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2.
Ann Surg Oncol ; 2024 Apr 13.
Article En | MEDLINE | ID: mdl-38615151

BACKGROUND: Up to 41% of intra- and extra-adrenal paragangliomas are linked to germline mutations with autosomal dominant transmission, which necessitates genetic testing for patients and their relatives.1-4 Certain alterations, such as the succinate dehydrogenase (SDH) subunit B gene mutation, are associated with a significant risk of extra-adrenal, malignant, and metastatic disease forms.4-7 This highlights the need for routine genetic counseling and diligent surveillance, as well as surgeon awareness of hereditary paraganglioma-pheochromocytoma syndrome (HPPS). METHODS: We present a multimedia article featuring a step-by-step video of a complex retroperitoneal resection, enriched with perioperative management insights. RESULTS: A 17-year-old female presented with episodes of hypertension, tachycardia, and diffuse diaphoresis. CT revealed a paraaortic mass adjacent to the left renal hilum later confirmed by a SPECT/CT with iodine-123 meta-iodobenzylguanidine.8 Additional imaging with gallium-68 DOTATATE was not performed then due to unknown mutation status. The patient underwent robotic removal of the tumor and adjacent lymph nodes. Pathology confirmed a poorly differentiated paraganglioma with 0/6 lymph node metastases. Genetic tests revealed SDHB gene mutation, indicative of HPPS.9,10 At 12 months, the patient remained disease-free on CT with normalized metanephrines levels and no detectable circulating tumor DNA. Familial screening detected her mother, maternal uncle, and maternal grandfather to be SDHB mutation carriers, although phenotypically silent. CONCLUSIONS: Robotic-assisted resection can be safe and effective for retroperitoneal malignant paragangliomas. However, management extends beyond surgery and requires cascade genetic testing to address familial risks. Because of the high probability of cancer associated with SDHB mutation, lifelong patient surveillance is imperative.

3.
Cureus ; 16(3): e56336, 2024 Mar.
Article En | MEDLINE | ID: mdl-38633941

Autosomal dominant hereditary paraganglioma-pheochromocytoma syndrome (HPPS) is a rare genetic disorder characterized by neuroendocrine tumor development associated with pathogenic variants in succinate dehydrogenase (SDH) enzyme complex genes. Particularly, HPPS linked to SDHB mutation poses a significant clinical challenge due to its association with aggressive tumor features and a high risk of malignancy. Our report underscores the diversity in the presentation of patients with SDHB-mutated paraganglioma and the feasibility of managing it with a minimally invasive surgical approach. In the first case, a 17-year-old female was diagnosed with a metabolically active, poorly differentiated extra-adrenal retroperitoneal paraganglioma that required challenging robotic resection. Cascade genetic testing revealed an SDHB mutation not only in her but also in three family members, pointing to the inherited nature of the syndrome. Conversely, the second case involves a 37-year-old male with an asymptomatic well-differentiated left paraaortic paraganglioma incidentally found during an unrelated medical examination. Robotic converted-to-open resection allowed the successful removal of the mass. Subsequent germline testing confirmed a deleterious SDHB mutation, initiating a process of familial cascade testing. Both patients remained symptom- and recurrence-free at 12 and six months, respectively. Through these cases, and supported by a literature review, we highlight the variable clinical presentations of HPPS, arising from the same genetic alteration. The successful application of minimally invasive surgical techniques, combined with genetic evaluation, emphasizes the necessity for a comprehensive, tailored approach to treatment and surveillance. This strategy not only addresses the immediate clinical needs but also fosters proactive management of at-risk family members, ensuring a multidisciplinary approach to this complex hereditary condition.

