GALNT6, GALNT14, and Gal-3 in association with GDF-15 promotes drug resistance and stemness of breast cancer via ß-catenin axis.

N-acetylgalactosaminyltransferases (GALNTs) are a polypeptide responsible for aberrant glycosylation in breast cancer (BC), but the mechanism is unclear. In this study, expression levels of GALNT6, GALNT14, and Gal-3 were assessed in BC, and their association with GDF-15, ß-catenin, stemness (SOX2 and OCT4), and drug resistance marker (ABCC5) was evaluated. Gene expression of GALNT6, GALNT14, Gal-3, GDF-15, OCT4, SOX2, ABCC5, and ß-catenin in tumor and adjacent non-tumor tissues (n = 30) was determined. The same was compared with GEO-microarray datasets. A significant increase in the expression of candidate genes was observed in BC tumor compared to adjacent non-tumor tissue; and in pre-therapeutic patients compared to post-therapeutic. GALNT6, GALNT14, Gal-3, and GDF-15 showed positive association with ß-catenin, SOX2, OCT4, and ABCC5 and were significantly associated with poor Overall Survival. Our findings were also validated via in silico analysis. Our study suggests that GALNT6, GALNT14, and Gal-3 in association with GDF-15 promote stemness and intrinsic drug resistance in BC, possibly by ß-catenin signaling pathway.

Uncommon toxic epidermal necrolysis -like presentation of cutaneous lupus erythematosus: A series of 6 cases.

TEN like Lupus erythematosus is an uncommon life-threatening variant of Lupus erythematosus. It is usually associated with flares of systemic lupus erythematosus and also because of widespread skin erosions, it can cause acute skin failure. It is often confused with drug induced TEN, however the management of both the diseases is different and hence correct diagnosis becomes crucial. In this study we aimed to assess the clinical characteristics and outcome of TEN like LE in the Indian population. All patients satisfying ACR/EULAR 2019 criteria for SLE and clinically diagnosed with TEN like LE were retrospectively reviewed. A total of 6 patients were identified. All patients were female. Except 1 patient who presented de-novo, the others had pre-existing symptoms of connective tissue disease. Half of the patients had palmoplantar involvement. Mucosal involvement was only mild. Majority had systemic involvement in the form of nephritis followed by arthralgia, autoimmune hepatitis and autoimmune hemolytic anemia.

Predicting intracranial involvement: Unveiling perineural spread in COVID-19-associated mucormycosis, a novel phenomenon.

This study aimed to investigate the risk factors associated with intracranial involvement in COVID-19-associated mucormycosis (CAM) and to develop a nomogram model for predicting the risk of intracranial involvement, with a specific focus on perineural spread. An ambispective analysis was conducted on 275 CAM patients who received comprehensive treatment. Univariable and multivariable logistic regression analyses were performed to identify independent risk factors, and a nomogram was created based on the results of the multivariable analysis. The performance of the nomogram was evaluated using a receiver operating characteristic (ROC) curve, and the discriminatory capacity was assessed using the area under the curve (AUC). The model's calibration was assessed through a calibration curve and the Hosmer Lemeshow test. In the results, the multivariable logistic regression analysis revealed that age (OR: 1.23, 95% CI 1.06-3.79), HbA1c (OR: 7.168, 95% CI 1.724-25.788), perineural spread (OR: 6.3, 95% CI 1.281-19.874), and the disease stage were independent risk factors for intracranial involvement in CAM. The developed nomogram demonstrated good discriminative capacity with an AUC of 0.821 (95% CI 0.713-0.909) as indicated by the ROC curve. The calibration curve showed that the nomogram was well-calibrated, and the Hosmer Lemeshow test yielded a P-value of 0.992, indicating a good fit for the model. In conclusion, this study found that CAM particularly exhibits perineural spread, which is a predictive factor for intracranial involvement. A nomogram model incorporating age, HbA1c, disease stage, and perineural spread was successfully developed for predicting intracranial involvement in CAM patients in both in-patient and out-patient settings.

Discovery of perineural spread in COVID-19-associated mucormycosis reveals a new predictive model for intracranial complications which is crucial for early intervention.

GALNT14 in association with GDF-15 promotes stemness and drug resistance through ß-catenin signalling pathway in breast cancer.

