Extraovarian Fibroma with Minor Sex Cord Elements-a Rare Tumour at Rare Site.

Sex cord stromal tumour (SCST) of ovary account for 5-8% of all ovarian malignancies. These include fibroma, thecoma, fibro-thecoma, Leydig cell tumour, granulosa cell tumour, and Sertoli cell tumour. Although considered primary ovarian malignancy, SCST has been described in extraovarian location also. Extraovarian SCST is a rare occurrence, and so far, only 25 cases have been reported in literature and only one amongst them was extraovarian fibroma with minor sex cord element. Considering the rarity, diagnosis and proper treatment remain the real challenge in these tumours. To the best of our knowledge, this is the second case of extraovarian fibroma with minor sex cord element to be reported. Herein, we report a case of 41-year-old lady who presented with large abdomino-pelvic mass which was considered ovarian tumour; however, intraoperatively both ovaries and uterus were found to be normal, and the mass was actually arising from sigmoid mesocolon.

Heterotopic Pancreas: Unusual Cause of Usual Diarrhoea.

Heterotopic pancreas (HP) also known as ectopic pancreas, pancreatic crest or accessory pancreas is the normal pancreatic tissue, found in a remote area other than its natural location, with no anatomic or vascular connection to main pancreatic tissue. It is a rare congenital anomaly and has been reported at many locations such as stomach (antrum) and small intestine. HP is usually an incidental finding and asymptomatic, however there are reports of pancreatitis, obstruction, perforation and malignant transformation as uncommon manifestations. Diagnosis of HP is primarily based on histological examination either by biopsy or surgical excision. Surgery is the standard treatment for symptomatic HP patients. Herein, we present a case of a 58-year-old female, who presented to us with intractable diarrhoea due to HP in the jejunum and underwent minimally invasive surgery for definitive diagnosis and treatment.

Gastric Schwannoma-Two Unusual Cases with Usual Presentation and Review of Literature.

Schwannomas, also known as neurilemmomas, are characteristically benign, slow growing neoplasms originating from Schwann cell sheath. Gastrointestinal schwannomas are very rare with stomach being the most affected organ representing approximately 0.2% of all gastric tumours. Malignant transformation is rarely reported in these tumors and complete surgical resection with negative margins is the recommended treatment. Herein, we report our experience of 2 cases of gastric submucosal spindle cell tumour (SMT), which were preoperatively assumed to be as gastrointestinal stromal tumor (GIST), however postoperatively confirmed as schwannoma on immunohistochemical examination.

Preoperative Albumin-Globulin Ratio and Its Association with Perioperative and Long-Term Outcomes in Patients Undergoing Pancreatoduodenectomy.

BACKGROUND: Systemic inflammatory response is involved in natural progression of cancers by different pathways. Albumin-globulin ratio (AGR) has been reported to have impact on prognosis in various solid tumors. OBJECTIVE: To study the significance of AGR on perioperative and long-term outcomes in patients undergoing PD. METHODS: This is a post hoc analysis of the pancreatic surgery database from January 2012 to March 2017. Cutoff value for AGR was calculated by using the receiver operating curve, and the study cohort was divided into group I (AGR ≥1) and group II (AGR <1). Two groups were compared for perioperative and long-term survival outcomes. RESULTS: Two groups were comparable with respect to clinicodemographic variables. Groups I and II had similar perioperative outcomes (p > 0.05) like median hospital stay (14 vs. 15 days), clinically relevant postoperative pancreatic fistula (16.6 vs. 15.7%), hemorrhage (3.1 vs. 2.6%), bile leak (1.4 vs. 0.65%), overall morbidity (30.1 vs. 28.9%), and postoperative mortality (2.7 vs. 3.9%). With a median follow-up of 3 years, median survival, overall survival, and disease-free survival were similar in both groups. CONCLUSION: AGR at the cutoff value of ≥1 was not associated with adverse perioperative and long-term oncological outcomes after PD.

Minimally Invasive Surgery for Pelvic Exenteration in Primary Colorectal Cancer.