4.
Eur J Surg Oncol ; 50(2): 107948, 2024 Feb.
Article En | MEDLINE | ID: mdl-38183864

BACKGROUND: Most patients with epithelial ovarian cancer (EOC) present with significant peritoneal spread. We assessed collaborative efforts of surgical and gynecological oncologists with expertise in cytoreductive surgery (CRS) in the management of advanced EOC. METHODS: Using a prospective single-center database (2014-2022), we described the operative and oncologic outcomes of stage IIIC-IVA primary and recurrent EOC perioperatively managed jointly by gynecological and surgical oncologists both specializing in CRS and presented components of this collaboration. RESULTS: Of 199 identified patients, 132 (66 %) had primary and 53 (27 %) had recurrent EOC. Due to inoperable disease, 14 (7 %) cases were aborted and excluded from analysis. Median peritoneal cancer index (PCI) in primary and recurrent patients was 21 (IQR: 11-28) and 21 (IQR: 6-31). Upper abdominal surgery was required in 95 % (n = 125) of primary and 89 % (n = 47) of recurrent patients. Bowel resections were performed in 83 % (n = 110) and 72 % (n = 38), respectively. Complete cytoreduction (CC-0/1) with no disease or residual lesions <2.5 mm was achieved in 95 % (n = 125) of primary and 91 % (n = 48) of recurrent patients. Ninety-day Clavien-Dindo grade III-IV morbidity was 12 % (n = 16) and 21 % (n = 11), respectively. Median follow-up was 44 (95%CI: 33-55) months. Median overall survival in primary and recurrent EOC was 68 (95%CI: 45-91) and 50 (95%CI: 16-84) months. Median progression-free survival was 26 (95%CI: 22-30) and 14 (95%CI: 7-21) months, respectively. CONCLUSIONS: Perioperative collaboration between surgical and gynecological oncologists specializing in CRS allows safe performance of complete cytoreduction in the majority of patients with primary and recurrent EOC, despite high tumor burden.


Ovarian Neoplasms , Humans , Female , Carcinoma, Ovarian Epithelial/surgery , Carcinoma, Ovarian Epithelial/pathology , Ovarian Neoplasms/pathology , Prospective Studies , Neoplasm Recurrence, Local , Peritoneum/pathology , Cytoreduction Surgical Procedures , Retrospective Studies
5.
Ann Surg Oncol ; 30(12): 7848-7857, 2023 Nov.
Article En | MEDLINE | ID: mdl-37633853

BACKGROUND: It is thought that low-grade (LG) appendiceal cancer (AC) demonstrates predominantly intraperitoneal recurrence (IPR) after cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), whereas high-grade (HG) tumors progress both intra- and extraperitoneally (EPR). However, evidence supporting this conception is lacking; therefore, we assessed recurrence in various AC histologies. METHODS: A retrospective, cohort study was conducted by using a single-center database (1998-2022). Recurrence patterns (IPR, EPR, combined) were identified for LG, HG, high-grade with signet ring cells (SRC), and goblet cell carcinoma (GCC). RESULTS: We included 432 complete (CC-0/1) CRS/HIPECs: 200 LG, 114 HG, 72 SRC, and 46 GCC. Median follow-up was 78 (95% confidence interval [CI] 70-86) months. Overall, 34% (n = 148) of patients recurred. IPR was the most common (LG 16%, HG 27%, SRC 36%, GCC 26%) with median time to recurrence (MTR) of 21 (IQR: 12-40) months. EPR (liver, lung, pleura, lymph nodes, or bones) occurred in LG 3%, HG 9%, SRC 22%, and GCC 7%. MTR was 11 (IQR: 4-16) months. Combined pattern occurred in LG 0%, HG 8%, SRC 7%, and GCC 0%. MTR was 13 (IQR: 7-18) months. Iterative surgery was performed in 53% IPR, 18% EPR, and 51% combined. Median post-recurrence survival was longer after IPR compared with EPR and combined recurrence: 36 (95% CI 25-47) versus 13 (95% CI 7-19) and 18 (95% CI 6-30) months (p < 0.01). CONCLUSIONS: After complete CRS/HIPEC, IPR was the predominant pattern in all AC histologies and occurred later. Post-recurrence survival after IPR was longer. Knowing AC recurrence patterns can help to understand its biology and plan follow-up and post-relapse management.