BACKGROUND: Altered glycosylation plays a role in carcinogenesis. GALNT14 promotes cancer stem-like properties and drug resistance. GDF-15 is known to induces drug resistance and stemness markers for maintenance of breast cancer (BC) stem-like cell state. Currently there is lack of data on association of GDF-15 and GALNTs. In this study, the expression and interaction of GALNT14 and GDF-15 with stemness (OCT4 and SOX2) and drug resistance (ABCC5) markers were evaluated in BC. METHODS: We investigated tumour tissue from 30 BC patients and adjacent non-tumour tissues. Expression of serum GALNT14 from BC patients and matched healthy controls was evaluated. Expression of GALNT14, GDF-15, OCT4, SOX2, ABCC5, and ß-catenin in BC tissue was determined by RT-PCR. Knockdown of GALNT14 and GDF-15 in the MCF-7 cell line was done through siRNA, gene expression and protein expression of ß-catenin by western blot were determined. RESULTS: A significant increase in the expression of GALNT14, GDF-15, OCT4, SOX2, ABCC5, and ß-catenin was observed in BC tumour tissues compared to adjacent non-tumour tissues. The serum level of GALNT14 was significantly high in BC patients (80.7 ± 65.3 pg/ml) compared to healthy controls (12.2 ± 9.12 pg/ml) (p < 0.000). To further analyse the signalling pathway involved in BC stemness and drug resistance, GALNT14 and GDF-15 were knocked down in the MCF-7 cell line, and it was observed that after knockdown, the expression level of OCT4, SOX2, ABCC5, and ß-catenin was decreased, and co-knockdown with GALNT14 and GDF-15 further decreased the expression of genes. CONCLUSION: It can be concluded that GALNT14, in association with GDF-15, promotes stemness and intrinsic drug resistance in BC, possibly through the ß-catenin signalling pathway.

Gallbladder perforation: Diagnostic accuracy of new CT difficulty score in predicting complicated laparoscopic cholecystectomy.

PURPOSE: To formulate and evaluate the diagnostic performance and utility of a new CT difficulty score in predicting difficult laparoscopic surgery in cases of gallbladder (GB) perforation. METHODS: This prospective single centre study included a total of 48 diagnosed cases of GB perforation on CT between December 2021 and June 2023, out of which 24 patients were operated. A new 6-point CT difficulty scoring system was devised to predict difficult laparoscopic approach, based on patterns of inflammation around the perforated GB that were found to be surgically relevant. The pre-operative imaging findings on CT were studied in detail and correlation coefficients of various imaging findings were calculated to predict difficult surgery. RESULTS: On CECT, the type of perforation, according to the revised Niemeier's classification could be exactly delineated in all 48 patients. A CT difficulty score of ≥ 3 was found to a good predictor difficult laparoscopic approach, with statistical significance (p = 0.001), sensitivity of 94.44%, specificity of 83.33%, PPV of 94.44% and NPV of 83.33%. Inflammatory changes around duodenum showed maximum correlation coefficient of 0.744 (p = 0.0001), around colon showed a correlation coefficient of 0.657 (p = 0.0005), and in the omentum had a correlation coefficient of 0.5 (p = 0.013)). Inter-observer agreement was also calculated for various findings and it was found to have moderate to strong agreement (κ value 0.5-1.0). CONCLUSION: The CT difficulty scoring system can be an effective tool in predicting difficult laparoscopic surgery in cases of GB perforation in an emergency setting which can help in decision making and improved patient outcome.

Circulating miRNA-21 Levels in Breast Cancer Patients Before and After Chemotherapy and Its Association with Clinical Improvement.

Breast cancer is the most frequent type of cancer in women, many patients experience recurrences and metastasis. miR-21 (microRNA-21) as biomarker is under investigation for breast cancer. At present, there is very limited information available regarding effect of chemotherapy on miR-21 expression in breast cancer and its correlation with the clinical improvement. Hence, this study was planned to evaluate the effect of chemotherapy on miR-21 in metastatic breast cancer and its relationship with the clinical outcome. Females, aged-18-90 years diagnosed with Invasive Ductal Carcinoma of breast and candidate of neoadjuvant chemotherapy including Adriamycin (60 mg/m2), Cyclophosphamide (600 mg/m2) with or without Taxane (75-175 mg/m2) were included in the study. Before and after 42 days of staring of chemotherapy sample was collected for circulatory miR-21 and RECIST 1.1 criteria was applied to assess the clinical status. Blood samples for routine clinical biomarkers including liver function test and renal function tests was also collected. miR-21 expression before and after chemotherapy was assessed using standard method based on real time PCR. Expression of miR-21, RECIST criteria and other liver and kidney related biomarkers were compared before and after chemotherapy. After neoadjuvant chemotherapy expression of miR-21 was significantly increased by 5.65-fold. There was significant improvement in clinical scores based on RECIST criteria (0.046). No significant correlation was observed between miR-21 expression and difference in RECIST score (r = - 0.122, p = 0.570). Neoadjuvant chemotherapy causes clinical improvement in breast cancer patients however it is not correlated with the miR-21 expression which significantly increased after chemotherapy.