BACKGROUND: Minimally invasive surgery (MIS) for pelvic exenteration is not a well-established technique. The aim was to assess the safety and feasibility of MIS for pelvic exenteration in locally advanced primary colorectal cancer and to compare the perioperative outcomes with open surgery. METHODS: This is a retrospective analysis of patients, who had undergone pelvic exenteration for primary colorectal adenocarcinoma from May 2013 to July 2018. The short-term outcomes like perioperative details and histopathological characteristics were compared between the two groups. RESULTS: MIS was performed in 23 patients and open pelvic exenteration was carried out in 72 patients. The mean operative time was significantly more in the MIS group (640 vs. 432 min, p = 0.00). The intraoperative blood loss (900 vs. 1550 ml, p = 0.00) and the requirement for blood transfusion (170 vs. 250 ml, p = 0.03) was significantly less in the MIS group. The overall morbidity (60% vs. 49%, p = 0.306) was comparable between the two groups. The median length of hospital stay in the MIS group was 11 d, compared to 12 d in the open surgery group, (p = 0.634). The rate of R0 resection (87% vs. 89%, p = 0.668) was comparable between the two groups. CONCLUSION: MIS is feasible and safe for total pelvic exenteration and posterior exenteration in carefully selected locally advanced primary colorectal cancer, when performed by an experienced surgical team in high volume centers. An R0 resection with adequate margin can be achieved with good perioperative outcomes in MIS. Long-term oncological outcomes would require further follow up to confirm.

Retroperitoneal Sarcomas: Prognostic Factors and Outcomes of a Series of Patients Treated at a Single Institution.

Retroperitoneal sarcomas (RPS) are rare heterogeneous tumors arising in the retroperitoneum with unique biological and behavioral patterns that are thought to be closely linked to histology. The aim of the study was to audit our results and analyze various clinico-pathological factors including surgical excision, histology, and their implications on the recurrences and survival outcomes in RPS. Retrospective analysis of patients treated at a tertiary referral center in India from March 2008 to July 2017 was performed. The clinico-pathological variables were analyzed for their association with tumor recurrence and survival with special emphasis on histological subtype. The primary outcome was overall survival (OS). One hundred consecutive patients operated for RPS were analyzed. Of these, 27 were operated for recurrent tumors. Liposarcomas (LPS) and leiomyosarcomas (LMS) constituted 50% (n = 50) and 30% (n = 30) of patients respectively. Complete tumor excision was achieved in 83%, with 43% patients undergoing adjacent organ resection. At a median follow-up of 25.3 months, the median disease-free survival (DFS) and overall survival (OS) were 30 months and 87.8 months respectively. On multivariate analysis, tumor grade was the only factor to significantly affect survival (p = 0.001 for DFS and 0.005 for OS). There was no difference in survival outcomes between infiltrative and adhesive tumors with respect to adjacent organ invasion (p = 0.361 for OS). Tumor grade remains an important prognostic factor affecting disease-free and overall survival in retroperitoneal sarcomas irrespective of tumor size, site, and histology.

To Do or Not to Do?-A Review of Cancer Surgery Triage Guidelines in COVID-19 Pandemic.

COVID-19 pandemic has emerged as a global health emergency involving more than 200 countries so far. The number of affected population is on rising, so is the mortality. This crisis has overwhelmed the healthcare infrastructures in many affected countries. Due to overall rising cancer incidence and specific concerns, a cohort of cancer patients forms a distinct subset of the population in whom a correct and timely treatment has a huge impact on the outcome. During this period, oncology care is definitely affected owing to many factors like lockdowns, reduced beds and deferral of elective cases to halt the spread of the pandemic. Surgery remains the best line of defence in many solid organ tumours especially in early stage and is potentially curative. China, the source of this pandemic, has taken more than 3 months to enter the post transitional phase of this pandemic. Deferring cancer surgeries for this long period may have a direct impact on the long-term outcomes of cancer patients. Many surgical oncology associations across the globe have come up with triage guidelines for surgical care of cancer patients; however, these are based on expert opinion rather than actual data. Herein, we intend to review these guidelines with respect to the risk of disease progression in cancer patients. In the absence of actual data on cancer surgery care during this pandemic, clinical decisions should be based on careful consideration of disease-related and patient-related factors. While some of the cancer surgeries can be safely delayed for some time, how long we can delay surgeries safely cannot be answered/ explained by any means. Thorough evaluation and discussion by an expert and experienced multidisciplinary team appears to be the most effective way forward.

Correction to: Twelve Hundred Consecutive Pancreato-Duodenectomies from Single Centre: Impact of Centre of Excellence on Pancreatic Cancer Surgery Across India.

The authors' affiliations are updated as reflected here.