7.
Eur J Surg Oncol ; 49(8): 1495-1503, 2023 08.
Article En | MEDLINE | ID: mdl-37068984

BACKGROUND: Ovarian carcinosarcoma (OCS) is an uncommon and aggressive malignancy, with poor response to current treatment approaches and no clear guidelines. Our aim is to evaluate the outcomes of an OCS cohort after cytoreduction with hyperthermic intraperitoneal chemotherapy (CRS/HIPEC). METHODS: A descriptive cohort study was performed. Patients who underwent CRS/HIPEC for peritoneal dissemination from tubo-ovarian malignancies (1999-2021) were retrospectively reviewed. Patients with confirmed histopathologic diagnosis of FIGO stage III/IV OCS were included. Overall (OS) and progression-free survival (PFS) were determined with the Kaplan-Meier method. RESULTS: Of 267 patients with tubo-ovarian malignancies reviewed, 7.5% (20/267) had OCS. Of these, 16 underwent CRS/HIPEC, including 9 for a new diagnosis and 7 for disease recurrence. Median age at surgery was 66.5 (IQR: 54.5-74.5) years. Nine (56.2%) patients were FIGO stage IV. Median peritoneal cancer index was 22 (IQR: 14-28). Complete cytoreduction was achieved in 15/16 (93.7%) cases. HIPEC agents included carboplatin (n = 7), cisplatin+doxorubicin (n = 4), and melphalan (n = 5). Major complications occurred in 4/16 (25%), with no 90-day mortality. Median follow-up was 41.8 months. Median PFS was 11.7 (95%CI: 10.5-17.1) months. Malignant bowel obstruction occurred in 3/16 (18.7%). Median OS from CRS/HIPEC was 21.3 (95%CI: 16.3-31.6) months, not reached for newly diagnosed vs 19.7 months for recurrent patients (p = 0.23). CONCLUSIONS: CRS/HIPEC showed promising survival and abdominal disease control with low rates of malignant obstruction in patients with advanced stage OCS. Collaborative studies with larger cohorts and longer follow-up may further elucidate the role of CRS/HIPEC in OCS.


Carcinosarcoma , Hyperthermia, Induced , Ovarian Neoplasms , Peritoneal Neoplasms , Female , Humans , Middle Aged , Aged , Hyperthermic Intraperitoneal Chemotherapy , Cytoreduction Surgical Procedures/methods , Cohort Studies , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Retrospective Studies , Peritoneal Neoplasms/drug therapy , Hyperthermia, Induced/methods , Neoplasm Recurrence, Local/pathology , Ovarian Neoplasms/pathology , Carcinosarcoma/therapy , Survival Rate , Combined Modality Therapy
9.
Eur J Surg Oncol ; 49(1): 179-187, 2023 01.
Article En | MEDLINE | ID: mdl-36253240

INTRODUCTION: There are no available data on the efficacy of adjuvant chemotherapy (ACT) in stage IVA/B high-grade mucinous appendiceal cancer treated with CRS/HIPEC. We evaluated the association between ACT and survival in this cohort. MATERIALS AND METHODS: A single-institution retrospective cohort study using a prospective database was conducted. Stage IVA/B high-grade mucinous appendiceal cancer patients who underwent CRS/HIPEC with CC-0/1 were included. Survival was compared between ACT and no chemotherapy (NoCT) patients. Subgroup analysis was performed with adjustment for confounding variables. RESULTS: We identified 180 patients: 77 ACT and 103 NoCT. ACT regimens included 5-FU/capecitabine (13%), oxaliplatin-based (63%), and irinotecan-based (21%), combined with bevacizumab in 27% of cases. Median number of cycles was 9 (IQR: 6-12). Median overall survival (OS) did not significantly differ between ACT and NoCT (53 vs 75 months, p = 0.566). Multivariable Cox regression showed no OS benefit for ACT vs NoCT in patients with neoadjuvant chemotherapy (HR 1.14; 95%CI: 0.38-3.39) or without it (HR 1.33; 95%CI: 0.69-2.57), with signet ring cell (HR 0.89; 95%CI: 0.38-2.06) or other histologies (HR 1.11; 95%CI: 0.50-2.46), positive lymph nodes (HR 1.60; 95%CI: 0.74-3.49), or peritoneal cancer index ≥20 (HR 1.08; 95%CI: 0.55-2.11) after adjusting for other factors. CONCLUSIONS: In our cohort, colon-type ACT was not associated with better OS in stage IVA/B mucinous appendiceal cancer after CRS/HIPEC, even after adjusting for confounders. This may be due to different tumor biology than colon cancer or small sample size. Prospective collaborative studies are needed.


Adenocarcinoma, Mucinous , Appendiceal Neoplasms , Hyperthermia, Induced , Peritoneal Neoplasms , Humans , Appendiceal Neoplasms/pathology , Hyperthermic Intraperitoneal Chemotherapy , Cytoreduction Surgical Procedures , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Retrospective Studies , Peritoneal Neoplasms/drug therapy , Adenocarcinoma, Mucinous/pathology , Chemotherapy, Adjuvant , Survival Rate , Combined Modality Therapy
10.
Ann Surg Oncol ; 30(4): 2520-2528, 2023 Apr.
Article En | MEDLINE | ID: mdl-36463354

BACKGROUND: 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. METHODS: 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. RESULTS: 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/m2 for 90 min) without reduction of dose or time. Major morbidity (p = 0.435) and mortality (p = 1.000) were comparable between the two groups. No postoperative acute kidney injury was seen in either group. Adjuvant chemotherapy was administered to 46.2% of the NE and 35.9% of the no-NE patients (p = 0.553), with similar starting times (p = 0.903) between the groups. CONCLUSIONS: Nephrectomy 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.