Human epidermal growth factor receptor 2/neu expression in urothelial carcinomas.

Introduction: Urothelial carcinomas of the bladder are more common in males, making them the sixth-most common cancer in men and the tenth-most common cancer overall, worldwide. Current guidelines do not recommend routine testing for human epidermal growth factor receptor (HER2/neu) expression on the biopsy specimens of patients with urothelial carcinoma. This study was aimed at determining the expression pattern of HER2/neu and its usefulness in muscle-invasive and nonmuscle-invasive urothelial carcinoma. Methods: HER2/neu expression was assessed in 89 specimens of urothelial cancer by immunohistochemistry (IHC), and equivocal cases were subjected to fluorescent in situ hybridization (FISH). Results: On IHC for HER2/neu, 17.9% (7/39) of the muscle-invasive bladder cancers (MIBCs) showed a 3+ expression, whereas 22% (11/50) of the non-muscle invasive cancers were positive with a score of 3+. A significant correlation between HER2/neu status and muscle invasion could not be established in the current study (P = 0.74, Fisher's exact test). Three cases of muscle-invasive (7.7%) and 2 cases (4%) among nonmuscle invasive cancers showed equivocal expression. All the cases with equivocal (2+) expression on IHC were subjected to FISH and none showed gene amplification on hybridization and were considered as negative. Conclusion: Overexpression of HER-2/neu was seen in 17.9% of MIBCs and 22% of non-MIBCs. There are no norms for routine testing of HER2/neu expression in the biopsy specimens of urothelial carcinoma. There is an unmet need to establish guidelines for HER2/neu scoring, similar to that for breast and gastric cancers, to determine the proportion of positive cases and help in identification of those who may benefit from targeted therapies.

Outcomes of transcutaneous retrobulbar Amphotericin B (TRAMB) as an adjuvant therapy for rhino-orbital-cerebral mucormycosis (ROCM) following COVID-19.

PURPOSE: To assess the outcomes of transcutaneous retrobulbar Amphotericin B (TRAMB) for rhino-orbital-cerebral-mucormycosis (ROCM) post-COVID-19, as an adjuvant to standard systemic antifungal therapy. METHODS: In this prospective cohort study involving ROCM patients with clinical/radiological orbital involvement, 44 eyes with ROCM stage ≥ 3B received TRAMB for 7 consecutive days with liposomal Amphotericin-B (3.5 mg/ml) with a minimum clinical and radiological follow-up of 3 months. All patients received standard systemic antifungal therapy also as per institutional protocol. Data pertaining to demography, systemic status, clinical involvement, imaging, surgical/medical management were also recorded. Potential eyes for exenteration were excluded. Please confirm if the author names are presented accurately and in the correct sequence (given name, middle name/initial, family name). Author 12 Given name: [Mahendra Kumar ] Last name [Garg]. Also, kindly confirm the details in the metadata are correct.All author names are presented accurately. Details in the metadata are correct. Thank you. RESULTS: Forty-four eyes of 42 patients were included, out of which 30 had diabetes mellitus & 22 had received steroid/oxygen treatment during COVID-19 infection. Forty eyes showed improvement or stable disease on follow-up on radiology. Four eyes which showed progression of the disease in orbit were reaugmented with TRAMB. No patient required exenteration. Subconjunctival haemorrhage occurred in six eyes and temporary blurring of vision in four eyes after TRAMB which resolved spontaneously. CONCLUSION: TRAMB, as an adjuvant to standard systemic antifungal therapy, is associated with a significant reduction or stabilisation of orbital involvement. TRAMB should be considered as an adjuvant therapy for ROCM to reduce disease progression as well as to preserve globe or sight. It has a promising role in preventing potential orbital exenterations.

