Primary pulmonary epithelioid inflammatory myofibroblastic sarcoma: a rare entity and a literature review.

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of inflammatory myofibroblastic tumor (IMT) harboring anaplastic lymphoma kinase (ALK) gene fusions and is associated with high risk of local recurrence and poor prognosis. Herein, we present a young, non-smoking male who presented with complaints of cough and dyspnoea and was found to harbor a large right lower lobe lung mass. Biopsy showed a high-grade epithelioid to rhabdoid tumor with ALK and desmin protein expression. The patient initially received 5 cycles of crizotinib and remained stable for 1 year; however, he then developed multiple bony metastases, for which complete surgical resection was performed. Histopathology confirmed the diagnosis of EIMS, with ALK gene rearrangement demonstrated by fluorescence in situ hybridization. Postoperatively, the patient is asymptomatic with stable metastatic disease on crizotinib and has been started on palliative radiotherapy. EIMS is a very rare subtype of IMT that needs to be included in the differential diagnosis of ALKexpressing lung malignancies in young adults.

A proposal for changing nomenclature from pseudomyxoma peritonei (PMP) to abdomino-peritoneal mucinous carcinoma (APM) based on its long journey and experience from tertiary oncology center in India.

INTRODUCTION: Pseudomyxoma peritonei (PMP) is a generalized term, usually known as "jelly belly" since 1884. Incidence is very low, 1-3 per million people per year. Because of its indolent nature, it is usually diagnosed at an advanced stage, thereby impacting the quality of life. The 5-year survival rate varies from 23 to 86% in world literature. Even 10 years and 20 years of survival have been described. With our experience, we like to propose rename of PMP as abdomino-peritoneal mucinous carcinoma (APM) as we strongly feel the time has come to specify the term and standardize the management strategy. METHODOLOGY: In the premier institute of India and as a tertiary referral center, we experienced the maximum number of advanced cases of APM. From 2012 to 2021, we analyzed all the APM patients based on a prospectively maintained computerized database in the department of surgical oncology and found the reasons for renaming from this traditional one. RESULTS: We included a total of 87 patients who underwent surgical intervention. Thirty-five patients underwent cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), and 52 patients underwent debulking. In CRS-HIPEC patients, CC-0 was achieved in 28 patients (80%), CC-1 in 4 patients (11.4%), and CC-2 in 3 patients (8.6%). Palliative intent HIPEC was done in 3 patients (8.6%). Clavien-Dindo grade III and IV morbidity was observed in 18.8% of patients with 90 days mortality of 5.7%. CONCLUSION: With our long-term experience and advancement of scientific evidence, we like to propose a new name for PMP as APM. We strongly believe this paper will give a clear picture of this rare disease and standard management outlines.

BMP4 enhances anoikis resistance and chemoresistance of breast cancer cells through canonical BMP signaling.

Bone morphogenetic proteins (BMPs) regulate cell fate during development and mediate cancer progression. In this study, we investigated the role of BMP4 in proliferation, anoikis resistance, metastatic migration, and drug resistance of breast cancer cells. We utilized breast cancer cell lines and clinical samples representing different subtypes to understand the functional effect of BMP4 on breast cancer. The BMP pathway was inhibited with the small molecule inhibitor LDN193189 hydrochloride (LDN). BMP4 signaling enhanced the expression of stem cell genes CD44, ALDH1A3, anti-apoptotic gene BCL2 and promoted anoikis resistance in MDA-MB-231 breast cancer cells. BMP4 enhanced self-renewal and chemoresistance in MDA-MB-231 by upregulating Notch signaling while LDN treatment abrogated anoikis resistance and proliferation of anoikis resistant breast cancer cells in the osteogenic microenvironment. Conversely, BMP4 downregulated proliferation, colony-forming ability, and suppressed anoikis resistance in MCF7 and SkBR3 cells, while LDN treatment promoted tumor spheroid formation and growth. These findings indicate that BMP4 has a context-dependent role in breast cancer. Further, our data with MDA-MB-231 cells representing triple-negative breast cancer suggest that BMP inhibition might impair its metastatic spread and colonization.

Upfront cytoreductive surgery versus neoadjuvant chemotherapy in advanced epithelial ovarian cancer in Indian patients.

In cases of ovarian carcinoma, primary cytoreductive surgery (CRS) is the standard treatment up to stage IIIB, but patient selection for neoadjuvant chemotherapy (NACT) in selected cases is controversial. A total of 200 patients with advanced ovarian cancer were analyzed retrospectively, according to specific selection criteria. Primary CRS was performed in 95 patients (47.5%) and interval CRS after 3-6 cycles of NACT was performed in 105 patients (52.5%). After median follow-up of 35 months, 5-year overall survival was 53.7% in the upfront CRS group and 42.2% in the NACT group. Primary CRS is the standard in advanced stages of ovarian carcinoma, but in certain subset of patients, NACT is preferred. Identifying that group is challenging but feasible. Proper selection of patients is key to successful outcomes.