External validation and comparison of the original, alternative and updated-alternative fistula risk scores for the prediction of postoperative pancreatic fistula after pancreatoduodenectomy.

BACKGROUND: Many postoperative pancreatic fistula (POPF) predictions models were developed and validated in western populations. Direct use of these models in the large Indian/Asian population, however, requires proper validation. OBJECTIVE: To validate the original, alternative and updated alternative fistula risk score (FRS) models. METHODS: A validation study was performed in consecutive patients undergoing pancreatoduodenectomy (PD) from January 2011 to March 2018. The area under the receiver operating curve (ROC) and calibration plots were used to assess the performance of original-FRS (o-FRS), alternative FRS (a-FRS) and updated alternative FRS (ua-FRS) models. RESULTS: This cohort consisted of 825 patients of which 66% were males with a median age of 55 years and mean body mass index of 22.6. The majority of tumors (61.8%) were of periampullary origin. Clinically relevant POPF was observed in 16.8% patients. Area under curve (AUC) of ROC for the o-FRS was 0.65, 0.69 for a-FRS and 0.70 for ua-FRS, respectively (p = 0.006). CONCLUSIONS: In this large Indian cohort of predominantly periampullary tumors, the ua-FRS performed better than the a-FRS and o-FRS, although differences were small. Since the AUC value of the ua-FRS is at the accepted threshold there might be room for improvement for a FRS.

Centralisation of Pancreatoduodenectomy in India: Where Do We Stand?

BACKGROUND: The volume-outcome relationship dictates that high-volume centres lead to improved patient outcomes after pancreatoduodenectomy (PD). We conducted a retrospective review to fathom the situation in India for PD and whether referral to high-volume centres would make a positive impact. METHOD: A systematic literature search in MEDLINE was performed, and all articles published from Indian centres from 01.03.2008 to 30.11.2019 were scrutinised. Any series with less than 20 patients, case reports, abstracts, unpublished data and personal communications were excluded. RESULTS: A total of 36 unique series including 6226 patients from 24 institutes across India were identified. Amongst the 24 institutes, 2 institutes reported less than 10 cases/year, 11 reported 10-25 cases/year and 11 reported ≥26 cases/year. Overall perioperative morbidity was 42.4%, 43.4% and 41% for centres doing <10, 10-25 and ≥26 cases/year, respectively. Operative mortality also improved with increasing number of cases/year (5.1% vs. 6.6% vs. 3.2%, respectively). CONCLUSION: With increasing volume of cases per year, trend towards improved PD outcomes is observed. To optimise the use of healthcare facilities, it would be pragmatic to consider building an organised referral system for complex surgeries to deliver unsurpassed patient care with maximum utilisation of the available healthcare infrastructure.

Twelve Hundred Consecutive Pancreato-Duodenectomies from Single Centre: Impact of Centre of Excellence on Pancreatic Cancer Surgery Across India.

BACKGROUND: Pancreato-duodenectomy (PD) is a technically challenging operation with significant morbidity and mortality. Over the period of time, Tata Memorial Centre has evolved into a high-volume centre for management of pancreatic cancer. Aim of this study is to report the short- and long-term outcomes of 1200 consecutive PDs performed at single tertiary cancer centre in India. METHODS: 1200 PDs were performed from 1992 to 2017. Prospectively maintained database was used to retrospectively assess the short- and long-term outcomes. RESULTS: Study cohort was divided into periods A and B (500 and 700 patients, respectively). Both groups were comparable for demographic variables. Overall morbidity and mortality in entire cohort were 31.2% and 3.9%, respectively. Period B documented significant reduction in post-operative mortality (5.4% vs 2.8%), post-pancreatectomy haemorrhage (5.8% vs 3%) and bile leaks (3.4% vs 1.3%). However, incidence of delayed gastric emptying and clinically relevant post-operative pancreatic fistula was higher in period B. With median follow-up of 25 months, 3-year overall survival and disease-free survival for patients with pancreatic cancer were 43.7% and 38.7%, respectively, and that for periampullary tumours were 65.9% and 59.4%, respectively. Period B also corresponded with dissemination of technical expertise across diverse regions of India with specialised training of 35 surgeons. CONCLUSION: Our study demonstrates the feasibility of delivering high-quality care in a dedicated high-volume centre even in a country with low incidence of pancreatic cancer with marked disparities in medical care and socio-economic conditions. Improved outcomes underscore the need to promote regionalisation via a dedicated training programme.