Study of MicroRNA (miR-221-3p, miR-133a-3p, and miR-9-5p) Expressions in Oral Submucous Fibrosis and Squamous Cell Carcinoma.

Oral squamous cell carcinoma (OSCC) is one of the common types of cancer. Its progression follows a transition from oral potentially malignant disorders (OPMDs) such as oral submucous fibrosis (OSMF). Epigenetic modifiers, especially microRNAs (miRNAs), have an appreciable role in the regulation of various carcinogenic pathways which are being used as biomarkers. miRNAs may also be helpful in the differentiation of oral submucous fibrosis from oral squamous cell carcinoma. Three miRNAs, miR-221-3p, miR133a-3p, and miR-9-5p, were found differentially expressed in many cancers in the literature search supported by our preliminary database search-based screening. The literature and our functional enrichment analysis in an earlier study have reported these miRNAs to regulate carcinogenesis at various steps. In the present study, the expression of these miRNAs was examined in 34 histopathologically confirmed OSCC, 30 OSMF, and 29 control (healthy volunteers) human samples. There was a significant downregulation of miRNA-133a-3p in OSCC compared to OSMF and controls, whereas there was up-regulation in oral submucous fibrosis compared to controls. There was no significant difference in the expression of miR-221-3p between OSCC and OSMF, but an upregulation in OSCC compared to controls. miR-9-5p was also found upregulated in both OSCC and OSMF. Further, miR-133a-3p expression was negatively correlated with age, smoking, drinking status, and AJCC staging, whereas miR-9-5p expression was only positively associated with tobacco/ areca nut chewing. The ROC plots, logistic regression model generated, and the correlation between the expression of miR-9-5p and miR-133a-3p in blood and tissue suggests that these could be used as risk stratification biomarkers.

Effect of Cytomegalovirus Infection on Initial Presentation and Overall Prognosis of Biliary Atresia Patients.

Background and Aim: Biliary atresia is known to have a multifactorial etiology and perinatal infection with hepatotropic viruses such as cytomegalovirus (CMV) is a probable trigger in a subset of patients. The aim of the current study is to evaluate the effects of CMV association of biliary atresia on the initial presentation of patients and their response to Kasai portoenterostomy. Patients and Methods: We conducted a retrospective, single-center study on 20 patients of biliary atresia and classified them into two groups based on their CMV immunoglobulin M (IgM) positivity. We compared the age of initial presentation, the liver biochemistry at presentation, immediate and delayed follow-up, rate of jaundice clearance following Kasai portoenterostomy, and histopathology of liver between the two groups. Data were reported in terms of means, and P < 0.05 was considered significant. Results: Out of 20 cases of biliary atresia, 60% (n = 12) were CMV IgM positive. Infants with CMV-positive status were noted to be older at presentation (88.5 days [65-150 days] vs. 83 days [45-160 days] P < 0.05) were more jaundiced at presentation (total bilirubin - 13.51 mg/dl [9.09-15.99 mg/dl] vs. 11.83 mg/dl [6.5-13.5 mg/dl] P < 0.05), had higher alkaline phosphatase (751.2 IU/L [387-1951 IU/L] vs. 621.75 IU/L [172-857 IU/L] P < 0.05), higher gamma-glutamyl transferase levels (505.58 IU/L [376-1127 IU/L] vs. 376.75 IU/L [186-624 IU/L] P < 0.05), and had higher incidence of splenomegaly. The rate of resolution of jaundice postKasai portoenterostomy was also evidently less in CMV-positive patients. Four out of 12 patients have bilirubin >2 mg/dl at a 6-month follow-up. Conclusion: CMV-associated biliary atresia patients have delayed initial presentation and impaired jaundice clearance postKasai portoenterostomy. The role of antiviral therapy should be studied in this subset of patients.

Extranodal Rosai-Dorfman disease: A rare mimicker of metastatic renal cell carcinoma.

A 29-year-old female presented with the history of pain in the abdomen and a palpable lump in the right hypochondrium, lumbar, and the umbilical regions for the past 2 months. On evaluation with contrast-enhanced computed tomography (CECT) and positron emission tomography CT, she was found to have a heterogeneously enhancing mass entirely replacing the mid and the lower pole of the right kidney, the pelvis, and the upper ureter with loss of fat planes with the inferior vena cava, psoas muscle, and the hepatic flexure, along with pericardial deposits and soft tissue lesions at multiple paravertebral regions and the right thigh. Owing to a high suspicion of metastatic renal cell carcinoma (RCC), a right cytoreductive nephrectomy was performed. Histopathology revealed extranodal Rosai-Dorfman disease, which was mimicking a metastatic RCC on imaging. The patient was started on oral steroids to control the distant lesions and to prevent progression of the disease and is doing well at follow up.

