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The best management of patients who have unresectable mucinous appendiceal cancer (MAC) with peritoneal spread after a failed attempt at cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is unclear. This study aimed to assess outcomes after systemic chemotherapy (SCT) for patients with unresectable peritoneal metastases from high-grade MAC.A single-center retrospective cohort study was conducted using a prospective CRS/HIPEC database. The study included high-grade MAC patients with peritoneal carcinomatosis who were deemed surgical candidates, but had an aborted CRS/HIPEC or only palliative HIPEC due to unresectable disease. Overall survival (OS) was compared.Of 72 identified patients, 20 received SCT and 52 did not (NoCT). The groups were balanced by age (p = 0.299), sex (p = 0.930), histopathologic subtype (p = 0.096), preoperative chemotherapy (p = 0.981), and postoperative major complication rates (p = 0.338). Both groups had extensive disease (median peritoneal cancer index at exploration, 39 vs 39). The median number of cycles was 12 (interquartile range [IQR], 6-15), and the median time between the procedure and SCT was 7 weeks (IQR, 5-10 weeks). The median follow-up period was 65 months. The median OS was significantly higher for the SCT group (26 months; 95 % confidence interval [CI], 10.8-41.5 months) than for the NoCT group (12 months; 95 % CI, 9.6-14.4 months) (p0.001), with hazard ratio (HR) of 0.22 (95 % CI, 0.08-0.66; p = 0.007) after adjustment for other factors.Systemic chemotherapy is associated with improved OS for high-grade MAC patients with unresectable peritoneal metastases who are deemed surgical candidates but underwent an unsuccessful CRS/HIPEC attempt. Further prospective studies with a larger sample are required to identify patient subgroups who benefit the most from SCT.

PURPOSE The 2-year Russian oncology residency focuses on diagnosis and treatment of malignancies but lacks evidence-based medicine (EBM) and patient communication skills (PCS) training. To overcome these educational disparities, the 5-year national program, the Higher School of Oncology (HSO), involving Russian expatriate physicians trained in the United States was established. METHODS A retrospective study was conducted. Highly motivated oncology residents were enrolled in the program through the three-step selection process. US-trained Russian expatriate physicians acted as mentors. EBM skills were taught through weekly online journal clubs and clinical case presentations. PCS training included live seminars and simulations after journal clubs. EBM knowledge was assessed using Fresno test among newly enrolled and postgraduate year (PGY) 2-5 HSO residents. PCS were evaluated via simulation exam including two clinical scenarios (maximum score 100 each) among 17 PGY2 HSO residents and seven non-HSO trainees. RESULTS Overall, 54 residents were enrolled over 5 years (8-13 annually); four were released from the program. The mean age was 24 ± 1 years, and 56% were females. Median scores of Fresno test were significantly higher among PGY 2-4 HSO residents compared with newly enrolled participants: 111 (IQR, 71-128) versus 68 (IQR, 42-84), P = .042; moreover, performance correlated with year of program participation (rs = 0.5; P < .0001). PCS assessment score was significantly higher among HSO residents than non-HSO trainees: 71 (IQR, 58-84) versus 15 (IQR, 10-30) for scenario number 1 ( P < .0001) and 78 (IQR, 71-85) versus 22 (IQR, 4-58) for scenario number 2 ( P = .005), respectively. CONCLUSION The involvement of Western-trained expatriates in remote education improves EBM and PCS among oncology trainees from their home country. This strategy can be useful in overcoming global medical education disparities in other specialties and in countries facing similar challenges.

Diaphragmatic resection (DR) is often required during cytoreductive surgery/hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) to achieve complete cytoreduction (CC). While CC provides the best survival, requiring a DR may indicate unfavorable tumor biology. We assessed how DR during CRS/HIPEC affects outcomes. A retrospective cohort study was conducted using a prospective single-center database from October 1994–May 2020. Peritoneal surface malignancy patients who underwent CRS/HIPEC with CC-0/1/2 were assigned to DR and NoDR groups. Survival was measured using the Kaplan–Meier method. Subgroup analysis was performed for patients with peritoneal cancer index (PCI) ≥ 20 to eliminate confounding of more extensive disease in DR. Of 824 CRS/HIPECs, 774 were included: 134 DR and 640 NoDR. PCI was significantly higher in DR: 29 versus 21, p < 0.001. CC-0/1 rate was 89% in DR and 95% in NoDR (p = 0.003). Neither 100-day morbidity nor mortality differed between the groups (p = 0.355 and p = 1.000). Median follow-up was 64 months. Median overall survival (OS) was significantly lower in DR (32 vs. 96 months, p < 0.001). Subgroup analysis by tumor type in patients with PCI ≥ 20 showed significantly shorter OS in DR than NoDR in appendiceal (40 vs. 196 months, p < 0.001) and colorectal (14 vs. 23 months, p = 0.003), but not in ovarian tumors (32 vs. 42 months, p = 0.893), whereas median PCI did not differ among subgroups. DR during CRS/HIPEC does not increase morbidity and mortality. It is associated with worse survival in appendiceal and colorectal tumors, even after adjusting for tumor burden but does not appear to impact ovarian cancer survival.