Hyperthermia, Induced , Peritoneal Neoplasms , Humans , Hyperthermic Intraperitoneal Chemotherapy , Mitomycin , Combined Modality Therapy , Melphalan , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Peritoneal Neoplasms/therapy , Propensity Score , Retrospective Studies , Nephrectomy/adverse effects , Cytoreduction Surgical Procedures/adverse effects , Survival Rate
12.
Ann Surg Oncol ; 29(11): 6581-6589, 2022 Oct.
Article En | MEDLINE | ID: mdl-35641710

BACKGROUND: 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. METHODS: 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. RESULTS: 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) (p < 0.001), with hazard ratio (HR) of 0.22 (95 % CI, 0.08-0.66; p = 0.007) after adjustment for other factors. CONCLUSION: 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.


Appendiceal Neoplasms , Hyperthermia, Induced , Peritoneal Neoplasms , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Appendiceal Neoplasms/pathology , Cytoreduction Surgical Procedures , Humans , Hyperthermic Intraperitoneal Chemotherapy , Peritoneal Neoplasms/secondary , Prospective Studies , Retrospective Studies
13.
Ann Surg Oncol ; 29(6): 3390-3401, 2022 Jun.
Article En | MEDLINE | ID: mdl-35133518

BACKGROUND: Recurrence after cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) for appendiceal tumors (AT) with mucinous carcinomatosis peritonei (MCP) is common. The evidence favoring iterative procedures (iCRS/HIPEC) is limited, and its benefit is not clear for all patients. METHODS: Retrospective (1998-2020) cohorts of AT patients with MCP recurrence after the first CRS/HIPEC were analyzed. Outcomes were compared within tumor grades between iCRS/HIPEC patients and matched control patients without iCRS/HIPEC using propensity score matching (1:1). Post-recurrence survival (PRS) was measured from the date of recurrence after the first CRS/HIPEC to death or last contact. RESULTS: Overall, 55 iCRS/HIPEC patients were identified: 36 low-grade (LGMCP) patients, 13 high-grade (HGMCP) patients, and 6 HGMCP patients with signet-ring features (HGMCP-S). Nine patients had a third CRS/HIPEC. The median peritoneal cancer index (PCI) scores were 33, 19 and 10, with CC-0/1 achieved for 94.4%, 78.2% and 88.9% of the patients after the first, second, and third CRS/HIPEC, respectively. No 90-day postoperative mortality occurred. The median progression-free survival from the first CRS/HIPEC was 19.7 months for the iCRS/HIPEC patients versus 14.2 months for the matched control patients (p = 0.43). The median PRS was 80.2 months for iCRS/HIPEC versus 36.2 for the control patients (p < 0.001). For the iCRS/HIPEC versus the matched control patients, the median PRS by tumor grade was 174.1 versus 51.9 (p < 0.001) for the LGMCP, 42.0 versus 12.4 (p = 0.02) for the HGMCP, and 15.4 versus 8.1 months (p = 0.61) for the HGMCP-S patients, respectively. CONCLUSIONS: Selected low- and high-grade appendiceal cancer patients with MCP recurrence able to undergo iterative CRS/HIPEC procedures showed favorable outcomes and such patients should be considered for surgery when feasible. This survival benefit with iCRS/HIPEC is not evidenced in recurrent MCP with signet ring cell morphology.


Adenocarcinoma, Mucinous , Adenocarcinoma , Appendiceal Neoplasms , Appendix , Hyperthermia, Induced , Peritoneal Neoplasms , Adenocarcinoma/pathology , Adenocarcinoma, Mucinous/pathology , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Appendiceal Neoplasms/pathology , Appendix/pathology , Combined Modality Therapy , Cytoreduction Surgical Procedures , Humans , Hyperthermic Intraperitoneal Chemotherapy , Neoplasm Recurrence, Local/pathology , Peritoneal Neoplasms/pathology , Retrospective Studies , Survival Rate
15.
Ann Surg Oncol ; 29(2): 873-882, 2022 Feb.
Article En | MEDLINE | ID: mdl-34546479

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.