FOXM1 mediates GDF-15 dependent stemness and intrinsic drug resistance in breast cancer.

BACKGROUND: Stemness, a key component of breast cancer (BC) heterogeneity, is responsible for chemoresistance. Growth differentiation factor-15 (GDF-15) induces drug resistance and stemness in BC cells. In this study, the expressions and interactions of GDF-15, FOXM1, and stemness (OCT4 and SOX2), and drug resistance (ABCC5) markers were evaluated in BC. METHODS AND RESULTS: 40 diagnosed BC patients and 40 healthy controls were included in this study. Serum GDF-15 was significantly raised (p < 0.001) in BC patients. Expressions of GDF-15, OCT4, SOX2, and FOXM1 in BC tissue and cell lines (MCF-7 and MDA-MB-231) were determined by RT-PCR, while phosphorylated AKT (p-AKT) was analyzed by Western blot. Not only were the fold change expressions higher in cancer tissue as compared to surrounding control tissue, but a higher expression was observed for all the genes along with p-AKT in MDA-MB-231 cells compared to MCF-7. Tissue GDF-15 was significantly associated with ABCC5 (p < 0.001), OCT4 (p = 0.002), SOX2 (p < 0.001), and FOXM1 (p < 0.001). To further analyze the signaling pathway involved in stemness and drug resistance in BC, GDF-15 knockdown was performed, which reduced the expression of p-AKT, FOXM1, OCT4 and SOX2, and ABCC5, whereas recombinant GDF-15 treatment reversed the same. In silico analyses in UALCAN revealed a similar picture for these genes to that of BC tissue expression. CONCLUSIONS: GDF-15 promotes stemness and intrinsic drug resistance in BC, possibly mediated by the p-AKT/FOXM1 axis.

Critical appraisal of epigenetic regulation of galectins in cancer.

Galectins are defined as the glycan-binding protein containing either one or two carbohydrate-binding domains and participate in various biological functions such as developmental processes, vascularisation programs, cell migration, and immune-regulation and apoptosis. Galectins are also linked to many diseases, including cancer. They are widely spread in extracellular and intracellular spaces, and their altered expression in cancer leads to tumor progression, metastasis, angiogenesis and stemness through different signalling pathways. Promoter methylation, microRNA, and histone modification constitute the epigenetic changes that regulate galectin activity in cancer. Our review discusses the concept of epigenetics in cancer and how the aforementioned factors i.e., promoter methylation, histone modification, change in miRNAs expression affect the glycomic changes in malignancies.

Alveolar Soft Part Sarcoma of Uterine Corpus in a Young Female: A Case Report With Review of Literature.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma, often occurs in adolescents and young adults with a particular predilection for the deep soft tissue of extremities. Occurrence of ASPS in the female genital tract is very uncommon and poses a significant diagnostic challenge. A case of ASPS of the uterine corpus is described in a young unmarried female, who presented to the out-patient clinic of our Institute with complaints of abnormal uterine bleeding for the past 9 mo. She was being treated with oral contraceptive pills, progesterone and tranexamic acid. Following radiological imaging and hysteroscopy, a therapeutic curettage of the lesion was done. The histopathologic features raised a differential diagnosis of a myriad of morphologic mimickers. The diagnosis was clinched by exclusion of mimickers by relevant immunohistochemical markers and strong nuclear expression of TFE3 on immunohistochemistry. The patient is on regular follow-up with oral contraceptives and antifibrinolytic medication. Despite being infrequent at this location, ASPS should be kept in differential diagnosis in young females presenting with abnormal uterine bleeding.

Pilomatricoma with florid osseous metaplasia: A common tumor with rare histopathological finding.

Pilomatricoma, also known as calcifying epithelioma of Malherbe, is a common benign skin adnexal tumor with differentiation toward hair cortex cells, commonly seen in head and neck region of children. It is usually solitary. Herein, we present a rare case of pilomatricoma with extensive osseous metaplasia as a thigh swelling in an adult female, considered clinically to be a sebaceous cyst. Florid osseous metaplasia is extremely rare in cases of pilomatricoma. To date, fewer than 10 cases of pilomatricoma with extensive osseous metaplasia at different sites have been reported in the English literature.