Introduction Genital necrosis (GN) is a rare complication of cytoreductive surgery with hyperthermic intraoperative chemotherapy (CRS/HIPEC) which can be confused with necrotizing fasciitis. We present an analysis of GN after CRS/HIPEC to define its natural history. Methods We identified patients with GN after CRS/HIPEC at two peritoneal surface malignancy institutions. Patient demographic, surgical, and postoperative data were extracted from prospective databases. Results Of 1597 CRS/HIPECs performed, 13 patients (0.8%) had GN. The median age was 57 years (IQR: 49–64) and 77% (n = 10) were male. Mitomycin-C was the perfusion agent in all cases of GN (100%). The median time to GN onset after CRS/HIPEC was 64 days (IQR: 60–108) and 2 (15%) patients were receiving systemic chemotherapy at the time of GN onset. Symptoms included severe pain (100%), edema (100%), labial or scrotal skin ulceration (92%), signs of infection (39%), and fever (15%). Seven (54%) patients had thrombocytosis >400 ∗109/L, whereas coagulation tests were within normal reference range in 100% cases. All patients initially underwent conservative treatment, with antibiotic therapy administered in 62% (n = 8). Surgical debridement was performed in 9 (70%) cases with median time after GN onset of 57 (IQR: 8–180). Conclusion GN is a debilitating complication after CRS/HIPEC with delayed onset and a protracted clinical course. Optimal treatment results could be achieved with initial conservative management until complete lesion demarcation followed by surgical debridement. The pathophysiology of GN is unclear, and we call for other researchers attention to better understand the complication and prevention.

Surgeons may hesitate to perform nephrectomy (NE) during cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) due to a potential increase in morbidity. However, no data are available regarding the impact of NE on outcomes, so the authors decided to assess its safety during CRS/HIPEC.A single-center propensity score-matched study was conducted using a prospective database (1994-2021). The study included patients who underwent NE during CRS/HIPEC with completeness of cytoreduction (CC) of 0, 1, or 2. Control subjects (no-NE) were selected in a 1:3 ratio using propensity score-matching weighted by age, histology, peritoneal cancer index (PCI), CC-0 or CC-1 rate, and length of surgery.Among 828 patients, 13 NE and 39 no-NE control subjects were identified. The indications for NE included tumor involvement of the ureter, hilum, and/or kidney with preserved (n = 8), decreased (n = 2), or absent (n = 3) function. NE patients received more intraoperative intravenous (IV) fluids (16,000 vs 11,500 mL; p = 0.045) and had a greater urine output (3200 vs 1913 mL; p = 0.008). NE patients received mitomycin C (40 mg for 90 min) or melphalan (50 mg/mNephrectomy performed during CRS/HIPEC does not seem to increase postoperative morbidity or to delay adjuvant chemotherapy, and NE can be performed if required for complete cytoreduction. The NE patients in our cohort did not have a reduction of mitomycin C or melphalan dose or perfusion time.

During cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), surgeons are reluctant to perform unprotected pelvic anastomosis despite lack of supporting data. We analyzed pelvic anastomosis outcomes and factors that influence ostomy creation in CRS/HIPEC patients. A single-center, descriptive study of patients with rectal resection during CRS/HIPEC was conducted using a prospective database. Surgical variables were reviewed. Multinomial logistic regression of outcomes (end or protective ostomy) was performed with pre- and intraoperative factors as predictors. Overall, 274 of 789 CRS/HIPEC patients underwent rectal resection, including 243 (89%) with pelvic anastomosis [232 (85%) without ostomy, 11 (4%) with protective ileostomy] and 31 (11%) with no anastomosis [16 (6%) with end colostomy, 15 (5%) with end ileostomy]. The median age was 57 and 29% (79) were male. Of 243 pelvic anastomosis patients, 3 (1.2%) had rectal anastomotic leaks, including 1 with a protective ileostomy. Other anastomotic leaks occurred in 3.6%. Overall, 13% had Clavien-Dindo complications ≥ IIIB and the readmission rate was 30%. Mortality at 30 days and 100 days was 0.4% and 2.2%, respectively. Male gender and primary rectal cancer were associated with protective ileostomy [odds ratio (OR) = 7.01, 95% CI: 1.6–31.5, p = 0.011, and OR = 16.4, 95% CI: 3–88.4, p = 0.001, respectively). Male gender and prior pelvic surgery were associated with end colostomy (OR = 13.9, 95% CI: 3.7–53, p