Targeting the Somatostatin Receptor 2 with the Miniaturized Drug Conjugate, PEN-221: A Potent and Novel Therapeutic for the Treatment of Small Cell Lung Cancer.

Small cell lung cancer (SCLC) is an aggressive neuroendocrine carcinoma with a 95% mortality rate with no improvement to treatment in decades, and new therapies are desperately needed. PEN-221 is a miniaturized peptide-drug conjugate (∼2 kDa) designed to target SCLC via a Somatostatin Receptor 2 (SSTR2)-targeting ligand and to overcome the high proliferation rate characteristic of this disease by using the potent cytotoxic payload, DM1. SSTR2 is an ideal target for a drug conjugate, as it is overexpressed in SCLC with limited normal tissue expression. In vitro, PEN-221 treatment of SSTR2-positive cells resulted in PEN-221 internalization and receptor-dependent inhibition of cellular proliferation. In vivo, PEN-221 exhibited rapid accumulation in SSTR2-positive SCLC xenograft tumors with quick clearance from plasma. Tumor accumulation was sustained, resulting in durable pharmacodynamic changes throughout the tumor, as evidenced by increases in the mitotic marker of G2-M arrest, phosphohistone H3, and increases in the apoptotic marker, cleaved caspase-3. PEN-221 treatment resulted in significant antitumor activity, including complete regressions in SSTR2-positive SCLC xenograft mouse models. Treatment was effective using a variety of dosing schedules and at doses below the MTD, suggesting flexibility of dosing schedule and potential for a large therapeutic window in the clinic. The unique attributes of the miniaturized drug conjugate allowed for deep tumor penetration and limited plasma exposure that may enable long-term dosing, resulting in durable tumor control. Collectively, these data suggest potential for antitumor activity of PEN-221 in patients with SSTR2-positive SCLC.

Cutting-edge strategies for borderline resectable pancreatic cancer.

Worldwide, pancreatic ductal adenocarcinoma (PDAC) accounts for more than 400 000 deaths every year, being the 12th most common cancer and the seventh most frequent cause of death from cancer. Regardless of the advances in diagnosis and treatment, PDAC continues to have dismal outcomes and fewer than 25% of patients survive for 1 year. In the absence of metastatic disease, radical surgery remains the most important factor for improving survival and possibly offer cure. However, approximately 80% of patients cannot be offered surgery owing to locally advanced or metastatic disease at presentation. At presentation, only 10%-20% patients are eligible for resection, 30%-40% are unresectable/locally advanced and 50%-60% are metastatic. One promising development in recent years has been the inclusion of a new subgroup within the locally advanced tumors of borderline resectable pancreatic cancer (BRPC) comprising approximately 5%-10% of the total patient population. Although its exact definition has been refined over the past few years depending on the vascular involvement around the tumor, the term was initially proposed for tumors that are at a high risk of having margin positivity after resection. Various treatment approaches are still evolving for this entity. Herein, we reviewed the current status of different treatment modalities for BRPC.

Bladder cancer demographics and outcome data from 2013 at a tertiary cancer hospital in India.

BACKGROUND: Bladder cancer (BCa) is the ninth most common cancer accounting for 3.9% of all cancer cases as per the Indian Cancer Registry data. There is a scarcity of data on urinary Bca from India. AIM: The aim of this study was to know demographic background, stage distribution, utilization of various treatment modalities, and oncological outcome in Indian patients presenting with bladder cancer to a tertiary care cancer center in Mumbai. METHODOLOGY: We performed a retrospective audit of all patients registered as urinary BCa in our hospital from January 1, 2013 to December 31, 2013. Electronic medical records of these patients were checked for most of the information gathered. RESULTS: Median age of patients at presentation was 59 years with a range of 18-88 years. There were 84% male and 16% female patients. Forty seven percent of patients had nonmuscle invasive bladder cancer (NMIBC), 36% had muscle invasive bladder cancer and locally advanced disease, and 17% had metastatic disease. Eight patients were treated with trimodality bladder preservation protocol. Recurrence was seen in 38 (22.6%) patients with NMIBC. Out of them. 44.7% and 55.3% were in low- and high-grade tumors, respectively. Overall survival and disease-free survival estimated for 3 years were 63% and 57%, respectively. CONCLUSION: Bladder cancer has a varied spectrum of presentation. Bladder cancer patients presenting to our hospital generally have a higher stage and grade of disease compared with that in the west.