Protective Diversion Stoma in Low Anterior Resection for Rectal Cancer: A Meta-Analysis of Randomized Controlled Trials.

BACKGROUND: With the establishment of the oncological safety and due to the potential of low anterior resection (LAR) with sphincter salvage in improving the quality of life of patients with low and mid rectal cancers, it has become a popular treatment modality. A potential complication of the procedure is anastomotic dehiscence which results in a significant increase in postoperative morbidity and mortality. METHODS: A literature search for randomized controlled trials (RCTs) that compared the role of protective diversion stoma with no stoma in LAR of the rectum was performed in PubMed. The effect size for dichotomous and continuous data was displayed as relative risk (RR) and weighted mean difference (WMD), respectively, with their corresponding 95% confidence intervals. A fixed effect or random effects model was used to pool the data according to the result of a statistical heterogeneity test. RESULTS: Five RCTs were identified and included in the analysis. These yielded 390 patients who had undergone a protective diversion ileostomy at the time of the surgery (LAR) and 378 who had not, resulting in a total of 768 patients, all of whom were included in the meta-analysis. The fashioning of an ileostomy significantly decreased the anastomotic leak (AL) rates (RR 0.33, 95% CI 0.21-0.51, p < 0.000) and the reoperation rates (RR 0.26, 95% CI 0.15-0.45, p < 0.000). CONCLUSION: This meta-analysis found that a protective diversion ileostomy in LAR for rectal cancer decreases the AL rates by one third and the reoperation rates by one fourth. Thus, we conclude that fashioning such a stoma is beneficial.

Milky Mesentery: Acute Abdomen with Chylous Ascites.

BACKGROUND: Clinical presentations of intestinal lymphangiectasia include pitting edema, chylous ascites, pleural effusion, diarrhea, malabsorption and intestinal obstruction. CASE CHARACTERISTICS: An 8-year-old male child presented to the emergency department with clinical features of peritonitis, raising suspicion of appendicular or small bowel perforation. INTERVENTION/OUTCOME: Diagnosis of chylous ascites with primary intestinal lymphangiectasia made on laparotomy. MESSAGE: Acute peritonitis may be a presentation of primary intestinal lymphangiectasia and chylous ascites.

Palliative Surgery for Metastatic Fungating Phyllodes Tumors: A Series of Two Cases.

INTRODUCTION: Local treatment of metastatic cystosarcoma phyllodes is classically viewed with skepticism because it does not provide survival benefit. When these advanced tumors ulcerate, they reduce quality of life dramatically because of associated pain, infection, malodor, massive discharge, and bleeding. CASE PRESENTATIONS: Two patients with metastatic cystosarcoma phyllodes presented to our hospital with recurrent disease featuring foul-smelling, ulcerated chest wall masses that caused physical pain and social exclusion. The first patient underwent radical chest wall resection with pedicled lattisimus dorsi flap reconstruction. The second patient underwent wide local excision with split-thickness skin grafting. There was significant improvement in the quality of life for these patients after the operations. DISCUSSION: Though both patients finally succumbed to progressive metastatic disease, palliative resection allowed them to have good social and family support to the end. Palliative surgery plays an important role in alleviating suffering of patients with metastatic fungating cystosarcoma phyllodes and has the potential to improve their quality of life significantly.

Preoperative therapy in locally advanced esophageal cancer.

Esophageal cancer is an aggressive malignancy associated with dismal treatment outcomes. Presence of two distinct histopathological types distinguishes it from other gastrointestinal tract malignancies. Surgery is the cornerstone of treatment in locally advanced esophageal cancer (T2 or greater or node positive); however, a high rate of disease recurrence (systemic and loco-regional) and poor survival justifies a continued search for optimal therapy. Various combinations of multimodality treatment (preoperative/perioperative, or postoperative; radiotherapy, chemotherapy, or chemoradiotherapy) are being explored to lower disease recurrence and improve survival. Preoperative therapy followed by surgery is presently considered the standard of care in resectable locally advanced esophageal cancer as postoperative treatment may not be feasible for all the patients due to the morbidity of esophagectomy and prolonged recovery time limiting the tolerance of patient. There are wide variations in the preoperative therapy practiced across the centres depending upon the institutional practices, availability of facilities and personal experiences. There is paucity of literature to standardize the preoperative therapy. Broadly, chemoradiotherapy is the preferred neo-adjuvant modality in western countries whereas chemotherapy alone is considered optimal in the far East. The present review highlights the significant studies to assist in opting for the best evidence based preoperative therapy (radiotherapy, chemotherapy or chemoradiotherapy) for locally advanced esophageal cancer.