Low-grade appendiceal mucinous neoplasms (LAMN) are tumors that frequently present with peritoneal spread of either acellular mucin (AM) or cellular mucin (CM). We aim to determine how mucin types and distribution affect survival. PATIENTS AND METHODS: A retrospective cohort study was conducted using a prospective database. Newly diagnosed LAMN patients with AM versus CM treated with cytoreductive surgery/hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) were compared. Postoperative pathology reports were reviewed to assess each involved abdominal zone. Survival was analyzed using the Kaplan-Meier method.Of 121 identified patients, 50 (41%) had peritoneal lesions with AM and 71 (59%) with CM. Peritoneal cancer index was lower in AM versus CM (mean: 19 ± 13 vs 28 ± 10, p = 0.004), but complete cytoreduction (CC) rates were similar (98% vs 96%, p = 0.642). The 5-year progression-free survival (PFS) was higher in AM versus CM (96% vs 69.8%, p = 0.002). CM patients had zones with both types of lesions: with and without cells. The CM subgroup analysis showed significant differences in 5-year progression-free survival (PFS) among patients with 1-3, 4-7, and 8-10 zones with cells (95.2%, 68.4%, and 35.7%, respectively, p 0.001), but PFS was not affected by the number of zones with any lesion type. There was no difference in overall survival (OS) between groups.Despite comparable CC rates after CRS/HIPEC, CM patients have shorter PFS than AM patients. In CM patients, more zones with cells, but not the total number of involved zones, negatively impact PFS. Mucin type does not impact OS. It is important to assess and report mucin cellularity in LAMN specimens.

Despite meticulous preoperative diagnostics, aborted hyperthermic intraperitoneal chemotherapy (AHIPEC) is a common, unsuccessful outcome of curative cytoreductive surgery (CRS)/HIPEC. The aim of this study was to evaluate the association between AHIPEC and preoperative risk factors of patients with mucinous appendiceal cancer (AC). A single-institute, case–control study was conducted using a prospective database. Potentially resectable patients with peritoneal carcinomatosis of mucinous AC origin with AHIPEC between October 1994 and February 2019 were identified. Preoperative risk factors were reviewed. Analysis was conducted by tumor grade: low-grade, high-grade, and signet ring cell carcinoma (high-S). All available tumor-type-matched successful CRS/HIPEC controls were obtained from the same database. Univariable and multivariable analyses were performed. Overall, 21, 44, and 15 AHIPEC cases and 153, 133, and 53 tumor-matched controls were identified for low-grade, high-grade, and high-S populations, respectively. Multivariable analysis revealed preoperative cancer antigen (CA) 19-9 > 1 upper limit of normal (ULN) [odds ratio (OR) 6.32; p = 0.014], CA125 > 2 ULN (OR 7.02; p = 0.039), C-reactive protein (CRP) > 2.5 mg/L (OR 13.7; p = 0.001), and previous HIPEC (OR 7.5; p = 0.031) were significantly associated with AHIPEC in the low-grade population. Preoperative CRP > 2.5 mg/L (OR 7.77; p

Soft-tissue myoepithelioma (STM) is an extremely rare benign tumor with predominant occurrence in head, neck, pelvic girdle and limbs. These tumors lack specific clinical and morphological features and can easily be confused with more common neoplasms. It may lead to incorrect diagnosis and management. Here, we present a clinical case of a young man with retroperitoneal STM which simulated a pancreatic tumor and required a distal pancreatectomy. Performed literature review highlights current data about clinical, morphologic, immunohistochemical and genetic evaluation, treatment and prognosis of STM. J Med Cases. 2020;11(1):16-21 doi: https://doi.org/10.14740/jmc3407

