.1% to 15.0% (P = 0.006). The proportion of negative appendicectomies was reduced from 22.7% to 17.3%, but the change was not statistically significant (P = 0.08). There was no difference in perforation rate before and after the intervention (13.6% v 13.3%; P = 0.86). CONCLUSION: The ACS model provides a safe surgical environment for patients and is associated with a reduced complication rate. Under the ACS model, there was an increase in the number of patients treated conservatively overnight, but this did not lead to an overall increase in perforation rate or length of stay.

Acute-care surgical service: a change in culture.

The provision of acute surgical care in the public sector is becoming increasingly difficult because of limitation of resources and the unpredictability of access to theatres during the working day. An acute-care surgical service was developed at the Prince of Wales Hospital to provide acute surgery in a more timely and efficient manner. A roster of eight general surgeons provided on-site service from 08.00 to 18.00 hours Monday to Friday and on-call service in after-hours for a 79-week period. An acute-care ward of four beds and an operating theatre were placed under the control of the rostered acute-care surgeon (ACS). At the end of each ACS roster period all patients whose treatment was undefined or incomplete were handed over to the next rostered ACS. Patient data and theatre utilization data were prospectively collected and compared to the preceding 52-week period. Emergency theatre utilization during the day increased from 57 to 69%. There was a 11% reduction in acute-care operating after hours and 26% fewer emergency cases were handled between midnight and 08.00 hours. There was more efficient use of the entire theatre block, suggesting a significant cultural change. Staff satisfaction was high. On-site consultant-driven surgical leadership has provided significant positive change to the provision of acute surgical care in our institution. The paradigm shift in acute surgical care has improved patient and theatre management and stimulated a cultural change of efficiency.