Congenital Melanocytic Nevus with Scrotal Mass: A Diagnostic Dilemma.

Congenital melanocytic nevus is deposition of pigment producing cells of melanocytic lineage in the dermis. We present an extremely rare case of congenital melanocytic nevus with a scrotal mass associated with deposition of melanin in the brain. The mass may mimic like a testicular tumour on clinical presentation.

Fetus Acardiac Amorphous Presenting as Placental Tumor: A Rare Case and Differentiating the Two.

Fetus acardius is a rare manifestation of twin reversed arterial perfusion and is a parasite due to vascular circulation from donor twin and lacks any resemblance to human embryos. Antenatal diagnosis is challenging as there are no well-defined features. We report here a case which presented as placental mass, the diagnosis of which was evident after delivery. Antenatal diagnosis, review of the literature, and differential diagnosis from the placental mass are discussed. A primigravida in the late third trimester had ultrasonography (USG) showing a 7 cm × 5 cm mass adjacent to the placenta. She had no complaints and fetal biometry was normal. She delivered a healthy baby; placenta showed an attached mass without identifiable fetal body or limbs, and was covered with skin having scanty hairs, which was identified as an amorphous fetus. Early diagnosis is possible by early USG; an acardiac amorphous fetus may present as placental mass. Characteristic feature on USG supplemented by magnetic resonance imaging may help in reaching a correct diagnosis and optimal management.

Concurrent acute myositis and Guillain-Barre syndrome in Cytomegalovirus infection - a rare case report.

BACKGROUND: Cytomegalovirus (CMV) is a double stranded DNA virus and ubiquitous in nature. Association of Guillain-Barre syndrome (GBS) and CMV is well known but CMV acute myositis is a rare condition. Restriction of movements of limbs due to severe pain in myositis may obscure the diagnosis of GBS and this may easily miss. CASE PRESENTATION: Here we describe a 29-year-old male presenting with pain and swelling of bilateral lower limbs which progressed rapidly with increasing serum creatine kinase levels with positive IgM CMV antibodies. In view of no improvement in clinical condition, patient was further evaluated and found to have concurrent GBS. He was treated with plasmapheresis and improved. CONCLUSION: Cytomegalovirus infection presenting as acute myositis is a uncommon and further association with GBS is a rare occurrence.

Tuberculosis and ovarian malignancy: Sometimes mimics, sometimes coexists.

Tuberculosis is a disease prevalent all over the world with India contributing to a larger share. Pulmonary tuberculosis presents with generalized symptoms of malaise, low grade fever and cough. On the other hand, genital tuberculosis presents with a variety of symptoms in each age group and is often underdiagnosed and missed. In an unmarried female, the usual presentations are menstrual complaints or presence of a solid cystic mass and ascites. In reproductive age group, patients may present with primary or secondary infertility or rarely with tubo-ovarian masses with peritoneal deposits, omental thickening and lymph node enlargement, hence mimicking ovarian carcinoma. In postmenopausal females, it can present as postmenopausal bleeding, leucorrhea or pyometra giving suspicion of endometrial carcinoma. We hereby report two cases operated with provisional diagnosis of ovarian malignancy but final histopathology ruled out malignancy in first and confirmed coexistence of malignancy and tuberculosis in another.

Endobronchial pulmonary blastoma - an unusual presentation of a rare lung malignancy and review of literature.

Biphasic pulmonary blastoma (BPB) is an extremely rare highly aggressive malignant tumor that arises from fetal lung tissue and has the classical biphasic histology of epithelial and mesenchymal components. It is usually seen in adults with a slight male predominance and smokers. Previously grouped along with well-differentiated fetal adenocarcinoma (WDFA), and pleuropulmonary blastoma (PPB), now it is considered a separate variant and grouped under sarcomatoid neoplasms. Symptoms include chest pain, cough, hemoptysis and it is asymptomatic in at least one-third of the cases. A biopsy is essential for diagnosis and surgical excision is the treatment of choice. Prognosis is poor with 5-year survival less than 20% and recurrence occurring within 12 months of surgery. An aggressive multimodality approach is required for its management and active follow up surveillance is needed to look for recurrence.