Peritoneal surface malignancies (PSM) can disseminate into the pleural cavity, increasing morbidity and mortality. While cytoreductive surgery with hyperthermic intraperitoneal chemoperfusion (CRS/HIPEC) improves outcomes for PSM with intra-abdominal spread, the optimal approach for patients with pleural dissemination from PSM remains unclear. It seems reasonable to apply peritoneal carcinomatosis management principles to patients with pleural lesions using CRS and hyperthermic intrathoracic chemotherapy (HITHOC). We conducted a descriptive study to evaluate outcomes of PSM patients who underwent CRS/HITHOC for pleural dissemination using a high-volume PSM center’s prospective database from October 1994–June 2020. CRS/HITHOC was performed via either diaphragmatic window during CRS/HIPEC (CRS/HIPEC+HITHOC) or thoracotomy as a separate procedure (CRS/HITHOC). Of 852 completed CRS/HIPECs, 18 HITHOCs in 15 patients were identified: 10 CRS/HIPEC+HITHOCs, and 8 CRS/HITHOCs. CRS/HIPEC+HITHOC primary tumors included: 4 appendix, 4 ovary, 1 colon, and 1 unknown. All (n = 8) CRS/HITHOC patients had recurrent appendiceal neoplasms. Complete cytoreduction was achieved in 90% of CRS/HIPEC+HITHOCs and 75% of CRS/HITHOCs. Major complications occurred in 20% of CRS/HIPEC+HITHOCs and 13% of CRS/HITHOCs with no 30-day mortality in either group. After median follow-up of 22 months, overall survival at 1, 3, and 5 years was 93.3%, 67.9%, and 67.9%, while 1-, 3-, and 5-year progression-free survival was 70.9%, 20.3%, and 20.3%. Intrapleural recurrence occurred in 1 CRS/HIPEC+HITHOC and 2 CRS/HITHOC patients. CRS/HITHOC performed via diaphragm or thoracotomy at high-volume centers is a safe option for PSM with pleural dissemination. Further comparative studies with longer follow-up are needed to evaluate survival by tumor type.

The authors have requested that this preprint be removed from Research Square.

Background. Clinical decisions in patients with peritoneal dissemination of low-grade appendiceal mucinous neoplasms (LAMN) diagnosed during pregnancy are challenging. However, their slow progression and favorable prognosis allow deferring definitive treatment until after spontaneous delivery, a reasonable period of breastfeeding, and fertility preservation. Case Presentation. Two pregnant patients were incidentally diagnosed with LAMN and extensive peritoneal spread at 20 weeks gestation and at cesarean section. Treatment with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in both cases was delayed until spontaneous delivery at term and breastfeeding in the first patient and breastfeeding and fertility preservation in the second patient. Both patients remain disease-free for over 5 years, and their children are healthy. The literature review highlights the challenges that physicians face in treating pregnant patients with stage IV appendiceal tumors. Conclusion. Pregnancy management decisions in patients with peritoneal spread from mucinous appendiceal tumor should be based on understanding the tumor biology and prognosis. Definitive treatment in pregnant patients with favorable tumors, such as LAMN, may be delayed until spontaneous delivery without compromising maternal survival.

10512 Background: Disparities in current global oncology care are a major concern for many societies. Two-year oncological training in Russia is deficient in evidence-based medicine (EBM) and effective patient communication skills, despite the fact that these dictate the ability to provide optimal cancer care. We report a 3.5-year sustained program aimed at implementing Western-oriented education among young Russian oncologists to combat these educational deficiencies. Methods: The Higher School of Oncology (HSO) is a 5-year national competitive program established to supplement the traditional 2-year Russian oncology curriculum with an emphasis on patient communication, critical appraisal of oncologic articles, multidisciplinary cancer care, and program development. A total of 35 PGY1-4 residents (8-9 residents annually) have enrolled in the program. Expatriate Russian physicians practicing in the US led 140 online educational seminars with journal clubs and clinical case presentations. Communication skills were evaluated by an independent private educational group. Results: Significant improvement of EBM knowledge was marked among HSO residents, from inability to explain basic concepts to practicing and teaching EBM. Beginning in the 3rd year of the program, residents organized educational courses and conducted journal clubs for HSO and non-HSO colleagues. Residents of the program had higher patient communication scores compared to that of residents of standard Russian programs. HSO residents promoted the spread and popularization of EBM and a patient-centered approach among attending physicians, improving the quality of cancer care. The HSO also triggered imitation of similar projects in other residency programs. Conclusions: The HSO project has pioneered EBM training and led to systemic changes in cancer patient care for Russian oncologists. This model has shown success and may be useful in overcoming global medical educational disparities in other specialties and in other countries facing similar challenges.