Appendiceal Neoplasms , Hyperthermia, Induced , Peritoneal Neoplasms , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Appendiceal Neoplasms/therapy , Combined Modality Therapy , Cytoreduction Surgical Procedures , Follow-Up Studies , Humans , Hyperthermic Intraperitoneal Chemotherapy , Peritoneal Neoplasms/drug therapy , Prospective Studies , Retrospective Studies , Survival Rate
16.
Ann Surg Oncol ; 28(13): 9126-9135, 2021 Dec.
Article En | MEDLINE | ID: mdl-34263367

BACKGROUND: 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). METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.


Hyperthermia, Induced , Peritoneal Neoplasms , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Chemotherapy, Cancer, Regional Perfusion , Combined Modality Therapy , Cytoreduction Surgical Procedures , Female , Follow-Up Studies , Humans , Neoplasm Recurrence, Local/therapy , Peritoneal Neoplasms/drug therapy , Retrospective Studies , Survival Rate
17.
Eur J Surg Oncol ; 47(9): 2352-2357, 2021 09.
Article En | MEDLINE | ID: mdl-33933342

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.


Cytoreduction Surgical Procedures/adverse effects , Genitalia, Female/pathology , Genitalia, Male/pathology , Hyperthermic Intraperitoneal Chemotherapy/adverse effects , Peritoneal Neoplasms/therapy , Anti-Bacterial Agents/therapeutic use , Antibiotics, Antineoplastic/administration & dosage , Combined Modality Therapy , Debridement , Edema/etiology , Female , Fever/etiology , Humans , Male , Middle Aged , Mitomycin/administration & dosage , Necrosis/diagnosis , Necrosis/etiology , Necrosis/therapy , Pain/etiology , Peritoneal Neoplasms/pathology , Retrospective Studies , Skin Ulcer/etiology , Time Factors
18.
JCO Glob Oncol ; 7: 353-360, 2021 03.
Article En | MEDLINE | ID: mdl-33667114

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.


Internship and Residency , Physicians , Adult , Evidence-Based Medicine , Female , Humans , Retrospective Studies , Russia , Schools , United States , Young Adult
19.
J Surg Oncol ; 123(4): 1030-1044, 2021 Mar.
Article En | MEDLINE | ID: mdl-33524164

BACKGROUND: Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is predominantly performed and studied in academic centers. While developing CRS/HIPEC programs in nonacademic hospitals can increase accessibility, its safety and oncological efficacy remains unclear. We evaluated CRS/HIPEC outcomes in a nonacademic setting. METHODS: A single-center descriptive study was conducted using a prospective database. Data of all CRS/HIPEC attempts in peritoneal surface malignancies (PSM) patients from October 1994 to November 2019 were extracted. Surgical and survival outcomes were measured. Center experience was assessed by quartiles of cases. RESULTS: Overall, 856 patients underwent 948 CRS/HIPEC attempts: 788 (83%) completed CRS/HIPECs, 144 (15%) aborted HIPECs, and 16 (2%) complete cytoreductions (CC-0/1) without chemoperfusion. For completed CRS/HIPECs, median peritoneal cancer index was 24 (interquartile range: 10-33) and CC-0/1 rate was 88%. Major complications occurred in 23.5% with 30- and 100-day mortality of 1.0% and 2.3%, respectively. Median overall survival was 68 months (95% confidence interval [CI]: 50-86). Median progression-free survival was 37 months (95%CI: 28-46). Incomplete cytoreduction and major complication rates decreased over time, while mortality remained low and constant. CONCLUSIONS: CRS/HIPEC at a nonacademic center with advanced surgical and auxiliary services is a safe option to treat PSM with favorable surgical and oncological outcomes.


Chemotherapy, Adjuvant/mortality , Chemotherapy, Cancer, Regional Perfusion/mortality , Cytoreduction Surgical Procedures/mortality , Hyperthermia, Induced/mortality , Hyperthermic Intraperitoneal Chemotherapy/mortality , Peritoneal Neoplasms/mortality , Aged , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Male , Middle Aged , Peritoneal Neoplasms/pathology , Peritoneal Neoplasms/therapy , Prognosis , Prospective Studies , Randomized Controlled Trials as Topic , Survival Rate
20.
Case Rep Oncol Med ; 2020: 8853704, 2020.
Article En | MEDLINE | ID: mdl-33123396

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.

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